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Mast cell sarcoma

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Disease definition

Mast cell sarcoma is a rare, neoplastic disease characterized by locally destructive sarcoma-like growth of a solitary mass, composed of atypical mast cells, and without systemic involvement. It can affect any organ and the symptoms depend on the location. Cells are medium to large, pleomorphic or epithelioid, with oval, bilobed or multilobulated nuclei, sometimes prominent multinucleated giant cells. The disease closely resembles other neoplasms and may share associated markers, however the tumor is positive for mast cell tryptase.

ORPHA:66661

Classification level: Disorder

Prevalence: <1 / 1 000 000

Inheritance: -

Age of onset: All ages

ICD-10: C96.2

ICD-11: 2A21.2

UMLS: C0036221

MeSH: D012515

A summary on this disease is available in Français, Español, Italiano, Nederlands, Polski
Detailed information

Logo ERN: produced/endorsed by ERN(s) Logo FSMR: produced/endorsed by FSMR(s)

Guidelines
Emergency guidelines
Français (2024.pdf) - Orphanet Urgences
Clinical practice guidelines
English (2015) - Hematology Am Soc Hematol Educ Program Logo ERN
Diagnostic Keys
Français (2024) - Les clés du diagnostic Logo FSMR
The documents contained in this website are presented for information purposes only. The material is in no way intended to replace professional medical care by a qualified specialist and should not be used as a basis for diagnosis or treatment.