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Amr S. Bugshan, Mohammed A. Alsaati, Faiyaz A. Syed, Khalid S. Almulhim, Adel I. Abdulhady
American Journal of Case Reports, Volume 21; doi:10.12659/ajcr.928307

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Amr S. Bugshan, Mohammed A. Alsaati, Faiyaz A. Syed, , Adel I. Abdulhady
Published: 24 November 2020
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John Emmanuel Custodio, Kevin Paul Ferraris, Joseph Erroll Navarro, Kenny Seng, Jose Carlos Alcazaren, Ma Angelle Dantes
Published: 9 November 2020
Neuro-Oncology, Volume 22; doi:10.1093/neuonc/noaa215.880

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Bei-Bei Wang, Jun-Ru Ye, Yun-Lei Li, Yi Jin, Zhong-Wei Chen, Jian-Min Li,
World Journal of Clinical Cases, Volume 8, pp 4966-4974; doi:10.12998/wjcc.v8.i20.4966

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, Nita Sahara, Ery Kus Dwianingsih,
Published: 23 September 2020
Dermatology Reports, Volume 12; doi:10.4081/dr.2020.8777

Abstract:
Langerhans Cell Histiocytosis (LCH) is a rare disease characterized by the clonal proliferation of Langerhans cells, which are immunoreactive to S-100 and CD-1a/ CD207 (Langerin). Cutaneous involvement is the most common presentation of LCH in children. It is suggested that the patients with single-system LCH limited to the skin have a better prognosis than those with systemic involvement. Three histologic reactions of cutaneous LCH have been reported and are associated with the clinical types of LCH. These histological reactions include: proliferative, granulomatous, and xanthomatous. This study presents the clinicopathological features of ten cutaneous LCH cases collected from Dr. Sardjito General Hospital Yogyakarta Indonesia between 2014-2018. The ten cases showed various clinical features, in which some features mimic other diseases. The microscopic features of skin biopsies showed granulomatous reaction in 80% of cases and proliferative reaction in the other 20%. Five patients (50% of cases) who died had systemic manifestation of thrombocytopenia, anemia, icterus, hepatosplenomegaly, and revealed the granulomatous type from their skin biopsy specimens. The clinical recognition of LCH and subsequent histological reaction determination are important since some cases may develop multisystem disease and have a poor prognosis.
, Michael Bonert, Asghar Naqvi, Chunjie Wang, Kiril Trpkov, Peer Dettmar, Hanns-Olof Wintzer, Robert Stoehr, Ondřej Hes, Sean R. Williamson, et al.
American Journal of Surgical Pathology; doi:10.1097/pas.0000000000001562

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Ahmed H. K. Abdelaal, Mohamed Sedky, Seham Gohar, Iman Zaki, Asmaa Salama, Omayma Hassanain,
Published: 16 July 2020
SICOT-J, Volume 6; doi:10.1051/sicotj/2020024

Abstract:
Introduction: Skeletal involvement in children with Langerhans cell histiocytosis (LCH) is a common feature of the disease. Several options for the treatment of these skeletal lesions have been reported. We describe our experience in the treatment of skeletal involvement of LCH in this retrospective case series study, entailing anatomic distribution, pattern of healing, skeletal deformities, and functional outcome of skeletal LCH. Methods: A retrospective analysis was conducted for patients diagnosed with LCH and having skeletal lesions in the period between 2007 and 2015. Out of a total of 229 cases, 191 (83.4%) had skeletal involvement. Bone healing was divided into partial and complete based on the size of lesion and cortical changes in plain radiograph. Skeletal deformities were serially measured. Time to pain control, resumption of weight bearing, and the final functional status of the patient were reviewed. Results: The mean age at presentation was 4.4 years (3 m–14.8 y) and the mean follow-up period was 53.3 months (0.2–120.7). After screening of skeletal and extra-skeletal lesions, 59 patients (31%) had M-S (Multisystem) LCH and 132 (69%) had S-S (Single system) LCH. Unifocal bone lesions were found in 81 (42.5%) patients, and multifocal bone lesions in 110 patients (57.5%). Single or multiple bone lesions were found in the craniofacial bones in 152 patients (79.5%), femur in 19 patients, (10%), ribs in 18 patients (9.4%), spine in 15 patients (8.1%), pelvis in 14 patients (7.3%), scapula in 8 patients (4.1%), humerus in 6 (3.1%), clavicle in 6 patients (3.1%), tibia in 3 patients (1.5%), radius in 3 patients (1.5%), and the ulna in 2 patients (1%) patients. No lesions were found in the fibula, hand, or foot. Out of all skeletal lesions, 179 (93.7%) patients were treated either medically or conservatively and 12 patients (6.2%) were treated surgically. The mean time to complete healing was 5.2 months (2–12). Skeletal complications included: pathologic fractures (9 vertebra plana, 5 long bone, 1 iliac bone), deformities (9 thoracolumbar kyphosis, 2 cervical spine subluxations, 2 coxa vara deformity of the proximal femur and one flattening of iliac bone). Conclusion: Non-operative treatment can lead to adequate bone healing in few months period. Partial or complete remodeling of bone deformities can be observed without surgical correction. However, surgical intervention might be indicated when cervical spine affection may lead to instability and subsequent neurological affection. Functional impairment is rarely caused by skeletal lesions in LCH.
Jake E Ryan, Vijayakumar Ganesh, Afroditi Karathanasi, Michele Moschetta, Matin Sheriff, John B Schofield, Stergios Boussios
Journal of B.U.ON. : official journal of the Balkan Union of Oncology, Volume 25, pp 612-617

