Sarcomatoid renal cell carcinoma with von Hippel-Lindau disease: a case report

We report a 68-year-old woman who had bilateral renal cell carcinoma (RCC) associated with von Hippel-Lindau (VHL) disease. Surgical resection of a central nervous system hemangioblastoma had been done previously. This time, synchronous bilateral RCCs were found in her kidneys, with metastases to lungs and liver. Right radical nephrectomy was performed to remove the primary tumor in the right kidney. Histopathological examination of the tumor revealed clear cell RCC with a sarcomatoid component. After surgery, transcatheter arterial embolization was performed for the tumor in the left kidney and interferon therapy was commenced. The left renal tumor decreased in size and interferon therapy was effective against the metastatic lung tumors. However, 4 years after resection of the right RCC, the tumor in the left kidney increased progressively in size and partial left nephrectomy was performed. Histopathological examination of the resected tumor also showed clear cell type RCC with a sarcomatoid component. The patient eventually died of her disease at 5 years after resection of the right RCC. RCC associated with VHL is usually of the clear cell type has a relatively good prognosis. Sarcomatoid RCC is rare in VHL patients and, to our knowledge, the present report is the first case of sarcomatoid RCC associated with VHL in the Japanese literature.     

Solitary pancreatic metastasis from renal cell carcinoma

A case of asynchronous bilateral renal cell carcinoma with pancreatic metastasis is described. Left nephrectomy and tumorectomy in the right kidney were performed. Solitary metastasis to the pancreas without symptoms was treated by distal pancreatectomy with tumor and splenectomy. Although bilateral renal cell carcinomas were histologically renal cell carcinoma (clear cell subtype, grade 2), the resected pancreatic tumor was renal cell carcinoma with sarcomatoid change. Therefore, the metastatic tumor had a more malignant potential than the primary tumor. The pancreatic metastasis was seen at 6 years 10 months and 2 years 6 months after left nephrectomy and enucleation of the right renal tumors, respectively. The patient is alive without disease and is being treated by alpha-interferon for 12 months after distal pancreatectomy. A careful long-term follow-up of the patient with renal cell carcinoma seems to be necessary.     

Sarcomatoid Squamous Cell Carcinoma of the Right Renal Pelvis with Liver Metastasis: Case Report

We report on a man with sarcomatoid squamous cell carcinoma of the right kidney with liver metastases. Bilateral renal lithiasis and well differentiated squamous cell carcinomas of the right renal pelvis were also noted. This uncommon case is discussed and the literature is reviewed.     

Simultaneous occurrence of renal cell and transitional cell carcinoma in the same kidney and ureter. A case report

An unusual case of 2 concurrent primary renal tumors within the same kidney is reported. A 70-year-old woman presented with gross hematuria when she was in the hospital for cerebral infarction. Excretory urography revealed a marked expansion of the right kidney with no renal function. CT scan showed a mass arising from the right kidney, the hydronephrotic right renal pelvis, and a mass in the lower right ureter. Selective renal angiogram showed marked neovascularity of the mass. There was an encasement of the intrarenal artery to the lower pole. Angiographic findings were highly suggestive of a renal cell carcinoma with a second neoplasm in the renal pelvis. Subsequently, the patient underwent right radical nephroureterectomy and partial cystectomy. Section of the removed specimen revealed a 4.0 X 3.8 cm solid tumor confined to the kidney in the upper pole and a transitional cell carcinoma arising from the renal pelvis. In addition, transitional cell carcinoma was present in the distal ureter.     

Renal cell carcinoma, primary lesion which was not easily identified: report of two cases

We report two cases of renal cell carcinoma, primary lesions of which were not diagnosed at an early stage. The first case was a 58-year-old woman, who had nephrectomy due to staghorn calculus of left kidney. Fifteen months later, she died of metastatic cancer whose origin was unknown until the left kidney was re-examined. It was sarcomatoid renal cell carcinoma. The second case was 43-year-old woman, who had amputation of right index finger due to metastatic tumor. Renal cell carcinoma was highly suspected, but no tumor could be found in her kidneys although various urological examinations were performed. Two years later, abdominal CT scan showed a space-occupying lesion (SOL) of left kidney. She had left nephrectomy, and the origin was finally identified.     

