Malignant Epithelioid Angiomyolipoma in the Kidney and Liver of a Patient with Pulmonary Lymphangioleiomyomatosis: Lack of Response to Sirolimus

Futoshi Higa; Teruhito Uchihara; Syusaku Haranaga; Satomi Yara; Masao Tateyama; Yoshinori Oshiro; Masayuki Shiraishi; Toshio Kumasaka; Kuniaki Seyama; Jiro Fujita

doi:10.2169/internalmedicine.48.2411

CASE REPORTS

Malignant Epithelioid Angiomyolipoma in the Kidney and Liver of a Patient with Pulmonary Lymphangioleiomyomatosis: Lack of Response to Sirolimus

Futoshi Higa, Teruhito Uchihara, Syusaku Haranaga, Satomi Yara, Masao Tateyama, Yoshinori Oshiro, Masayuki Shiraishi, Toshio Kumasaka, Kuniaki Seyama, Jiro Fujita

Author information

Futoshi Higa
Department of Medicine and Therapeutics, Prevention, Control of Infectious Diseases, School of Medicine, University of the Ryukyus
Teruhito Uchihara
Department of Medicine and Therapeutics, Prevention, Control of Infectious Diseases, School of Medicine, University of the Ryukyus
Syusaku Haranaga
Department of Medicine and Therapeutics, Prevention, Control of Infectious Diseases, School of Medicine, University of the Ryukyus
Satomi Yara
Department of Medicine and Therapeutics, Prevention, Control of Infectious Diseases, School of Medicine, University of the Ryukyus
Masao Tateyama
Department of Medicine and Therapeutics, Prevention, Control of Infectious Diseases, School of Medicine, University of the Ryukyus
Yoshinori Oshiro
Department of Urology, School of Medicine, University of the Ryukyus
Masayuki Shiraishi
Department of Pathology, School of Medicine, University of the Ryukyus
Toshio Kumasaka
Department of Pathology, School of Medicine, Juntendo University
Kuniaki Seyama
Department of Respiratory Medicine, School of Medicine, Juntendo University
Jiro Fujita
Department of Medicine and Therapeutics, Prevention, Control of Infectious Diseases, School of Medicine, University of the Ryukyus

Keywords: angiomyolipoma, epithelioid variant, lymphangioleiomyomatosis, sirolimus, gefitinib

JOURNAL OPEN ACCESS

2009 Volume 48 Issue 20 Pages 1821-1825

DOI https://doi.org/10.2169/internalmedicine.48.2411

Details

Abstract

A 26-year-old woman with lymphoangioleiomyomatosis (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.

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