Multimodal Treatment Using Surgery, Radiotherapy, and Chemotherapy in a Patient With a Perivascular Epithelioid Cell Tumor of the Uterus
- 1 December 2005
- journal article
- case report
- Published by Ovid Technologies (Wolters Kluwer Health) in Journal of Pediatric Hematology/Oncology
- Vol. 27 (12), 681-684
- https://doi.org/10.1097/01.mph.0000193475.06870.d5
Abstract
Perivascular epithelioid tumor (PEComa), a recently defined tumor, is a very rare disease affecting various organs, most often the uterus. This tumor displays a variety of histologic and clinical features and at this point is regarded as a tumor with uncertain malignant potential. A 9-year-old girl with abdominal pain and vaginal spotting was diagnosed with PEComa of the uterus with metastasis. She received chemotherapy comprising vincristine, ifosfamide, and doxorubicin, as well as radiotherapy after surgery. After this multimodal treatment, there was no evidence of recurrence or further metastasis. She remains disease-free 1.5 years after her initial diagnosis. PEComa of the uterus displaying malignant characteristics may have a more favorable response to more aggressive therapy.Keywords
This publication has 14 references indexed in Scilit:
- Lack of response of a metastatic renal perivascular epithelial cell tumor (PEComa) to successive courses of DTIC based-therapy and imatinib mesylatePediatric Blood & Cancer, 2005
- Perivascular epithelioid cell (PEC) tumors of the uterus: a clinicopathologic study of two cases with aggressive featuresLaboratory Investigation, 2005
- Perivascular epithelioid cell tumor (PEComa) with perirenal manifestationInternational Journal of Urology, 2005
- Perivascular Epithelioid Cell Tumor of the Oral MucosaArchives of Pathology & Laboratory Medicine, 2005
- Perivascular Epithelioid Cell Tumor of the Uterus: A Case ReportInternational Journal of Gynecological Pathology, 2004
- Perivascular epithelioid cell tumor of the uterus: Immunohistochemical, ultrastructural and molecular studyPathology International, 2003
- Late pulmonary metastasis in uterine PEComaJournal of Clinical Pathology, 2003
- Perivascular Epithelioid Cell Tumor (`PEComa') of the UterusThe American Journal of Surgical Pathology, 2002
- Abdominopelvic Sarcoma of Perivascular Epithelioid Cells. Report of Four Cases in Young Women, One with Tuberous SclerosisLaboratory Investigation, 2001
- PEC and SugarThe American Journal of Surgical Pathology, 1992