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PEComa of the colon resistant to sirolimus but responsive to doxorubicin/ifosfamide

PEComa of the colon resistant to sirolimus but responsive to doxorubicin/ifosfamide

作     者:Wolfgang Scheppach Nikolaus Reissmann Thomas Sprinz Ekkehard Schippers Bjoern Schoettker Justus G Mueller 

作者机构:Department of MedicineJuliusspital WuerzburgD-97070 WuerzburgGermany Department of SurgeryJuliusspital WuerzburgD-97070 WuerzburgGermany Onkologische SchwerpunktpraxisD-97070 WuerzburgGermany Department of PathologyUniversity of Wuerzburg97070 WürzburgGermany 

出 版 物:《World Journal of Gastroenterology》 (世界胃肠病学杂志(英文版))

年 卷 期:2013年第19卷第10期

页      面:1657-1660页

核心收录:

学科分类:1002[医学-临床医学] 100214[医学-肿瘤学] 10[医学] 

主  题:Perivascular epithelioid cell tumor Colon Liver metastases Mammalian target of rapamycin inhibitor Sirolimus Chemotherapy Doxorubicin Ifosfamide 

摘      要:A 23-year-old male presented with a three-week-history of crampy abdominal pain and *** revealed a friable mass filling the entire lumen of the cecum;histologically,it was classified as perivascular epithelioid cell tumor(PEComa).An magnetic resonance imaging scan showed,in addition to the primary tumor,two large mesenteric lymph node metastases and four metastatic lesions in the *** patient underwent right hemicolectomy and left hemihepatectomy combined with wedge resections of metastases in the right lobe of the liver,the resection status was ***,the patient was treated with *** 4 mo of adjuvant mammalian target of rapamycin inhibition he developed two new liver metastases and a local pelvic *** visible tumor formations were again excised surgically,this time the resection status was R2 with regard to the pelvic *** patient was treated with 12 cycles of doxorubicin and ifosfamide under which the disease was stable for 9 *** clinical course was then determined by rapid tumor growth in the pelvic *** line chemotherapy with gemcitabine and docetaxel was ineffective,and the patient died 23 mo after the onset of *** case report adds evidence that,in malignant PEComa,the mainstay of treatment is curative *** not achievable,the effects of adjuvant or palliative chemotherapy are unpredictable.

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