Mast cell tumor natural history, complications and prognosis

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Overview

Common complications of mast cell tumor include episodes of anaphylaxis, osteoporosis, and progression to malignant mastocytosis or mast cell leukemia.

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