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(searched for: Case Report: Adult Retroperitoneal Rhabdomyosarcoma)
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Budi Martono, Sri Inggriani
Published: 1 July 2020
JBN (Jurnal Bedah Nasional), Volume 4, pp 62-68; https://doi.org/10.24843/jbn.2020.v04.i02.p04

Abstract:
Background: Rhabdomyosarcoma (RMS) is the most common type of soft tissue sarcoma in children, however, RMS is a rare malignancy in adults. Head and neck are the most common site for RMS, while intrabdominal RMS are rare in adults. Case: We present a rare case of a retroperitoneal abdominal mass, treated surgically with histopathology results of a retroperitoneal RMS. We discuss the clinical presentation, image findings, and treatment for this case. Conclusion: Intraabdominal tumours need to be identified quickly and precisely. CT scan or MRI can help clinicians to determine the staging, therefore plans the best treatment for the patient. In our case, surgery and radiotherapy showed promising outcome. The lack of literature and consensus on a standardized approach to systemic treatment and outcome in retroperitoneal pleomorphic RMS in adults makes our case a rare presentation of rhabdomyosarcoma and thus the need for reporting.
Published: 1 January 2000
Pathology - Research and Practice, Volume 196, pp 67-72; https://doi.org/10.1016/s0344-0338(00)80024-2

The publisher has not yet granted permission to display this abstract.
Chengli Miao,
Published: 4 August 2017
The publisher has not yet granted permission to display this abstract.
Till M. Theilen, Thambipillai Sri Paran, Daniel Rutigliano, Leonard Wexler, Yukio Sonoda, Michael P. Laquaglia
Published: 13 April 2011
Surgical endoscopy, Volume 25, pp 2748-2755; https://doi.org/10.1007/s00464-011-1583-7

Abstract:
Retroperitoneoscopy (RS) has been successfully introduced in adult oncology for diagnostic procedures, staging, and surgical treatment. Its value for children has rarely been reported. This report describes the authors’ experience using RS in the diagnosis and staging of cancer for children and adolescents. All RS procedures performed at the authors’ institution between 2004 and 2010 were reviewed. The authors’ operative technique entails a 10- to 12-mm flank incision followed by finger and balloon dissection of the retroperitoneal areolar tissue, with carbon dioxide (CO2) insufflation used to push the peritoneal lining medially. One to two additional working ports are placed above the iliac rim and below the costal margin. In cases of peritoneal tear with leakage of CO2 and progressive retroperitoneal impingement, a Veress needle is placed in the umbilicus for pressure release. This review included 16 patients with a median age of 16.4 years (range, 4.4–29.8 years) who underwent RS for lymph node sampling (9 cases), diagnostic biopsy (6 cases), or resection of a metastatic nodule (1 case). Four complications were encountered (3 conversions to open surgery and 1 self-limited gross hematuria). The mean operative time was 123.3 ± 33.5 min. The patients required 1.1 ± 0.8 days of intravenous analgesia on the average. The mean hospital stay was 1.7 ± 0.6 days. The authors believe that RS is a safe surgical technique for access to the retroperitoneum in pediatric patients. In cases of a peritoneal tear, placement of a Veress needle in the umbilicus effectively prevents conversion to open surgery. Retroperitoneoscopy should be considered for children who need biopsies, lymph node dissections, or resections of primary tumors in the retroperitoneum.
Published: 26 September 2022
Journal: Cureus
Abstract:
Spindle cell rhabdomyosarcoma (SC-RMS) is an unprecedented version of embryonal rhabdomyosarcomas (RMSs) that emerges from the mesenchyme with the capacity to differentiate into skeletal muscle cells. Retroperitoneal RMS is extremely rare in the adult population. We present the case of a primary spindle cell retroperitoneal RMS with compression features. Investigation-based diagnosis of RMS is difficult due to the lack of specificity of clinical findings. Radiology does not help in making an accurate diagnosis. Surgical removal of the tumor followed by chemotherapy and radiotherapy is the best possible treatment for RMS in adults. SC-RMS has a poor long-term prognosis. To our knowledge, such cases of retroperitoneal SC-RMS compressing the abdominal viscera and resulting in hydroureteronephrosis have never been reported before.
Published: 28 May 2018
Journal of Medical Case Reports, Volume 12; https://doi.org/10.1186/s13256-018-1607-1

