ALK-positive histiocytosis with KIF5B - ALK fusion in the central nervous system

Source: Acta Neuropathologica - Category: Neurology Source Type: research

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Abstract PURPOSE: The 2-part, phase 1/2a, open-label study (NCT01677741) sought to determine the safety, tolerability, pharmacokinetics, and preliminary activity of dabrafenib in pediatric patients with advanced BRAF V600-mutated cancers. EXPERIMENTAL DESIGN: This phase 1 dose-finding part treated patients aged 1 to 52 weeks and 52% undergoing treatment at data cutoff date. The most common grade 3/4 adverse events suspected to be related to study drug were maculopapular rash and arthralgia (2 patients each). No dose-limiting toxicities were observed. Pharmacokinetic analyses showed a dose-dependent increase i...
Source: Clinical Cancer Research - Category: Cancer & Oncology Authors: Tags: Clin Cancer Res Source Type: research
We describe a rare case of orbital Langerhans cell histiocytosis in a 39-year-old female patient who presented right orbital pain and edema of the upper right eyelid. Surgery showed a friable lesion and underlying bone irregularity. Morphological aspects and immunohistochemical profile favored the diagnosis of Langerhans cell histiocytosis, which was confirmed with evidence of Langerin expression. The staging tests did not reveal any organ involvement, so we decided to follow the algorithm proposed by Euro Histio Net: in case of unifocal disease and in a single organ, clinical surveillance was pref...
Source: Acta Medica Portuguesa - Category: General Medicine Tags: Acta Med Port Source Type: research
We present a case of single lesion self-limited LCH in a full-term male infant with interesting morphology. Physical examination revealed a painless, 6 millimeter, well-demarcated, papule encircled by erythema with central hemorrhage. An infectious workup was negative and a punch biopsy was obtained, which showed a dermal infiltrate of histiocytes consistent with a diagnosis of LCH. The lesion healed without intervention within three weeks. Our case highlights the need for dermatologists to consider LCH in the differential diagnosis for lesions...
Source: Dermatology Online Journal - Category: Dermatology Source Type: research
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: - Category: Research Source Type: clinical trials
Ishwor Gurung, Yan Gao, Kai Han, Xue-Biao PengIndian Journal of Dermatology 2019 64(5):414-416 Langerhans cell histiocytosis (LCH) is a proliferative disease commonly seen in the pediatric population but rarely encountered in the adult population. The exact etiology remains unclear. It has various clinical features and is very likely to be misdiagnosed. Histopathology and immunohistochemistry are very important for the diagnosis of LCH. Treatment protocols remain controversial. Herein, we report a rare adult onset LCH, which is confined to the skin. A 50-year-old Chinese man presented with a nodule with itchy rashes on th...
Source: Indian Journal of Dermatology - Category: Dermatology Authors: Source Type: research
We report a rare case of mandibular condyle unifocal HL mimicking a temporomandibular joint disorder. CASE REPORT: A 44-year-old patient presented with a left temporomandibular disorder with painful left preauricular swelling. The imaging assessment found a bone lesion of the left mandibular condyle. A curettage with biopsy was used to diagnose HL. Six months later, the patient had no more pain. DISCUSSION: The craniofacial clinical expressions of HL mainly concern the bones, which can cause: pain, swelling, fracture, compression of noble organs. The other sites are: oral cavity, skin, lymph nodes, or eyes. Isolate...
Source: Revue de Medecine Interne - Category: Internal Medicine Tags: Rev Med Interne Source Type: research
Casper Royle, of Devon, was diagnosed with Langerhans cell histiocytosis when he was three months old. It affects just 50 British children a year.
Source: the Mail online | Health - Category: Consumer Health News Source Type: news
Extramammary Paget Disease (EMPD) is a rare intraepithelial adenocarcinoma that arises in apocrine gland bearing skin and presents as a chronic rash that resembles several disorders such as eczema, cutaneous T cell lymphoma, Langerhans cells histiocytosis, intraepithelial neoplasia, fungal infections and psoriasis. EMPD is often associated with underlying or distant malignancies. The epidemiology of EMPD in Canada is poorly understood. Further, the risk of developing second malignancies in Canadian EMPD patients was not evaluated prior to the presented study.
Source: Journal of Investigative Dermatology - Category: Dermatology Authors: Tags: Clinical Research and Epidemiology Source Type: research
Publication date: Available online 30 August 2019Source: Blood Cells, Molecules, and DiseasesAuthor(s): Prateek Bhatia, Minu Singh, Madhulika Sharma, Ajay Kumar, Nandita Kakkar, S. Radhika, Amita Trehan, Deepak BansalAbstractLangerhans cell histiocytosis (LCH) is an inflammatory myeloid neoplasia of children with systemic involvement and poor outcome. The altered RAS-RAF-MEK-ERK cell signalling pathway due to somatic mutation of BRAF V600E is the most common genetic abnormality associated with the disease. In the current study, we highlight the frequency of BRAF V600E in our cohort of LCH cases (n = 31) and its relatio...
Source: Blood Cells, Molecules, and Diseases - Category: Hematology Source Type: research
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