Interdigitating dendritic cell sarcoma

Orpha code: 86900OMIM code: 267730

Definition

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
Classification

Disease

Synonyms
Interdigitating cell sarcoma
Reticulum cell sarcoma
Interdigitating cell sarcoma
Reticulum cell sarcoma
ORPHA code
86900
OMIM code
267730
ICD10 code
C96.4
ICD11 code
2B31.4

No additional description.

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