Kidney Cancer:
Renal Cell Carcinoma (RCC) The American Cancer Society estimates that approximately 28,000 new cases of renal cell carcinoma will be detected in 1998. It is further estimated that over 11,000 deaths will result from this form of kidney cancer. Neither chemotherapy nor radiation therapy have been shown to be effective against renal cell carcinoma that has spread beyond the kidney (metastatic). Thus, the early detection of these cancers, which are generally incurable except by surgical means, should translate into improvements in survival due to earlier operative intervention at a potentially curable stage. The cause of renal cell carcinoma is unknown, although tobacco and industrial carcinogens have been implicated. There is an association of renal cell carcinoma with several clinical syndromes such as Von Hippel Lindan Disease, Adult Polycystic Kidney Disease, and Acquired Renal Cystic Disease. Renal Cell Carcinoma has also been reported to run in certain families (a familial type). Q: What are the symptoms/signs of RCC? Dr. Green: Symptoms/signs of RCC are generally caused by either invasion of the tumor beyond the confines of the kidney causing pain, hematuria (blood in the urine), or a flank mass. Symptoms of advanced disease (metastatic) include weight loss, fever, hypertension (elevated blood pressure), night sweats, and the sudden onset of a varicocele (varicose veins of the testis) in a male patient. Q: Do all cases of RCC have symptoms/signs? Dr. Green: No. Many patients with RCC are asymptomatic and an increased number of RCCs are now being detected incidentally because of the increased use of computerized tomography (CT) scanning and ultrasonography. Q: What is Staufer's Syndrome? Dr. Green: Paraneoplastic syndromes occur in approximately thirty percent of patients with RCC, and account for such presenting symptoms as hypertension, hypercalcemia, pyrexia or hepatic dysfunction. RCC with hepatosplenomegaly, elevated alkaline phosphatase and serum haptoglobin, and prolonged prothrombin (bleeding) time is referred to as Staufer's Syndrome. Q: What is the classic triad? Dr. Green: Pain, hematuria, and a flank mass constitute the classic triad; however, this is only seen in approximately ten percent of patients and usually only those with advanced disease. Q: How does one diagnose RCC? Dr. Green: Although there are no prospective studies in the uroradiology literature, it is generally accepted that computerized tomography is the most sensitive imaging technique. However, abdominal ultrasonography (USG) is a useful adjunct to CT, especially in differentiating hyperdense cysts from solid tumors. Q: Are these imaging studies (CT & USG) one hundred percent accurate in diagnosing RCC? Dr. Green: No. Some benign tumors present as solid renal lesions and may be misdiagnosed as renal cell cancer. The most common of these rare lesions are angiomyolipomas (renal hamartoma) and oncocytomas. Q: Do all cancers in the kidneys arise primarily from the kidneys themselves? Dr. Green: No. The kidney is a frequent site of metastatic cancers from other sites. The most common sources of metastatic cancer to the kidney occur from primary lung and breast cancers and should be suspected in patients with these neoplasms. Q: How do you treat someone with RCC? Dr. Green: Prior to treatment one needs an evaluation primarily aimed at determining the extent of cancer spread (staging). Currently, the most commonly employed method used is the American Joint Committee for Clinical Staging, referred to as the TNM Clinical Classification. Q: Why is staging of RCC important? Dr. Green: RCC can grow locally into very large masses and invade through the surrounding fascia into adjacent organs. They also metastasize through lymphatic channels into regional and mediastinal nodes or by hematogenous routes primarily to the lungs, bone, and brain. Treatment options and prognoses are based upon clinical and pathologic stage. Cancer outside the confines of the kidney carries a less favorable prognosis than one confined to the kidney. Q: What is involved in staging? Dr. Green: As mentioned previously, the abdominal CT and ultrasound are the main imaging studies. Other studies to complete staging include a chest x-ray, a chest CT if the primary cancer is large or if the chest x-ray is indeterminate, and a bone scan. Also needed is a complete blood panel that assesses the hematologic system, liver and kidney function. It is my practice to reserve head CT, MRI, and renal angiography for patients with vascular involvement or those undergoing "parenchyma-sparing" surgery. Because survival depends on surgical removal, as no effective chemo or radiotherapy has been identified against this disease, the evaluation of any patient presenting with RCC should include a history and physical examination with a determination made of the patient's ability to undergo major surgery and tolerate general anesthesia. Q: What factors influence prognosis? Dr. Green: Other than metastases, the factors which are associated with poor prognosis include cancer size, extension through the kidney covering (Gerota's fascia), involvement of contiguous organs, spread to regional or distant lymph nodes, and vena caval involvement. In general the propensity for metastases increases with larger lesions. Q: How do you treat RCC? Dr. Green: The mainstay of treatment for primary RCC is surgical removal. Surgery is the only currently known curative therapeutic modality. Radical nephrectomy is the procedure of choice for clinically localized RCC. Q: How long can one expect to live after surgery for RCC? Dr. Green: Survival data varies with pathologic stage. Overall, 5-year survival ranges from 60-82% for small RCC confined to the kidney. For RCC that extends into major veins, adrenal glands, or perinephric tissue, but not beyond Gerota's fascia, the 5-year survival averages 50 percent. Q: What is a "nephron-sparing" operation? Dr. Green: "Nephron-sparing" surgery is the local excision of small RCC only... the remainder of the kidney is left in place, i.e., not surgically removed as in a radical nephrectomy. The technique is becoming popular for the management of small RCC in that recent data has shown that the results may be equal to the radical nephrectomy in these select cases. This procedure was initially developed for patients who developed RCC in a solitary kidney. The book is still open on this procedure as to whether it is as effective as radical nephrectomy; it will require longer follow-up on patients that have undergone these procedures. Q: Are there other treatment options? Dr. Green: Yes. 1. Radiation therapy has been applied to RCC as both an adjuvant to surgical therapy and as a treatment for metastatic lesions. Palliative radiotherapy has been successful in treating painful metastases and is a good tool for pain management. It is not effective as a single primary treatment modality. 2. Hormonal therapy protocols were based upon observations that progestinal agents inhibited the growth of renal cell tumors. Although early reports were encouraging, no studies have substantiated the initial results. Other hormonal agents, including tamoxifen, have proven equally ineffective. 3. Chemotherapy in treating renal cell has been poor, although cytotoxic drugs are the cornerstone of therapy for most solid malignancies. 4. Immunotherapy has shown some responses; however, evidence that any of them have had an impact on patient survival is lacking. 5. Adoptive immunotherapy relies on activation of memory T-lymphocytes. Some favorable responses are occurring. This treatment is in the investigational phase? Summary: Renal Cell Carcinoma presents a diagnostic and therapeutic challenge. The increased utilization of abdominal imaging is detecting more tumors at a very early stage. The task will be to improve the differential diagnoses of these tumors to exclude those which do not need surgical therapy. At present, no systemic agent has been proven to be uniformly effective in the management of metastatic or locally advanced RCC. The future focus may be in "gene therapy" and creating "tumor vaccines". | 
