Retroperitoneal Sclerosing Angiomyolipoma with Long-Term Follow up: A Case Report with Unique Clinicopathologic and Genomic Profile

Liwei Jia, Vandana Panwar, Michelle Parmley, Elena Lucas, Ivan Pedrosa, Payal Kapur

Research output: Contribution to journalArticlepeer-review

Abstract

Sclerosing angiomyolipoma (sAML) is a rare variant of the perivascular epithelioid tumors exhibiting distinct morphology with extensive stromal hyalinization, which makes it challenging to recognize. It often lacks an adipose tissue component and melanocytic markers may be expressed only focally, further posing a diagnostic challenge. Here, we report a case of sAML of the left pararenal retroperitoneum in a 52-year-old woman with 92 months of clinical follow up and discuss the histologic features, immunoprofile, molecular alterations, and differential diagnoses that can aid in the diagnosis of this unique and rare entity.

Original languageEnglish (US)
JournalInternational Journal of Surgical Pathology
DOIs
StateAccepted/In press - 2021

Keywords

  • perivascular epithelioid tumors (PEComas)
  • sclerosing angiomyolipoma
  • transcription factor E3 (TFE3) gene rearrangement

ASJC Scopus subject areas

  • Surgery
  • Anatomy
  • Pathology and Forensic Medicine

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