Case report
Malignant Langerhans cell tumor: A case with a favorable outcome associated with the absence of blood dendritic cell proliferation

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Abstract

Malignant Langerhans cell tumor is a rare malignant proliferation of Langerhans cells, with a negative prognosis due to its dissemination throughout the body, leading to death within 1 year. This disease has to be distinguished from Langerhans cell histiocytosis. The favorable evolution of a case of Langerhans cell tumor, characterized by the absence of metastasis 18 months after its occurrence, may be due to the initial treatment, which consisted of complete and large resection of the tumor. The authors searched for abnormal dendritic cells or progenitors in the blood but found no large amounts or proliferation of CD34+ or CD1a+ cells at the diagnosis and 1 year later. This case report shows that malignant Langerhans cell tumor is not always a lethal disease. The condition may be related to surgical treatment and the absence of malignant cells in the blood when the diagnosis was performed.

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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