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This neoplasm is not reportable.
Name
Cutaneous mastocytosis
ICD-O-3 Morphology
9740/1: Mastocytoma
Effective
2001 and later
Reportability
This neoplasm is not reportable
Primary Site(s)
No primary site specified
Cutaneous primary sites only (C440-C449, C510-C512, C518-C519, C600-C602, C608-C609, C632)
This hematopoietic disease is not reportable for the U.S.
Canada, refer to your specific province for reportability requirements.
This hematopoietic disease is not reportable for the U.S.
Canada, refer to your specific province for reportability requirements.
Abstractor Notes
Alternate Names
Extracutaneous mastocytoma
Maculopapular cutaneous mastocytosis (MPCM)
Mast cell tumor, NOS
Mastocytoma (NOS)
Solitary mastocytoma of skin
Definition
The diagnosis of cutaneous mastocytosis (CM) requires the demonstration of typical clinical findings and histological proof of abnormal mast cell infiltration of the determine.
In cutaneous mastocytosis, there is no evidence of systemic involvement in the bone marrow or any other organ. In addition, the diagnostic criteria for systemic mastocytosis is not fulfilled; however, patients with cutaneous mastocytosis may present with one or two of the minor diagnostic criteria for systemic mastocytosis, such as an elevated serum tryptase level or abnormal morphology of mast cells in the bone marrow.
Three major variants of cutaneous mastocytosis are recognized:
1. Urticaria pigmentosa/maculopapular cutaneous mastocytosis: The most frequent form of CM. In children the lesions of UP tend to be larger and papular. Histopathology typically reveals aggregates of spindle-shaped mast cells filling the papillary dermis and extending as sheets and aggregates into the reticular dermis, often in perivascular and periadnexal positions. A subvariant, usually occurring in young children, presents as non-pigmented, plaque-forming lesions. In adults the lesions are disseminated and tend to be red or brown and macular or maculopapular.
2. Diffuse cutaneous mastocytosis: Occurs almost exclusively in childhood. The skin is diffusely thickened and may have an orange peel appearance. There are no individual lesions. Atypical cells are not present, which can help distinguish this disease from a mast cell sarcoma (9740/3) occurring in the skin.
3. Mastocytoma of skin: Occurs as a single lesion, almost exclusively in infants. These mast cell infiltrates may extend into the subcutaneous tissues. Extracutaneous mastocytomas has been reported in the lung. This isolated mast cell tumor has a non-destructive growth pattern.
In cutaneous mastocytosis, there is no evidence of systemic involvement in the bone marrow or any other organ. In addition, the diagnostic criteria for systemic mastocytosis is not fulfilled; however, patients with cutaneous mastocytosis may present with one or two of the minor diagnostic criteria for systemic mastocytosis, such as an elevated serum tryptase level or abnormal morphology of mast cells in the bone marrow.
Three major variants of cutaneous mastocytosis are recognized:
1. Urticaria pigmentosa/maculopapular cutaneous mastocytosis: The most frequent form of CM. In children the lesions of UP tend to be larger and papular. Histopathology typically reveals aggregates of spindle-shaped mast cells filling the papillary dermis and extending as sheets and aggregates into the reticular dermis, often in perivascular and periadnexal positions. A subvariant, usually occurring in young children, presents as non-pigmented, plaque-forming lesions. In adults the lesions are disseminated and tend to be red or brown and macular or maculopapular.
2. Diffuse cutaneous mastocytosis: Occurs almost exclusively in childhood. The skin is diffusely thickened and may have an orange peel appearance. There are no individual lesions. Atypical cells are not present, which can help distinguish this disease from a mast cell sarcoma (9740/3) occurring in the skin.
3. Mastocytoma of skin: Occurs as a single lesion, almost exclusively in infants. These mast cell infiltrates may extend into the subcutaneous tissues. Extracutaneous mastocytomas has been reported in the lung. This isolated mast cell tumor has a non-destructive growth pattern.
Definitive Diagnostic Methods
This data item does not apply
Treatments
Chemotherapy
Radiation therapy
Surgery
Corresponding ICD-10 Codes (Cause of Death codes only)
D47.0 Histiocytic and mast cell tumors of uncertain and unknown behavior
Corresponding ICD-10-CM Codes (U.S. only)
D47.01 Cutaneous mastocytosis (effective October 01, 2015)
D47.09 Other mast cell neoplasms of uncertain behavior (effective October 01, 2015)
Sources
WHO Classification of Tumours Editorial Board. Haematolymphoid tumours. Lyon (France): International Agency for Research on Cancer; 2024. (WHO classification of tumours series, 5th ed.; vol. 11). https://publications.iarc.who.int/637.
Section: Mastocytosis
Pages: Part A: 61-63
Section: Mastocytosis
Pages: Part A: 61-63
International Classification of Diseases for Oncology, 3rd edition (including revisions). Geneva: World Health Organization, 2001, 2011, 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
