title: Dramatic tumor response to everolimus for malignant epithelioid angiomyolipoma.
abstract: Malignant epithelioid variant of angiomyolipoma has aggressive characteristics, against which conventional cytotoxic agents have been reported to be disappointingly inactive, and the prognosis of unresectable or recurrent disease is dismal poor. A 52-year-old man with a history of left nephrectomy for epithelioid angiomyolipoma was referred to our institution. The computed tomographic scan showed a soft tissue dense mass around the Rex's recess and behind the spleen, and a large pelvic mass. Specimens obtained by percutaneous needle biopsy confirmed the recurrence of malignant epithelioid angiomyolipoma. Everolimus was initiated at 10 mg per day for recurrent disease. Computed tomographic scans 2 months later showed the tumors to be markedly decreased in size. The patient has continued with this treatment on an outpatient basis without signs of disease progression over 7 months, as of February 2011. In this case, treatment with everolimus resulted in dramatic tumor response for the malignant epithelioid variant of angiomyolipoma.
authors.affiliation: Department of Clinical Oncology, Aichi Cancer Center Hospital, 1-1 Kanokoden, Chikusa-ku, Nagoya 464-8681, Aichi, Japan. kouheis0824@yahoo.co.jp
authors.forename: Kohei
authors.initials: K
authors.lastname: Shitara
authors.forename: Yasushi
authors.initials: Y
authors.lastname: Yatabe
authors.forename: Ayako
authors.initials: A
authors.lastname: Mizota
authors.forename: Tsuyoshi
authors.initials: T
authors.lastname: Sano
authors.forename: Yuji
authors.initials: Y
authors.lastname: Nimura
authors.forename: Kei
authors.initials: K
authors.lastname: Muro
mesh_terms: D000284:Administration, Oral; D018207:Angiomyolipoma / Q000175:diagnosis* / Q000188:drug therapy* / Q000473:pathology; D000970:Antineoplastic Agents / Q000008:administration & dosage / Q000494:pharmacology / Q000627:therapeutic use*; D015622:Epithelioid Cells / Q000473:pathology*; D000068338:Everolimus; D006801:Humans; D007166:Immunosuppressive Agents / Q000627:therapeutic use; D007680:Kidney Neoplasms / Q000473:pathology / Q000601:surgery*; D008297:Male; D008875:Middle Aged; D009392:Nephrectomy; D049268:Positron-Emission Tomography; D012008:Recurrence; D020123:Sirolimus / Q000008:administration & dosage / Q000031:analogs & derivatives* / Q000494:pharmacology / Q000627:therapeutic use; D058570:TOR Serine-Threonine Kinases / Q000037:antagonists & inhibitors*; D014057:Tomography, X-Ray Computed
chemical_list: D000970:Antineoplastic Agents; D007166:Immunosuppressive Agents; D000068338:Everolimus; C546842:MTOR protein, human; D058570:TOR Serine-Threonine Kinases; D020123:Sirolimus