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

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

Thu Dang Anh Phan  1 , Nhi Thuy To  2 , Diem Thi Nhu Pham  1
Affiliations
Case Reports

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

Thu Dang Anh Phan et al. Fetal Pediatr Pathol. 2023 Apr.

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.

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