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Alice Brambilla, Francesco Pegoraro, Maria Luisa Coniglio, Roberto Caputo, Anna Perrone, Anna Maria Buccoliero, Claudio Favre, Elena Sieni
Published: 20 May 2020
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Published: 16 April 2020
Blood, Volume 135, pp 1319-1331; doi:10.1182/blood.2019000934

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Ivy Hoi Yee Ng, Elaine Yun Ning Cheung, Grace Yee Wai Kam
Journal of the Endocrine Society, Volume 4; doi:10.1210/jendso/bvaa046.644

Abstract:
Background Langerhans cell histiocytosis (LCH) is a myeloid neoplastic disorder that is rare in adults, with a reported incidence of 1-2 cases per million per year. Cranial diabetes insipidus (CDI) is the most common and often the first endocrine manifestation of LCH, when histiocytes infiltrate the pituitary. Anterior pituitary dysfunction, when present, usually develops after onset of CDI. Unlike most other etiologies of CDI, multisystem involvement is frequent in LCH, especially the bones, skin, and lungs. Case A 23-year-old man of good past health presented with 3 months of polyuria and polydipsia in 2015. CDI was confirmed by water deprivation test, and his symptoms resolved with DDAVP treatment. There were no anterior pituitary hormone deficiencies on presentation. Pituitary MRI showed loss of the posterior bright spot and a contrast enhancing pituitary stalk thickening (PST) of 5 mm, with no compression on the optic chiasm. Lumbar puncture showed no evidence of CNS infection or neoplastic cells. Beta HCG, AFP, IgG4, ANA, and chest Xray were unremarkable. Three months later he developed central hypogonadism, and was treated with testosterone after sperm banking. A repeat MRI showed persistent PST of 5 mm, and biopsy of the pituitary suggested lymphocytic infundibulo-hypophysitis (LIH). Unfortunately the biopsy was complicated with irreversible focal visual field defect. It was then noted that a bone scan had been arranged earlier for intermittent left hip pain, which revealed a 3.5 cm osteolytic lesion over the left ilium. The concomitant pituitary and bone lesions thus make LCH the more likely diagnosis. However bone biopsy showed nonspecific fibrosis only. The pathologist was then contacted for further immunohistochemical analysis. This identifies clusters of Langerhans cells with positive stain for S100, CD1a, and Langerin (LCH cell markers) in the pituitary tissue, and BRAF V600E mutation was detected by PCR, compatible with the diagnosis of LCH. Systemic chemotherapy was proposed due to multisystemic disease, but was declined by patient. At 4 years of follow-up, the PST spontaneously regressed to 2 mm but the CDI and central hypogonadism persisted. Conclusion Our case illustrates the diagnostic challenge in a patient presenting with CDI, given a pituitary biopsy with histological features mimicking LIH and an inconclusive bone biopsy. However, a diagnosis of LIH would not explain for the unusual bone lesion in a young man, and bone biopsies can be prone to crush artifacts limiting analysis. The importance of actively looking for and biopsying extracranial lesions before biopsying the pituitary cannot be understated as the latter entails risks of hypopituitarism and visual defect, as had happened to our patient. When clinical and pathologic findings do not match, communication with the pathologist for histological review had been essential in establishing the diagnosis of LCH.
Franck-Neil El Sissy, Gwenael Lorillon, Emmanuel Mandonnet, Marc Polivka, Homa Addle-Biassette,
Clinical Neuropathology, Volume 39, pp 64-69; doi:10.5414/np301225

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Guiqian Liu, Ting Liu, Chong Shen, Li Zhou, Ruoyun Ouyang
Published: 28 January 2020
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Shreya Sehgal, Muniraju Maralakunte, Lokesh Singh, Uma Debi
Archives of Clinical and Medical Case Reports, Volume 5, pp 43-47; doi:10.26502/acmcr.96550323

Abstract:
Langerhans cell histiocytosis (LCH) constitutes a rare group of disorder derived from the macrophages and dendritic cells. The cells in LCH show close similarity to the langerhans cells found in the skin and mucosa and stain positive for CD1a, S100, and CD207 antigens. These cells display the same intracytoplasmic organelles on electron microscopy as seen in Langerhans cells i.e. Birbeck granules. LCH is ten times more prevalent in the paediatric age group as compared to adult population. Females are affected more commonly as compared to the males. LCH can have single- or multi-system types of involvement. Unifocal or multifocal pattern is seen in singlesystem type of disease. The multisystem pattern is often seen in the paediatric population while unisystem pattern is seen in adults. We present case report of a 35 yrs old male patient with multisystemic involvement.
Renuka Verma, Sunita Singh, Rajnish Kalra, Vinay Kumar Malik
Published: 1 January 2020
Clinical Cancer Investigation Journal, Volume 9; doi:10.4103/ccij.ccij_72_19

