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Mast cell sarcoma

Specialty	Oncology
Usual onset	Can occur at any age.^[1]
Prognosis	The median survival time is less than 18 months

Mast cell sarcoma is an extremely aggressive^[2] form of sarcoma made up of neoplastic mast cells. A sarcoma is a tumor made of cells from connective tissue. Mast cell sarcoma is an extremely rare tumor. The largest analysis to date comprises 34 cases.^[3] Prognosis is extremely poor. People with a mast cell sarcoma have no skin lesions, and pathology examination of the tumor shows it to be very malignant with an aggressive growth pattern.^[4] Mast cell sarcoma should not be confused with extracutaneous mastocytoma, a rare benign mast cell tumor without destructive growth. In the cases observed, mast cell sarcoma terminated quickly as mast cell leukemia; one of the most aggressive human cancers.^[5]

References

^ Monnier, Jilliana; Georgin-Lavialle, Sophie; Canioni, Danielle; Lhermitte, Ludovic; Soussan, Michael; Arock, Michel; Bruneau, Julie; Dubreuil, Patrice; Bodemer, Christine; Chandesris, Marie-Olivia; Lortholary, Olivier; Hermine, Olivier; Damaj, Gandhi (4 October 2016). "Mast cell sarcoma: new cases and literature review". Oncotarget. 7 (40): 66299–66309. doi:10.18632/oncotarget.11812. PMC 5323235. PMID 27602777.
^ Chott A, Guenther P, Huebner A, Selzer E, Parwaresch R, Horny H, Valent P (2003). "Morphologic and immunophenotypic properties of neoplastic cells in a case of mast cell sarcoma". Am J Surg Pathol. 27 (7): 1013–9. doi:10.1097/00000478-200307000-00019. PMID 12826896. S2CID 46710032.
^ Matsumoto, Nana P.; Yuan, Ji; Wang, Jun; Shen, Qi; Chen, Xueyan; Kim, Young; Zuppan, Craig W.; Chang, Chung-Che; Cui, Wei; Chen, Dong; Shi, Min; Gisriel, Savanah D.; Chen, Mingyi; Xu, Mina L.; Pan, Zenggang (July 2022). "Mast cell sarcoma: clinicopathologic and molecular analysis of 10 new cases and review of literature". Modern Pathology. 35 (7): 865–874. doi:10.1038/s41379-022-01014-w. PMID 35105959.
^ Nancy Gould, "Diagnosis and Classification of Mastocytosis Archived 2004-10-27 at the Wayback Machine", The Mastocytosis Society.
^ Ansell, Stephen M., ed. (2008). Rare Hematological Malignancies. doi:10.1007/978-0-387-73744-7. ISBN 978-0-387-73743-0.^{[page needed]}

External links

CFU-GM/
and other granulocytes

CFU-GM

Myelocyte

AML	Acute myeloblastic leukemia M0 M1 M2 APL/M3
MP	Chronic neutrophilic leukemia

Monocyte

AML	AMoL/M5 Myeloid dendritic cell leukemia
CML	Philadelphia chromosome Accelerated phase chronic myelogenous leukemia

Myelomonocyte

AML	M4
MD-MP	Juvenile myelomonocytic leukemia Chronic myelomonocytic leukemia

Other

Histiocytosis

CFU-Baso

AML	Acute basophilic

CFU-Eos

AML	Acute eosinophilic
MP	Chronic eosinophilic leukemia/Hypereosinophilic syndrome

MEP

CFU-Meg

MP	Essential thrombocythaemia Acute megakaryoblastic leukemia

CFU-E

AML	Erythroleukemia/M6
MP	Polycythaemia vera
MD	Refractory anemia Refractory anemia with excess of blasts Chromosome 5q deletion syndrome Sideroblastic anemia Paroxysmal nocturnal haemoglobinuria Refractory cytopenia with multilineage dysplasia

CFU-Mast

Mastocytoma	Mast cell leukemia Mast cell sarcoma Systemic mastocytosis
Mastocytosis	Diffuse cutaneous mastocytosis Erythrodermic mastocytosis Adult type of generalized eruption of cutaneous mastocytosis Urticaria pigmentosa Mast cell sarcoma Solitary mastocytoma
Systemic mastocytosis	Xanthelasmoidal mastocytosis

Multiple/unknown

AML	Acute panmyelosis with myelofibrosis Myeloid sarcoma
MP	Primary myelofibrosis Biphenotypic acute leukaemia

Connective/soft tissue tumors and sarcomas

Not otherwise specified

Connective tissue neoplasm

Fibromatous

Fibroma/fibrosarcoma	Dermatofibrosarcoma protuberans Desmoplastic fibroma
Fibroma/fibromatosis	Aggressive fibromatosis Aggressive infantile fibromatosis Aponeurotic fibroma Collagenous fibroma Diffuse infantile fibromatosis Familial myxovascular fibromas Fibroma of tendon sheath Fibromatosis colli Infantile digital fibromatosis Juvenile hyaline fibromatosis Plantar fibromatosis Pleomorphic fibroma Oral submucous fibrosis Pachydermodactyly
Histiocytoma/histiocytic sarcoma	Benign fibrous histiocytoma Malignant fibrous histiocytoma Atypical fibroxanthoma Solitary fibrous tumor

Synovial-like

General	Myoma/myosarcoma
Smooth muscle	Leiomyoma/leiomyosarcoma
Skeletal muscle	Rhabdomyoma/rhabdomyosarcoma: Embryonal rhabdomyosarcoma Sarcoma botryoides Alveolar rhabdomyosarcoma
Leiomyoma Cutaneous Angioleiomyoma Angiolipoleiomyoma Genital leiomyoma Leiomyosarcoma Multiple cutaneous and uterine leiomyomatosis syndrome Multiple cutaneous leiomyoma Neural fibrolipoma Solitary cutaneous leiomyoma STUMP

Complex mixed and stromal

Mesothelial

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Classification	D ICD-10: C96.2 ICD-10-CM: C96.22 ICD-9-CM: 202.6 ICD-O: M9740/3 MeSH: D012515 SNOMED CT: 13583002
External resources	Orphanet: 66661

See also

References

External links