Renal Epithelioid Angiomyolipoma in Children

Dhruv Mahajan; Vishesh Jain; Sandeep Agarwala; Manisha Jana; Prashant P Ramteke

doi:10.15586/jkcvhl.v8i2.178

Dhruv Mahajan

Departments of Pediatric Surgery, All India Institute of Medical Sciences, Ansari Nagar, New Delhi, India.

Vishesh Jain

Departments of Pediatric Surgery, All India Institute of Medical Sciences, Ansari Nagar, New Delhi, India.

Sandeep Agarwala

Departments of Pediatric Surgery, All India Institute of Medical Sciences, Ansari Nagar, New Delhi, India.

Manisha Jana

Departments of Radiology, All India Institute of Medical Sciences, Ansari Nagar, New Delhi, India.

Prashant P Ramteke

Departments of Pathology, All India Institute of Medical Sciences, Ansari Nagar, New Delhi, India.

Keywords

angiomyolipoma, kidney tumor, pediatric cancer, renal epithelioid angiomyolipoma, tuberous sclerosis

Abstract

Renal angiomyolipoma is a rare cause of renal tumor in children. Most are associated with tuberous sclerosis, and the classic type is observed more commonly. Epithelioid angiomyolipoma is even rarer with only limited case reports and series published in literature, most of which are of adult patients. We describe a 12-year-old boy, a diagnosed patient of tuberous sclerosis, who presented with pain in the left flank. On evaluation, it was found to have a left renal mass with the clinical picture suggestive of renal cell carcinoma. Partial nephrectomy was performed and histopathology revealed epithelioid angiomyolipoma. The child was asymptomatic at follow-up after 3 months. Only a few such cases in children are found in literature, which are discussed alongside. Differential diagnosis of this rare tumor must be kept in mind in a renal tumor as surgery is generally curative in this possibly malignant tumor. Metastasis confers a poor prognosis. Chemotherapy is generally not effective, although various regimens have been tried. Tumor recurrence must be kept in mind and a follow-up after apparent complete remission is of paramount importance.

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