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Pilar Gandara-Vila, Catalina Barba-Montero, Jose Manuel Suarez-Peñaranda, Jose Manuel Somoza-Martín, Abel Garcia-Garcia, Andres Blanco-Carrion, Mario Perez-Sayans
Oral Surgery, Oral Medicine, Oral Pathology and Oral Radiology, Volume 129; doi:10.1016/j.oooo.2019.06.401

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Ko Kudo, Rika Kanezaki, Akie Kobayashi, Tomohiko Sato, Takuya Kamio, Shinya Sasaki, Imamura Masaru, Chihaya Imai, Masahiro Irie, Yoji Sasahara, et al.
Published: 13 November 2019
Blood, Volume 134, pp 3588-3588; doi:10.1182/blood-2019-127536

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, Githa Rahmayunita, Triana Agustin, Sondang P. Sirait, Riesye Arisanty
Published: 4 November 2019
by Wiley
Pediatric Dermatology, Volume 37, pp 180-183; doi:10.1111/pde.14054

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Yingyi He, Jianling Xie, Hui Zhang, Jiayi Wang, Xiaoling Su, Dianyue Liu
Journal of Pediatric Hematology/Oncology, Volume 42; doi:10.1097/mph.0000000000001625

Abstract:
Langerhans cell histiocytosis (LCH) is a rare disease with localized to disseminated clinical features. Thyroid involvement in LCH is rare and presenting as either a single-organ or multisystem disease, it is usually misinterpreted as another thyroid disorder. Therefore, the LCH diagnosis is often delayed. We report a pediatric case of LCH with thyroid involvement as the initial clinical manifestation progressing to respiratory failure. Clinicians should note insidious extrathyroidal laboratory abnormalities and consider infiltrative thyroid diseases, such as LCH. Systematic clinical and laboratory investigations are needed to prevent delayed diagnosis because the classic features of LCH may become evident only over time.
Minal Wade
GLOBAL JOURNAL FOR RESEARCH ANALYSIS pp 1-2; doi:10.36106/gjra/6900647

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Lindsey M. Voller, Kristin E. Totoraitis, Kevin J. Gaddis,
Published: 1 October 2019
JAAD Case Reports, Volume 5, pp 835-837; doi:10.1016/j.jdcr.2019.07.011

Abstract:
Langerhans cell histiocytosis (LCH) is a rare disorder arising from the abnormal proliferation and subsequent deposition of CD1a+ myeloid dendritic cells in various organs. Clinical features are heterogeneous and primarily depend on specific organ systems involved, ranging from skin rash and osteolytic skull lesions to treatment-refractory multiorgan failure in severe cases. Although LCH predominantly affects the pediatric population, an increasing number of adult-onset cases are being reported.1x1Aricò, M., Girschikofsky, M., Généreau, T. et al. Langerhans cell histiocytosis in adults: report from the International Registry of the Histiocyte Society. Eur J Cancer. 2003; 39: 2341–2348Google ScholarSee all References,2x2Crickx, E., Bouaziz, J., Lorillon, G. et al. Clinical spectrum, quality of life, BRAF mutation status and treatment of skin involvement in adult Langerhans cell histiocytosis. Acta Derm Venereol. 2017; 97: 838–842Google ScholarSee all References We describe the case of a 51-year-old man with disseminated (multisystem) LCH stemming from an in-frame, oncogenic BRAF mutation.
Yaozhu Pan, Rui Xi, Cunbang Wang, Lei Fang, Jiaofeng Bai, Yonggang Cai, Min Guo, Ruiyun Qiao, Xu Lan, Jiaojiao Yin, et al.
Journal of International Medical Research, Volume 47, pp 4522-4529; doi:10.1177/0300060519864807

Abstract:
Langerhans cell histiocytosis (LCH) is a disorder caused by clonal proliferation of CD1a+/CD207+ cells and characterized by varying degrees of organ involvement. Treatment of LCH is risk adapted; patients with multisystem disease and risk-organ involvement require more intensive therapy. Optimal therapies for multisystem, high-risk LCH remain uncertain. Recently, targeted therapy using inhibitors of mutated BRAF (the gene encoding serine/threonine-protein kinase B-Raf) has proven very effective in patients with multisystem refractory LCH. Herein, we report a case of LCH with involvement of the bones, liver, and lymph nodes. Using next-generation sequencing of the patient’s pathological sample, we identified a mutation in MAP2K1 in exon 3 (c.362G>C, p.Cys121Ser) and no mutation in BRAF; thus, high-risk, multisystem LCH with MAP2K1 mutation and wild-type BRAF was diagnosed. After four chemotherapy treatments (COEP regimen), the patient received autologous hematopoietic stem cell transplantation (auto-HSCT). Complete remission was confirmed by follow-up positron emission tomography–computed tomography, which showed no lesions in liver, lymph nodes, or bones compared with the pretreatment period. To date, the patient has sustained good health for 24 months. In conclusion, auto-HSCT may be an effective treatment option for high-risk, multisystem BRAF V600E-negative LCH.
Jason Signorelli, Sowmya Ravulapati, Jay L Patel, Michael W. Deininger, Srinivas K. Tantravahi
Published: 29 November 2018
Blood, Volume 132, pp 4315-4315; doi:10.1182/blood-2018-99-120290

