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.
Coding Manual:
Hematopoietic Coding Manual (PDF)
Abstractor Notes
Diagnostic Confirmation
This histology can be determined by positive histology (including peripheral blood) with or without genetics and/or immunophenotyping. Review the Definitive Diagnostic Methods, Immunophenotyping and Genetics Data sections below, and the instructions in the Hematopoietic Manual for further guidance on assigning Diagnostic confirmation.
Grade
Not Applicable
Module Rule
None
Alternate Names
Definition
Mast cell sarcoma (MCS) is an extremely rare entity characterized by localized destructive growth of highly atypical mast cells, which can be identified only through the application of appropriate immunohistochemical markers, such as antibodies specific for tryptase and KIT (CD117).
Although the disease is initially localized, distant spread followed by a terminal phase resembling mast cell leukemia occurs after a short interval of several months.
Although the disease is initially localized, distant spread followed by a terminal phase resembling mast cell leukemia occurs after a short interval of several months.
Definitive Diagnostic Methods
Bone marrow biopsy
Genetic testing
Immunophenotyping
Genetics Data
None
Immunophenotyping
Immunohistochemical markers anti-tryptase/chymase and anti-CD117
KIT (CD117)+ (expression/positive)
Treatments
Chemotherapy
Radiation therapy
Surgery
Transformations to
There are no known transformations
Transformations from
There are no known transformations
Same Primaries
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 (U.S. only)
C96.22 Mast cell sarcoma (effective October 01, 2015)
C96.20 Malignant mast cell neoplasm, unspecified (effective October 01, 2015)
Signs and Symptoms
Abdominal pain
Arthralgias
Bone pain
Dermatographism
Diaphoresis
Fatigue
Fever
Flushing
Fractures
Gastrointestinal distress
Headache
Hepatosplenomegaly
Hypotension
Myalgias
Neutropenia
Osteopenia/osteoporosis
Organ impairment
Pruritis
Respiratory problems
Syncope
Tachycardia
Thrombocytopenia
Urticaria
Weight loss
Diagnostic Exams
Progression and Transformation
Epidemiology and Mortality
Survival: aggressive course, death within months
Sources
Swerdlow SH, Campo E, Harris NL, Jaffe ES, Pileri SA, Stein H, Thiele J (Eds):
WHO Classification of Tumours of Haematopoietic and Lymphoid Tissues (Revised 4th edition)
IARC: Lyon 2017
Section: Mastocytosis
Pages: 69
WHO Classification of Tumours of Haematopoietic and Lymphoid Tissues (Revised 4th edition)
IARC: Lyon 2017
Section: Mastocytosis
Pages: 69
International Classification of Diseases for Oncology, Third Edition, Second Revision. Geneva: World Health Organization, 2020.
Section: ICD-O-3.2 (2020) Morphological Codes
Pages: http://www.iacr.com.fr/index.php?option=com_content&view=category&layout=blog&id=100&Itemid=577
Section: ICD-O-3.2 (2020) Morphological Codes
Pages: http://www.iacr.com.fr/index.php?option=com_content&view=category&layout=blog&id=100&Itemid=577