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. Only three cases have been are reported so far[when?]. 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.[3] 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.[4]

Mast cell sarcoma, burgeoning polylobar tissue lesion of the right colon

## See also[edit | edit source]

* Mastocytosis

## References[edit | edit source]

1. ↑ Monnier J., Georgin-Lavialle S., Canioni D., Lhermitte L., Soussan M., Arock M., Bruneau J., Dubreuil P., Bodemer C., Chandesris M., Lortholary O., Hermine O., Damaj G. et al Mast cell sarcoma: new cases and literature review. Oncotarget. 2016; 7: 66299-66309. Retrieved from https://www.oncotarget.com/article/11812/text/ Archived 2020-08-06 at the Wayback Machine
2. ↑ 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.
3. ↑ Nancy Gould, "Diagnosis and Classification of Mastocytosis Archived 2004-10-27 at the Wayback Machine", The Mastocytosis Society.
4. ↑ Ansell SM, ed. (2008). Rare Hematological Malignancies. Cancer Treatment & Research. Springer.

## External links[edit | edit source]

Classification|

* 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

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Myeloid-related hematological malignancy

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 thrombocythemia

* Acute megakaryoblastic leukemia

|

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
* 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

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Connective/soft tissue tumors and sarcomas

Not otherwise specified|

* Soft-tissue sarcoma
* Desmoplastic small-round-cell tumor

Connective tissue neoplasm|

| Fibromatous|

| Fibroma/fibrosarcoma:|

* 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|

* Myxoma/myxosarcoma
* Cutaneous myxoma
* Superficial acral fibromyxoma
* Angiomyxoma
* Ossifying fibromyxoid tumour

Fibroepithelial|

* Brenner tumour
* Fibroadenoma
* Phyllodes tumor

Synovial-like|

* Synovial sarcoma
* Clear-cell sarcoma

Lipomatous|

* Lipoma/liposarcoma
* Myelolipoma
* Myxoid liposarcoma
* PEComa
* Angiomyolipoma

* Chondroid lipoma
* Intradermal spindle cell lipoma
* Pleomorphic lipoma
* Lipoblastomatosis
* Spindle cell lipoma
* Hibernoma

Myomatous|

| general:|

* Myoma/myosarcoma

|

smooth muscle:|

* Leiomyoma/leiomyosarcoma

skeletal muscle:|

* Rhabdomyoma/rhabdomyosarcoma: Embryonal rhabdomyosarcoma
* Sarcoma botryoides
* Alveolar rhabdomyosarcoma

* Leiomyoma
* Angioleiomyoma
* Angiolipoleiomyoma
* Genital leiomyoma
* Leiomyosarcoma
* Multiple cutaneous and uterine leiomyomatosis syndrome
* Multiple cutaneous leiomyoma
* Neural fibrolipoma
* Solitary cutaneous leiomyoma
* STUMP

Complex mixed and stromal|

* Adenomyoma
* Pleomorphic adenoma
* Mixed Müllerian tumor
* Mesoblastic nephroma
* Wilms' tumor
* Malignant rhabdoid tumour
* Clear-cell sarcoma of the kidney
* Hepatoblastoma
* Pancreatoblastoma
* Carcinosarcoma

Mesothelial|

* Mesothelioma
* Adenomatoid tumor

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