Mięsak z komórek dendrytycznych grudki

Kod Orpha: 86902Kod OMIM:

Definicja

A rare dendritic cell neoplasm characterized by a proliferation of spindled to ovoid cells with morphological and immunophenotypic features of follicular dendritic cells. Conventional follicular dendritic cell sarcomas are negative for EBV. The tumor arises as a painless, slow-growing mass in lymph nodes (most often cervical), extranodal sites (such as tonsils, gastrointestinal tract, soft tissue, mediastinum, or lung, among others), or both. Paraneoplastic pemphigus may occur in rare cases. Predictive factors are tumor size, presence of coagulative necrosis, mitotic count, and presence of significant cytological atypia.

Dane
Klasyfikacja

Choroba

Kod ORPHA
86902
Kod OMIM
-
Kod ICD10
C96.4
Kod ICD11
2B31.5

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