title: Malignant epithelioid angiomyolipoma in the kidney and liver of a patient with pulmonary lymphangioleiomyomatosis: lack of response to sirolimus.
abstract: A 26-year-old woman with lymphangioleiomyomatosis (LAM) was hospitalized for the surgical excision of a giant abdominal tumor of right kidney origin. The pathological diagnosis of the tumor was conventional angiomyolipoma (AML). After 8 months, 2 liver tumors appeared and grew rapidly. The tumors were resected, and the pathological finding of these tumors was epithelioid AML. Thereafter, metastatic multiple lung tumors appeared, and there was local recurrence of the liver tumors. Sirolimus, an mTOR protein inhibitor, was used to treat epithelioid AML. However, the drug did not inhibit the rapid growth of the tumor at all. This finding suggests that sirolimus might not be effective against epithelioid AML, and in such cases, complete surgical resection should be the treatment of choice.
authors.affiliation: Department of Medicine and Therapeutics, Prevention, Control of Infectious Diseases, School of Medicine, University of the Ryukyus, Okinawa. fhiga@med.u-ryukyu.ac.jp
authors.forename: Futoshi
authors.initials: F
authors.lastname: Higa
authors.forename: Teruhito
authors.initials: T
authors.lastname: Uchihara
authors.forename: Syusaku
authors.initials: S
authors.lastname: Haranaga
authors.forename: Satomi
authors.initials: S
authors.lastname: Yara
authors.forename: Masao
authors.initials: M
authors.lastname: Tateyama
authors.forename: Yoshinori
authors.initials: Y
authors.lastname: Oshiro
authors.forename: Masayuki
authors.initials: M
authors.lastname: Shiraishi
authors.forename: Toshio
authors.initials: T
authors.lastname: Kumasaka
authors.forename: Kuniaki
authors.initials: K
authors.lastname: Seyama
authors.forename: Jiro
authors.initials: J
authors.lastname: Fujita
mesh_terms: D000328:Adult; D018207:Angiomyolipoma / Q000150:complications / Q000175:diagnosis* / Q000188:drug therapy; D017809:Fatal Outcome; D005260:Female; D006801:Humans; D007680:Kidney Neoplasms / Q000150:complications / Q000175:diagnosis* / Q000188:drug therapy; D008113:Liver Neoplasms / Q000150:complications / Q000175:diagnosis* / Q000188:drug therapy; D008175:Lung Neoplasms / Q000150:complications / Q000175:diagnosis* / Q000188:drug therapy; D018192:Lymphangioleiomyomatosis / Q000150:complications / Q000175:diagnosis* / Q000188:drug therapy; D009364:Neoplasm Recurrence, Local / Q000150:complications / Q000175:diagnosis / Q000188:drug therapy; D020123:Sirolimus / Q000627:therapeutic use*
chemical_list: D020123:Sirolimus