Document Detail

Malignant transformation of mastocytoma developed on skin mastocytosis into cutaneous mast cell sarcoma.
MedLine Citation:
PMID:  22498828     Owner:  NLM     Status:  In-Data-Review    
Mastocytosis is a group of disorders characterized by abnormal mast cell proliferation, involving the skin in 80% of cases. Cutaneous mastocytosis, which appears in childhood in 60% of cases, usually has a benign course with a gradually regressive evolution before puberty. Mast cell sarcomas, part of the systemic forms of mastocytosis, are very rare tumors characterized by a destructive growth of highly atypical mast cells, with secondary spread, poor prognosis, and low survival rates. We report the first known case of primary cutaneous mast cell sarcoma due to the transformation of a benign solitary mastocytoma in an adult suffering from an unregressive localized cutaneous mastocytosis. Histologic characteristics of the tumor, mutation analysis, and c-Kit expression were compared with available data. Wide surgical excision of the tumor followed by adjuvant local radiotherapy were performed, and for the first time the use of imatinib was attempted, as neoplastic mast cells expressed the CD117 marker. However, they failed to control the progression of sarcoma. To date, no treatment is known to be effective for this disease, which is associated with short-term survival of the patients.
Isabelle Auquit-Auckbur; Câlin Lazar; Sophie Deneuve; Cécile Guillemet; Nadège Cordel; France Blanchard; Pascal Joly; Philippe Courville
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Publication Detail:
Type:  Journal Article    
Journal Detail:
Title:  The American journal of surgical pathology     Volume:  36     ISSN:  1532-0979     ISO Abbreviation:  Am. J. Surg. Pathol.     Publication Date:  2012 May 
Date Detail:
Created Date:  2012-04-13     Completed Date:  -     Revised Date:  -    
Medline Journal Info:
Nlm Unique ID:  7707904     Medline TA:  Am J Surg Pathol     Country:  United States    
Other Details:
Languages:  eng     Pagination:  779-82     Citation Subset:  IM    
Departments of *Plastic Surgery ¶Pathology #Dermatology, Rouen University Hospital §Department of Oncology, Becquerel Institute, Rouen University Hospital, Rouen †Department of Plastic Surgery, Aesthetic and Reconstructive Surgery, Meulan Regional Hospital, Meulan ‡Department of ENT and Cervicofacial Surgery, Gustave Roussy Institute, Villejuif ∥Department of Dermatology, Pointe à Pitre University Hospital, Guadeloupe, France.
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