Mięsak ze splatających się komórek dendrytycznych

Kod Orpha: 86900Kod OMIM: 267730

Definicja

A rare dendritic cell tumor characterized by a neoplasm composed of spindle to ovoid cells with phenotypic features similar to those of interdigitating dendritic cells. Solitary lymph node involvement is common, although extranodal localization (in particular skin and soft tissue) has also been reported. Patients usually present with an asymptomatic mass, sometimes with systemic symptoms such as fatigue, fever, and night sweats. Generalized lymphadenopathy, splenomegaly, or hepatomegaly may be seen in rare cases. The clinical course is generally aggressive.

Dane
Klasyfikacja

Choroba

Synonimy
Interdigitating cell sarcoma
Reticulum cell sarcoma
Interdigitating cell sarcoma
Reticulum cell sarcoma
Kod ORPHA
86900
Kod OMIM
267730
Kod ICD10
C96.4
Kod ICD11
2B31.4

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