Sarcomatoid variant of Xp11 translocation renal cell carcinoma: A rare case report in an adult female.

Xp11 translocation renal cell carcinoma (XPTRCC) is a very rare kidney neoplasm, which has been predominantly reported in young patients. Sarcomatoid transformation in renal cell carcinomas is known. However, its occurrence in XPTRCC is unreported so far in the literature. We report a unique case of sarcomatoid transformation in a XPTRCC in a 23-year-old female, who presented with a huge right-sided renal mass and had metastatic deposits in lungs. Morphologically, clear cell morphology with papillary architecture along with foci of sarcomatoid transformation and rhabdoid differentiation were noted. Immunohistochemistry showed Pax-8 and TFE-3 expression in all components including the sarcomatous areas, whereas CK and EMA were expressed in conventional clear cell component. We present an extremely rare case of sarcomatous transformation in XPTRCC and discuss the case as determined by histopathology and immunocytochemistry. To our knowledge, this is the first case of sarcomatoid transformation XPTRCC being reported in the world literature.

Cytoreductive Surgery in the Management of Renal Tumours: Rationale, Current Evidence and Future Perspectives.

Renal cell carcinoma accounts for 3% of adult solid malignant tumours. Approximately 25% of the patients present with metastatic disease at presentation. In the era of immunotherapy (interferon alpha-2b and interleukin-2), studies showed significant survival benefit with cytoreductive nephrectomy (CRN) followed by interferon alpha-2b than interferon alpha 2-b alone. Introduction of targeted therapies (vascular endothelial growth factor receptor-tyrosine kinase inhibitors, mammalian target of rapamycin inhibitors) in 2005 generated a great interest in the management of metastatic renal cell carcinoma (mRCC) as these drugs exhibited tumour shrinkage in the primary tumour as well as in the metastatic site/s. Though there is no level 1 evidence, many studies have shown the usefulness of cytoreductive nephrectomy along with targeted therapy as against to targeted therapy alone. This review is aimed at the rationale behind the cytoreductive nephrectomy in mRCC, the current evidence and what is in store for the future. A detailed search on the management of mRCC was carried out on MEDLINE, Embase, CANCERLIT and Cochrane Library databases using the key words "cytoreductive nephrectomy", "immunotherapy" and "targeted therapy" since 1980 till 2015. Original articles, review articles, monograms, book chapters on metastatic renal cancer and textbooks on urologic oncology, oncology and urology were reviewed. Various international guidelines on this issue were also studied. An identical search was performed using the American Society of Clinical Oncology Abstract database. Trials in the progress or recently completed that were relevant to this paper were identified through clinicaltrials.gov. The latest information for new articles ahead of publication was last accessed in November 2015. CRN has remained an integral part to the management of metastatic renal cell carcinoma mainly for the patients with good performance status, life expectancy of more than 12 months and in the absence of adverse prognostic factors. It had shown measurable survival benefit in the era of immunotherapy (CRN + immunotherapy vs. immunotherapy alone). In the era of targeted therapy, many studies have shown significant survival benefit with CRN + targeted therapy. However, there is no clear level 1 evidence to support this. The ongoing trials (CARMENA and European Organisation for Research and Treatment of Cancer SURTIME) would perhaps guide us in the way in which we should manage mRCC disease in the future. Maybe we may find some answers on the issues of the effectiveness of targeted therapy, the timing of CRN and sequencing these treatment arms once the results of these ongoing and future trials are through.

Rationale for an early detection program for bladder cancer.

INTRODUCTION: A total of 356,557 new cases were diagnosed annually worldwide in 2009, it was estimated that 52,810 new patients were to be diagnosed with bladder cancer and there were 10,180 projected deaths from the disease in the USA. Despite being the fourth commonest cancer in men, we do not have an early detection/screening program for bladder cancer. The review was aimed at looking at the evidence for the rationale for an early detection program for bladder cancer. MATERIALS AND METHODS: A detailed search on bladder cancer epidemiology, diagnosis, pathology, tumor markers, treatment outcomes, screening, morbidity and mortality of bladder cancer was carried out on Pubmed central/Medline. Original articles, review articles, monograms, book chapters on bladder cancer, text books on urological oncology, oncology and urology were reviewed. The latest information for new articles before publication was last accessed in June 2010. DISCUSSION AND CONCLUSIONS: Bladder cancer is the fourth commonest cancer in men, the annual death rate from this disease is significant and every year there is an increase in its incidence globally. The prognosis of bladder cancer is stage and grade dependent; the lower the stage (T2 or less) the better is the survival. Delay in the diagnosis and treatment does alter the overall outcome. Therefore, there is a clear need for early detection of bladder cancer and screening program. Although we do not have an ideal marker for bladder cancer, it is time we maximize the potential of markers such as UroVysion, NMP22 along with cytology to start such a program. May be as a first step the early detection and screening program could be started in high-risk population. It is not worth waiting till we find the best marker as it would be unfair to our patients. The fear of unnecessary tests and treatment in bladder cancer after its detection in screening program is without any substance. The cost-effectiveness of such a program is certainly comparable to that is used for colon or breast and for prostate as well.