Abstract:
Intrascrotal embryonal rhabdomyosarcoma in adults is a rare tumor with high aggression and a poor prognosis. We report our patient's case and review the relevant literature to improve the understanding of this rare disease. A 21-year-old Han Chinese man presented to our hospital with a right intrascrotal mass of 1 year's duration. His physical examination revealed an enlarged right scrotum containing a huge tender mass measuring about 10 × 7 cm. Ordinary and contrast-enhanced ultrasonography showed a solid mass in the right scrotum, which was suspected to be a malignant tumor. An abdominopelvic computed tomographic scan revealed metastases in the retroperitoneal lymph nodes. The patient was diagnosed with malignant testicular tumor and underwent a right radical orchiectomy by an inguinal approach. Postoperative pathological examination suggested an intrascrotal embryonal rhabdomyosarcoma. Intrascrotal embryonal rhabdomyosarcoma is a rare but highly aggressive tumor. Clinical and imaging manifestations of this tumor are nonspecific, so the definitive diagnosis depends on postoperative pathology and immunohistochemistry. Early suspicion, radical orchiectomy, accurate pathologic diagnosis, and adjuvant chemotherapy and/or radiotherapy are the keys to optimal prognosis.
K Tazi, , A Koutani, A Ibn Attya, M Hachimi, A Lakrissa
Published: 1 June 2000
Progrès en Urologie, Volume 10

Abstract:
The authors report a case of paratesticular thabdomyosarcoma in a young adult. This tumour is more frequent in children than in adults. Rhabdomyosarcoma is the commonest variety of soft tissue sarcoma in children and young adults. It represents 6.5% of all malignant tumours in paediatrics with an annual incidence of 4 to 7 cases/million children. The primary paratesticular site is considered to have a good prognosis in comparison with other rhabdomyosarcomas, despite the frequency of retroperitoneal lymph node invasion. This superficial site allows rapid diagnosis and consequently often complete resection of the tumour. The multidisciplinary treatment of paratesticular rhabdomyosarcoma has improved control of the disease with a 2-year survival of 80%.
JOURNAL of CLINICAL AND DIAGNOSTIC RESEARCH, Volume 9; https://doi.org/10.7860/jcdr/2015/9805.5747

Abstract:
Para testicular rhabdomyosarcoma is a rare malignant tumour, which usually presents as a painless mass in the scrotum or groin. A case of para testicular rhabdomyosarcoma in a 17-year-old male is being reported here who presented with chronic scrotal pain. Paratesticular rhabdomyosarcoma is a rare non germ cell tumour of scrotal sac in children and young adult/teens which can invade testis at presentation. Embryonal variant is the most common type. 40% cases can have metastasis to retroperitoneal lymph node. Diagnosis can be done on high degree of clinical suspicion coupled with biopsy and immunohistochemistry. Multimodality approach of treatment is often beneficial for patients.
Raouah Mehdi, Bouchabaka Yassine, El Matlini Abdelali, Saadoune Mohamed, Laatitioui Sana, Igarramen Tariq, Bounid Oumayma, Darfaoui Mouna, Lalya Issam, El Omrani Abdelahamid, et al.
Cross Current International Journal of Medical and Biosciences, Volume 2, pp 121-124; https://doi.org/10.36344/ccijmb.2020.v02i08.003

Abstract:
Para testicular rhabdomyosarcoma is a rare malignant tumour, which usually presents as a painless mass in the scrotum or groin. A case of paratesticular embryonal rhabdomyosarcoma in a 16-year-old male, with a history of left testicular ectopia treated with orchiopexy, is being reported here who presented with chronic scrotal swelling. Paratesticular rhabdomyosarcoma is a rare non germ cell tumour of scrotal sac in children and young adult/teens which can invade testis at presentation. Embryonal variant is the most common type. 40% cases can have metastasis to retroperitoneal lymph node. Diagnosis can be done on high degree of clinical suspicion coupled with biopsy and immunohistochemistry. Multimodality approach of treatment is often beneficial for patients.
Thiago Lara Rocha, José Spila Neto, Paulo Adaias Piza, Alyna Araújo e Marcondes, Matheus Henrique Sena Ursi, Juliana Helena Chávez-Pavoni, Luciana Marques da Silva, Claudinéia de Araújo
Published: 16 August 2022
MOJ Public Health, Volume 11, pp 130-131; https://doi.org/10.15406/mojph.2022.11.00388

