Cobimetinib in Refractory Langerhans Cell Histiocytosis (LCH), and Other Histiocytic Disorders

Conditions:   Langerhan's Cell Histiocytosis;   Juvenile Xanthogranuloma;   Erdheim-Chester Disease;   Rosai Dorfman Disease;   Neuro-Degenerative Disease;   Histiocytic Sarcoma;   Histiocytic Disorders, Malignant Intervention:   Drug: Cobimetinib Sponsors:   Carl Allen;   Texas Children's Hospital;   North American Consortium for Histiocytosis;   Genentech, Inc. Not yet recruiting
Source: ClinicalTrials.gov - Category: Research Source Type: clinical trials

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Conditions:   Langerhan's Cell Histiocytosis;   Juvenile Xanthogranuloma;   Erdheim-Chester Disease;   Rosai Dorfman Disease;   Neuro-Degenerative Disease;   Histiocytic Sarcoma;   Histiocytic Disorders, Malignant Intervention:   Drug: Cobimetinib Sponsors:   Carl Allen;   Texas Children's Hospital;   North American Consortium for Histiocytosis;   Genentech, Inc. Not yet recruiting
Source: ClinicalTrials.gov - Category: Research Source Type: clinical trials
ConclusionThe BRAFV600E mutation rate in patients with LCH was lower than those reported in other studies. In addition, BRAFV600E mutations might not be correlated with age, gender, LCH classification type or prognosis for Chinese cases.
Source: Thoracic Cancer - Category: Cancer & Oncology Authors: Tags: ORIGINAL ARTICLE Source Type: research
s are a group of rare disorders that can solely involve the lungs or might be part of a multi-organ process. Multidisciplinary assessment, including lung histology, is key to reaching the final diagnosis, as some might be hereditary and linked to a risk of malignancy. Tissue for microscopic examination of the lungs is mainly accessed during surgical treatment for pneumothorax, and can suggest an underlying cystic disease. This multidisciplinary approach is reflected in this review article, with summaries of the clinico-radiological presentation of cystic lung disease and detailed histopathological descriptions of these unc...
Source: Diagnostic Histopathology - Category: Pathology Authors: Tags: Mini-Symposium: Cardiothoracic Pathology Source Type: research
This study was supported by the Shanghai Sailing Program [grant number 17YF1425200, 2017]; Chinese National Natural Science Funding [grant number 81702249, 2017]; Science and Technology Commission of Shanghai Municipality [grant number 17511103403, 2017]; The funder has no role in the study design, data collection and analysis, decision to publish, or preparation of the manuscript. Conflict of Interest Statement The authors declare that the research was conducted in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest. Acknowledgments We acknowledge the ex...
Source: Frontiers in Oncology - Category: Cancer & Oncology Source Type: research
Abstract Small round cell lesions of the bone encompass a heterogeneous group of tumors and tumor-like lesions, including Ewing sarcoma, small cell osteosarcoma, mesenchymal chondrosarcoma, neuroblastoma, non-Hodgkin's lymphoma (NHL), "Ewing-like" undifferentiated round cell sarcomas, metastasizing small cell carcinoma, along with plasma cell dyscrasia and Langerhan's cell histiocytosis. At the same time, there are tumor mimics, for example, chronic osteomyelitis, which has overlapping radiologic features with Ewing sarcoma and a primary intraosseous NHL. An exact diagnosis necessitates integration of cl...
Source: Indian Journal of Pathology and Microbiology - Category: Pathology Authors: Tags: Indian J Pathol Microbiol Source Type: research
ConclusionThe particularity of this observation is the bilaterality of the lesion on both forearms and it has not previously been reported. Langerhans cell histiocytosis should be included in the differential diagnosis of osteomyelitis and Ewing ’s sarcoma.
Source: Journal of Medical Case Reports - Category: General Medicine Source Type: research
Rationale: Langerhans cell sarcoma (LCS) is a rare, high-grade neoplasm characterized by overtly malignant cytologic features and a poor prognosis. Herein, we present a rare case of langerhans cell histiocytosis (LCH) that later transformed into langerhans cell sarcoma 11 months after the benign mass was excised from soft tissue in the right groin. Patient concerns: A 41-year-old patient who presented with a mass in the right groin for 3 years earlier after being bitten by ants. Diagnoses: The patient was diagnosed with langerhans cell sarcoma arising from antecedent langerhans cell histiocytosis. Interventions: ...
Source: Medicine - Category: Internal Medicine Tags: Research Article: Clinical Case Report Source Type: research
Genomic analyses of histiocytic neoplasms (including Langerhans Cell Histiocytosis (LCH) and the non-LCH Erdheim-Chester Disease (ECD)) have revolutionized our understanding of these disorders as clonal hematopoietic malignancies driven by MAPK signaling and led to FDA approval of vemurafenib for BRAFV600E-mutant ECD. Despite these advances, several questions about the pathogenesis of the histiocytoses remain unanswered. For example, the cell-of-origin of the histiocytoses is not definitively known. In addition, histiocytoses represent a spectrum of diseases, and genetic alterations across histiocytosis subtypes have not b...
Source: Blood - Category: Hematology Authors: Tags: 635. Myeloproliferative Syndromes: Basic Science: Identification of Novel Therapeutic Targets Source Type: research
This article describes the case of an 11-month-old boy with claudication, right lower limb shortening, and painless right leg volume increase. Image studies demonstrated an osteolytic lesion with small ossifications within, involved by right tibial diaphysis cortical thickening. The diagnostic hypotheses were osteoid osteoma, chronic osteomyelitis (Brodie's abscess), Ewing sarcoma, and Langerhans cell histiocytosis. Microorganism cultures were negative and the histopathological exam demonstrated osteoid osteoma. This report expands the knowledge on osteoid osteoma as a cause of painless limping and lower limb shortening in...
Source: Revista Brasileira de Ortopedia - Category: Orthopaedics Source Type: research
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