Recombinant factor VII [rFVII] can be useful for massive hemorrhage during radical nephrectomy

Radical nephrectomy for renal tumors can be associated with serious complications, e.g. massive bleeding or even table deaths. Various regimens like normovolemic hemodilution, autologous transfusion, hypotensive anesthesia, etc have been used in anticipation of hemorrhage in these operations. In our case there was not only massive hemorrhage but also a failure to clot and disseminated intravascular coagulation. All the routine regimens failed to stop bleeding and the generalized ooze. Recombinant Factor VII [rFVIIa] was used and it saved the day

Hnpcc hereditary nonpolyposis colorectal cancer in Pakistan

Hereditary nonpolyposis colorectal cancer [HNPCC] is the most common form of hereditary bowel cancer. Multiple generations are affected with colorectal cancer at relatively young age, between 25 and 45 years. To investigate the frequency of HNPCC in Pakistani population, due to the high incidence of colorectal cancer in younger Pakistani adults and prevalence of consanguinity in this region conducted this study. November 2008 and January 2010. Ninty histopathologically confirmed colorectal cancer patients between 12- 50 years and their families were interviewed using a detailed questionaire. The questions about family history of colorectal cancer, history of other cancers, age at diagnosis and consanguinity were asked. The pedigrees were drawn for all families based on given information. Hospital records were also reviewed to confirm cancers reported in relatives. Amsterdam criteria was used to label a family as HNPCC. Seventeen patients [18.9%] had one or more first or second degree relatives under age 50 years with colorectal cancers suggestive of HNPCC. Another 15 patients [16.7%] had first or second degree relatives with a family history of other extra-colonic cancers including ovarian, breast, endometrium, lung, parotid, brain and bladder cancer. Of these 30 patients [33.3%] reported that their parents were first degree cousins. 1. High frequency of HNPCC is seen in Pakistani population. 2. Higher proportion of colorectal cancer is seen in young Pakistanis. Strong prevalence of consanguineous marriages could be important factors for HNPCC occurrence in Pakistan. Future studies with large sample size along with genetic testing and screening programmes are warranted

Primitive neuroectodermal tumor/Ewing's sarcoma in adult uro-oncology: a case series from a developing country

Peripheral primitive neuroectodermal tumor/Ewing's sarcoma [PNET/EWS] is primarily a tumor of soft tissues and bones. Primary localization of PNET/EWS in genitourinary organs is rare. No data on this localization of PNET/EWS are available in literature from Pakistan. We searched our adult uro-oncology records from 1994 till date and identified all cases of adult genitourinary and adrenal PNET/EWS diagnosed on histology and immunohistochemistry. Their case records were reviewed to obtain data on demographics, presentation, pathologic features, management and outcome. Six cases were found; all were young and had aggressive disease at presentation. Four had renal PNET/EWS. One case each of prostate and adrenal PNET/EWS was seen. Surgery and chemotherapy formed the mainstay of management. Three patients [50%] died during treatment, two were lost to follow-up and one case with renal PNET/EWS showed good initial response to chemotherapy but was later on lost to follow-up. In conclusion, PNET/EWS should be considered in the differential diagnosis of genitourinary malignant tumors in young patients. These tumors are aggressive with poor outcome