Abstract:
Malignant rhabdoid tumour is a very rare neoplasm with a high lethality rate. Its neoplastic cells present rhabdomyoblastic features, although those cells must be differentiated from epithelioid sarcoma, melanoma, and rhabdomyosarcoma. Therefore, this is a case report of a 21-year-old man who presented a Malignantrhabdoid tumour in the head of the pancreas. Patient was submitted to partial pancreatomy and gastrectomy in block, splenectomy, and retroperitoneal lymphadenectomy. Anatomopathological analysis revealed invasive malignant neoplasm of the pancreas, which was classified as malignant epithelioid neoplasm according to the Department of Pathology, Emory University (Atlanta, United States of América). Systemic chemotherapy protocol consisted in ifosfamide, mesna and doxorrubicin, although after the first cycle of chemotherapy, patient died due to disease complications. According to literature only seven pancreatic neoplasms were described with rhabdoid features. Extra renal Malignant rhabdoid tumours diagnosis is challenging because of immunohistochemistry characteristics. Thus, this case report may contribute to medical literature, as recording and describing anatomo pathological and immunochemistry features of one more case of this rare neoplasm, which in this case was described in an uncommon age range and organ.
, Sobia Faisal, Iqbal A. M. Khyani, Bushra Sikandar
Published: 1 January 2016
World Journal of Oncology, Volume 7, pp 85-90; https://doi.org/10.14740/wjon978w

Abstract:
Rhabdomyosarcoma (RMS) is a soft tissue neoplasm of mesenchymal origin. It is a commonly encountered malignant tumor amongst pediatric patients, yet relatively rare in adults. It usually involves the head and neck region, genitourinary organs and retroperitoneal structures. In adults, the most commonly affected area is the head and neck region. We present here a case of a 30-year-old male patient with a primary squamous cell carcinoma of the tongue (T1, N0, M1), successfully cured with surgery and chemoradiotherapy and later on development of metachronous ipsilateral lesion on the left lower alveolus. Biopsy was consistent with spindle cell RMS. Immunohistochemistry demonstrated positivity for desmin, vimentin and myogenin, thus confirming the mesenchymal origin. With the best of our literature search, this is an exceptional case presenting two malignant lesions with diverse genetic origins, diagnosed at stage 1 and giving a favorable outcome.
A. Demir, , L. Türkeri
Published: 1 January 2004
International Urology and Nephrology, Volume 36, pp 577-578; https://doi.org/10.1007/s11255-004-0853-x

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Pedro Aguiar Jr, Barbara De Souza Gutierres, Daiane Pereira Guimarães
Published: 1 January 2014
Journal of Medical Cases, Volume 5, pp 538-540; https://doi.org/10.14740/jmc1933w

Abstract:
Soft tissue sarcomas are rare neoplasms, which account for less than 1% of adult malignancies. In addition, they account for only 2% of renal cancers. The main mesenchymal lesions of the kidney are benign and difficult to differentiate with well-differentiated sarcomas. The aim of this report was to present the experience of the Hospital Sao Paulo (HU/UNIFESP) in a case of well-differentiated liposarcoma, which affected the kidney of a young adult who has been treated with radiotherapy in childhood, as well as to raise the relevant literature. A 26-year-old male patient with a history of prostate rhabdomyosarcoma that has been submitted to surgical resection and adjuvant radiotherapy 23 years ago in the childhood was presented. He was asymptomatic during 22 years, then he developed back pain, and underwent investigation and one tumor was detected in the right kidney. Radical nephrectomy was performed with complete resection of the lesion and he is currently without evidence of disease recurrence. Renal liposarcoma usually has good prognosis and the treatment is based almost exclusively on tumor resection. The differentiation between primary renal or retroperitoneal site is important for the prognostic assessment of the disease. There is a relationship between exposure to radiation in childhood and the development of second malignancy in adulthood, especially soft tissue sarcomas. Metastases are rare in well-differentiated forms of the disease. Recurrences can be observed in 30% of all cases and have been described after 13 years of initial diagnosis, which justifies the extended follow-up of these patients.J Med Cases. 2014;5(10):538-540doi: http://dx.doi.org/10.14740/jmc1933w
J N Lin, K L Wang,
Published: 1 July 1988
International surgery, Volume 73

