Mast cell sarcoma

Name
Mast cell sarcoma
ICD-O-1 Morphology
9740/3
Effective 1978 - 1991
ICD-O-2 Morphology
9740/3
Effective 1992 - 2000
ICD-O-3 Morphology
9740/3
Effective 2001 and later
Reportable
for cases diagnosed 1978 and later
Primary Site(s)
See Module 7
Most common sites of involvement: larynx, large bowel, meninges, bone and skin.

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Grade
9 - Grade/differentiation unknown, not stated, or not applicable
Module Rule
None
Alternate Names
Definition
An exceedingly rare entity characterized by localized but destructive growth of a tumor consisting of highly atypical, immature mast cells. Although initially localized, distant spread is seen after a short interval. The cells may resemble those of Mast cell leukemia (9742/3), and a leukemic phase may occur.
Abstractor Notes
Extremely rare neoplasm characterized by a localized and destructive growth of highly atypical mast cells which can be identified only with the application of appropriate immunohistochemical markers.

If Mast cell sarcoma occurs simultaneously or after Mast cell leukemia, see M3 and Module 5:PH 9.
Definitive Diagnostic Methods
Bone marrow biopsy
Genetic testing
Immunophenotyping
Genetics Data
Somatic point mutation at codon 816 in KIT receptor gene (most common mutation is D816V)
Immunophenotyping
CD2+
CD9+
CD14-
CD15-
CD16-
CD25+
CD33+
CD45+
CD68+
CD117+
Immunohistochemical markers anti-tryptase/chymase and anti-CD117
Treatments
Blood thinners; anti-coagulant medications
Chemotherapy
Radiation
Surgery
Transformations to
There are no known transformations
Transformations from
There are no known transformations
Corresponding ICD-9 Codes
202.6 Malignant mast cell tumors
Corresponding ICD-10 Codes
C96.2 Malignant mast cell tumor
Corresponding ICD-10-CM Codes (effective October 1, 2015 U.S. only)
C96.2 Malignant mast cell tumor
Signs and Symptoms
Abdominal pain
Bone pain
Dermatographism
Diaphoresis
Flushing
Fractures
Gastrointestinal distress
Headache
Osteopenia/osteoporosis
Respiratory problems
Weight loss
Diagnostic Exams
Progression and Transformation
Mast cell sarcoma is initially localized, followed within months by distant spread and terminal phase.
Epidemiology and Mortality
Survival: aggressive course, death within months