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Mast cell sarcoma
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- Synonym(s): -
- Prevalence: -
- Inheritance: -
- Age of onset: All ages
- ICD-10: C96.2
- ICD-11: 2A21.2
- OMIM: -
- UMLS: C0036221
- MeSH: D012515
- GARD: -
- MedDRA: -
A summary on this disease is available in Español (2018) Italiano (2018) Nederlands (2018) Polski ()
Detailed information
General public
- Article for general public
- Deutsch (2018, pdf) - ÄZQ
Guidelines
- Emergency guidelines
- Français (2020, pdf) - Orphanet Urgences
- Clinical practice guidelines
- English (2015) - Hematology Am Soc Hematol Educ Program
- Français (2022) - PNDS


Additional information
Further information on this disease
Patient-centred resources for this disease
Research activities on this disease
Specialised Social Services
The documents contained in this web site 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.