Refine Search

New Search

Results: 51

(searched for: Metastatic Angiosarcoma: A Rare Malignancy)
Save to Scifeed
Page of 2
Articles per Page
by
Show export options
  Select all
T Patel, S K Ohri, M Sundaresan, J Jackson, L A Desa, A T Davey, J Spencer
Published: 1 June 1991
European Journal of Surgical Oncology, Volume 17, pp 295-9

Abstract:
A case of metastatic angiosarcoma of the ovary, complicated by disseminated intravascular coagulation, is described. The tumour was diagnosed by histopathologic examination and immunohistochemical studies. Angiosarcoma of the ovary, although extremely rare, appears to be a highly malignant neoplasm with an aggressive course and effective therapy is unknown.
Raymond A. Dieter, George B. Kuzycz, Jessica Gulliver, Robert S. Dieter
Published: 28 August 2019
The publisher has not yet granted permission to display this abstract.
Béla B. Toth, Terence J. Fleming, Jose A. Lomba, Jack W. Martin
Published: 31 July 1981
Oral Surgery, Oral Medicine, Oral Pathology, Volume 52, pp 71-74; https://doi.org/10.1016/0030-4220(81)90176-6

Abstract:
A case of angiosarcoma metastatic to the maxillary gingiva is presented. A review of the literature and an epidemiologic survey of angiosarcoma cases since 1944 at the M. D. Anderson Hospital and Tumor Institute prove this to be an extremely rare occurrence. Generally, metastasis to the oral/perioral tissues occurs as a late sequela secondary to diffuse metastatic disease. In this case, however, two extensive metastatic tumor surveys determined that the only metastasis was to the oral site. The lesion clinically mimicked an inflammatory process. It is hoped that this presentation will reinforce aggressive pursuit of innocuous-appearing oral lesions when the patient has a history of malignant disease and will further encourage dental involvement in the oral/dental assessment and are of the cancer patient.
, M. Rupolo, , , A. Brollo, , , U. Tirelli, S. Frustaci, S. Berretta
Published: 1 April 2006
Journal of Chemotherapy, Volume 18, pp 221-224; https://doi.org/10.1179/joc.2006.18.2.221

Abstract:
Angiosarcomas are rare soft tissue malignancies. Typically they originate from the skin of the scalp or face, whereas visceral sarcomas are very rare. We report the case of a 67-year-old man affected by a large angiosarcoma of the kidney. After surgical removal, a rapid peritoneal, visceral and cutaneous diffusion developed. Palliative chemotherapy, based on anthracycline and ifosfamide, which are normally used to treat all other high-grade spindle cell sarcomas, was totally inactive. On the basis of these results and of the biological characteristics of these rare neoplasms it is mandatory to develop other therapeutic approaches. Antiangiogenetic agents are of interest for this disease due to the peculiar origin of the cells of these sarcomas.
A Popović, P Harrigan, A J Sanfilippo, A E Weyman
Published: 1 July 1989
Srpski arhiv za celokupno lekarstvo, Volume 117

Abstract:
Cardiac angiosarcoma is the most common primary malignant tumour of the heart that is rarely diagnosed ante mortem. The authors present the case of a 73-year-old female with right atrial angiosarcoma that was followed-up echocardiographically for 20 months. Echocardiographic features suggestive of primary and metastatic malignant tumours of the right heart are discussed.
Zaitun Zakaria, Kantharuby Tambirajoo, Mohammad Taufiq Sattar, Michael A Farrell
Published: 1 January 2015
by 10.5137
Turkish neurosurgery, Volume 25; https://doi.org/10.5137/1019-5149.JTN.9042-13.1

Abstract:
Central nervous system (CNS) angiosarcoma, both primary and secondary, is an exceptionally rare malignant tumor. The behaviour of angiosarcoma is one of rapid progression with early metastatic spread but as with all sarcomas, the primary site may not clinically evident by the time metastases develop. Identification of the primary site of origin is masked by the carpet-like growth pattern that may occur in the left atrium, the most frequently encountered primary site for angiosarcoma. This rare tumor presents many diagnostic challenges. We describe the clinical and neuropathologic features of two patients with angiosarcoma of the brain and review the previously published cases described to date.
E Ventrillon, A Avril
Published: 1 October 1992
Revue francaise de gynecologie et d'obstetrique, Volume 87

Abstract:
Four cases of angiosarcoma of the breast, including three previously unpublished, form the starting point for a brief descriptive review of this extremely rare but fearsome tumour which accounts for only 0.04 per cent of breast malignancies. The contrast between the initially totally benign clinical appearance of this sarcoma and the dramatic prognosis of its advanced or metastatic forms against which adjuvant chemotherapy remains of only relative usefulness, leads the authors to chiefly discuss a diagnostic approach likely to speed recognition (histological diagnosis) and treatment (simple mastectomy) of a tumour with two troublesome features: its powerful metastatic potential being matched only by its extreme rarity which may cause clinicians, radiologists and histopathologists to use valuable time.
M Crespo Urigüen, A Miyar González, M A Martínez Menéndez, J M García García
Published: 1 July 1993
Revista Española de Enfermedades Digestivas, Volume 84

Abstract:
Primary spleen angiosarcoma is a malignant neoplasia based on vascular tissue and it is a rare tumor (65 cases published). The clinical aspects are obscure and the specific diagnosis is made by laparotomy. The prognosis is very poor in any case and survival isn't more than two years, whenever the spleen undergoes spontaneous rupture in which case the survival should be less than six months. The splenectomy before rupture could increase survival. Patients with or without metastatic disease may be treated by chemotherapy but with poor results. Radiotherapy is used for relief the pain produced for bone metastasis.
Michael A. Burnstine, Bartley R. Frueh,
Archives of Ophthalmology (2012), Volume 114, pp 93-96; https://doi.org/10.1001/archopht.1996.01100130089017

Abstract:
Angiosarcoma is a rare malignant endothelial cell tumor. Few reports of primary orbital angiosarcoma exist in the literature. A 46-year-old woman had a 3-month history of progressive diplopia and right-sided exophthalmos. Magnetic resonance imaging showed a right intraconal mass, consistent with a cavernous hemangioma. Ultrasound examination showed an isolated mass with moderate vascularity, consistent with a metastatic lesion. Sub-total excision through a lateral orbitotomy was performed. Tissue was stained with hematoxylineosin, periodic acid-Schiff, and immunohistochemical stains for factor VIII-related antigen and Ulex europaeus agglutinin I lectin. Lateral orbitotomy biopsy specimens showed an angiosarcoma, similar to a primary breast angiosarcoma that had been resected 2 years previously. The patient initially underwent orbital irradiation, which failed to control tumor progression. Subsequently, she underwent orbital exenteration for her only known metastasis. Despite a multidisciplinary and aggressive approach to this tumor, the tumor recurred, and the patient died.
A Alabassi, I S Fentiman
Published: 1 December 2003
International Journal of Clinical Practice, Volume 57

Abstract:
Breast sarcomas are rare lesions that can be derived from any of the mammary stromal cells. A phyllodes tumour may resemble a rapidly growing fibroadenoma but in an older age group. Behaviour ranges from benign to frankly malignant and cannot always be predicted from the histological appearance. For all the sarcomas spread is haematogenous rather than lymphatic and treatment is based on wide surgical clearance without axillary surgery. Angiosarcoma is a rare consequence of skin irradiation as part of breast conservation therapy. Fibrosarcomas have a tendency to relapse locally unless wide surgical margins have been achieved. Radiotherapy is not of value but chemotherapy can be used to palliate women with metastatic disease.
Journal of pediatric hematology/oncology, Volume 32, pp 192-194; https://doi.org/10.1097/mph.0b013e3181ca9ed7

Abstract:
We present a case of a rare malignancy, primary breast angiosarcoma, in a 15-year-old girl. She presented with a locally advanced tumor and suspected metastatic disease to the mediastinum and bones on PET imaging. The patient was treated with systemic chemotherapy and definitive radiation therapy to her left breast and achieved a complete response. She has no evidence of disease recurrence 44 months from her initial diagnosis.
A Ullah, S N Mattox, S Heneidi, J White, M Sinkler, C Wehrle
American Journal of Clinical Pathology, Volume 154; https://doi.org/10.1093/ajcp/aqaa161.062

