Mast cell sarcoma

Mast cell sarcoma
Classification and external resources
ICD-10	C 96.2
ICD-9	202.6
ICD-O:	M 9740/3
MeSH	D012515

Mast cell sarcoma is an extremely aggressive^[1] 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. Only 3 cases are reported so far. 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.^[2] 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. ^[3]

References

^ 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.
^ Nancy Gould, "Diagnosis and Classification of Mastocytosis", The Mastocytosis Society.
^ Ansell SM, ed (2008). Rare Hematological Malignancies. Cancer Treatment & Research. Springer.

Myeloid hematological malignancy/leukemia histology (ICD-O 9590–9989, C81–C96, 200–208)

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	AML (AMKL/M7) MP (Essential thrombocytosis)

CFU-E	AML (Erythroleukemia/M6) MP (Polycythemia vera) MD (Refractory anemia, Refractory anemia with excess of blasts, Chromosome 5q deletion syndrome, Sideroblastic anemia, Paroxysmal nocturnal hemoglobinuria, Refractory cytopenia with multilineage dysplasia)

CFU-Mast

Mastocytoma (Mast cell leukemia, Mast cell sarcoma, Systemic mastocytosis)

Mastocytosis: Diffuse cutaneous mastocytosis · Erythrodermic mastocytosis · Generalized eruption of cutaneous mastocytosis (adult type) · Generalized eruption of cutaneous mastocytosis (childhood type) · Mast cell sarcoma · Solitary mastocytoma · Systemic mastocytosis · Xanthelasmoidal mastocytosis

Multiple/unknown

AML (Acute panmyelosis with myelofibrosis, Myeloid sarcoma) · MP (Myelofibrosis) · Acute biphenotypic leukaemia

M: MYL

cell/phys (coag, heme, immu, gran), csfs

rbmg/mogr/tumr/hist, sysi/epon, btst

drug (B1/2/3+5+6), btst, trns

Connective/soft tissue tumors and sarcomas (ICD-O 8800–9059) (C45–C49/D17–D21, 171/214–215)

Not otherwise specified (8800–8809)

Soft tissue sarcoma · Desmoplastic small round cell tumor

Connective tissue neoplasm

Fibromatous (8810–8839)	Fibroma/fibrosarcoma: Dermatofibrosarcoma · Dermatofibrosarcoma protuberans · Desmoplastic fibroma Fibroma/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 Histiocytoma/histiocytic sarcoma: Benign fibrous histiocytoma · Malignant fibrous histiocytoma · Atypical fibroxanthoma Solitary fibrous tumor

Myxomatous (8840–8849)	Myxoma/myxosarcoma (Cutaneous myxoma, Superficial acral fibromyxoma) · Angiomyxoma · Ossifying fibromyxoid tumour

Fibroepithelial (9000–9039)	Brenner tumour · Fibroadenoma · Phyllodes tumor

Synovial-like (9040–9049)	Synovial sarcoma · Clear-cell sarcoma