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

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
• perivascular epithelioid cell neoplasms
• tuberous sclerosis complex
• kidney

Acknowledgements

We would like to express our deep gratitude to Dr. Kenneth Chang, KK Women’s and Children’s Hospital for providing molecular tests for the patient in this research.

Funding

The author(s) reported there is no funding associated with the work featured in this article.