Case reportMalignant Langerhans cell tumor: A case with a favorable outcome associated with the absence of blood dendritic cell proliferation☆
Section snippets
Case report
A 38-year-old woman noticed a single red, hardened tumor on her abdominal skin (Fig 1) in April 1998. There was no pain or pruritus. Two months later, this 1- × 0.8-cm tumor was excised with a safety margin of 3 cm. There was neither a superficial enlarged lymph node nor hepatosplenomegaly. Laboratory investigations revealed no evidence of systemic involvement or inflammation. Bone marrow biopsy revealed no histiocytic proliferation and no CD34+, CD1a+, or S100+ cells. X-ray findings of the
Discussion
This patient presented with a malignant Langerhans cell tumor. Indeed, this disease is defined by Tani et al1 as a “malignant neoplasm of Langerhans cells with the following criteria: (i) proliferation of typical Birbeck granule–containing tumor cells, and (ii) malignant cytological features such as atypia and frequent mitotic figures.” This cancer is characterized by a rapid dissemination from a single cutaneous tumor toward the whole skin and internal organs with a fatal outcome in spite of
Acknowledgements
We thank Vincent Atrache for his linguistic help.
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2009, Weedon's Skin Pathology: Third Edition
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Conflict of interest: None identified.