Clinical study of sarcomatoid renal cell carcinoma

Patients with sarcomatoid renal cell carcinoma are rare and have poor survival. We evaluated 14 patients who had renal cell carcinoma with a sarcomatoid component between 1982 and 2000. There were 9 men and 5 women with a median age of 59.5 years (range 32 to 77). Seven patients had a tumor on the right side and 7 on the left side. Thirteen patients had some symptoms and 11 had metastases at the initial visit. Most of them were stage T4 and high nuclear grade cancer and showed elevated acute phase reactants. There were 7 patients followed by interferon therapy, and the cause-specific 5-year survival rate was less than 10%. We confirmed that renal cell carcinoma with a sarcomatoid component often showed local invasion, distant metastasis and poor prognosis.     

A case of bilateral renal cell carcinoma

A case of asynchronous bilateral renal cell carcinoma is reported. A 52-year-old man with the chief complaint of asymptomatic gross hematuria visited our hospital on November 16, 1981. Intravesicular pyelography showed poor secretion of the left kidney, compression of its pelvis and calyces and normal visualization of the right kidney. On computer tomographic (CT) scanning, abdominal aortogram and left selective renal angiogram, a round tumor lesion on the left kidney was recognized. Under the diagnosis of left renal tumor, radical left nephrectomy was performed in December, 1981. Histological diagnosis was clear cell carcinoma of the left kidney. He was administered medroxyprogesterone acetate. In December, 1985, CT showed a space-occupying lesion laterally on the right kidney and another suspected tumorous lesion. On the right selective renal angiogram, a round hypervascular lesion about 3 cm in diameter was found on the upper portion of the right kidney. On February 10, 1986 simple surgical enucleation of the 2 renal tumors was performed. Histological diagnosis was the same as that of the left kidney. Postoperative course was uneventful. He is well without recurrence or metastasis 8 months after operation.     

Combined small cell carcinoma and sarcomatoid squamous cell carcinoma in the renal pelvis

We report here a case of combined small cell carcinoma and sarcomatoid squamous cell carcinoma in the renal pelvis. A 61-year-old female presented with right flank discomfort, microhematuria and progressive renal dysfunction. Following diagnosis of right renal pelvic carcinoma, radical nephroureterectomy with lymph node dissection was performed through a midline incision. The tumor was pathologically diagnosed to be combined small cell carcinoma and sarcomatoid squamous cell carcinoma in the renal pelvis. The patient had no evidence of recurrence or metastasis, 16 months postoperatively. Small cell carcinoma or sarcomatoid squamous cell carcinoma of the renal pelvis is very rare. We believe this is the first such case to be reported in the world.     

Solitary massive right ventricular metastasis of renal cell carcinoma without inferior vena cava or right atrium involvement

Right ventricular metastases from renal cell carcinoma without inferior vena cava or right atrium involvement are rare. A 67-year-old male presented with hematuria and congestive heart failure. Computed tomography revealed a left renal mass. In addition, an intra-cardiac mass was found during the preoperative workup, causing right ventricular outflow tract obstruction. His past medical history included previous coronary bypass grafting. The patient underwent combined radical nephrectomy and removal of right ventricular mass. Pathology confirmed renal cell carcinoma with extensive sarcomatoid features in both the left kidney and right ventricle. His postoperative recovery was unremarkable.     

The Ron proto-oncogene product is a phenotypic marker of renal oncocytoma

The proto-oncogene product Ron is the receptor for macrophage stimulating protein, a scatter factor that stimulates cell proliferation, prevents apoptosis, and induces an invasive cell phenotype. We investigated the expression of Ron, Ki-67 (proliferation index), p53, and bcl-2 (proapoptotic and antiapoptotic proteins, respectively) in 50 renal tumors (19 clear cell carcinomas, 18 oncocytomas, 7 papillary cell carcinomas, 5 chromophobe cell carcinomas, and 1 carcinoma with sarcomatoid areas). In addition, we studied Ron in normal kidney and in the renal carcinoma cell line Caki-1. Immunostaining and Western blot showed Ron in normal kidney and in all oncocytomas but never in renal cell carcinomas or in Caki-1. In addition, Western blot showed that Ron was expressed in phosphorylated, i.e., active, form. Bcl-2 was strongly expressed in oncocytomas, whereas Ki-67 and p53 were much less expressed in oncocytomas than in carcinomas. These results indicate in Ron a marker that differentiates oncocytoma from the other renal epithelial tumors. We therefore think that Ron may prove to be a new tool for a sound and precise diagnosis of oncocytoma, a benign tumor that cannot always be distinguished from carcinomas at histologic examination. The overexpression of bcl-2, but not p53 in oncocytoma, suggests that the MSP/Ron system sustains the growth of oncocytoma by opposing apoptosis.     

