Reclassification of a tubal leiomyosarcoma as an eGIST by molecular evaluation of c-KIT
School of Medicine
Michael V. Seiden
Base Sequence; Diagnosis, Differential; Fallopian Tube Neoplasms; Female; Gastrointestinal Stromal Tumors; Humans; Leiomyosarcoma; Middle Aged; Molecular Sequence Data; Proto-Oncogene Proteins c-kit
Female Urogenital Diseases and Pregnancy Complications | Neoplasms | Obstetrics and Gynecology | Oncology
BACKGROUND: Extragastrointestinal stromal tumors (eGISTs) are rare mesenchymal-derived tumors arising outside of the GI tract. eGISTs are often histologically confused with leiomyosarcoma. Distinction between eGIST and leiomyosarcoma is critical because of the unique responsiveness of eGISTs to the molecularly targeted agent imatinib.
CASE: A woman presented with a history of tubal spindle cell tumor that was initially diagnosed and treated as a leiomyosarcoma. Because of minimal response to sarcoma directed chemotherapy, the possibility that the tumor was in fact an eGIST was investigated and supported by immunohistochemical and mutational analyses of the c-Kit receptor tyrosine kinase. The patient currently has stable disease control on imatinib for the last 18 months.
CONCLUSIONS: The possibility of eGIST should be considered in the differential diagnosis of tumors with a spindle cell morphology in the gynecologic tract especially when involving the ovary, fallopian tube, or uterine serosa.
DOI of Published Version
Gynecol Oncol. 2006 May;101(2):363-6. Epub 2006 Jan 24. Link to article on publisher's site
Foster, Rosemary; Solano, Steven; Mahoney, Jennifer; Fuller, Arlan; Oliva, Esther; and Seiden, Michael V., "Reclassification of a tubal leiomyosarcoma as an eGIST by molecular evaluation of c-KIT" (2006). University of Massachusetts Medical School. Senior Scholars Program. Paper 180.