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Systemic mastocytosis with extensive large cutaneous mastocytomas: Surgical management
Author(s) -
McDermott William V.,
Topol Bruce M.
Publication year - 1985
Publication title -
journal of surgical oncology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.201
H-Index - 111
eISSN - 1096-9098
pISSN - 0022-4790
DOI - 10.1002/jso.2930300407
Subject(s) - medicine , hepatosplenomegaly , cosmesis , systemic mastocytosis , dermatology , histamine , disease , cutaneous mastocytosis , surgery , pathology
A case of systemic mastocytosis is reported with an observation period of 20 years. During these two decades multiple manifestations of the disease appeared including urticaria pigmentosis, episodic histamine release, gastro‐intestinal involvement and hepatosplenomegaly. The most extraordinary, and possibly unique phenomenon, has been the development of a massive proliferation of large mastocytomas mainly, but not exclusively, limited to the lower extremities. For different reasons (mechanical disability, bleeding, cosmesis), these tumors have required repeated admissions for surgical removal. The most successful technique has involved use of the ultrasonic scalpel.