Metastatic sarcomatoid carcinoma to liver and bone marrow in renal transplant recipient: due to exacerbation of quiescent renal cancer? A case report

Sarcomatoid carcinoma is a rare tumor with rapid growth and a poor prognosis. A 60-year-old man underwent kidney transplantation. Three months after transplantation, multiple tumors were found in the liver and bone, and the patient died several days later. Pathological examination of liver and bone marrow biopsies revealed metastatic sarcomatoid carcinoma. Pretransplantation, the patient's workup was positive only for mild thrombocytopenia and a complicated cyst with peripheral rim calcification (Bosniak IIF) in the right kidney. Retrospectively, we found the abdominal computed tomography film, which had been examined at another hospital 6 years previously. The calcified complicated cyst was a 3-cm enhancing solid mass in the right kidney, suggesting renal cell cancer. It is possible that the cancer developed from the calcified complicated cyst. In this case, immunosuppressants may have altered malignant cell proliferation, invasion, and the form of metastasis.     

A case of renal cell carcinoma during pregnancy

Renal cell carcinoma during pregnancy is extremely rare. This 30-year-old patient was first seen on Dec. 18, 1983 at the 7th week of her 2nd pregnancy. She had asymptomatic hematuria. Cystoscopy revealed normal findings and sonogram showed well defined echogenic mass in the upper pole of the right kidney. She was admitted to our hospital on account of gross hematuria and severe lumbago on Jan. 19, 1984. The pregnancy was stopped by artificial abortion. Renal angiography revealed a rich-vascularized tumor in the upper pole of the right kidney. Preoperatively, arterial embolization was done and nephrectomy was performed. Histological examination showed a well-differentiated adenocarcinoma. There was no involvement of the renal vein and no lymph-node metastases were found in the operation specimen. The post-operative course was uneventful. This rare case of renal cell carcinoma during pregnancy is herein reported along with some discussion.     

Small renal cell carcinoma presented with pleural metastasis and multiple bone metastasis ; a case report

The patient is a 69-year-old male. His chief complaint was chest pain. Because imaging studies suggested pleural mesothelioma associated with multiple bone metastases, right pleural tumor resection was performed. Pathological diagnosis was metastatic pleural tumor, and renal cell carcinoma (RCC) was suspected as its origin. Dynamic computed tomography showed a small tumor in the right kidney. The tumor was 15 mm in diameter and consistent with RCC. Laparoscopic radical nephrectomy was performed for the right kidney. Pathological diagnosis was RCC, clear cell carcinoma with sarcomatoid component, T1aN0M1, stage IV. Sorafenib therapy was started 46 days after the operation as a systemic therapy, and stable disease has been maintained. Generally, small RCC is assumed to have a good prognosis. However, a small percentage of patients with small RCC have distant metastasis at the time of diagnosis, and the prognosis is reported to be poor. We report this case, and a review of the literature.     

A case of synchronous contralateral renal cell carcinoma and ureteral transitional cell carcinoma

A case of synchronous contralateral renal cell carcinoma and ureteral transitional cell carcinoma is reported. A 56-year-old man, who had been incidentally found to have an abnormal mass in the upper pole of the right kidney on ultrasound sonography, was admitted on January 8, 1985. CT scanning and renal arteriography revealed right renal malignancy. Right radical nephrectomy was performed and histological examination showed adenocarcinoma, granular cell type of the right kidney. He was discharged on February 3, 1985. Two months postoperatively, he was rehospitalized for macroscopic hematuria. Left retrograde pyelogram showed obstruction at middle ureter and cytology of urine from left ureter was positive. So a left ureteral tumor was suspected, and partial resection of left ureter and ureteroureterostomy were performed. Histological examination revealed ureteral transitional cell carcinoma. He is now doing well at 6 months following the lat surgery, without any evidence of recurrence.     

Simultaneous double malignant tumors on functional solitary kidney: a case report]

A case of simultaneous double cancer on functional solitary kidney is reported. A 72-year-old man was admitted to the Department of Internal Medicine with angina, hypertension and diabetes mellitus. After treatment involving percutaneous transluminal coronary angioplasty, he was sent to the Department of Urology to check the microhematuria. Cystoscopy showed normal interior of urinary bladder. Drip infusion pyelography and computed tomography demonstrated a tumor mass on the upper pole of the right kidney and atrophic left kidney. Preoperative diagnosis was right renal cell carcinoma. However, an additional pelvic tumor was found during surgery, and a partial nephrectomy was performed. Histologic examination confirmed the presence of 2 separate and distinct malignant entities: a renal cell carcinoma and a non-invasive transitional cell carcinoma. Postoperative recovery has been uneventful and without hemodialysis for 16 months. This is the 19th case of simultaneous occurrence of renal cell carcinoma and transitional cell carcinoma in the same kidney and the 1st case in the patient with a functional solitary kidney in the Japanese literature.     