Abstract:
Casestudy: Angiosarcomas represent 1% of all soft tissue malignancies. Primary pleural angiosarcomas are aggressive and extremely rare. The etiology of pleural angiosarcoma is largely unknown but in the few reported cases it is associated with asbestos exposure. Common presenting symptoms are chest pain, cough, dyspnea, and weight loss. Localized tumors are generally resected, with other options including chemotherapy and radiotherapy. Limited literature describes this rare neoplasm’s metastatic potential and patterns. We present a 64-year-old male with shoulder pain and chronic cough. CT thorax showed a large 6.7 x 8 cm mass with central necrosis in the left upper lung invading the chest wall with partial destruction of the second and third ribs, and axillary adenopathy. Bilateral adrenal nodules were also identified. Histology of the adrenal lesion reveal sheets of infiltrative polygonal/round cells with large nuclei, prominent nucleoli and eosinophilic cytoplasm. Tumor cells were positive for CD31, CD34, FLI-1, AE1/AE3 and CK7 immunohistochemical stain, correlating with primary epithelioid angiosarcoma. Based on the patients new development of confusion, dizziness and ataxia, an MRI was performed showing multiple metastatic brain lesions. One month after initial presentation, the patient again presented with worsening symptoms. Work up showed enlargement of the bilateral adrenal masses, doubling of the brain lesions and an additional lesion posterior to the left kidney. This case shows the metastatic potential and pattern of an aggressive primary neoplasm that is not described in current literature. It also highlights the importance of timely intervention based on the rapid metastatic progression of primary pleural angiosarcomas.
A L Donfut, J Lemaitre, H Van De Walle, S Holbrechts, P Carlier, C Winant, E Lebrun
Published: 31 July 2014
Acta chirurgica Belgica, Volume 114

Abstract:
We describe the case of a 56-year-old man presenting a primary pulmonary epithelioid angiosarcoma versus malignant epithelioid hemangioendothelioma still alive, without recurrence at nearly two years after the beginning of the symptoms. The primary pulmonary angiosarcoma is extremely rare, being reported only in a handful of cases. Metastatic involvement of the lung (90%) is far more common than primary pulmonary involvement (10%). Various predisposing condition for the development of angiosarcoma have been described. Early diagnosis is not common, because of the rarity of angiosarcoma in the lung and consequent low index of suspicion. Due to the paucity of cases, there are no defined treatment regimens for this entity. However, there is a tendency for surgical intervention in all reported cases.
Zulma Cardona, Yasser A Haider, Yuval Eisenberg
Published: 1 April 2020
Journal of the Endocrine Society, Volume 4; https://doi.org/10.1210/jendso/bvaa046.2228

Abstract:
Angiosarcoma is a rare aggressive malignancy originating from the endothelium of blood and lymphatic vessels, accounting for 1% of all tissue sarcomas. These tumors usually invade skin and soft tissue, however they may also invade visceral organs and bones as metastatic disease. Approximately 50 cases have been reported in the literature describing primary adrenal angiosarcomas (PAA). This case represents the first report to describe concomitant Cushing’s syndrome in the setting of epithelioid PAA. A 38-year-old female with pre-diabetes mellitus and hyperlipidemia presented to the emergency department complaining of cough with bloody sputum. Physical exam was remarkable for bilateral lower extremity edema; no Cushing’s features appreciated. Bilateral subsegmental and lobar pulmonary emboli (PE) along with lytic lesions on the ribs were found on Computed Tomography (CT) PE protocol. Chest CT Angiography to prepare for arterial embolization to treat hemoptysis, demonstrated a large heterogeneous enhancing mass arising from the adrenal gland. MRI with adrenal protocol to evaluate incidentaloma revealed an indeterminate 5.2 x 5.6 x 5.2 cm right adrenal mass with central necrosis.Biochemical evaluation revealed a nonsupressible 1mg dexamethasone suppression test (3.3 ug/dL, ref: <1.8 ug/dL), slightly elevated urine metanephrines (191 ug/d, ref: 39 -143 ug/d) and normal plasma metanephrines (0.16 nmol/L, ref: 0.0 - 0.49 nmol/L). Given the biochemical evidence of mild hypercortisolism and uncertainty of etiology of other CT findings, the decision was made to pursue adrenalectomy to aid in the presumed oncologic diagnosis. She underwent successful adrenalectomy, however the postoperative course was complicated by hypotension resistant to fluids. ACTH stimulation test revealed a baseline cortisol <1.0 (ref: 0.0-9.0 ug/dL) with an ACTH of 8 pg/mL (ref: 6 to 58pg/mL), and 60 minute post stimulation cortisol level was 5.5 ug/dL (ref: >18.0 ug/dL). She was started on hydrocortisone replacement therapy, with clinical improvement in hypotension and was eventually tapered off steroids completely.Surgical pathology identified positive staining for CD31, CD34, factor 8, CAM 5.2, and Vimentin consistent with epithelioid angiosarcoma with lymphovascular invasion. There was additionally evidence of adrenocortical hyperplasia on the tissue resected. She established care with oncology and is currently completing 8 cycles of paclitaxel for metastatic disease to bones and lungs. Our patient’s pathology results, and postoperative course suggest the presence of concomitant non-ACTH dependent Cushing’s syndrome associated with epithelioid PAA. This case highlights the importance of thorough biochemical testing before adrenalectomy in the setting of PAA given the possibility of Cushing’s syndrome, which could contribute to post-surgical complications, and prolonged hospital stays.
, T. Stachowicz-Stencel, A. Balcerska, J. Godzinski, B. Kazanowska, , W. Madziara, A. Rybczynska, A. Kurylak, B. Zalewska-Szewczyk, et al.
Published: 1 July 2009
European Journal of Cancer Care, Volume 18, pp 411-420; https://doi.org/10.1111/j.1365-2354.2008.01063.x

The publisher has not yet granted permission to display this abstract.
P Garcia Bras, L Moura Branco, A Castelo, V Vaz Ferreira, T Branco Mano, A Galrinho, A T Timoteo, J Abreu, E Pinto, P Coelho, et al.
European Heart Journal – Cardiovascular Imaging, Volume 21; https://doi.org/10.1093/ehjci/jez319.658