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Thomas Burke, Alejandra Legarreta, Howard Lin, Brooks Scull, Olive S. Eckstein, Kenneth L. McClain, Carl E. Allen
Published: 29 November 2018
Blood, Volume 132, pp 4941-4941; doi:10.1182/blood-2018-99-114295

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Le Hong, Gen Sun, Long Peng, Yi Tu, Yong Li, Weidong Xiao
Published: 1 September 2018
Medicine, Volume 97; doi:10.1097/md.0000000000012378

Abstract:
Langerhans cell histiocytosis (LCH) is a relatively rare disorder characterized by the proliferation of abnormal Langerhans cells. Splenic involvement usually presents in children with multisystem LCH, and isolated LCH of the spleen is a very rare disease. Herein, we report a case of isolated splenic LCH in an adult man. We report a case of isolated splenic LCH that first manifested with recurrent left upper abdominal pain. Unenhanced CT revealed multiple nodular lesions located at the spleen. Magnetic resonance imaging speculated that these lesions were lymphomas. On the basis of histopathological and immunohistochemical findings, the diagnosis of isolated splenic LCH was confirmed. The patient underwent splenectomy. Histopathologic examination revealed the proliferation of Langerhans cell. Immunohistochemical staining revealed that cells of the tumor were positive for S-100, CD1a, CD45RO, and Vimentin. The patient is alive without recurrence 9 years after operation. Isolated LCH of the spleen may have a favorable prognosis and splenectomy is an effective therapeutic method.
Cp Eyth, S Synoracki, R Noppeney, S Lang, N Dominas
Forschung heute – Zukunft morgen, Volume 97; doi:10.1055/s-0038-1640013

Abstract:
Introduction: Langerhans cell histiocytosis (LCH, also known as histiocytosis X) is a rare disease involving clonal proliferation of Langerhans cells usually presenting in childhood and adolescence. Its manifestations ranges from isolated bone and skin lesions to multisystem disease. The clinical course can appear self-limiting as well as in a fulminant and lethal form. Unifocal LCH of the periorbital bone and soft tissue is rare. Treatment is guided by extent of the disease and ranges from surgical excision to chemotherapy. Case report: A 31-year-old Caucasian man presented to us with a short-term history of indolent swelling of his right medial canthus. Magnetic resoncance imaging showed a subcutaneous lesion with contrast agent uptake and local bone thickness reduction at the right base of the nose. Excision biopsy and histomorphological evaluation showed evidence for LCH. Subsequent staging could objectify unifocal manifestation and continuous follow-up was initiated. Discussion: Unifocal LCH of the periorbital bone and soft tissue is rare and normally appears in childhood and adolescence. The actual literature shows usually an affection of the upper- or lower eye lid or the lateral canthus. Affection of the medial canthus in the presented case is unusual, particularly in a young adult. Conclusion: Suspicious orbital space-consuming lesions should be further evaluated by biopsy. Here – also in young adults – LCH should be taken into consideration as a possible differential diagnosis.
, Nuria Arco Huguet, Veronica Sanmartin Novell, Felip Vilardell Villellas, Ana Velasco Sánchez, Elisa Martró Català, Rosa Maria Martí Laborda, Xavier Soria Gili
Published: 12 March 2018
by Wiley
Pediatric Blood & Cancer, Volume 65; doi:10.1002/pbc.27047

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Isotta M. Magaton, , Fatime Krasniqi, Christof Rottenburger, Rosanna Zanetti-Daellenbach, Peter Grendelmeier, Viola Heinzelmann-Schwarz, , Fabienne D. Schwab
Published: 5 October 2017
Case Reports in Oncology, Volume 10, pp 876-884; doi:10.1159/000480696

