Interdigitating dendritic cell sarcoma

Orpha code: 86900OMIM code: 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.

Disease data
Klasyfikacja

Disease

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

No additional description.

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