Mast cell tumor classification

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1];Associate Editor(s)-in-Chief: Suveenkrishna Pothuru, M.B,B.S. [2]

Overview

Based on the affected organ(s), mast cell tumor may be classified into either cutaneous mastocytosis or systemic mastocytosis. Mast cell tumor may be classified into seven subtypes based on WHO classification system.[1]

Classification

Based on the affected organ(s), mast cell tumor may be classified into two subtypes:

  • Cutaneous mastocytosis
  • Limited to the skin where mast cells infiltrate the skin
  • Systemic mastocytosis
  • Systemic mastocytosis is caused by mast cells accumulating in the tissues and can affect organs such as the liver, spleen, bone marrow, and small intestine
  • Localized mastocytosis

Mast cell tumor may be classified according to WHO classification into seven subtypes:[1]

  • Cutaneous mastocytosis
  • Urticaria pigmentosa
  • Maculopapular cutaneous mastocytosis
  • Diffuse cutaneous mastocytosis
  • Mastocytoma of skin
  • Indolent systemic mastocytosis
  • Smoldering systemic mastocytosis
  • Isolated bone marrow mastocytosis
  • Systemic mastocytosis with an associated (clonal) hematologic non–mast cell lineage disease
  • Aggressive systemic mastocytosis
  • Lymphadenopathic systemic mastocytosis with eosinophilia
  • Mast cell leukemia
  • Typical mast cell leukemia
  • Aleukemic mast cell leukemia
  • Mast cell sarcoma
  • Extracutaneous mastocytoma

References

  1. 1.0 1.1 Patnaik MM, Rindos M, Kouides PA, Tefferi A, Pardanani A (2007). "Systemic mastocytosis: a concise clinical and laboratory review". Arch Pathol Lab Med. 131 (5): 784–91. doi:10.1043/1543-2165(2007)131[784:SMACCA]2.0.CO;2. PMID 17488167.

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