Abstract:
Background: The clinical presentation of Langerhans cell histiocytosis (LCH) is heterogeneous ranging from single-organ involvement to systemic disease causing substantial morbidity and mortality. We describe an unusual course of severe multisystem LCH with spontaneous remission. Case Presentation: We report on a 45-year-old Caucasian woman with cervical cancer, FIGO stage IVB. Five months after the end of combined radiochemotherapy and brachytherapy, the patient was readmitted because of severe dysuria. Sterile leukocyturia was seen, and cystoscopy revealed only 3 unspecific small mucosal lesions compatible with postradiation cystitis. Incidentally, a computed tomography (CT) scan of the body showed diffuse micronodular and cystic lesions in lungs and hypodense lesions in the liver. Biopsies revealed infiltrations of CD1a and Langerin (CD207)-positive histiocytes in the lung, liver, and bladder. Additionally, positron emission tomography-CT (PET-CT) was compatible with bone involvement. Retrospective analysis revealed that the increase in alkaline phosphatase might have been a surrogate of bone marrow infiltration with osseous activity. Repeated pneumothoraces occurred, and only one course of vinblastine-prednisolone could be applied. Despite ongoing tobacco consumption and without further therapy, PET-CT showed considerable remission 2 months later. However, despite stable remission, documented by serial PET and conventional CT scans, persistent infiltration of the bladder by Langerhans histiocytes could still be demonstrated 17 months later. Unfortunately, cervical cancer recurred and progressed. Conclusion: Multisystem LCH may rapidly occur, may be oligosymptomatic and, even in high-risk cases, remission without specific therapy might occur. Whether alkaline phosphatase might be a surrogate to monitor osseous disease activity has to be further explored.
Supriya Gujjar Suresh, Saraswathy Jagadeesan, Arathi Srinivasan, Julius Xavier Scott
International Journal of Contemporary Pediatrics, Volume 4; doi:10.18203/2349-3291.ijcp20172702

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Hideyuki Nakashima, , Shuhei Minamiyama, Kei Watanuki, ,
Japanese Journal of Oral and Maxillofacial Surgery, Volume 63, pp 570-575; doi:10.5794/jjoms.63.570

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Miho Ogawa, Masayuki Kobayashi, Koji Jimbo, Toyotaka Kawamata, Kazuaki Yokoyama, Nobuhiro Ohno, Yoichi Imai, Satoshi Takahashi, Arinobu Tojo
Published: 2 December 2016
Blood, Volume 128, pp 4883-4883; doi:10.1182/blood.v128.22.4883.4883

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Jie Shen, Shicheng Feng
Molecular and Clinical Oncology, Volume 6, pp 67-70; doi:10.3892/mco.2016.1077

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Nadira Musabbir, Asm Bazlul Karim, Kaniz Sultana, Syeda Afria Anwar
Northern International Medical College Journal, Volume 7, pp 158-160; doi:10.3329/nimcj.v7i2.29663

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Deepak Chellapandian, Polyzois Makras, Gregory Kaltsas, Cor Van Den Bos, Anne-Sophie Carret, Sheila Weitzman, Maarten Egeler, Oussama Abla
Published: 3 December 2015
Blood, Volume 126, pp 2209-2209; doi:10.1182/blood.v126.23.2209.2209

Abstract:
Introduction: Langerhans cell histiocytosis (LCH) is a monoclonal disorder characterized by proliferation and accumulation of atypical Langerhans cells and in up to 55% of all cases somatic mutations in BRAF proves to be the driver. Although uncommon, it is potentially fatal and carries significant morbidity. Bone involvement in LCH can be destructive, painful and often associated with pathologic fractures. There is no consensus in the treatment strategies for bone LCH which could vary from simple curettage with biopsy and/or intralesional steroids to more toxic systemic chemotherapy. However, the number of treatments for this disease is limited and other options need to be explored. Bisphosphonates are osteoclast inhibitors that can target certain osteoclast markers expressed by the multinucleated giant cells in the skin, bone and lymph nodes LCH lesions and can potentially be used to alleviate bone pain and possibly control the progression of disease activity. Purpose: To evaluate the efficacy and safety of bisphosphonates in treating bone LCH and extra-osseous disease. Methods: An international multicenter retrospective chart review was conducted in children and adults with LCH who received bisphosphonates between 1995 and 2014. Results: Eighteen patients were identified from 4 centers. All received bisphosphonates therapy either at diagnosis or at ≥ 1st reactivation. Median age at start of bisphosphonates was 23.7 years (range 5.7-38.3 years), and median follow-up time post-bisphosphonate therapy was 2.8 years (range 0.9-5.0 years). Patients had either single system bone disease or bone lesions as part of their multisystem disease. Patients were treated with different bisphosphonates with majority received zoledronic acid (n=10), followed by pamidronate (n=4) and alendronate (n= 3); one patient received both pamidronate and zoledronic acid. All patients reported significant reduction in pain to either no or mild pain after administration of bisphosphonates. Thirteen of 18 patients (72%) achieved complete remission (CR) in their bone lesions, including lesions in skin (n=1), lung (n=1) and pituitary (n=1); 2 had partial response and 3 had no response. Among the 13 CR patients, 12 had no active disease for a median of 4.1 years (range 2.8 - 5.1 years) and 1 developed radiographic neurodegeneration after 2 years. Bisphosphonate therapy was well tolerated by all patients with no major toxicity. Progression-free survival (PFS) was 75 ± 11% at 3 years, with a trend favoring better PFS (P=0.24) in patients with no or first reactivation compared with those having ≥ 2 reactivations. Conclusion: Bisphosphonates is a well-tolerated medication that can significantly improve bone pain in patients with bone LCH, and may even be effective in treating extra-osseous disease. A prospective randomized trial evaluating the role of bisphosphonates in multifocal bone LCH is warranted. Disclosures No relevant conflicts of interest to declare.
Shaodong Yang, Xinming Chen, Jiali Zhang, Qiong Fang
International Journal of Clinical and Experimental Pathology, Volume 8, pp 13647-13650

