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Lack of response of a metastatic renal perivascular epithelial cell tumor (PEComa) to successive courses of DTIC based‐therapy and imatinib mesylate
Author(s) -
Rigby Heather,
Yu Weiming,
Schmidt Matthias H.,
Fernandez Conrad V.
Publication year - 2005
Publication title -
pediatric blood and cancer
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.116
H-Index - 105
eISSN - 1545-5017
pISSN - 1545-5009
DOI - 10.1002/pbc.20305
Subject(s) - medicine , perivascular epithelioid cell , dacarbazine , imatinib mesylate , imatinib , tyrosine kinase inhibitor , discontinuation , regimen , clear cell sarcoma , pdgfra , pathology , oncology , chemotherapy , epithelioid cell , sarcoma , cancer , immunohistochemistry , gist , myeloid leukemia , stromal cell
An 11 year‐old girl presented with two large abdominal masses in the left flank and epigastrium and left supraclavicular lymphadenopathy. Subsequent investigations led to the diagnosis of metastatic perivascular epithelioid cell tumor (PEComa) arising from the left kidney. Effective treatment for this rare tumor is not yet known. The tumor did not respond to an initial treatment of two cycles of a dacarbazine (DTIC) based regimen. She was placed on a trial of imatinib mesylate based on tumor expression of c‐KIT, a tyrosine kinase targeted by this drug. This report highlights the first documented case of the use of imatinib for PEComa. Lack of response and adverse effects of the drug required discontinuation of therapy. © 2005 Wiley‐Liss, Inc.