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 Abstractor Notes and Module 5 Rule PH9

Help me code for diagnosis year :

Grade
9 - Grade/differentiation unknown, not stated, or not applicable
Module Rule
M3 Module 5: PH9
Alternate Names
Malignant mast cell tumor
Malignant mastocytoma
MCS
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. Reported to occur in the larynx, large bowel, meninges, bone and skin.
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. Mast cell sarcoma is initially localized, followed within months by distant spread and terminal phase.
Definitive Diagnostic Methods
Immunohistochemical markers
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
No Transformations
Transformations from
No 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
None
Diagnostic Exams
None
Recurrence and Metastases
None
Epidemiology and Mortality
None