Abstract:
Langerhans cell histiocytosis (LCH) is a rare disorder characterized by the proliferation of pathologic Langerhans cells. Its clinical presentation is highly variable, that range from single-system, limited disease to severe, multi-organ disease with high mortality. LCH usually affects children and young adults. The most frequent sites for LCH are the bone, skin, lung, pituitary gland, and lymph nodes. Salivary gland involvement by LCH is extremely rare, and only a few cases of LHC involving the parotid glands have been reported in the English literature. To our knowledge, the involvement of the sublingual gland as a part of single or multisystem LCH has not been previously described. Herein we reported the first case of primary LCH of the sublingual gland. A 40-year-old woman presented with a 2-month history of a painless mass on the right sublingual area. Excision of the lesion including the right sublingual gland was performed. Histopathological diagnosis of LCH was rendered. The patient remains free of symptoms 17 months after surgery.
Huanyu Ding, Yang Li, Caishun Ruan, Yuan Gao, Hehua Wang, Xiangsong Zhang, Zhihong Liao
Endocrinology, Diabetes & Metabolism Case Reports, Volume 2015; doi:10.1530/edm-15-0055

Abstract:
Summary Erdheim-Chester disease (ECD), one type of systemic non-Langerhans cell histiocytosis, has been rarely seen and is characterized by the accumulation of foamy CD68+CD1a- histiocytes. We reported a case of ECD and reviewed the clinical features of 13 cases of ECD reported so far in China. A 53-year-old male was diagnosed with central diabetes insipidus in March 2014, followed by fever, splenomegaly and anemia in July 2014. His initial pituitary magnetic resonance imaging (MRI) revealed the absence of high signal at T1-weighted image in posterior pituitary without any lesion. A further positron emission tomography/computer tomography (PET/CT) images showed elevated metabolic activity of 18F-2-fluro-D-deoxy-glucose (FDG) and low 13N-NH3 uptake in the posterior pituitary, and multi-organ involvement. Biopsy at right femur lesion revealed that granulomatous infiltration of foamy histiocytes and Touton giant cells surrounded by fibrosis tissues. Immunohistochemistry stain was positive for CD68, negative for CD207/Langerin and S-100. The diagnosis of ECD was confirmed and the treatment with pegylated interferon was effective. ECD was a possible immune-related disorder concluding from the IgG4 immunohistochemistry results. We summarized the pathological manifestations for ECD and its differential diagnosis from Langerhans cell histiocytosis (LCH) and Rosai-Dorfman disease (RDD). ECD should be considered by both pathologists and clinicians in the differential diagnosis when central diabetes insipidus is accompanied with multi-organ involvement, especially skeletal system involvement, or recurrent fever. Learning points ECD should be considered when central diabetes insipidus is accompanied with multisystem involvement, especially symmetric/asymmetric bone lesions, or recurrent fever. PET/CT scanning was helpful for locating pituitary lesion, discovering multiple system involvement and indicating the biopsy sites. Conducting proper immunohistochemistry stains was important for diagnosing ECD. ECD might be correlated with immune disorder.
Lintao Bi, Butong Sun, Zhenxia Lu, Zhangzhen Shi, Dan Wang, Zhenxing Zhu
Experimental and Therapeutic Medicine, Volume 9, pp 2137-2140; doi:10.3892/etm.2015.2396

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Pistamaltzian Nikolaos, Nikolaidi Adamantia, Raftogiannis Maria, Economou Aggelos, Mourtzoukos Spryridon, Athanasiadis Ilias
Forum of Clinical Oncology, Volume 6, pp 6-9; doi:10.1515/fco-2015-0002

Abstract:
A 35 year old male patient received treatment for testicular cancer of pure seminoma histology. He underwent initially a right inguinal orchiectomy and afterwards he received 3 cycles of BEP chemotherapy, as his imaging studies showed enlarged para-aortic lymph nodes. Five months after completion of chemotherapy treatment, a thoracic CT revealed multiple micronodular lesions in both lungs. The patient was advised about the need of salvage chemotherapy, but he opted to undergo further investigation. A lung lesion biopsy was performed, and histology was compatible with diagnosis of Langerhans cell histiocytosis (LCH). LCH is quite an unusual clinical entity that affects usually -but not solely- the lungs of young adults. The hallmark of the disease, is the presence of the activated Langerhans cells. As its clinical, laboratory and radiological findings are nonspecific, a transbronchial biopsy is sometimes necessary to establish diagnosis. Treatment is generally symptomatic and reserved for patients with multisystem organ involvement. LCH’s relationship with cancer is complex and no certain correlation is evident. More troublesome can be its differential diagnosis from a cancer with metastatic spread to the lungs or other affected organs, and although rare, it has to be kept in mind when suspicious signs arise
, R. Maarten Egeler, Kudakwashe Chikwava, Kathleen Patterson, Ronald Jaffe
Pediatric and Developmental Pathology, Volume 18, pp 127-138; doi:10.2350/15-01-1593-oa.1

