Malignant Pigmented Epithelioid Angiomyolipoma of the Kidney in a Child with Tuberous Sclerosis Complex

Fetal Pediatr Pathol. 2023 Apr;42(2):285-290. doi: 10.1080/15513815.2022.2088911. Epub 2022 Jun 23.

Abstract

Introduction: Pigmented epithelioid angiomyolipoma is a variant of epithelioid angiomyolipoma (EAML) that has not previously been described in children with tuberous sclerosis.

Case presentation: A 15-year-old boy with tuberous sclerosis had a rapidly enlarging renal mass associated with a left lung nodule. Microscopically it was a pigmented EAML, confirmed by immunohistochemistry.

Discussion/conclusion: The pigmented variant of EAML can arise and metastasize from the kidney of a teenager with tuberous sclerosis.

Keywords: Pigmented; epithelioid angiomyolipoma; kidney; perivascular epithelioid cell neoplasms; tuberous sclerosis complex.

Publication types

  • Case Reports

MeSH terms

  • Adolescent
  • Angiomyolipoma* / complications
  • Angiomyolipoma* / diagnosis
  • Angiomyolipoma* / pathology
  • Child
  • Epithelioid Cells / pathology
  • Humans
  • Kidney / pathology
  • Kidney Neoplasms* / complications
  • Kidney Neoplasms* / diagnosis
  • Kidney Neoplasms* / pathology
  • Male
  • Tuberous Sclerosis* / complications
  • Tuberous Sclerosis* / diagnosis
  • Tuberous Sclerosis* / pathology