Renal oncocytoma with cystic degeneration: a case report

A 45-year-old man was referred to our department because of a right renal mass which was incidentally found at a health screening. Ultrasound sonography, computerized tomography and magnetic resonance imaging showed a hypovascular tumor 3 cm in diameter with the fluid at the upper pole of the right kidney, implicating that the tumor was renal cell carcinoma originating from a renal cyst wall, or with central necrosis. A radical nephrectomy was therefore performed. The tumor was dark-brown and contained brown fluid. The histopathological findings showed renal oncocytoma with cystic degeneration.     

A case of renal cell carcinoma similar to rhabdoid tumor of kidney in an adult; trial of radiation and chemotherapy]

We report a case of sarcomatoid renal cell carcinoma in a 30-year-old woman. The pathological findings indicated renal cell carcinoma (solid sarcomatoid and pleomorphic type) similar to malignant rhabdoid tumor of kidney (MRTK). We treated her with combination chemotherapy (vincristine, adriamycin, cyclophosphamide and actinomycin D) and local irradiation after nephrectomy. The number of bone metastatic foci increased and brain metastasis occurred. However, irradiation was effective on each focus. She died 8 months after the operation caused by brain metastasis. We conclude that sarcomatoid renal cancer is highly malignant and chemotherapy-resistant, but radiation may be partially effective.     

Bilateral renal cell carcinoma in a patient receiving long-term dialysis

Bilateral renal cell carcinoma developing in a 55-year-old male receiving long-term dialysis is reported. The patient, who had undergone maintenance hemodialysis for 12 years, was admitted in July, 1986 for the purpose of extensive examination of a right renal mass. CT scan and ultrasonography demonstrated an enlarged bilateral kidney associated with multiple cysts, containing a solid mass in the right upper pole. The patients underwent right nephrectomy under the diagnosis of right renal tumor. The solid tumor 4 x 3 cm in size revealed a grade 1 to 2 renal cell carcinoma pathologically. Two small tumors were recognized in other portions of the kidney. Multiple cysts of varying sizes, the largest 2 cm in diameter, replaced the renal parenchyma. The cyst walls frequently contained hyperplastic changes. The patient has been subsequently followed up for 2 years and 9 months and underwent left nephrectomy for suspected left renal tumor. The surface of the left kidney was covered by numerous cysts. The result of pathological examination was renal cell carcinoma, which was recognized in a total of 7 regions of the left kidney. The patient remains well on hemodialysis, with no evidence of recurrence or metastasis.     

