A Rare Case Of Kidney Tumours

ADRENAL ANGIOMYOLIPOMA : A RARE CASE OF KIDNEY TUMOURS

Abstract : Adrenal angiomyolipoma is a rare entity . Only four cases have been reported to the best of our knowledge till date. We report a 25 years old female who presented with epigastric pain. A computerized tomography (CT) scan showed right adrenal mass with features of angiomyolipoma as suggested by soft tissue density right adrenal mass with fatty component with HU value -125 to -42 HU which is diagnostic. FNAC was done to confirm the diagnosis of adrenal angiomyolipoma. The patient was kept under observation as the size of the tumour was less than 4 cm.

Introduction : Angiomyolipoma is a benign mesenchymal tumor consisting of varying amounts of mature adipose tissue, smooth muscle and thick walled blood vessels. It arises from perivascular epitheliod cells and is commonly seen in the kidney. The extrarenal sites reported include the bone, colon, heart, lung, parotid gland, skin, spermatic cord, gynecologic organs and retroperitoneum with the most common extrarenal site being the liver.

Case Study : A 25-years old female presented with epigastric pain for which she underwent a routine USG abdomen which revealed a right adrenal mass. CT Scan of the whole abdomen was performed for further evaluation. There was no evidence of tuberous sclerosis. Computerized tomography (CT) scan showed a well defined soft tissue density, right adrenal mass with fat attenuation with HU value -125 to -42 HU less than 4 cm without evidence of calcification and haemorrhage. Serum catecholamine, cortisol and urinary VMA were within normal limits. As the symptoms presented by the patient are not related to the findings, there is no haemorrhage present and the mass is less than 4cm, the patient is being kept under observation. A USG abdomen will be performed every three months.

Discussion : Angiomyolipoma is a benign hamatomatous tumor. About half of the angiomyolipomas are associated with tuberous sclerosis and in these cases, they are usually multiple and bilateral. It has been estimated that approximately 80% of the severe and complete forms of tuberous sclerosis have angiomyolipoma. In the previous reported cases; two were associated with tuberous sclerosis, while the other two were sporadic. On CT scan, the presence of even a small amount of fat as evidenced by HU less than 10, suggests the diagnosis of angiomyolipoma. Attenuation beyond - 30 HU is almost diagnostic of angiomyolipoma which is seen in our case. On magnetic resonance imaging (MRI), the typical features of the fatty component include bright signal intensity on non-fat suppressed images, with dropout of signal on fat suppressed images. However, these features can also be seen with other lipomatous tumors (lipoma, liposarcoma, teratoma or myelolipoma) and are not specific for angiomyolipoma. The size of the tumor, despite the clear fat plane between it and the adjacent structures, could not rule out the malignancy. Grossly, angiomyolipomas appear well circumscribed and depending on the relative amount of adipose tissue, they range from a glistening yellow (''fatty'') appearance to a more white-tan and firm appearance depending on the relative amount of adipose tissue. Histology typically shows a variable mixture of mature adipocytes, thick-walled blood vessels and spindled and epithelioid stromal cells often radiating out from blood vessel walls. Most angiomyolipomas show predominance of adipocytes but some contain mainly spindled stromal cells and thick walled vessels with little adipose tissue. A diagnostically helpful feature is the staining of the stromal cells for HMB-45 by immunohistochemistry. Management should be the same as that for any adrenal mass. Assessment of functional status of the tumor should be done although all adrenal angiomyolipomas reported so far were nonfunctional. Surgery is indicated if the patient is symptomatic or the tumor is more than 6 cm since the risk of malignancy increases with size. Also, the risk of spontaneous rupture increases with size, owing to the presence of abundant and abnormal elastin-poor vascularity in the tumor. Laparoscopic adrenalectomy is an option and had been successfully done for a 6 cm adrenal angiomyolipoma. Since it is a benign disease, its prognosis is good. Currently, there is no universally agreed protocol on follow-up but an ultrasound three to six months following the surgery with annual clinical examination for large tumors is recommended.

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