| Abstract|| |
A 77 year old Saudi male admitted to the surgical unit of our hospital because of a left renal mass. A diagnosis of left renal carcinoma was subsequently made and a left nephrectomy was performed. Histological examination of the nephrectomy specimen showed a renal cell carcinoma containing a large sarcomatoid component. In addition, numerous schistosoma ova, some of them calcified, were identified within the carcinomatous part of the tumor. To the best of knowledge, this is the first report of simultaneous renal schistosomiasis and sarcbmatoid renal cell carcinoma.
Keywords: Renal cell carcinoma, Sarcomatoid, Schistosomiasis.
|How to cite this article:|
Arafah MI. Sarcomatoid Renal Cell Carcinoma Associated with Renal Schistosomiasis: A Case Report and Review of Literature. Saudi J Kidney Dis Transpl 1996;7:305-8
|How to cite this URL:|
Arafah MI. Sarcomatoid Renal Cell Carcinoma Associated with Renal Schistosomiasis: A Case Report and Review of Literature. Saudi J Kidney Dis Transpl [serial online] 1996 [cited 2013 May 23];7:305-8. Available from: http://www.sjkdt.org/text.asp?1996/7/3/305/39495
| Introduction|| |
Sarcomatoid renal cell carcinoma is an uncommon variant of renal cell carcinoma (RCC) characterized microscopically by intimate association of typical RCC with a sarcomatoid component  . It appears to be a distinct clinicopathological entity in view of aggressive behavior with a potential for distant metastases and poor prognosis. The reported incidence varies from 0.1 % to 10% of all RCC ,,, . The association of schistosomiasis with urinary bladder neoplasms is well known. Such association with renal cell carcinoma is, however, extremely rare with only one case reported in the literature  .
| Case Report|| |
A 77 year old Saudi farmer from Al Taif area presented to King Faisal Specialist -hospital in November 1993 with a seven year history of left loin pain which increased in severity over the two months prior to admission. On physicai examination, the patient appeared weak and a large nontender mass was felt upper abdomen. Rectal examination revealed a slightly enlarged prostate which appered clinically benign. There were no clinical abnormalities in the caediovascular or respitatory system. His hemoglobin was slightly low at 110 g/L ; creatinine was 109 µmol/L, serum urea and electrolytes were in the normal range. The liver function tests revealed albumin of 25 g/ L, alkaline phosphatase: 225 U/L, alanine ammotransferase: 41 U/L, aspartate amino transferase: 24 U/L, total billirubin:57/µmol/L and total protein: 52 g/L. Urine examination was unremarkable. Abdominal computed tomography (CT) scan showed a 2 cm stone in the left renal pelvis causing marked hydronephrosis to the extent that the left kidney appeared as a fluid-filled sac with virtually no functioning tissue. Inside this kidney there were areas of higher density which could be due to hemorrhage associated with hydronephrosis, but the possibility of a tumor could not be ruled out. Bone scan revealed no evidence of metatasis to bone. Chest x-ray was normal. The urinary bladder was not evaluated.
This patient was treated by radical left nephrectomy and the specimen was sent for histopathological examination. The postoperative period was uneventful and the patient was discharged after ten days from admission. The patient did not comply with follow-up appointments. Upon inquiry from the family, the patient remained well for about one year after surgery; then he was admitted to another hospital with a diagnosis of acute abdomen and expired 20 days after admission. No autopsy was performed.
| Pathology Findings|| |
The left kidney was markedly distended, measuring 22x18x8 cm and weighing 2,392 grams. The outer surface was irregular. On sectioning, a partially necrotic and hemorrhagic mass measuring 19x13x8 cm was seen occupying most of the kidney. The surrounding renal tissue showed markedly dilated calyces and renal pelvis with atrophic renal parenchyma. There was a 2 cm oval stone in the renal pelvis. Microscopic examination of cells with frequent mitotic figures. H & E stain x 100 the mass showed features of renal cell carcinoma with mixed trabecular and papillary patterns [Figure - 1]. The majority of the neoplastic cells had basophilic granular cytoplasm. Cells with clear cytoplasm were present in focal areas. In addition to the described features, there were areas with pleomorphic malignant spindle, polygonal, and giant cells. These cells were arranged irregularly in interlacing fascicles. The morphological pattern was indistinguishable fo-cally from malignant fibrous histiocytoma. Other areas showed malignant spindle cells with monomorphic proliferation resembling a fibrosarcoma [Figure - 2]. A focal hemangiopericytoma like pattern was also identified. Carcinoma and sarcoma components were roughly equal. Multiple schistosoma ova were present in the carcinomatous component [Figure - 3] and in the necrotic areas. These ova were surrounded by foreign body type giant cells with focal fibrosis. The neoplasm was extending beyond the renal capsule. The surgical resection margin was, however, free with no evidence of renal blood vessels involvement. The kidney parenchyma showed marked fibrosis, chronic inflammation with almost total loss of renal tissue. The renal pelvis revealed surface ulceration with inflammation and fibrosis. No schistosoma ova were, however, detected in the renal pelvis or in the ureter. One of the paraaortic lymph nodes contained deposits of renal cell carcinoma.