Abstract:
Background Primary cardiac tumors are extremely rare and are usually diagnosed late due to the non-specific symptomatology. Surgery is the main treatment option and despite chemotherapy, the prognosis remains poor. Cardiac invasion by metastatic tumors, while more common, also entails an unsatisfactory outcome. Purpose To review patients (P) with malignant cardiac tumours that were diagnosed by transthoracic echocardiography (TTE) or by transesophageal echocardiogram (TEE) in a tertiary center between 1997 and 2019. Methods Retrospective analysis of clinical data from the digital files, echocardiographic assessment of tumor location and morphology, histology results and survival outcomes. Results A total of 33 malignant cardiac tumors were diagnosed: 12 primary tumors (A) and 21 metastatic tumors (B). A Regarding primary cardiac tumors, the most common types were angiosarcomas (6 cases), 2 undifferentiated pleomorphic sarcomas, 1 right ventricle (RV) sarcoma, 1 primary cardiac lymphoma, 1 myxofibrosarcoma and 1 fibrosarcoma. The mean age of the P at time of diagnosis was 43 ± 15 years, 50% female gender. The most frequent presentation was heart failure symptoms (50% of P) followed by arrhythmias (20%). One patient had a rare presentation with pruritus and polyarthralgias. On TTE, the most prevalent tumor location was in the right-heart chambers (70%) - mostly the right atrium (50%), with mean dimensions of 40 ± 18 mm x 27 ± 11 mm. 85% of patients had preserved systolic left ventricular function and there was moderate or severe pericardial effusion in 38%. The most frequent distant metastatic involvement of primary tumors at diagnosis was pulmonary (33%) and hepatic (33%). 50% of P were submitted to tumor resection and 40% were submitted to chemotherapy. In the case of angiosarcomas, the most common immunohistochemical markers were vimentin, CD31 and CD34. The authors found a mortality rate of 81% in P with primary cardiac tumors, with a median time of follow-up of 6 months (minimum of 20 days and maximum of 18 years). In the latter case, the P was submitted to heart transplantation after diagnosis of a sarcoma of the RV and is still alive and well. B Regarding secondary cardiac invasion, there was a diagnosis of the following primary tumor sites: 6 thymomas, 4 cases of lymphoma, 3 lung carcinomas, 3 hepatocellular carcinomas, 2 bladder carcinomas, 1 parathyroid carcinoma, 1 soft tissue sarcoma and 1 melanoma. The mean age of P with metastastic involvement of the heart was 57 ± 22 years, 65% male. On TTE/TEE the authors also found a right-sided chambers predominance (60%), with pericardial metastasis in 35%. As expected, the mortality rate was also extremely high (90%), with a median time of follow up of 1.5 months (minimum of one week, maximum of 44 months) Conclusion Cardiac malignant tumors generally present in a late stage with a dismal prognosis. When possible, heart transplantation can be an option with a good outcome.
Julie A. Teague, Alan Pham, Ilana Gory
Published: 1 January 2012
Journal: Pathology
The publisher has not yet granted permission to display this abstract.
Published: 6 April 2018
Clinical Sarcoma Research, Volume 8, pp 1-6; https://doi.org/10.1186/s13569-018-0092-z

Abstract:
This review of the literature combined with a clinical case will allow the illustration of a favorable outcome of this variable low grade malignancy, display a role for limb salvage surgery with intralesional treatment, and offer a clinical example of epithelioid hemangioendothelioma, a rare malignancy. The case report presents a case of solitary epithelioid hemangioendothelioma (EHE) of the calcaneus in a 60-year-old male. Primary vascular tumors of the bone are rare; however, EHE is one of the most common primary malignant vascular tumors to occur in bone. A review of the literature found few cases that involved the calcaneus; those cases found that involved the calcaneus were either part of a multifocal or metastatic disease process. Our case presents a 45-month clinical follow-up of solitary EHE in the calcaneus treated with surgical excision by curettage and cementing. This case has clinical follow-up greater than 2 years post-operatively and could be a guide for treatment of a rare disorder with a substantial paucity of literature.
David J. Rosenfeld, Mark A. Cappel,
Published: 14 March 2021
International Journal of Dermatology, Volume 60, pp 1334-1342; https://doi.org/10.1111/ijd.15500

The publisher has not yet granted permission to display this abstract.
Epameinondas Dogeas, Ali A. Mokdad, Mitu Bhattatiry, , Patricio M. Polanco, John C. Mansour, Michael A. Choti,
Published: 12 April 2021
Journal of Surgical Research, Volume 264, pp 481-489; https://doi.org/10.1016/j.jss.2021.02.043

The publisher has not yet granted permission to display this abstract.
L. López, , L. Cancelo, A. Gómez, F. Uresandi, V. Atxotegui
Published: 1 April 2004
Archivos de Bronconeumología, Volume 40, pp 188-190; https://doi.org/10.1016/s1579-2129(06)60213-9

The publisher has not yet granted permission to display this abstract.
Published: 1 September 2018
The Permanente Journal, Volume 22, pp 17-168; https://doi.org/10.7812/tpp/17-168

Abstract:
Angiosarcomas are rare, malignant vascular tumors that affect endothelial cells of blood vessels. Angiosarcomas most commonly occur on the scalp or face of elderly individuals and are highly aggressive, with a 5-year survival rate below 15%. Cutaneous angiosarcomas often metastasize to the lung, where they can present with cystic lesions, solid lesions, pneumothorax, and/or hemothorax. We report the case of an 83-year-old woman who presented with a scalp lesion, which was initially thought to be caused by scalp trauma but was later found to be an angiosarcoma. She initially refused any therapy for the tumor. She returned several months later with a cough and shortness of breath and was found to have multiple pulmonary cysts. She was treated with paclitaxel, but her tumor did not respond to the therapy and she died 2 months later. We discuss the common presentation of cutaneous angiosarcomas and their tendency to metastasize to the lung and present as cystic lesions. We also review the common conditions that can cause cystic changes in the lungs.
P. Roy, M.A. Clark,
Published: 30 November 2004
European Journal of Surgical Oncology, Volume 30, pp 982-986; https://doi.org/10.1016/j.ejso.2004.07.027

Abstract:
Stewart–Treves syndrome is an angiosarcoma associated with long-standing lymphoedema, most commonly seen as a rare complication of breast cancer treatment, and is associated with a poor outcome. We present results from six patients supporting the use of early radical surgery to improve prognosis. Six patients with Stewart–Treves syndrome were diagnosed and treated at our centre over an 11-year period. Five patients had forequarter amputation and the sixth had a through-hip amputation. Four of the six patients are alive and well following surgery (at 3, 16, 23, and 135 months after amputation); one patient died from metastatic disease at 15 months and the second died due to an unrelated malignancy. Early diagnosis and treatment by radical ablative surgery confers a reasonable prognosis with this rare but aggressive disease. A nihilistic approach is unjustified.
P. Roy, M.A. Clark,
Published: 1 November 2004
European Journal of Surgical Oncology, Volume 30, pp 982-986; https://doi.org/10.1016/s0748-7983(04)00215-x

The publisher has not yet granted permission to display this abstract.
P Garcia Bras, S Aguiar Rosa, L Ferreira, L Moura Branco, A Castelo, V Vaz Ferreira, J Branco Ferrao, F Martins, L Sousa, A Fiarresga, et al.
European Heart Journal – Cardiovascular Imaging, Volume 21; https://doi.org/10.1093/ehjci/jez319.094

Abstract:
Introduction Primary cardiac tumors are rare entities and 75% are benign. Angiosarcoma is the most common malignant primary cardiac tumor. We report the case of cardiac angiosarcoma presenting with an atrial arrhythmia. Clinical case A 39-year-old female patient with no past medical history presented to the emergency department with heart palpitations and atypical chest pain. Electrocardiogram on admission showed atrial flutter with a heart rate of 153 beats per minute. Laboratory analysis were performed showing elevated D-dimer levels (2210 ug/L). A thoracic CT scan was performed, which ruled out pulmonary embolism, but showed multiple pulmonary nodules and a right atrial (RA) mass measuring 48 mm that could correspond to a thrombus or neoplasia. The patient was admitted in the Cardiology ICU of our hospital and was started on beta-blocker and amiodarone with conversion to sinus rhythm. Additional exams were performed: - Transthoracic echocardiogram (TTE) revealed an heterogenous 32,6 x 17,7 mm mass in the lateral wall of the RA with an adherent mobile mass near the tricuspid valve with 28 mm diameter (possible adherent thrombus). - Cardiac magnetic resonance imaging confirmed a RA tumor with invasion of the atrial free wall and compression of the superior vena cava. Due to the unclear etiology of the RA mass, ultrasound-guided intracardiac biopsy was performed. Pathological examination revealed spindle cell proliferation, consistent with the diagnosis of angiosarcoma. Immunohistochemical staining was positive for Vimentin, CD34 and CD31, with 70% Ki67 expression. Later on, the patient developed melena with significant drop of hemoglobin levels, requiring daily red blood cell transfusions and anticoagulation had to be stopped. The patient was transferred to the Internal Medicine ward and thoracic-abdomen-pelvis staging computed tomography (CT) scan showed a significant increase in the number of pulmonary nodules, bilateral ovarian masses, 4 hepatic nodules and ileum metastization. During hospitalization, the patient developed right leg deep venous thrombosis and thoracic CT scan revealed bilateral pulmonary embolism. After improvement of the clinical status, palliative chemotherapy was started and the patient was discharged, maintaining regular outpatient follow-up in the Oncology Department for 1 month. Cardiac angiosarcoma generally presents in a late stage of the disease with metastatic involvement. When surgical treatment is not possible, despite agressive chemotherapy, the prognosis remains poor. Abstract P229 Figure. Echocardiogram: right atrium mass
, E. Llinares-Tello, , L. Gràcia-Sánchez, J. Robles-Barba, C. Gámez-Cenzano
Revista Española de Medicina Nuclear e Imagen Molecular (English Edition), Volume 38, pp 183-185; https://doi.org/10.1016/j.remnie.2018.11.001