Management of urological cancers during pregnancy.

Urological cancers during pregnancy pose many challenges for clinicians. As these tumors are very rare among pregnant women and their symptoms might mimic those of common pregnancy-related disorders, the diagnosis is often delayed. Once a urological tumor is diagnosed in a pregnant patient, appropriate steps should be taken to treat her and the fetus at the same time. A delay in treatment can risk the mother's life whereas an early treatment can risk the life of the fetus. Common urological cancers that might occur during pregnancy include renal cancer, bladder cancer and adrenal tumors, particularly pheochromocytoma. Ultrasonography and MRI, which use no ionizing radiation, are considered to be safe during pregnancy. Surgical intervention is safest if performed during the second trimester. The treatment outcomes of these cancers in pregnant women are no different from those in the general population. Early suspicion and timely intervention are crucial for successful treatment.

Treatment of locally advanced and metastatic bladder cancer.

BACKGROUND: There is a significant variation in the treatment strategies adopted for the treatment of locally advanced T3b, T4a, N1-3 and metastatic bladder cancer. There is increasing evidence that we would be able to offer them some benefit in terms of disease-free survival and improving the quality of life. This article is aimed at reviewing the current literature on the treatment strategies in locally advanced and metastatic bladder cancer. MATERIALS AND METHODS: Extensive literature search was done on Medline/Pubmed from 1980-2007 using the key words - treatment of locally advanced, metastatic bladder cancer. Standard textbooks on urology, urologic oncology and monograms were reviewed. Guidelines such as National Comprehensive Cancer Network guidelines, European Urology Association guidelines and American Urology Association guidelines were also studied. RESULTS AND CONCLUSIONS: There is a place for radical cystectomy in locally advanced T3b-T4 and N1-3 bladder cancer. Radical cystectomy alone rarely cures this subgroup of patients. There is increasing evidence that meticulous surgical clearance and extended lymphadenectomy has significant impact on disease-free survival. Adjuvant chemotherapy has been found to be effective in terms of recurrence-free survival and better than cystectomy alone. Neoadjuvant chemotherapy followed by radical cystectomy also has beneficial effects in terms of downstaging the disease and improving recurrence-free survival. This perioperative chemotherapy (adjuvant/neoadjuvant) has 5-7% survival benefit and 10% reduction in the death due to cancer disease. Excellent five-year survival rates have been achieved in patients achieving pT0 stage at surgery following chemotherapy (around 80%) and overall 40% five-year survival in node positive patients, which is promising. Though practiced widely, perioperative chemotherapy is not considered as a standard of care as yet. Current ongoing trials are likely to help us in reaching a consensus over this. There is no role of preoperative or postoperative radiotherapy in locally advanced/metastatic bladder cancer except in non TCC bilharzial/squamous cell carcinoma of bladder. Use of nomograms and prognostic factor evaluation may help us in the future in predicting the disease relapse and may help us in tailoring the treatment accordingly. Newer and more effective chemotherapeutic drugs and ongoing trials will have a significant impact on the treatment strategies and outcome of these patients in the future.

Treatment algorithm in hormone-resistant prostate cancer: Practical guidelines.

Treatment of hormone-resistant prostate cancer can be a challenging situation. The first important step in treating this condition is to assess if one has achieved the castrate level or not. If the castrate levels are not achieved, attempt should be made to achieve so. If the castrate level is achieved, then androgen withdrawals may be of help. Supportive care, care of the clinical problems forms an integral part of the treatment. Cancer-specific chemotherapy is certainly an option in progressive disease.