Renal angiomyolipoma (report of 2 cases).
BL Jain, DR Mathur, SM Rai, MC Vyas, IN Ramdeo
Department of Pathology, S.N. Medical College, Jodhpur.
B L Jain
Department of Pathology, S.N. Medical College, Jodhpur.
Renal angiomyolipoma, a rare benign renal neoplasm constitutes less than 3 percent of all renal neoplasm. Two rare cases of renal angiomyolipoma were reported from the Department of Pathology, Dr. S.N. Medical College, Jodhpur in last fifteen years. Both were not associated with tuberous sclerosis. The relevant literature on the subject is reviewed.
|How to cite this article:|
Jain B L, Mathur D R, Rai S M, Vyas M C, Ramdeo I N. Renal angiomyolipoma (report of 2 cases). J Postgrad Med 1992;38:46-7,46A
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Jain B L, Mathur D R, Rai S M, Vyas M C, Ramdeo I N. Renal angiomyolipoma (report of 2 cases). J Postgrad Med [serial online] 1992 [cited 2022 Dec 4 ];38:46-7,46A
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Angiomyolipoma is a rare benign tumour characterized by the presence of a variable mixture of blood vessels, smooth muscles and fat. There are two types of renal angiomyolipoma reported in the literature. First one is associated with tuberous sclerosis and another without associated tuberous sclerosis. Two cases of renal angiomyolipoma without tuberous sclerosis have been reported.
Case No 1:
A 35 year old Hindu female presented with a gradually increasing painless swelling in the right lumber region extending to right hypochondrium, right iliac fossa from 6 months but from the last two months the patient also had dull aching pain in the same region and low grade intermittent fever.
Examination revealed pallor and a firm, non-tender lump extending from the right hypochondrium to the right iliac fossa; it was bimanually palpable, with well-outlined margins. Her haemoglobin was 9.0 gm%, rest haematological and biochemical parameters were normal. Blood pressure and renal function tests were also normal. Urine examination showed albuminuria and microscopic haematuria. IVP showed non-functioning right kidney due to tumour mass, and left kidney functioning normally. Clinically the provisional diagnosis was renal cell carcinoma of the right kidney. Right renal nephro-ureterectomy was performed. The resected mass measured 13 x 10 x 8 cm. Outer surface was dark brown and smooth and part of remaining normal kidney was light brown. Cut surface showed yellowish homogenous, slimy rounded tumour arising from upper pole of kidney. The tumour was 9.0 cm in diameter, and it was capsulated producing a bulging smooth mass (see [Figure:1])
Histopathologically it was diagnosed as angiomyolipoma of kidney. Tumour composed of adipose tissue, smooth muscles and blood vessels (See [Figure:2]). The adipose tissue was composed of uniform fat cells with large cytoplasmic vacuoles and small peripheral nucleus. Smooth muscles are haphazardly arranged in interlacing bundles and sheets. The vascular components consisting of large thick walled tortuous blood vessels.
Case No 2:
A 35 year old Hindu female presented with the complaints of pain in abdomen and lump in left lumber region which extended from left hypochondrium to left lumber region, non tender, cystic to firm and bimanually palpable. Urine examination revealed microscopic haematuria. IVIP showed non-functioning left kidney and normal functioning right kidney. Left nephro-ureterectomy performed. Grossly there was enlarged kidney measuring 14 x 10 x 5 cm. Outer surface was nodular, cut surface - renal parenchyma was replaced by multdocular cystic space consisting of stag horn calculus. There was a tumour mass measuring 8 x 5 x 4.5 cm lying on one side of kidney. Histologically diagnosis of renal angiomyolipomas with pyelonephritis was made.
The term renal angiomyolipoma was first used by Morgan et al1 in 1951. It has been generally referred to ahamartoma. Fischer2 (1911) and later on (Critchley et al3 (1932) found that 40-80% of case of renal angiomyolipoma were associated with one or more features of tuberous sclerosis complex i.e. mental retardation, epilepsy, retinal phecomas, adenoma sebeceum, hamartomas of liver, heart or bone, cerebral angioma and cerebral neoplasms.
Hajdu and Foote4 reported that about 90% cases (77 out of 86) of renal angiomyolipoma occurred in patients without tuberous sclerosis. The association of angiomyolipoma with tuberous sclerosis is generally accepted, but it is less appreciated that the same lesion more commonly exists without evidence of tuberous sclerosis.
There are possible two forms of angiomyolipoma reported by many workers5. One form is small, asymptomaic and usually found at the time of autopsy in association with tuberous sclerosis. Another form is a large, symptomatic tumour without association of tuberous sclerosis. Most symptomatic patients complain of flank pain and palpable renal mass and less commonly associated with gross haematuria. There is 100 percent cure after complete nephrectomy. In out both the cases tumour mass was not associated with tuberous sclerosis and both recovered completely after excision.
Morgan GS, Straumflord JV, Hall EJ. Angiomyolipoma of the kidney. J Urol 1951; 65:525-527.|
|2||Fisher W. Dienierenturomorenbei der Tuberosen Hirsklerose Zeigler Beite. Z Path Anat UZ allg Path 1911; 50:235-282.|
|3||Critchley M, Earl CX. Tuberous sclerosis and allied conditions. Brain 1932; 55:311-346.|
|4||Hajdu SI, Foote FW Jr. Angiomyoliporna of the kidney. Report of 27 cases and review of the literature J Urol 1969; 102:396-401.|
|5||Farrow GM, Harrison EG Jr, Utz DC, Jones DR. Renal angiornyoliporna. A clinicopathological study of 32 cases. Cancer 1968; 22:654-670.