The publisher has not yet granted permission to display this abstract.
Vaia Florou, Breelyn A. Wilky, Jonathan C. Trent
Journal of Clinical Oncology, Volume 37; https://doi.org/10.1200/jco.2019.37.15_suppl.e22543

Abstract:
E22543 Background: Angiosarcoma (AS) is a rare endothelial malignancy with only 2–3 new cases per one million diagnosed yearly but is among the most aggressive soft tissue sarcomas. Some cutaneous angiosarcomas may have a mutational landscape similar to UV light exposed melanoma. These findings offer a rationale for the early limited evidence of checkpoint inhibitor activity in cutaneous angiosarcomas. Methods: This is a retrospective review of patients with locally advanced or metastatic angiosarcoma, who were treated with checkpoint inhibitors at our institution. Descriptive statistics were used to analyze our findings. Results: Since 2016, we have treated seven angiosarcoma patients with checkpoint inhibitors either in the context of clinical trials or off label [Pembrolizumab + Axitinib (NCT02636725; n = 1), a CTLA-4 inhibitor (NCT02694822; n = 2), Pembrolizumab (n = 4)]. Five patients had cutaneous AS, one primary breast AS and one radiation associated breast AS. The average age was 66 years old and 5/7 (71%) patients were females. All patients had received prior systemic therapies (range 1-7, mean 3) and 2/7 (29%) patients had metastatic disease at the time of treatment. The mean number of checkpoint inhibitor doses was 6. At twelve weeks, 5/7 patients (71%) had partial response, by RECIST, of their lesions either on imaging or clinical exam and two (29%) had progressive disease. 6/7 patients are alive to date and, thus far, 3/7 patients (43%) progressed (median 3.4 months)- one achieved partial response after pembrolizumab was switched to ongoing Nivolumab/Ipilimumab, one died of progressive disease at 31 weeks (primary breast AS) and one was placed on subsequent therapy. No patient experienced any toxicities more than grade 2. Conclusions: This is a single institution, retrospective review of patients with AS treated with checkpoint inhibitors, first reported in the literature. Checkpoint inhibitors may be an effective and safe treatment option for this aggressive sarcoma. Prospective clinical trials are needed to systemically study this effect.
, K Kerl, R Dummer, N Schoenewolf, A Cozzio
Journal of the European Academy of Dermatology and Venereology, Volume 24, pp 1230-1234; https://doi.org/10.1111/j.1468-3083.2010.03624.x

Abstract:
BACKGROUND: Cutaneous angiosarcoma (AS) is a rare malignant tumour of endothelial origin with very poor prognosis, frequent recurrences and high metastatic potential. Clinical suspicion is often raised too late, but histological findings and immunohistochemical assays have proved to be very helpful in the diagnostic process.\ud \ud PATIENTS AND METHODS: Over the last 13 years, nine patients with AS were found in our archives. Clinical features, evolution, treatment and outcome were analysed and all biopsy specimens were reviewed by a trained dermatopathologist, with subsequent immunohistochemical assessment.\ud \ud RESULTS AND CONCLUSIONS: Cutaneous AS was clinically diagnosed in 4 of 9 patients, while systemic lupus erythematosus was the most common misdiagnosis. Radiotherapy was the most prescribed treatment, but many different combinations of surgery, chemotherapy and radiotherapy were observed. Mean disease-free and overall survival (15.4 and 23.7 respectively) were consistent with previous series, with local recurrence rate (2/9) lower than previously reported data. CD31 was positive in all patients. Vimentin, D2-40 and VEGFR-3 were expressed by the vast majority, Factor VIII by 3/7 and CD34 by about 1/3 of patients. Cytokeratin was negative in all patients. The patients with the most unfavourable course showed a strong expression of Ki-67, while those with the best outcome only had a slight positive Ki-67 staining. Larger studies regarding tumour cell expression of Ki-67 and other markers such as D2-40 will be helpful to evaluate a potential prognostic value of these stainings
Christina Kelly Lopes, Gerald Post, Lindsay Lambert, Benjamin Lewis, Thaddeus David Allen, Angel Patel, Bruce A Littlefield
Molecular cancer therapeutics, Volume 18; https://doi.org/10.1158/1535-7163.targ-19-c098

Abstract:
Eribulin is a microtubule dynamics inhibitor that is currently in clinical use for treatment of certain patients with advanced breast cancer or liposarcoma. FDA approval for both indications was based on extension of overall survival. Based on eribulin’s known vascular remodeling effects, its activity against at least one sarcoma type, and literature reports of taxane activity in angiosarcoma, we hypothesized that eribulin may show activity against angiosarcoma. Due to a paucity of preclinical cell-based, xenograft, and PDX models for human angiosarcoma, we sought other alternatives for preclinical testing in this disease. While rare in humans, angiosarcoma is common in some breeds of domestic dogs, where it is known in veterinary parlance as hemangiosarcoma (HSA). Accordingly, we undertook a small veterinary clinical trial to evaluate eribulin against spontaneous HSA in pet dogs. Canine HSA most frequently occurs in the spleen, so splenectomy is a common first-line treatment; however, non-splenic sites also occur and these are often unresectable. In keeping with eribulin’s clinical use in the metastatic setting, we selected metastatic canine HSA, which we defined as dogs with histologically confirmed HSA with either non-resectable or post-splenectomy residual disease confirmed by imaging (PET/CT, CT). Of 19 pets initially screened, 12 dogs were imaged; 6 of these had imageable disease and thus began eribulin treatment. Eribulin was administered using the human clinical schedule (d1,8Q21), with the first cycle at 0.3 mg/m2 followed by intra-patient dose escalation to 0.6 mg/m2 after determination of tolerability. Of the 6 dogs entering treatment, 5 completed cycle 1, 3 completed cycle 2, and 2 completed the full per-protocol 3 cycles of treatment. These 2 dogs survived 121 and 179 days after initial diagnosis, contrasting with the 0.9 month (~27 days) median survival time reported by Wendelburg et al. (JAVMA, 2015) for 52 post-splenectomy canine HSA patients with residual metastases. Although limited in scope, our results with spontaneous metastatic canine HSA in pet dogs provides a preclinical foundation for further exploration of eribulin in malignancies of vascular origin in both the veterinary and human settings. Citation Format: Christina Kelly Lopes, Gerald Post, Lindsay Lambert, Benjamin Lewis, Thaddeus David Allen, Angel Patel, Bruce A Littlefield. Preclinical evaluation of eribulin in a veterinary clinical trial of metastatic spontaneous hemangiosarcoma in pet dogs [abstract]. In: Proceedings of the AACR-NCI-EORTC International Conference on Molecular Targets and Cancer Therapeutics; 2019 Oct 26-30; Boston, MA. Philadelphia (PA): AACR; Mol Cancer Ther 2019;18(12 Suppl):Abstract nr C098. doi:10.1158/1535-7163.TARG-19-C098
A.K. Yoder, A. Farooqi, C. Wernz, A. Subramaniam, J. Zheng, V. Ravi, R.P. Goepfert, E.M. Sturgis, D. Mitra, A.J. Bishop, et al.
International Journal of Radiation Oncology - Biology - Physics, Volume 114; https://doi.org/10.1016/j.ijrobp.2022.07.1821

The publisher has not yet granted permission to display this abstract.
J. C. Fanburg, J. M. Meis-Kindblom, A. E. Rosenberg
The American journal of surgical pathology, Volume 19, pp 1029-1038; https://doi.org/10.1097/00000478-199509000-00006