Sarcomatoid differentiation in renal cell carcinoma: a study of 101 cases

Sarcomatoid renal cell carcinoma is not a distinct histologic entity and represents high-grade transformation in different subtypes of renal cell carcinoma. It is not known whether any particular histologic type has a predilection for sarcomatoid change or whether the primary histologic type of renal carcinoma undergoing sarcomatoid change affects prognosis. Of 952 consecutively histologically subtyped renal cell carcinomas, the incidence of sarcomatoid differentiation was 8% in conventional (clear cell) renal carcinoma, 3% in papillary renal carcinoma, 9% in chromophobe renal carcinoma, 29% in collecting duct carcinoma, and 11% in unclassified renal cell carcinoma. One hundred one renal cell carcinomas with sarcomatoid change were studied, and clinicopathologic parameters were correlated with outcome. The mean age of patients was 60 years (range, 33-80 years), and the male-to-female ratio was 1.6:1. The median tumor size was 9.2 cm (range, 3-25 cm). The primary histologic subtype of the carcinoma component was conventional (clear cell) renal carcinoma in 80 cases, papillary renal carcinoma in eight, chromophobe renal carcinoma in seven, collecting duct carcinoma in two, and unclassified renal cell carcinoma in four. The sarcomatoid component resembled fibrosarcoma in 54 cases, malignant fibrous histiocytoma in 44, undifferentiated sarcoma (not otherwise specified) in three with focal rhabdomyosarcomatous component in two of them. The spindled elements accounted for 1% to 99% of the sampled tumor (median, 40%; mean 45%). The histologic grade of the spindled elements was intermediate to high in 92 cases and low in nine cases. Most cases were TNM stages III and IV (seven stage I, six stage II, 63 stage III, and 25 stage IV). Follow-up was available in 88 patients; 61 (69%) patients died of disease and had a median survival time of 19 months. Distant metastases, most frequently to the lungs, were documented in 51 (66%) of 77 patients who had available clinical information regarding distant metastasis. The disease-specific survival rate was 22% and 13% after 5 and 10 years, respectively, compared with a cohort of renal cell carcinomas without sarcomatoid change with a 5-and 10-year disease-specific survival of 79% and 76%, respectively. Kaplan-Meier survival analysis showed that tumors with high TNM stage (p = 0.0027), at least 50% sarcomatoid component (p = 0.0453), and angiolymphatic invasion (p = 0.0282) were associated with decreased survival rates. The primary histologic subtype of the carcinoma component and the type and grade of the sarcomatoid component did not affect survival. In a Cox proportional hazard regression model, TNM stage appeared to be the only significant variable in predicting outcome among renal cell carcinomas with sarcomatoid change (p = 0.018; risk ratio, 6.984 and 8.439). Compared with a cohort of renal cell carcinomas without sarcomatoid change, sarcomatoid tumors tended to present at a more advanced stage (p = 0.0001). Also, when adjusted for stage, necrosis, and tumor size, patients with tumors with sarcomatoid differentiation had a worse prognosis than did patients with tumors without sarcomatoid change (p = 0.0001). In conclusion, sarcomatoid change in renal cell carcinoma portends a worse prognosis. Because tumors with even a small component of sarcomatoid change may have an adverse outcome, this finding, when present, should be noted in the surgical pathology report.     

Immunohistochemical expression of hypoxia inducible factor-1alpha and its downstream molecules in sarcomatoid renal cell carcinoma

PURPOSE: Sarcomatoid renal cell carcinomas, highly aggressive variants of renal cell carcinoma subtypes, often present with or develop metastases soon after the primary diagnosis. Most metastatic cases do not respond to immunotherapy or aggressive chemotherapy. Recently targeted therapies, particularly those targeting hypoxia inducible pathway molecules, have been tested clinically on metastatic clear cell renal cell carcinoma with promising initial results. No such studies are available on sarcomatoid renal cell carcinoma. We investigated the hypoxia inducible pathway marker immunohistochemical expression profile, and any potential therapeutic implications that such expression may have, in these tumors. MATERIALS AND METHODS: Immunohistochemical staining for hypoxia inducible factor-1alpha, glucose transporter 1, carbonic anhydrase IX and vascular endothelial growth factor was performed in 22 clear cell and 12 nonclear cell sarcomatoid renal cell carcinomas. The immunoreactivity in the tumors was graded from 0 to 3+ (0-no staining, 1+-1% to 25% cells positive, 2+-26% to 50% cells positive and 3+-greater than 50% cells positive). The results were then compared with various clinical parameters to assess for associations. RESULTS: Most clear cell renal cell carcinomas over expressed (2+ or 3+) hypoxia inducible factor-1alpha (in 59%), carbonic anhydrase IX (95%), glucose transporter 1 (91%) and vascular endothelial growth factor (95%). None of the nonclear cell sarcomatoid renal cell carcinomas showed 2+ or 3+ expression of hypoxia inducible factor-1alpha, carbonic anhydrase IX or glucose transporter 1, but 92% showed diffuse positivity for vascular endothelial growth factor. Over expression of carbonic anhydrase IX showed no association with survival, unlike that reported in (nonsarcomatoid) clear cell renal cell carcinoma. There was significant discordance in the staining grades among hypoxia inducible factor-1alpha, carbonic anhydrase IX and glucose transporter 1 in clear cell renal cell carcinoma, suggesting that mechanisms other than hypoxia inducible pathway may be involved in some sarcomatoid clear cell renal cell carcinoma. CONCLUSIONS: Hypoxia inducible pathway markers continue to be over expressed in sarcomatoid clear cell renal cell carcinoma, and can be of diagnostic usefulness in such high grade tumors. Over expression of vascular endothelial growth factor in the clear and nonclear cell groups raises the possibility that vascular endothelial growth factor targeted therapies may have a role in the management of sarcomatoid renal cell carcinoma, and deserve further investigation.