[Langerhans cell sarcoma: a case report and literature review].
OBJECTIVE
To explorer the clinical features, diagnosis and therapy of Langerhans cell sarcoma (LCS).


METHODS
The clinical data of a case of LCS originated from cervical lymph nodes was analyzed. The pathological biopsy was studied by cell morphology, immunohistochemistry and electron microscopy, and the related literature was reviewed.


RESULTS
The giant tumor cells were characterized by markedly malignant proliferation, irregular nuclei and obviously chromatin abnormality, the positive S-100, CD1a and Langerin (CD207) tumor cells were revealed by immunohistochemistry, and Birbeck granules could be found by electron microscopy. All of them supported the diagnosis of LCS. The patient's condition progressed rapidly and died of multiple organ failure in a short time.


CONCLUSIONS
LCS is an extremely rare neoplastic proliferation of Langerhans cells with overtly malignant cytologic features and spreads aggressively. The diagnosis of LCS mainly relies on pathological cell morphology, immunohistochemistry and electron microscopy if necessary. The treatment includes chemotherapy, surgery and radiotherapy, etc, but lack of generally accepted optimal treatment regimen currently. In short, LCS has intensive invasiveness and poor prognosis.
Department of Hematology & Pathology, Capital Medical University, Beijing, China.
Jia
J
Zhang
Jing-shi
JS
Wang
Xue-jing
XJ
Wei
Zhao
Z
Wang
D000368:Aged; D005260:Female; D006801:Humans; D007150:Immunohistochemistry; D054752:Langerhans Cell Sarcoma / Q000175:diagnosis* / Q000628:therapy*; D007801:Langerhans Cells