Abstract:
Maffucci's syndrome is classically defined as the association of multiple enchondromas and hemangiomas. Spindle-cell hemangioendothelioma (SCH), a recently described vascular tumor of purported low malignant potential, has both cavernous hemangioma and Kaposi-like features. We report six patients with Maffucci's syndrome in whom all vascular lesions were SCH. The enchondromas involved the small and long tubular bones of the extremities in all of these patients; flat bones were also involved in three patients. The SCH usually arose in the extremities, distal to the knees and elbows. Five of the six patients had multiple and separate nodules of SCH, and in four patients there was recurrent or persistent SCH within 6 months to 4 years after initial removal. One patient also had a vascular tumor in the spleen mainly with features of a low-grade angiosarcoma with separate SCH-like foci. None of the SCH have metastasized within a follow-up period averaging 20 years. Five patients are alive 14 to 31 years after presentation. One patient died from metastatic dedifferentiated chondrosarcoma. The patient with the low-grade splenic angiosarcoma is alive approximately 2 years after diagnosis. Reappraisal of the older literature suggests that some of the vascular tumors occurring in Maffucci's syndrome, previously diagnosed as hemangiomas, may in fact be SCH. The apparent association between Maffucci's syndrome and SCH, the presence of SCH in other congenital syndromes, and the young patient age and multicentric distribution of SCH unassociated with Maffucci's syndrome raise the possibility that SCH may be a manifestation of a congenital mesodermal disorder with a genetic background related to Maffucci's syndrome. Although the behavior of SCH appears to be one of a locally recurrent or persistent multicentric lesion that does not metastasize, the association of SCH-like foci in a low-grade angiosarcoma of the spleen raises the possibility that SCH may rarely be associated with a higher grade lesion. Therefore, SCH, at least in the setting of Maffucci's syndrome, should be carefully monitored.
Lori A. Erickson
Published: 24 February 2014
The publisher has not yet granted permission to display this abstract.
, Jesse K. McKenney, Julia A. Bridge, Sharon W. Weiss
The American journal of surgical pathology, Volume 26, pp 1175-1183; https://doi.org/10.1097/00000478-200209000-00008

Abstract:
Rhabdomyosarcomas (RMSs) are classified into embryonal (ERMS), alveolar (ARMS), and pleomorphic (PRMS) subtypes. ERMS, including botryoid variants, typically occurs in young children, ARMS typically occurs in older children and young adults, and PRMS occurs in older adults. Although ARMSs show thin fibrous bands separating nests of cells, abundant extracellular matrix production is rare in RMS. In the course of reviewing hyalinizing sarcomas we discovered a distinctive RMS in adults that closely mimicked osteosarcoma or chondrosarcoma because of the extensive matrix production. Four RMSs with hyalinized matrix were retrieved from our files. These cases were evaluated with respect to patient age and sex, tumor site and size, growth pattern, nuclear grade, cellularity, mitotic figures/20 high power fields, vascular invasion, necrosis, the presence of rhabdomyoblasts, multinucleated cells, and alveolar growth pattern. Immunohistochemistry for desmin, myogenin, MyoD1, actin, cytokeratin, S-100 protein, collagen II, and CD99 was performed. Reverse transcriptase polymerase chain reaction for the ARMS-associated PAX3/FKHR and PAX7/PKHF was also performed on three cases. The cases involved the forearm, hand, orbit, and nasopharynx of a 40-year-old woman, a 50-year-old man, an 18-year-old man, and a 21-year-old man, respectively. The tumors ranged from 3.7 to 8 cm and consisted of lobules and infiltrating cords of small round malignant cells embedded in a densely hyalinized matrix having both a chondroid and osteoid-like appearance. No definite lacunae or matrix calcification was present. An alveolar pattern was only present focally, and tumor giant cells were not present. One case had a single focus of rhabdomyoblastic differentiation with strap cells. Mitotic activity was >20 mitotic figures/20 high power fields in three of four cases. Immunohistochemically, one case strongly expressed desmin, whereas three cases expressed it focally, with a dot-like pattern. Myogenin was only focally positive, but MyoD1 was present in nearly every cell of each case. Two cases expressed actin and one expressed CD99. No case expressed cytokeratin, S-100 protein, or collagen II. Only one case contained adequate RNA for reverse transcriptase polymerase chain reaction, and this case was negative for the ARMS-associated gene fusions. Follow-up showed one patient to be dead of metastatic disease at 60 months despite intensive therapy, another patient to be disease free at 26 months, and the third patient to be disease free at 5 months. The fourth case is recent. These cases are a distinctive-appearing rhabdomyosarcoma easily mistaken for variants of chondrosarcoma, osteosarcoma, or even sclerosing epithelioid fibrosarcoma because of their hyalinizing appearance compounded by their typically focal and dot-like desmin expression. These four cases are essentially identical to the three unusual RMSs recently reported by Mentzel and Katenkamp as “sclerosing, pseudovascular rhabdomyosarcoma in adults.” Although the focal alveolar architecture and the primitive cytologic appearance of these hyalinizing RMS suggest a relationship with ARMS, the presence of abundant strap cells in one case, the predominant expression of MyoD1 rather than myogenin, and the absence of ARMS-associated fusions genes point more strongly toward a variant of ERMS. However, the late adult age in two cases is unusual for both EMRS and ARMS, suggesting that sclerosing RMS may prove to be a distinct subtype of RMS. Study of additional cases will be necessary to more fully elucidate its place among RMS and its prognostic significance.
, , Grace A. Tye, Yueyi I. Liu, Naveen N. Parti,
Published: 1 October 2009
Seminars in Ultrasound, CT and MRI, Volume 30, pp 387-407; https://doi.org/10.1053/j.sult.2009.06.001

Abstract:
Hypervascular hepatocellular lesions include both benign and malignant etiologies. In the benign category, focal nodular hyperplasia and adenoma are typically hypervascular. In addition, some regenerative nodules in cirrhosis may be hypervascular. Malignant hypervascular primary hepatocellular lesions include hepatocellular carcinoma, fibrolamellar carcinoma, and peripheral cholangiocarcinoma. Vascular liver lesions often appear hypervascular because they tend to follow the enhancement of the blood pool; these include hemangiomas, arteriovenous malformations, angiosarcomas, and peliosis. While most gastrointestinal malignancies that metastasize to the liver will appear hypovascular on arterial and portal-venous phase imaging, certain cancers such as metastatic neuroendocrine tumors (including pancreatic neuroendocrine tumors, carcinoid, and gastrointestinal stromal tumors) tend to produce hypervascular metastases due to the greater recruitment of arterial blood supply. Finally, rare hepatic lesions such as glomus tumor and inflammatory pseudotumor may have a hypervascular appearance.
Alexandra W. Acher, Amir A. Rahnemai-Azar, Sharon M. Weber,
Published: 4 June 2022
The publisher has not yet granted permission to display this abstract.
, R. Locke, A. Sandison, M. Jenkins, M. Hamady
Published: 10 February 2010
CardioVascular and Interventional Radiology, Volume 34, pp 20-24; https://doi.org/10.1007/s00270-009-9776-3

