A rare giant renal angiomyolipoma in a 17-year-old male: A case report

Abstract

Renal angiomyolipoma is an uncommon benign tumor that mostly affects adults and is highly associated with tuberous sclerosis. It’s very uncommon for it to affect adolescents. In this case, a 17-year-old male with abdominal pain and distension was diagnosed with sporadic giant renal angiomyolipoma. Histopathology confirmed the diagnosis after a successful nephrectomy. Though rare in teenagers, giant renal angiomyolipoma can be managed with surgery. Histopathology is a mainstay of confirming the diagnosis.

Conclusion

This case highlights the difficulties in managing big retroperitoneal tumors in teenagers and the necessity of taking sporadic angiomyolipoma into account when making a differential diagnosis.

Highlights

•

Bening renal mass.
•

Renal angiomyolipoma are uncommon.
•

Giant sporadic Renal Angiomyolipoma is very rare in adolescent and paediatric group.
•

Partial or total nephrectomy seems to be a reasonable option to treat sporadic giant renal angiomyolipoma.

1 Introduction

Rare giant mesenchymal tumors with variable ratios of smooth muscle cells, blood vessels, and adipose tissue make up renal angiomyolipoma. . There are very few occurrences recorded in pediatric and adolescent populations; they are usually seen in adults from the second decade of life and are frequently linked to tuberous sclerosis complex, however 80 % of case are sporadic and usually affect adult group , . Tumors bigger than 10 cm in diameter are known as giant renal angiomyolipoma, they are rare and present serious management challenges because of the possibility of consequences like hemorrhage and rupture. . We report a rare instance of a 17-year-old male with a giant renal angiomyolipoma.

2 Case presentation

A case of 17 years old male presented with a 4 years history of gradual painless hard surfaced and immobile abdominal mass which began solely on his left upper quadrant progressing further towards the midline associated with early satiety, loss of appetite and occasionally, non-projectile vomiting of food contents no history of constipation no blood in stool, no history of hematuria or change in urination habit, not epileptic and no history of mental illness reported, no history of the same condition in the family.

General examination: alert, not pale, not jaundice, slight dyspneic, no lymphadenopathy.

The vital signs were essentially within a normal range.

Per Abdominal Examination: massive distended abdomen with palpable mass on the left lumbar region which extends towards the left hypochondriac region, immobile and mildly tender on palpation, it was firm on consistency irregular in shape and does not cross the midline. Deep palpation was difficult due to the distention but there were no signs of hepatomegaly nor splenomegaly.

Laboratory results: urinalysis microscopic hematuria, CBC = Hg 11g/dl other parameters essential were normal, creatinine 112g/dl, BUN 4g/dl.

Abdominal CT-Scan: reviled a well-defined solid heterogeneous retroperitoneal mass originated from the left kidney containing markedly hypodense area with some necrotic components. no signs of local invasion to the near structures, hence strongly suggested angiomyolipoma ( Fig. 1 ).

Diagnosis of renal angiomyolipoma was reached based on the history, clinical findings and abdominal CT-Scan with contrast, hence laparotomy was done, where a huge well capsulated mass measures (32×27 × 19cm) and weigh 4.8kg, originating from the left kidney was found with no invasion to nearby structure and no mesenteric lymph node identified, trans peritoneal left nephrectomy was done with total mass excision with minimal bleeding ( Fig. 2 .), drainage inserted and abdomen was closed. The whole mass was sent for histopathology. Patient was sent to general ward on day three the drain was removed and discharged home, follow up was done for six months (creatinine monitoring and urine output) on outpatient clinic with no any significant complication reported.