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Jung-Eun Choi, Hae Ri Lee, Jung Hun Ohn, Min Kyong Moon, Juri Park, Seong Jin Lee, Moon-Gi Choi, Hyung Joon Yoo, Jung Han Kim, Eun-Gyoung Hong
Endocrinology and Metabolism, Volume 29, pp 394-9; doi:10.3803/enm.2014.29.3.394

Abstract:
We report the rare case of an adult who was diagnosed with recurrent multisystem Langerhans cell histiocytosis (LCH) involving the pituitary stalk and lung who present with central diabetes insipidus and was successfully treated with systemic steroids and chemotherapy. A 49-year-old man visited our hospital due to symptoms of polydipsia and polyuria that started 1 month prior. Two years prior to presentation, he underwent excision of right 6th and 7th rib lesions for the osteolytic lesion and chest pain, which were later confirmed to be LCH on pathology. After admission, the water deprivation test was done and the result indicated that he had central diabetes insipidus. Sella magnetic resonance imaging showed a mass on the pituitary stalk with loss of normal bright spot at the posterior lobe of the pituitary. Multiple patchy infiltrations were detected in both lung fields by computed tomography (CT). He was diagnosed with recurrent LCH and was subsequently treated with inhaled desmopressin, systemic steroids, vinblastine, and mercaptopurine. The pituitary mass disappeared after two months and both lungs were clear on chest CT after 11 months. Although clinical remission in multisystem LCH in adults is reportedly rare, our case of adult-onset multisystem LCH was treated successfully with systemic chemotherapy using prednisolone, vinblastine, and 6-mercaptopurine, which was well tolerated
Kimber Foust, , Scott Genshaft, Tisha Wang
Published: 1 October 2013
Chest, Volume 144, pp 666A-666A; doi:10.1378/chest.1704447

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J. Haroche
Published: 1 June 2013
by BMJ
Annals of the Rheumatic Diseases, Volume 71, pp 8.3-8; doi:10.1136/annrheumdis-2012-eular.1506

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, Mauricio Saab Assef, Rodrigo Azevedo Rodrigues, Carla Pagliari, Mauricio Saab Assefa, Rodrigo Azevedo Rodriguesa
Published: 1 January 2013
Autopsy Case Reports, Volume 3, pp 39-44; doi:10.4322/acr.2013.006

Abstract:
Langerhans cell histiocytosis (LCH) is a rare disease characterized by\ud proliferation of Langerhans-type cells that express CD1a, Langerin (CD207) and\ud S100 protein. Birbeck granules are a hallmark by ultrastructural examination.\ud LCH presents with a wide clinical spectrum, ranging from solitary lesions of\ud a single site (usually bone or skin) to multiple or disseminated multisystemic\ud lesions, which can lead to severe organ dysfunction. Most cases occur in\ud children. Gastrointestinal tract involvement is rare and has been associated\ud with systemic illness and poor prognosis especially in children under the age of\ud 2 years. Adult gastrointestinal LCH is very rare. We report a case of a previously\ud healthy, nonsmoking 48-year-old male who was referred for routine screening\ud colonoscopy. Two sessile, smooth, firm and yellowish LCH polyps measuring\ud 0.2 cm and 0.3 cm were detected in the sigmoid colon. Fifteen months later a\ud second colonoscopy found two histologically confirmed hyperplastic polyps at\ud the sigmoid colon. No other LCH lesions were seen. A third colonoscopy after\ud 28 months of follow-up found a submucosal 0.5 cm infiltrated and ulcerated\ud LCH polyp in the cecum, close to the ostium of the appendix. The patient\ud had been asymptomatic for all this period. Imaging investigation for systemic\ud or multiorgan disease did not find any sign of extracolonic involvement. On\ud histology all lesions showed typical LCH features and immunohistochemical\ud analysis showed strong and diffuse staining for CD1a and CD207. This case\ud illustrates two distinct clinicopathologic features not previously reported in this\ud particular clinical setting: metachronous colonic involvement and positivity for\ud CD207
, Mirna Djuric, Istvan Klemt, Zivka Eri, Milana Panjkovic, Dragana Tegeltija, Djordje Povazan
Vojnosanitetski pregled, Volume 70, pp 1159-1161; doi:10.2298/vsp1312159l

