Mast cell tumor natural history, complications and prognosis

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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

Common complications of mast cell tumor include episodes of anaphylaxis, osteoporosis, and progression to malignant mastocytosis or mast cell leukemia. The prognosis of mast cell tumor varies with the subtype, location, and extent of the tumor. The cutaneous mastocytosis is associated with the most favorable prognosis and mast cell leukemia is associated with poor prognosis.

Natural History

• Mast cell tumor usually occurs as a sporadic disease that is often transient and limited in children and progressive in adults.
• Systemic mastocytosis may develop in childhood cases of urticaria pigmentosa that persist beyond puberty, and in approximately 40% of adults with urticaria pigmentosa, usually of long standing.
• Patients with mast cell tumor often initially have symptom-free intervals interspersed among symptomatic periods.
• Over time, symptom-free intervals shorten, and finally symptoms become chronic with intensity which fluctuates but with an overall trend toward steadily increasing intensity.

Complications

Common complications of mast cell tumor include:

• Anaphylaxis
• Osteoporosis
• Systemic mastocytosis may progress to malignant mastocytosis and/ or mast cell leukemia

Prognosis

The prognosis varies with the location and extent of the tumor:

Subtype	Prognosis
Cutaneous mastocytosis	Childhood cases: resolve spontaneously Adults: may progress to systemic form
Systemic mastocytosis	Prognosis varies with degree of hematological and organ involvement Indolent systemic mastocytosis: good prognosis Mast cell leukemia: poor prognosis
Localized mastocytosis	Mastocytoma: benign tumor with good prognosis Mast cell sarcoma: poor prognosis