Abstract:
Primary malignant tumours of the aorta are rare. They present with aneurysm formation, arterial occlusion, and embolic phenomenon. We report the case of a 56-year-old man whose initial presentation and investigations lead to emergency endovascular stenting of a descending thoracic aneurysm with a contained leak. Initial response was favourable, yet the patient presented again with worsening symptoms. The circum-aortic haematoma expanded by 50% on subsequent imaging, but no endoleak was identified. When altered bone marrow signal was identified on magnetic resonance imaging, the possibility of malignancy was considered. A metastatic skin lesion was then biopsied, which demonstrated morphological and immunohistochemical features consistent with metastases from a pleomorphic sarcoma of the aorta.
Js Ross, J Chung, Je Elvin, J-A Vergilio, S Ramkissoon, J Suh, E Severson, S Daniel, Gm Frampton, D Fabrizio, et al.
Published: 15 February 2018
Journal: Cancer Research
Abstract:
Background: The tropomysin receptor kinase A family includes the 3 NTRK1, NTRK2 and NTRK3 genes and plays major roles in neuronal development. The recent evidence of remarkable efficacy for kinase inhibitors (TKI) targeting NTRK across a wide variety of malignancies that harbor NTRK gene fusions has stimulated great interest in determining the type of cancers driven by these therapy defining NTRK genomic alterations. Methods: A consecutive series of 12,214 locally aggressive, relapsed and metastatic breast malignancies (mBM) were subjected to comprehensive genomic profiling (CGP) using DNA extracted from 40 µm of FFPE sections and adaptor ligation-based libraries to a mean coverage depth 719X for up to 315 cancer-related genes. The results were analyzed for all classes of genomic alterations (GA) including base substitutions, insertions and deletions, select fusions and rearrangements, and copy number changes. Tumor mutational burden (TMB) was determined on 1.1 Mbp of sequenced DNA. Microsatellite instability (MSI) status was calculated by a customized algorithm. Results: 16 (0.13%) mBM (all female) harbored NTRK gene fusions. The median age was 51 years (range 34 to 70 years). There were 9 ductal carcinomas, 2 lobular carcinomas, 3 secretory carcinomas 1 metaplastic carcinoma and 1 angiosarcoma. Tumor stages at the time of sequencing were 12 Stage IV, 1 Stage III and 3 stage I (all 3 secretory carcinomas). In 9 cases, clinical receptor status was known: 3 (33%) were ER+/HER2- and 6 (66%) ER-/HER2- (all TNBC) with all 9 (100%) of cases HER2-. All 3 SCA were TNBC. 10 NTRK fusions involved NTRK1 featuring a variety of fusion partners (CGN, GATAD2B, LMNA, MDM4, PEAR1, and TPM3) and 6 involved NTRK3 (all ETV6 fusions). There were no NTRK2 fusions. NTRK fusion+ mBM featured a mean of 4.25 GA per sample. The most frequent non-fusion partner co-altered genes in this series of NTRK fusion+ mBM were: TP53 at 25%, IKBKE, PIK3C2B CCND1 at 19%, and AKT, PIK3CA, MYC, CDH1, CDKN2A, PTEN, FGF3, FGF4 and FGF19 all at 13%. The median TMB for NTRK fusion+ mBM was 0.9 mutations/Mb and no cases (0%) had a TMB > 10 mutations/Mb and no cases (0%) feared high microsatellite instability (MSI high). Clinical response assessment to NTRK TKI therapies in this series is ongoing. Conclusions:NTRK gene fusions although extremely uncommon in breast malignancies occur across a variety of tumor types, is universally HER2 negative, more frequent in TNBC than in ER+ tumors, is associated with a moderate frequency of additional genomic alterations and a complete absence of either high TMB or high MSI. This study confirms that a CGP assay, when applied to a large cohort of near universal clinically advanced disease can identify extremely rare alterations that can lead a small number of patients to highly effective precision therapies. Citation Format: Ross JS, Chung J, Elvin JE, Vergilio J-A, Ramkissoon S, Suh J, Severson E, Daniel S, Frampton GM, Fabrizio D, Hartmaier RJ, Albacker LA, Ali SM, Schrock AB, Miller VA, Stephens PJ, Gay LM. NTRK fusions in breast cancer: Clinical, pathologic and genomic findings [abstract]. In: Proceedings of the 2017 San Antonio Breast Cancer Symposium; 2017 Dec 5-9; San Antonio, TX. Philadelphia (PA): AACR; Cancer Res 2018;78(4 Suppl):Abstract nr P2-09-15.
P Garcia Bras, L Moura Branco, A Galrinho, A Castelo, V Ferreira, T Mano, J Reis, A Grazina, A T Timoteo, J Abreu, et al.
Published: 1 October 2021
European Heart Journal, Volume 42; https://doi.org/10.1093/eurheartj/ehab724.1837

Abstract:
Background: Primary cardiac tumors are extremely rare and are usually diagnosed late due to the nonspecific symptoms. Surgery is the main treatment option, and despite chemotherapy, the prognosis remains poor. Cardiac invasion by metastatic tumors, while more common, also entails an unsatisfactory outcome. The aim of this study was to review patients (P) with malignant cardiac tumors that were diagnosed by transthoracic echocardiogram (TTE) or transoesophageal echocardiogram (TOE) in a tertiary center between 1995 and 2020. Methods: Retrospective analysis of clinical data, echocardiographic assessment of tumor location and morphology, histology results and survival outcomes. Results: A total of 44 malignant cardiac tumors were diagnosed: 12 primary tumors (A) and 32 metastatic tumors (B). A: Regarding primary cardiac tumors, the most common types were angiosarcomas (6), undifferentiated pleomorphic sarcomas (2), fibrosarcomas (2), myxofibrosarcoma (1) and primary cardiac lymphoma (1). The mean age of P at time of diagnosis was 43±15 years, 50% male. The most frequent presentation was heart failure symptoms (50%) followed by arrhythmias (20%). 1 P presented with pruritus and polyarthralgias. On TTE, the most prevalent tumor location was in the right-heart chambers (70%) – mostly the right atrium (50%), with mean dimensions of 40±18x27±11 mm. 85% of P had preserved biventricular systolic function and there was severe pericardial effusion in 38%. The most frequent metastatic involvement at diagnosis was pulmonary (33%) and hepatic (33%). 50% of P were submitted to tumor resection and 40% to chemotherapy. Regarding angiosarcomas, the most common immunohistochemical markers were vimentin, CD31 and CD34. The authors found a mortality rate of 10P (83%) in P with primary cardiac tumors, with a median time to mortality of 5.5 (IQR 2–10) months (Figure 1), in a median follow-up of 6.2 (IQR 2.5–15) months (minimum of 20 days and maximum of 19 years). In the latter case, the P was submitted to heart transplantation after diagnosis of a fibrosarcoma and is still alive and well. B: Regarding secondary cardiac invasion, there was a diagnosis of the following primary tumor sites: 12 lung carcinomas, 7 thymomas, 4 lymphomas, 3 hepatocellular carcinomas, 2 bladder carcinomas, 1 parathyroid carcinoma, 1 soft tissue sarcoma, 1 uterine sarcoma and 1 melanoma. The mean age of P was 57±19 years, 57% male. On TTE/TOE the authors found a right-sided chambers predominance (12 P, 38%) vs left-sided chambers in (8 P, 25%), with pericardial metastasis also present in 13 P (41%). Cardiac tamponade occurred in 6P (19%). The mortality rate was 75% (24P), with a median time to mortality of 1.1 (IQR 0.6–3.8) months (minimum of 7 days, maximum of 44 months), in a median follow-up of 2.3 (IQR 0.8–14) months. Conclusion: Cardiac malignant tumors generally present in a late stage with a dismal prognosis. When possible, heart transplantation can be an option with a favourable outcome. Funding Acknowledgement: Type of funding sources: None. Figure 1. Kaplan-Meier analysis
Kimberley M Heinhuis, , Anne Miek Koenen, Winette T A van der Graaf, Rick L Haas, Jos H Beijnen, Alwin D R Huitema, Winan J van Houdt, Neeltje Steeghs
Published: 10 September 2020
by BMJ
Journal: BMJ Open
Abstract:
Introduction Angiosarcoma is a rare and aggressive malignancy with a high metastatic potential and recurrence rate. Despite optimal treatment with surgery, with or without radiation, the prognosis remains poor and, therefore, new treatment strategies are warranted. Recently, propranolol has effectively been repurposed for the treatment of infantile haemangioma. Propranolol is a β3-sparing antagonist of the β-adrenergic receptor. In infantile haemangioma, the β1, β2 and β3 receptors are highly expressed. Angiosarcoma has several similarities with haemangioma, including its high β-adrenergic receptor expression and the supposedly important role of vascular endothelial growth factor in malignant growth. As a result, propranolol has been administered small scale in individual angiosarcoma cases with promising results. The precise effect of propranolol, however, is not yet established. Methods and analysis The goal of this neoadjuvant window of opportunity study is to prospectively evaluate the activity of propranolol monotherapy in patients with cutaneous angiosarcoma. The neoadjuvant setting provides a good opportunity to rapidly evaluate both the clinical response and histological response, without a significant delay in standard anticancer treatment. Fourteen patients with primary, recurrent or metastatic cutaneous angiosarcoma will be included. Propranolol will be administered orally in an escalating dose during 3–6 weeks, before the initiation of standard treatment. The primary endpoint is clinical response according to Response Evaluation Criteria in Solid Tumours, as measured on consecutive coloured photographs or CT/MRI. The histological response will be determined as secondary endpoint, comparing the difference in proliferation index before and after propranolol by measuring the change in immunohistochemistry staining of Ki-67. The study will be considered positive when at least three patients have a response to propranolol. Ethics and dissemination Ethical approval was obtained from the Medical Ethical Committee of the Netherlands Cancer Institute. Independent of the outcome, results of this study will be shared and submitted for publication in an international peer-reviewed journal. Trial registration number NL8118; registry through the Netherlands Trial Register.
Published: 4 December 2015
Journal: Histopathology
Histopathology, Volume 68, pp 33-44; https://doi.org/10.1111/his.12865