Abstract:
Introduction. Langerhans cell histiocytosis (LCH) is an uncommon disease of unknown etiology characterized by uncontrolled proliferation and infiltration of various organs by Langerhans cells. Case report. We presented a 54-year-old man, heavy smoker, with dyspnea, cough, hemoptysis, headache and ataxia, who died shortly after admission to our hospital. On the autopsy, tumor was found in the posterior segment of the right upper pulmonary lobe as well as a right-sided occipitoparietal lesion which penetrated into the right ventricle resulting in internal and external hematocephalus. Histologically and immunohistohemically, the diagnosis of primary lung adenocarcinoma with brain metastasis was made (tumor cells showed positivity for CK7 and TTF-1 which confirmed the diagnosis). In the lung parenchyma around the tumor, as well as in brain tissue around the metastatic adenocarcinoma histiocytic lesions were found. Light microscopic examination of the other organs also showed histiocytic lesions involving the pituitary gland, hypothalamus, spleen and mediastinal lymph nodes. Immunohistochemical studies revealed CD68, S-100 and CD1a immunoreactivity within the histiocytes upon which the diagnosis of Langerhans' cells histiocytosis was made. Conclusion. The multisystem form of LCH with extensive organ involvement was an incidental finding, while metastatic lung adenocarcinoma to the brain that led to hematocephalus was the cause of death.
, N. Rakesh, Manjushree Agarwal
Journal of Clinical and Experimental Dentistry, Volume 4; doi:10.4317/jced.50728

Abstract:
Langerhans cell histiocytosis (LCH), is a rare, proliferative disorder in which the accumulation of pathologic Langerhans cells leads to local tissue infiltration and destruction. We present a case of a 32 years old, completely edentulous female patient who presented with erythema of hard palate, maxillary alveolar mucosa and mucosa over the distobuccal part of mandibular alveolar ridge with foci of ulcerations. Histopathologic features were suggestive of LCH which was confirmed by immunohistochemistry which was CD1a positive, confirmatory for LCH. Bone scan revealed multiple bone involvement. At this stage, disease had already progressed to multisystem involvement with endocrinal abnormalities (primary hypothyroidism and hyperprolactinemia), requiring aggressive treatment. Therefore, this case is a reminder of the possibility of occurrence of this rare disease in the oral cavity which might manifest itself in multiple presentations thus easily leading to the misdiagnosis and therefore, it could be easily overlooked by dentists. Key words:Langerhans cell histiocytosis, immunohistochemistry, bone scan.
Chen-Xi Xia, Rong Li, Zhi-Hong Wang, Fa-Jin Lv, Xiao-Qiong Tang, Qi-Fu Li, Su-Hua Zhang
Published: 1 January 2012
Endocrine Journal, Volume 59, pp 47-54; doi:10.1507/endocrj.ej11-0243

Abstract:
Goiter is a very common clinical problem; however, Langerhans cell histiocytosis (LCH) with thyroid involvement that presents as a goiter is very rare. In this article, we report one case of thyroid LCH. An 18-year-old male patient presented with goiter, polyuria, polydipsia, and lymphadenectasis of the neck, and LCH was confirmed by a lymph node biopsy and pathological investigation. Without a thyroidectomy, the goiter shrank after nine cycles of chemotherapy. In addition, we summarize the reported thyroid LCH cases in the literature from the last 10 years. LCH usually involves other organs, such as the lungs, bones, skin, pituitary gland, and lymph nodes. Thyroid LCH is more common in adults than in children, and it may coexist with a thyroid carcinoma. Without any unique thyroid manifestations, either clinically or by imaging, it is difficult to distinguish thyroid LCH from other thyroid diseases. Pathology is the gold standard for the diagnosis of LCH. A fine needle aspiration biopsy (FNAB) may help to diagnosis LCH, although sometimes it leads to misdiagnosis. Chemotherapy is recommended for multi-system LCH. Younger patients with widespread disease or who are non-responsive to chemotherapy have poor outcomes.
Aatur D. Singhi, Elizabeth A. Montgomery
American Journal of Surgical Pathology, Volume 35, pp 305-310; doi:10.1097/pas.0b013e31820654e4

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Maki Inoue, Chihiro Nakagawa, Kenji Ogura, Ei Kamio, Mamoru Tsukuda
Nippon Jibiinkoka Gakkai Kaiho, Volume 112, pp 752-756; doi:10.3950/jibiinkoka.112.752

Abstract:
We report a case of Langerhans cell histiocytosis (LCH), a rare disease caused by the proliferation of abnormal Langerhans cells, coincident with severe external ear canal inflammation. A 27-year-old man with a 1-year history of external ear canal discharge was found in computed tomography (CT) to have left temporal bone erosion with tissue granulation. Chest X-ray showed pulmonary fibrosis, necessitating transbronchial lung biopsy that yielded a definitive diagnosis of multisystem, multisite LCH involving the bone, skin, lung, pituitary, thyroid, and lymph node systems. He underwent combination chemotherapy directed by the Japan LCH Study Group. LCH should therefore be considered in the case of a patient with severe external ear canal inflammation coincident with temporal bone erosion.
, K. Sidani, F. El Fata, P. Arcand, M.C. Quintal, A. Abela
International Journal of Pediatric Otorhinolaryngology, Volume 72, pp 775-786; doi:10.1016/j.ijporl.2008.02.001

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