KMID : 0363220190570090538
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Korean Journal of Dermatology 2019 Volume.57 No. 9 p.538 ~ p.543
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Langerhans Cell Sarcoma of the Scalp with Concomitant Multiple Cervical Lymph Node and Adjacent Soft Tissue Involvement: A Case Report
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Han Man-Hoon
Kim Jun-Young Lee Weon-Ju Lee Seok-Jong
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Abstract
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Currently, the World Health Organization classifies Langerhans cell tumors into Langerhans cell histiocytosis and Langerhans cell sarcoma (LCS). LCS is a neoplastic proliferation of Langerhans cells showing malignant cytological features and aggressive clinical behavior with grave prognosis. Only a few cases have been reported in the available literature; therefore, to date, no definitive treatment has been established. A 64-year-old woman presented with a 1-year history of an asymptomatic, slow-growing erythematous nodule measuring 0.7 cm on her scalp. The patient also reported a 3-month history of a painful swelling on the right side of her neck. Histopathological examination of a scalp biopsy specimen revealed sheets of atypical cells with hyperchromatic nucleoli and clear cytoplasm. Immunohistochemical studies revealed malignant cells positive for CD1a, CD31, CD68, and S-100 expression. Additionally, positron emission tomography-computed tomography and fine-needle aspiration revealed LCS of the cervical lymph nodes and surrounding soft tissue. We recommended surgical excision and adjunctive chemotherapy; however, the patient refused treatment and died of the disease 28 months later.
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KEYWORD
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Langerhans cell sarcoma, Lymph node, Parotid gland, Scalp, Soft tissue
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