Chondrosarcoma of right big toe with metastases to left orbital and left infra temporal region.

To report a case of chondrosarcoma of right big toe with left orbital and left infra temporal metastases. Chondrosarcoma is the second most frequent primary malignant tumour of the bone. A 56 year old man had history of trauma on his right big toe, which was amputated and the biopsy in 2011 at Sindh Institute of Urology and Transplantation (SIUT) revealed chondrosarcoma with clear margins. Eventually the patient was presented with swelling of the left eye, pain and gradual loss of vision of that eye. Later a CT scan of his chest, brain and orbit showed pulmonary and pleural based nodule, with mediastinal and hilar lymphadenopathy representing metastatic deposit in left orbit, extending to left infra temporal region. A treatment of palliative chemotherapy was started with doxorubicin and ifosfamide, after which he was referred for radiotherapy. At that time he had loss of vision, pain and exopthalamus, and palliative radiotherapy was delivered to the left orbit with the prescribed dose of30 Gy/300cGy×10 fraction. Thereafter his case will be followed up at the oncology OPD after a 03 month interval.

Outcome of trimodality protocol for invasive bladder cancer patients at Karachi, Pakistan.

OBJECTIVES: To evaluate the outcomes of patients with muscle invasive bladder cancer managed by trimodality protocol with assessment of factors that may predict treatment response, risk of recurrences and survival of such patients in our population. METHODS: A prospective, single arm study was conducted between July 2006 and December 2009 at the Sindh Institute of Urology and Transplantion, Karachi. One hundred and sixteen patients with muscle invasive bladder cancer T2-T3N0M0 were treated with concurrent chemoradiation (total dose 6500 cGy) after maximal transurethral resection. Complete response was defined as no tumour seen on check cystoscopy and biopsy. The disease control and overall survival were determined by Kaplan and Meier method and statistical inferences with the log-rank test. Cox regression analysis was used to find different prognostic factors. RESULTS: At the median follow up of 36 months (14-43), out of total 116, 62(51.6%) surviving patients the bladder was functioning well, while 18(15%) had local recurrence; in 9 patients superficial tumour recurred and required further transurethral resection and intravesical drug therapy and nine patients who had muscle invasive recurrence; underwent radical cystectomies. Concurrent chemoradiation was well tolerated. The overall survival at 3 years was 54%. Initial complete response, primary tumour stage and absence of hydronephrosis were the most important prognostic factors for survival (all p=<0.0001). CONCLUSION: Trimodality treatment was found to be an effective therapy in patients with invasive bladder cancer and complete TURBT, tumour stage and no hydronephrosis at time of presentation were found important prognostic factors for treatment response, disease free and over all survival rates.

Renal transplant in a tuberous sclerosis patient with bilateral giant renal angiomyolipomas and concurrent renal carcinoma.

Co-existence of angiomyolipoma (AML) and renal cell carcinoma (RCC) in the same tumor mass is very rare and only eight cases have been reported. We present a case of a young female with tuberous sclerosis complex (TSC) with bilateral huge renal AMLs. Both tumors were removed, one of which revealed co-incidental RCC. She was subsequently successfully transplanted a kidney from her brother and is maintaining normal graft function eight months post-transplant. No recurrence or metastases of RCC has been detected till the last follow-up.

Renal tumors in young adults: a single-center experience from a developing country.

PURPOSE: To determine the pattern and outcome of renal tumors in young adults in a large surgical series in Pakistan. MATERIALS AND METHODS: We retrospectively analyzed 133 young adults (age: ≥ 16 to ≤ 40 years) with 136 renal tumors, who underwent surgical treatment for suspected renal cancer from 1994 till 2010. The clinical and pathological parameters were determined and their impact on final outcome was analyzed. RESULTS: The mean age of the patients was 33.3 ± 6.2 years. Of 136, 121 (88.9%) renal tumors were malignant and 15 (11%) were benign. Among malignancies, 76 (62.7%) patients had stage I or II tumors, 22 (18.1%) stage III, and 23 (19%) stage IV at surgery. The overall cancer-specific survival for malignant tumors at 1, 5, and 10 years was 97%, 83%, and 83%, whereas the cancer-free survival (CFS) was 80%, 63%, and 37%, respectively. Patients with age ≤ 35 years had 1 and 5-year CFS of 83% and 71%, respectively, as compared with 76% and 49% for patients > 35 years (P = .02; odds ratio = 2.3; P = .03). Regarding tumor size, 1 and 5-year CFS for tumors ≤ 10 cm was 93% and 75%, while tumors > 10 cm showed CFS of 56% and 41%, respectively (P = .0001; odds ratio = 4.2; P = .0001). For stage I tumors, CFS at 1 and 5 years was 98% and 84%; for stage II, 82% and 63%; and for stage III, 62% and 50%,respectively. One-year survival for stage IV was 48% only (P = .0001). CONCLUSION: A wide heterogeneity of renal tumors is seen in young adults with delayed presentation.

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.