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Name
Systemic mastocytosis with an associated hematological neoplasm
ICD-O-1 Morphology
9741/3: Malignant mastocytosis
Effective
1978 - 1991
ICD-O-2 Morphology
9741/3: Malignant mastocytosis
Effective
1992 - 2000
ICD-O-3 Morphology
9741/3: Malignant mastocytosis
Effective
2001 and later
Reportable
for cases diagnosed
1978 and later
Primary Site(s)
C421
Primary site must be bone marrow (C421)
Coding Manual:
Hematopoietic Coding Manual (PDF)
Abstractor Notes
If the diagnosis is "systemic mastocytosis with an associated hematological neoplasm (AHN)" the patient has a history of myelodysplastic syndrome, myeloproliferative neoplasm, myelodysplastic/myeloproliferative neoplasm or acute leukemia. This would be a second primary.
Smoldering systemic mastocytosis may progress to aggressive systemic mastocytosis (ASM) or mast cell leukemia. Progression to either of these neoplasms is the same primary.
Smoldering systemic mastocytosis may progress to aggressive systemic mastocytosis (ASM) or mast cell leukemia. Progression to either of these neoplasms is the same primary.
Diagnostic Confirmation
None
Grade
Not Applicable
Module Rule
None
Alternate Names
Aggressive systemic mastocytosis
AHN
ASM
Lymphadenopathic (systemic) mastocytosis with eosinophilia
Malignant mastocytosis
SM
SM-AHNMD
Smoldering systemic mastocytosis
Systemic mastocytosis, NOS
Systemic mastocytosis with an associated hematological neoplasm (AHN)
Systemic mastocytosis with AHNMD
Definition
Criteria for the diagnosis of systemic mastocytosis have been established. Five variants are recognized.
Smoldering systemic mastocytosis: The mast cell burden is high, organomegaly is often found, and multilineage involvement is typically present. The clinical course is usually stable for many years, but there may be progression to aggressive systemic mastocytosis or mast cell leukemia. Skin lesions are found in most patients.
Systemic mastocytosis with an associated hematological neoplasm (AHN): This fulfills the general criteria for systemic mastocytosis as well as the criteria for an AHN. In most cases, a myeloid disease of non-mast cell lineage is detected, such as a myelodysplastic syndrome, myeloproliferative neoplasm, myelodysplastic/myeloproliferative neoplasm or acute leukemia.
The AHN should be abstracted as a second primary.
Aggressive systemic mastocytosis (ASM): Mast cells in bone marrow smears may be increased in number, but account for less than 20% of all nucleated bone marrow cells. Most patients with ASM have no skin lesions.
Smoldering systemic mastocytosis: The mast cell burden is high, organomegaly is often found, and multilineage involvement is typically present. The clinical course is usually stable for many years, but there may be progression to aggressive systemic mastocytosis or mast cell leukemia. Skin lesions are found in most patients.
Systemic mastocytosis with an associated hematological neoplasm (AHN): This fulfills the general criteria for systemic mastocytosis as well as the criteria for an AHN. In most cases, a myeloid disease of non-mast cell lineage is detected, such as a myelodysplastic syndrome, myeloproliferative neoplasm, myelodysplastic/myeloproliferative neoplasm or acute leukemia.
The AHN should be abstracted as a second primary.
Aggressive systemic mastocytosis (ASM): Mast cells in bone marrow smears may be increased in number, but account for less than 20% of all nucleated bone marrow cells. Most patients with ASM have no skin lesions.
Definitive Diagnostic Methods
Bone marrow biopsy
Genetic testing
Histologic confirmation
Genetics Data
KIT D816V
Immunophenotyping
None
Treatments
Chemotherapy
Immunotherapy
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)
C96.21 Aggressive systemic mastocytosis (effective October 01, 2015)
D47.02 Systemic mastocytosis (effective October 01, 2015)
Signs and Symptoms
Abdominal pain
Anemia
Arthralgias
Bone pain
Dermatographism
Diaphoresis
Eosinophilia
Fatigue
Fever
Flushing
Fractures
Gastrointestinal distress
Headache
Hepatosplenomegaly
Hypotension
Leukocytosis
Myalgias
Neutropenia
Osteopenia/osteoporosis
Organ impairment
Pruritis
Respiratory problems
Syncope
Tachycardia
Thrombocytopenia
Urticaria
Weight loss
Diagnostic Exams
Progression and Transformation
None
Epidemiology and Mortality
Age: primary second decade of life
Sex: no male or female predominance
Survival: patients with cutaneous involvement only follow indolent course and may have a normal life expectancy. Patients with no skin lesions and aggressive disease may survive only a few 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
