Recurrence of Pigmented Epithelioid Angiomyolipoma of the Kidney With Xp11 Translocation: A Case Report

Abstract

This case report pertains to a 70-year-old male patient with a medical history marked by atrial fibrillation, ankylosing spondylitis, and Crohn's disease. Eight years prior, the patient underwent a left radical nephrectomy due to the presence of a pigmented epithelioid angiomyolipoma (PEComa) in the kidney. Notably, pathological examination revealed an unusual subtype of PEComa characterized by Xp11 gene translocation, indicating a more aggressive clinical profile. Following a five-year observation period without recurrence, the patient was discharged. However, eight years after initial treatment, he presented with vague symptoms of left loin discomfort and fullness, which had persisted for several weeks. Subsequent evaluation via computed tomography (CT) scanning showed a small lesion at the site of the renal bed. Surgical resection confirmed the return of the identical tumour. Key clinical points elucidated by this case include the varied behaviour of PEComas, the essential need for prolonged surveillance, and a recognition that recurrences can transpire even after extended disease-free intervals. Prior studies suggest recurrence rates of up to 31.8% for this specific PEComa subtype, emphasising the requirement for prolonged follow-up protocols.

Keywords: adequate follow-up; laproscopic nephrectomy; local excision; renal malignant epithelioid angiomyolipoma; xp11 translocation pecoma.

Figure 1. Coronal image of the CT scan showing the original tumour

The yellow arrow points to the left kidney tumour.

Figure 2. Axial image of the CT scan showing the tumour recurrence site

The yellow arrow points to the left nephrectomy bed recurrence.

Figure 3. Coronal image of the CT scan showing the tumour recurrence site

The yellow arrow points to the left nephrectomy bed recurrence.