Abstract:
Twenty-six primary testicular tumors in children (less than 15 years old) seen within a period of nine years (1978-1986) were analyzed. Of these, 16 (61.5%) were benign mature or immature teratomas and ten (38.5%) malignant. This is obviously different from most of the western series in which a 75-80% rate of malignancy has usually been reported. Sixteen benign testicular tumors, which were either mature or immature teratomas, showed no evidence of tumor one to nine years after high inguinal orchiectomy. Serum alpha-fetoprotein (AFP) levels were available in 14 of these patients and all of them were below 50 ng/ml. In the ten malignant tumors, eight were yolk sac tumors and two embryonal rhabdomyosarcomas. High inguinal orchiectomy only was performed for stage I disease with sequential monitoring of AFP level for two years. For stage II or III tumors, chemotherapy and/or irradiation were added in the treatment regimen. Retroperitoneal node dissections were not performed in this series. Eight out of these ten cases were alive without evidence of disease after one to seven years, one expired for an unknown cause and one was lost to follow-up. We advocate this conservative approach to childhood testicular tumors which have a better outcome than adult tumors.
Tamara L. Densmore, Jacob C. Langer, Jean P. Molleston, Louis P. Dehner,
Published: 7 December 1996
Medical and pediatric oncology, Volume 27, pp 556-560; https://doi.org/10.1002/(sici)1096-911x(199612)27:6<556::aid-mpo9>3.3.co;2-z

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, Vani H.N., Nabanita Kora
Published: 2 December 2021
Dubai Diabetes and Endocrinology Journal, Volume 27, pp 162-164; https://doi.org/10.1159/000520177

Abstract:
Tumour-induced hypoglycaemia is a rare complication/condition mainly seen in adults. It is caused due to increased production of insulin or insulin-like growth factor (IGF) 2 tumour cells. We present a 3-year-old paediatric patient with non-islet cell tumour induced hypoglycaemia (NICTH) secondary to rhabdomyosarcoma. She presented with abdominal mass and refractory hypoglycaemia, requiring high glucose infusion and steroids. Critical sample analysis during hypoglycaemia showed suppression of insulin, IGF-1, C-peptide, growth hormone, and ketones, with a high cortisol level. CT scan of abdomen and pelvis showed a huge retroperitoneal mass, later diagnosed as rhabdomyosarcoma. In a resource-limited setting, where IGF-2 is not possible, low serum insulin and IGF-1 levels during hypoglycaemia aids in diagnosis of NICTH. This is one of the first few reported paediatric cases with NICTH from India, and we believe that reporting this case would add more information to the existing literature. Thus, NICTH should be suspected in all malignancies presenting with intractable hypoglycaemia irrespective of their age.
R Mazanet, K H Antman
Published: 1 December 1991
Seminars in oncology, Volume 18

Abstract:
Adjuvant therapy is currently established in the treatment of osteosarcoma, Ewing's sarcoma and rhabdomyosarcoma. Of the 12 reported randomized studies of adjuvant chemotherapy for soft tissue sarcoma, only 2 show a significant overall survival advantage for chemotherapy (the most important endpoint). In three randomized trials, the survival of the observation arm exceeds that of the chemotherapy arm. In two additional studies, subset analyses currently indicate a significant DFS advantage for adjuvant chemotherapy in extremity lesions, but no significant improvement in survival. Although initial NCI reports showed significantly prolonged survival for the subset of chemotherapy-treated extremity primaries, survival on longer follow-up is no longer significantly different. In the subset analysis of retroperitoneal sarcomas in the same NCI study, the survival of the control group is superior to the treatment group. Doxorubicin associated cardiotoxicity has occurred in about 10% of treated patients, occasionally contributing to treatment-related deaths. Based on these data, adjuvant chemotherapy should be considered investigational for adult soft-tissue sarcomas of any primary site. Future randomized trials should include patients at high risk for metastases (large, high-grade lesions) with a reasonable likelihood of local control by radical resection, or resection with uninvolved margins and subsequent radiotherapy. Low-grade sarcomas are currently cured by surgical resection in 80% of cases, and thus should not be included in adjuvant trials.
, J. El Fenni, S. Chaouir, T. Amil, A. Hanine, M. Ben Ameur
Published: 30 June 2004
Journal de radiologie, Volume 85, pp 779-781; https://doi.org/10.1016/s0221-0363(04)97683-4

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