Primary histiocytic sarcoma presenting as diffuse leptomeningeal disease: Case description and review of the literature
Zanelli M.;Fanni D.;
2017-01-01
Abstract
Histiocytic sarcoma is a rare malignant neoplasm arising most commonly in lymph nodes, intestinal tract, skin and soft tissue. The incidence of primary CNS histiocytic sarcoma is even rarer with a total of just 27 cases reported in the literature so far. Herein we describe the first autopsy case of histiocytic sarcoma presenting as a diffuse leptomeningeal disease in absence of a CNS tumor-forming parenchymal lesion. The clinical, pathological and immunophenotypic features are described and an updated literature review on primary CNS histiocytic sarcoma is included.I documenti in IRIS sono protetti da copyright e tutti i diritti sono riservati, salvo diversa indicazione.