Abstract:
Although most breast spindle cell lesions (BSCLs) are rare, they constitute a wide spectrum of diseases, ranging from reactive processes to aggressive malignant tumours. Despite their varied histogenesis and behaviour, some lesions show an overlap of morphological features, making accurate diagnosis a challenging task, particularly in needle core biopsies. Clinical history and immunohistochemistry can help in making a correct diagnosis in morphologically challenging cases. To make an accurate diagnosis, it is important to maintain a wide differential diagnosis and be familiar with the diverse morphological appearances of these different entities. BSCLs can generally be classified into bland‐looking and malignant‐looking categories. In the former, the commonest diagnosis is scarring. However, it is important to distinguish low‐grade spindle cell metaplastic breast carcinoma from other benign entities, as the management is clearly different. In the malignant category, it is important to differentiate metaplastic carcinoma from other malignant primary and metastatic malignant spindle cell tumours of the breast, such as malignant phyllodes tumour, angiosarcoma, and melanoma. This review focuses on the classification and histological and molecular diagnosis of various BSCLs, with an emphasis on the diagnostic approach, including in core biopsies.
, A. Benedict Cosimi
Published: 1 April 2005
Journal: The oncologist
The oncologist, Volume 10, pp 269-281; https://doi.org/10.1634/theoncologist.10-4-269

Abstract:
Learning Objectives: After completing this course, the reader will be able to: List the indications for liver transplantation in patients with hepatobiliary malignancies.Describe the MELD and PELD system.Discuss the management and treatment algorithm of a patient with hepatobiliary malignancy. Access and take the CME test online and receive 1 hour of AMA PRA category 1 credit at CME.TheOncologist.com Liver transplantation for hepatic malignancies has emerged from an exotic and desperate approach to a well-documented and proven treatment modality for these unfortunate patients. However, early unsatisfactory results emphasized that only a highly selected patient population would benefit from transplantation. Currently, <10% of all liver transplants performed are for hepatocellular cancer (HCC). There is no controversy that hepatoblastoma is an excellent indication in pediatric patients with unresectable tumors. Similarly, liver transplantation for HCC in the adult population yields good results for patients whose tumor masses do not exceed the Milan criteria. It remains to be determined whether patients with more extensive tumors can be reliably selected to benefit from the procedure. Adjunctive procedures like radiofrequency ablation, chemoembolization, or cryotherapy might be indicated to limit tumor progression for patients on waiting lists. Epitheloid hemangioendothelioma is also an appropriate indication for liver transplantation, unlike angiosarcoma. Metastatic liver disease is not an indication for liver transplantation, with the exception of cases in which the primary is a neuroendocrine tumor, for which liver transplantation can result in long-term survival and even cure in a number of patients. And finally, while gallbladder cancers are never an indication for liver transplantation, rare cases of cholangiocellular cancer might qualify if aggressive combination therapies, including chemotherapy and radiotherapy followed by OLT, are carried through. Survival in these selected patients can approach that for patients with cholestatic liver disease.
E. V. Rozengauz, A. L. Dolbov, A. G. Karakhanova
Published: 12 December 2020
Medical Visualization, Volume 24; https://doi.org/10.24835/1607-0763-2020-4-42-50

Abstract:
Epithelioid hemangioendothelioma of the liver (EHEL) is a rare (1 case per 1 000,000 people) primary malignant neoplasm from the group of mesenchymal tumors. EHEL is the most aggressive representative of the hemangioendothelioma range with metastatic response and relapse rate, and therein is close to angiosarcoma.The purpose of this publication is to summarize our own experience in comparison with published data and to describe a disease symptom that was not investigated before.Materials and methods. We studied five cases of epithelioid hemangioendothelioma of the liver, confirmed by histological examination. All patients underwent standard computer tomography with bolus contrast agent.Results. The analysis of the images revealed typical for this pathology symptoms such as local retraction of the liver contour, a specific contrast pattern – “the lollipop sign”. We have revealed a new symptom: “the beading sign”, which was detected in four of five cases. In three of five cases, this symptom allowed us to determine the nature of the disease before histological examination.Conclusion. EHEL is a difficult disease to diagnose due to its rare frequency and similarity to other focal liver lesions. In aspect of radiology, it is advisable to focus on specific symptoms of this pathology: retraction of the contour, “lollipop-sign” and “beading-sign”.
Published: 27 June 2014
Current Treatment Options in Oncology, Volume 15, pp 476-481; https://doi.org/10.1007/s11864-014-0299-z

Abstract:
Sarcomas are rare malignant tumors that develop from mesenchymal tissue. Most sarcomas are idiopathic, however, a significant minority develops as a consequence of prior radiation exposure. Although the absolute risk of developing a radiation-associated sarcoma is small, these tumors represent significant clinical challenges. For recurrent, unresectable or metastatic disease, the standard treatment is systemic chemotherapy. Radiation-associated sarcomas tend to be undifferentiated sarcomas, angiosarcomas, or leiomyosarcomas, which are variably sensitive to chemotherapy. The best general approach is to treat each radiation-associated sarcoma as one would its sporadic histologic counterpart. There are limited data to guide the best treatment for radiation-associated sarcoma, therefore, the standard chemotherapy options are reasonable choices. These include doxorubicin, ifosfamide, gemcitabine, docetaxel, and pazopanib. Patients with radiation-associated sarcomas may have received prior anthracyclines to treat antecedent malignancies such as breast cancer or lymphoma. Thus, if additional doxorubicin cannot be used, liposomal doxorubicin is a reasonable substitute. More prospective research is needed on how radiation-associated sarcomas respond to systemic therapy. Future clinical trials of new agents in sarcoma should identify and include patients with radiation-associated sarcoma.
, E. Rustemi, C. Masetti, J. Kreshak, , D. Vanel, A. Toni, M. Mercuri
Published: 31 January 2011
European journal of radiology, Volume 77, pp 43-50; https://doi.org/10.1016/j.ejrad.2010.08.015

Abstract:
Periprosthetic osteolysis is a well known phenomenon caused by wear particle-induced bone resorption, particularly common and extensively reported in total hip arthroplasty. Its typical radiographic feature is a radiolucent area adjacent to an implant, sometimes associated with a soft tissue mass. Osteolytic changes may be caused by numerous other pathologic processes, including infection, metabolic disease, and neoplasia. Four cases of massive periprosthetic bone destruction associated with a large soft tissue mass around a failed total hip replacement are presented. In three cases, a diagnosis of periprosthetic osteolysis was correctly made and managed by revision surgery. However, in one case angiosarcoma of the ipsilateral hemipelvis went long unrecognized despite aggressive clinical course, requiring hind-quarter amputation and ultimately resulting in the patient's death. Periprosthetic malignancy in the form of either primary sarcoma or metastatic cancer is a very rare yet reported event in the setting of previous hip replacement, likely leading to catastrophic consequences when diagnosis is not established in a timely manner. The differential diagnosis of periprosthetic osteolysis should consider the entire spectrum of conditions that can present with radiolucent changes. Thorough review of patient's history and course of symptoms, along with careful evaluation of standard roentgenograms should be always performed and possibly integrated with imaging modalities such as CT, MRI, and bone scintigraphy in order to increase diagnostic accuracy. If uncertainty remains, biopsy should always be considered to rule out malignancy.
, P H Wünsch
Published: 8 October 2007
by BMJ
Journal of Clinical Pathology, Volume 61, pp 499-503; https://doi.org/10.1136/jcp.2007.052696

