|Year : 1983 | Volume
| Issue : 2 | Page : 120-122A
Primary malignant haemangio-pericytoma of kidney (a case report).
AA Mondal, SS Choudhury, PK Mukherjee
A A Mondal
|How to cite this article:|
Mondal A A, Choudhury S S, Mukherjee P K. Primary malignant haemangio-pericytoma of kidney (a case report). J Postgrad Med 1983;29:120-122A
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Mondal A A, Choudhury S S, Mukherjee P K. Primary malignant haemangio-pericytoma of kidney (a case report). J Postgrad Med [serial online] 1983 [cited 2022 May 26 ];29:120-122A
Available from: https://www.jpgmonline.com/text.asp?1983/29/2/120/5538
Hemangiopericytoma is an uncommon tumour of the somatic soft tissues. Stout and Murray in 1942 originally suggested the name "Hemangiopericytoma" for a rare, richly vascular tumour, composed of capillaries surrounded by cells, described by Zimmerman as pericytes. The primary location varies and these tumours can occur almost anywhere in the body. Although hemangiopericytoma of retroperitoneum is relatively uncommon among retroperitoneal sarcomas, it arising in retroperitoneum represents 25% of total in any reported series. Renal and perirenal origin has been documented in only ten cases in the world literature. In India only one case has been reported by Sanyal et al in 1976. Black and Heineman first reported a case of renal hemangiopericytoma in 1955 while the maximum number is reported by Farrow et al who described 5 such cases. This case is being reported for its rarity in literature and the mode of involvement of kidney seems to place this case as the 3rd case in the world literature.
S.K., a 50 year old male, was admitted in the Medical College and Hospital on 16-6-1981 with a history of discomfort in the right flank for 3 months, a palpable mass in the same region and passage of smoky urine for one month's duration. There was no past history of any renal colic. There were no bowel disturbances.
Examination revealed a man of moderate build. He was neither anaemic nor jaundiced. There was no peripheral lymphadenopathy or bone tenderness. Abdominal examination revealed a firm, non-tender mass of 20 cm x 12 cm in size, occupying the right hypochondrium and right lumbar regions. It was ballotable. The right renal angle was full. The liver and spleen were not enlarged. External genitalia were normal.
Routine haematological investigations, blood urea and serum creatinine were within normal limits. There was microscopical haematuria. X-ray of the chest was normal. Straight X-ray of abdomen showed a large mass occupying the right hypochondrium and the lumbar region. Intravenous pyelography revealed a non-functioning right kidney. Left kidney showed normal structural pattern and function. Cystoscopy showed no abnormality of bladder. A diagnosis of right renal tumour was made. An explorative laparotomy was done on 23-6-1981 followed by right radical nephrectomy. The whole of right kidney was hugely enlarged and its capsule was infiltrated by the tumour but it had invaded no adjacent organs. No enlarged regional lymph nodes were found.
The nephrectomy specimen measured 18 cm x 10 cm x 6 cm and weighed 985 g. The external surface was brown and smooth. The tumour had invaded the capsule. The cut section showed that almost whole of the right kidney was replaced by the tumour. A rim of renal tissue could be identified. The colour of the tumour varied from greyish white to reddish brown and yellow with focal areas of necrosis and haemorrhages. The tumour was multinodular and firm in consistency. The renal vein and pelvis were free from the tumour [Fig. 1]. Multiple sections were taken from the tumour.
The tumour was highly cellular. It was composed of many chink like vascular channels lined by a single layer of endothelial cells. These vascular channels were separated by solid aggregates of rounded to fusiform tumour cells. These cells were oriented around the vessels as whorls or collarettes. The cells had pale cytoplasm, indistinct cellular membranes and prominent, round to oval, vesicular nuclei. There were scattered mitotic figures all over the tumour [Fig. 2]. Although it was very much evident that the tumour was malignant haemangiopericytoma, reticulin stain was done which showed well defined outlines of individual vessels, lined by a single layer of endothelial cells. The tumour cells were perivascular. The reticulin fibres radiating from the vessel wall enclosed the tumour cells individually and in groups. So a diagnosis of primary malignant hemangiopericytoma was made.
The patient was advised to attend the Out Patients' Department but he did not turn up.
Hemangiopericytoma may occur wherever capillaries are found but the kidney is one of the least common sites. The tumour occurs at any age and equally in both sexes. There are no characteristic signs or symptoms. The discovery of a lump is usually the initial complaint. These tumours tend to show insidious growth with or without involvement of the surrounding tissue. The majority are benign but 20% of cases reveal malignancy. In the latter, growth and invasion are rapid and metastasis is prompt through haematogenous route. The grade of malignancy is higher in the larger deep seated tumours developing in the mediastinum and in the lower extremities.
The majority of renal hemangiopericytomas reported to date have been firmly attached to the external surface of the kidney, apparently arising from the renal capsule but not invading the renal parenchyma. It has been suggested that there is some factor inherent in the ranal capsule which can produce such tumour. But the peculiarity of our case was that almost whole of the right kidney was replaced by the tumour. This is similar to only 2 cases reported by Farrow et al who showed invasion of renal parenchyma by the tumour. As with hemangiopericytoma elsewhere in the body, outcome is difficult to predict. The histological picture is a poor indicator of the aggressive behaviour of the tumour. Some show aggressive growth without known metastases.
Complete surgical excision is the treatment of choice, regardless of their location due to the malignant potentiality of the tumour, the high incidence of local recurrence and the unpredictable behaviour of the tumour. In the cases reported, nephrectomy had been the only form of initial treatment. Lymph node dissection has little to offer in these tumours as metastases are more frequently haematogenous than lymphogenous. However, there is no experience in lymph node dissection in patients with renal hemangiopericytoma. The role of radiotherapy and chemotherapy is still on trial.
We are grateful to the Principal, Medical College and Hospital, Calcutta for his kind permission to publish this case.
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