Renal epithelioid angiomyolipoma with malignant features: Histological evaluation and novel immunohistochemical findings

Sachiko Konosu-Fukaya, Yasuhiro Nakamura, Fumiyoshi Fujishima, Atsuko Kasajima, Keely M. Mcnamara, Yayoi Takahashi, Kensuke Joh, Hideo Saito, Naomasa Ioritani, Yoshihiro Ikeda, Yoichi Arai, Mika Watanabe, Hironobu Sasano

Research output: Contribution to journalArticlepeer-review

14 Citations (Scopus)

Abstract

Renal epithelioid angiomyolipoma (EAML) is a potentially malignant tumor type whose characteristics and biomarkers predictive of malignant behavior have not been elucidated. Here, we report three cases of renal EAML with malignant features but without histories of tuberous sclerosis complex. Case 1 involved a 29-year-old man with a 12-cm solid mass in the right kidney who underwent radical right nephrectomy. Case 2 involved a 22-year-old woman with a retroperitoneal mass who underwent radical right nephrectomy and retroperitoneal tumorectomy. Local recurrence was detected 7 years post-surgery. Case 3 involved a 23-year-old man with a 14-cm solid mass in the left kidney who underwent radical left nephrectomy. Microscopically, the tumors in all cases demonstrated proliferation of epithelioid cells with atypia, mitotic activity, necrosis, hemorrhage, and vascular invasion. Epithelioid cells in all cases were immunohistochemically positive for melanocytic and myoid markers and weakly positive for E-cadherin and β-catenin. Immunohistochemistry revealed activation of the mammalian target of rapamycin pathway. Here, we report the morphological and immunohistochemical features of clinically or histologically malignant renal EAML.

Original languageEnglish
Pages (from-to)133-141
Number of pages9
JournalPathology international
Volume64
Issue number3
DOIs
Publication statusPublished - 2014 Mar

Keywords

  • E-cadherin
  • Epithelioid angiomyolipoma
  • Malignant angiomyolipoma
  • mTOR
  • β-catenin

ASJC Scopus subject areas

  • Pathology and Forensic Medicine

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