Symptoma

Langerhans Cell Sarcoma

To date, only 30 cases of Langerhans cell sarcoma, including the present case, have been reported. We report a new case of Langerhans cell sarcoma that presented with multifocal cutaneous involvement. [ncbi.nlm.nih.gov]

Neoplastic cells can have moderate or abundant cytoplasm, foamy or vacuolated Focal necrosis often present Eosinophils are few or absent in background Lymph nodes LCS may show variety of patterns; often a mixture present Diffuse effacement Vaguely nodular [basicmedicalkey.com]

  • Splenectomy

    The patient underwent a splenectomy which revealed multifocal areas of necrosis without viable tumor. The patient is in remission 16 months after initial presentation without signs of recurrence. [pagepress.org]

    Click for larger image Since the patient's fever did not subside after treatment with antibiotics and steroids, a splenectomy and multiple mesenteric lymph node biopsies were performed. [kjronline.org]

  • Mediastinal Lymphadenopathy

    FDG PET/CT demonstrated heterogeneous high-grade activity in the pulmonary mass located in the right upper lung (standardized uptake value of 20), with central necrosis, bilateral upper mediastinal lymphadenopathy, right supraclavicular lymphadenopathy [ncbi.nlm.nih.gov]

    CT scan imaging showed bulky left tonsil, left parapharyngeal and retropharyngeal space, heterogeneously enhancing conglomerate lymph node mass in bilateral cervical (left greater than right) areas, and left axillary and mediastinal lymphadenopathy [omicsonline.org]

  • Chronic Cough

    The patient was an 81-year-old man who showed symptoms such as chronic cough and weight loss. [ncbi.nlm.nih.gov]

    The patient was a 34-yr-old man who presented with complaints of fever and a chronic cough. He was a smoker and had a history of pulmonary tuberculosis that had been treated and cured. [synapse.koreamed.org]

    Lung involvement is particularly common in smokers and can cause a chronic cough or shortness of breath. [dana-farber.org]

  • Long Arm

    We found inactivation of paired box 5 (PAX-5) in the lymphoma cells by methylation, along with duplication of part of the long arm of chromosomes 16 and 17 in the sarcoma cells. [ncbi.nlm.nih.gov]

  • Neck Swelling

    In this report we represent the case of a 77-year-old woman with a 1-month history of left neck swelling and pain. A diagnosis of LCS was rendered from pathological findings of the cervical lymph node biopsy. [ncbi.nlm.nih.gov]

    We present the case of a 38 year old woman with a 2 month history of left neck swelling and pain. A diagnosis of LCS was made based on pathological findings of the biopsy of the tonsil. [omicsonline.org]

  • Neck Mass

    We report a case of 86 year-old male of Caucasian descent who presented with an enlarging right neck mass over 2 months with an underlying unknown cause of anemia. [ncbi.nlm.nih.gov]

Even though these results may be inflated due to a strong referral bias, the study highlights the possible progression of unifocal bone disease and the importance of a comprehensive workup and follow-up. [28] Hester CC, Silkiss RZ. [eyewiki.aao.org]

We suggested palliative treatment with chemotherapy and radiation. The patient refused any treatment and died 2 weeks later. [ncbi.nlm.nih.gov]

Chemotherapy alone was not considered an effective treatment. [pagepress.org]

KEYWORDS: Head and neck; Langerhans cell sarcoma; Management; Prognosis [ncbi.nlm.nih.gov]

Etiology FDC express CD21 which is EBV receptor, however the correlation is not well documented Hyaline-vascular Castleman disease may be a predisposing factor to FDC p53 pathway may play a role Epidermal growth factor receptor(EGFR) expression has [slideshare.net]

Cervical LNs most often affected o Also common LNs: maxillary, mediastinal, mesenteric and retroperitoneum o Extranodal sites: tonsil, spleen, oral cavity, GI tract, liver, soft tissue, skin, and breast o Common sties of metastasis: LNs, lung, and liver · Etiology [hemepathreview.com]

Although the etiology of FDCS is unknown, the condition has been associated with Epstein-Barr virus ( 7 ). [e-crt.org]

[…] the characteristic granuloma-like lesions of LCH consists of Langerhans cells, macrophages, eosinophils, T lymphocytes and plasma cells, it is thought that clonal expansion of defective immature Langerhans cells is the key pathogenetic element. [3] Etiology [eyewiki.aao.org]

The etiology of LCH/LCS is unknown in most cases, and there is not clear understanding of the pathogenesis. [intechopen.com]

Abbreviations Langerhans cell sarcoma (LCS) Synonyms Dendritic/histiocytic sarcoma, Langerhans cell type Malignant histiocytosis X Malignant Langerhans cell tumor Definitions Neoplastic proliferation of overtly malignant Langerhans cells CLINICAL ISSUES Epidemiology [basicmedicalkey.com]

Epidemiology 80 cases was documented until 2011 in English literature Pancreat Dis Int，Vol 10，No 4 • August 15，2011 Hepatobiliary Onset age is various (9-82), mean age is about 50 Female: male: 1.2:1 Presentation: Painless, slow-growing mass Lab: [slideshare.net]

Histiocytic disorders of children and adult, introduction to the problem, overview, historical perspective and epidemiology. [path.upmc.edu]

Unlike in LCH, the pathophysiology and clinical course of LCS are unclear due to its rarity. Here, we report the case of a 73-year-old male patient who was diagnosed with cutaneous LCH and pulmonary LCS at the same time. [ncbi.nlm.nih.gov]

These image findings may be seen in cases of LCS due to the similar pathophysiology. However, unlike LCH, in which punctate, calcification in the thymus has been described, thymic calcification could not be seen in our 2 patients ( 10 ). [kjronline.org]

The lesions of LCH may involve the bones, skin, hypothalamus-pituitary region, as well as other organs. [2] Pathophysiology While the characteristic granuloma-like lesions of LCH consists of Langerhans cells, macrophages, eosinophils, T lymphocytes and [eyewiki.aao.org]

Spontaneous regression has been reported, perhaps suggesting an incomplete understanding of the pathophysiology 26. A number of factors could be responsible for the worse prognosis in intraabdominal, extranodal fdcs. [current-oncology.com]

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