Immunohistochemical studies revealed positivity for cytokeratin and vimentin in the carcinomatous area. The sarcomatous areas showed, however, weak positivity for vimentin only. Cytogenetic studies were not performed.
| Discussion|| |
Schistosomiasis, also known as bilharziasis, is caused by infection with parasitic trematodes that colonize venous circulation.
There are more than 200 million persons infected with schistosoma worldwide, which causes as many as 200 thousand deaths annually  . The distribution of infection intensity is not uniform; most infected individuals (50%-75%) carry light infection, while a small percentage (5-15%) carry extremely heavy infection.
Schistosomiasis caused by S. hematobium and 5. mansoni are endemic in Saudi Arabia  . The prevalence of the disease varies from one area to another; it is upto 90% in settled populations in Aljouf, Skakah and in the South, 5% in northern Hijaz and Najd and none in the Eastern province. This variation in prevalence is due to irregular distribution of the snail vectors in different regions  . The prevalence of schistosomiasis in Al Taif region is 0.2% for S1. hematobium and 7.7% for S. mansoni  .
In areas endemic for schistosomiasis, there is an increased incidence of certain types of carcinoma. For example; in areas endemic for S. hematobium, squamous cell carcinoma of the urinary bladder is more frequent than transitional cell carcinoma  . In addition, bladder cancer is encountered at younger age in patients with schistosoma egg-positive cases than those of egg-negative cases  .
The actual mechanism of carcinogenesis associated with chronic schistosomiasis is unclear. Experimental studies indicate, however, that carcinogeneic nitrosamines produced by chronic bacterial infection together with chronic irritation caused by schistosoma hematobium eggs may induce bladder cancer. Also, high rate of P53 gene mutation (86%) was found in cases with squamous cell carcinoma of the bladder with schistosomiasis  .
The kidney is one of the many organs which may be involved by schistosomiasis. The lesions described were nephritis, nephrotic syndrome, hydronephrosis and amyloidosis.
In addition, direct infestation of the kidney by schistosomal eggs has also been described.
The exact pathogenesis of renal cell carcinoma is unknown. There are several medical and genetic conditions associated with RCC. Examples of these are von Hippel-lindau's disease, hereditary form of renal cell carcinoma, adult form of polycystic kidney disease and acquired cystic kidney disease in patients on chronic hemodialysis ,,, . Suvarna, et al has reported a case of sarcomatoid renal cell carcinoma arising in hemodialysis associated acquired cystic kidney disease  .
There are several epidemiologic studies to define the environmental and occupational risk factors associated with renal cell carcinoma. The risk factors implicated are tobacco, cadmium, arsenic, obesity, meat consumption, regular use of phenacetin, thorotrast contrast used in the 1920s, working with coke-ovens in steel plants and exposure to asbestos , . Other occupational risk factors were found to be associated with employment as a truck driver, exposure to gasoline, other hydrocarbons and insecticides and pesticides  .
The current data on the carcinogenesis of renal cell carcinoma indicate that the cells of the proximal tubule undergo environmental or genetic insult, leading to the deletion of important gene(s) on the P21 region of Chromosome 3. This is followed by additional chromosomal loss or mutation. These changes then follow the unregulated growth and expansion of malignant clone(s). This is supported by activation of several growth factor pathways such as tumor growth factor or interlukine 6  .
The role of schistosomiasis in renal cell carcinogenesis is not understood. There is only one case of such association reported in the literature  . Our case represents, however, the first documented association between the sarcomatoid variant of renal cell carcinoma and schistosomiasis in a Saudi patient. The association between these two pathologies could be a coincidental finding. The other possibility is that renal carcinoma in this patient is related to end-stage renal disease rather than to schistosomiasis or to renal stone. Mendley and To back suggested that an altered microenvironment of the cells in the end-stage renal failure may cause the release of certain growth factors that enhance proliferation of renal cell carcinoma  .
In patients residing in countries where schistosomiasis is endemic and presenting with renal masses, the possibility of renal schistosomiasis should be considered and investigated. Pathologists and clinicians should be aware of the possible association between schistosomiasis and renal cell carcinoma.
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Maha I Arafah
Department of Pathology, King Khalid University Hospital, P.O. Box 7805, Riyadh 11472
[Figure - 1], [Figure - 2], [Figure - 3]