Abstract:
Background:The distribution and reactivity pattern of neural cell adhesion molecule (NCAM/CD56) in gastrointestinal stromal tumours (GISTs) and their mesenchymal mimics have not been investigated in the KIT era.Methods:275 histologically and immunohistochemically well characterised primary and metastatic intra-abdominal mesenchymal lesions were analysed by conventional immunohistochemistry, with emphasis on GIST and GI smooth muscle neoplasms.Results:CD56 expression was seen in 18/21 (86%), 4/5 (80%), 26/34 (76%), and 32/168 (19%) of primary GI leiomyomas, schwannomas, leiomyosarcomas, and GISTs, respectively. Reactivity in GISTs was mostly focal. Of 6% strongly staining GISTs, 71% were either malignant clinically or assigned a high risk prognostic group. CD56 expression in GISTs varied greatly with histological type (seen in 50% and 7% of epithelioid and spindled GISTs, respectively) and anatomical site (in 33%, 10%, 1%, and 0% of rectal, gastric, small intestinal and oesophageal GISTs, respectively). A variable, but inconsistent expression was seen in miscellaneous lesions including dedifferentiated liposarcoma, abdominopelvic PEComa, myo/fibroblastic sarcoma and malignant fibrous histiocytoma. Mesenteric fibromatoses, angiosarcoma/Kaposi sarcoma, reactive tumefactive fibrogenic lesions and 12/13 primary anorectal and oesophageal melanomas were negative.Conclusion:Results confirmed the ubiquity and non-specificity of CD56 as a neurogenic marker. Except for a subset of epithelioid gastric and high-grade rectal GISTs, CD56 expression is rare in GISTs, contrasting with true leiomyomatous and neurogenic neoplasms. CD56 plays a limited role in the differential diagnosis of GIST. Its potential role as a marker of adverse outcome in GISTs remains to be further investigated.
A. Comandone, A. Boglione, P. Pochettino, E. Berno, M. Inguì, M. Papotti, P. Borasio, G. Maggi, E. Brach del Prever, G. Gino
Journal of Clinical Oncology, Volume 27; https://doi.org/10.1200/jco.2009.27.15_suppl.e21509

Abstract:
E21509 Background: Primary sarcomas of the lungs and mediastinum are rare and few data are reported on treatment and results of therapy. Methods: We reviewed our experience from 1980 and 2008 including 31 patients (pts). Pts characteristics: median age 41 (19–80 y), male/female 19/12; symptoms at diagnosis: dyspnoea (42%), chest and shoulder pain (39%), cough (35%), hemophtoae (13%), discomfort (10%). 4 pts had a previous history of mediastinal radiation for Hodgkin's and non-Hodgkin's linfomas. 5 mediastinal tumours were located as follows: 2 in anterior part, 1 in posterior and 2 in the middle (sarcomas of the heart). 26 lung sarcomas presented as a singular mass in 23 cases and as a metastatic disease in 3. Results: In 20/31 cases the tumour was immediately resected (3 mediastinal masses and 17 lung sarcomas). In 8/31 cases only biopsy was possible. FNA was done in 25 pts. Neoadjuvant chemotherapy was performed in 4 cases (3 resected). Resection was complete in 11/23 cases and in 12/23 incomplete. The histology were: peripheral nerve tumour 7, leiomyosarcoma 4, MFH 2, fibrosarcoma 2, liposarcoma 1, angiosarcoma 2, undifferentiated sarcoma 1, solitary fibrous tumour 2, rhabdomyosarcoma 2, synovialsarcoma 2, pulmonary artery sarcoma 1, pleuropolmonary blastoma 1, malignant hemangiopericytoma 1, mixoid chondrosarcoma 1, ectopic osteosarcoma 1, aggressive fibromatosis 1. Only 4 pts received neoadjuvant chemotherapy, 11 adjuvant CT, 5 exclusive CT + RT for inoperable disease. Radiotherapy was completed in 26 pts (21 adjuvant). Local relapse or metastatic progression were recorded in 16/23 pts and 12 received one or more lines of palliative CT. Data about survival are disposable only for the more recently recorded pts (1998–2008: 17 pts). Of these only 8 are alive (2 with disease). Volume of disease, complete resection and grading are the dominant prognostic factors. Conclusions: Primary sarcomas of the lungs and mediastinum have a very severe prognosis. Surgical resection is the fundamental therapy, but in the future the role of neoadjuvant CT will increase. No significant financial relationships to disclose.
D. J. Indelicato, S. R. Keole, A. H. Shahlaee, C. G. Morris, C. P. Gibbs, M. T. Scarborough, S. Islam, R. B. Marcus
Journal of Clinical Oncology, Volume 27; https://doi.org/10.1200/jco.2009.27.15_suppl.e21501

Abstract:
E21501 Background: Primary sarcomas of the lungs and mediastinum are rare and few data are reported on treatment and results of therapy. Methods: We reviewed our experience from 1980 and 2008 including 31 patients (pts). Pts characteristics: median age 41 (19–80 y), male/female 19/12; symptoms at diagnosis: dyspnoea (42%), chest and shoulder pain (39%), cough (35%), hemophtoae (13%), discomfort (10%). 4 pts had a previous history of mediastinal radiation for Hodgkin's and non-Hodgkin's linfomas. 5 mediastinal tumours were located as follows: 2 in anterior part, 1 in posterior and 2 in the middle (sarcomas of the heart). 26 lung sarcomas presented as a singular mass in 23 cases and as a metastatic disease in 3. Results: In 20/31 cases the tumour was immediately resected (3 mediastinal masses and 17 lung sarcomas). In 8/31 cases only biopsy was possible. FNA was done in 25 pts. Neoadjuvant chemotherapy was performed in 4 cases (3 resected). Resection was complete in 11/23 cases and in 12/23 incomplete. The histology were: peripheral nerve tumour 7, leiomyosarcoma 4, MFH 2, fibrosarcoma 2, liposarcoma 1, angiosarcoma 2, undifferentiated sarcoma 1, solitary fibrous tumour 2, rhabdomyosarcoma 2, synovialsarcoma 2, pulmonary artery sarcoma 1, pleuropolmonary blastoma 1, malignant hemangiopericytoma 1, mixoid chondrosarcoma 1, ectopic osteosarcoma 1, aggressive fibromatosis 1. Only 4 pts received neoadjuvant chemotherapy, 11 adjuvant CT, 5 exclusive CT + RT for inoperable disease. Radiotherapy was completed in 26 pts (21 adjuvant). Local relapse or metastatic progression were recorded in 16/23 pts and 12 received one or more lines of palliative CT. Data about survival are disposable only for the more recently recorded pts (1998–2008: 17 pts). Of these only 8 are alive (2 with disease). Volume of disease, complete resection and grading are the dominant prognostic factors. Conclusions: Primary sarcomas of the lungs and mediastinum have a very severe prognosis. Surgical resection is the fundamental therapy, but in the future the role of neoadjuvant CT will increase. No significant financial relationships to disclose.
, A. Fourquet, Y. Kirova
Published: 28 December 2020
Cancer/Radiothérapie, Volume 25, pp 21-25; https://doi.org/10.1016/j.canrad.2020.05.019

The publisher has not yet granted permission to display this abstract.
Page of 2
Articles per Page
by
Show export options
  Select all
Back to Top Top