Abbreviations & Acronyms B = bladder HR = hazard ratio K = kidney LRFS = local recurrence-free survival MFS = metastasis-free survival NR = not reported OS = overall survival P = paratesticular RMS = rhabdomyosarcoma Objectives: To report our institutional experience in the management of adult genitourinary sarcoma. Methods: This was a retrospective analysis of data on adult genitourinary sarcoma treated at the West China Hospital, Sichuan University, Chengdu, Sichuan, China from 1985 to 2010. Clinicopathological parameters were analyzed to determine their impact on overall, recurrence-free and metastasis-free survivals. Results: A total of 46 women and 142 men were included, with a median age of 42 years. Of these, 152 cases were high-grade. The most common site was the paratesticular region. Surgical resection was carried out in 155 patients (82.4%), with negative margin in 106. After a minimum follow up of 5 years, 20 patients (11.6%) survived disease-free, 14 (8.1%) were alive with disease and 138 (80.2%) died of disease. Survival rates at 1, 3 and 5 years were 91.3%, 64.0% and 47.7%. In univariate analyses, liposarcoma, high grade, metastasis at diagnosis, a lack of surgical resection and positive margin were predictive of unfavorable survival. In multivariate analyses, high grade, a lack of surgical resection and chemotherapy were independent predictors of poor survival. Conclusions: Adult genitourinary sarcoma is an aggressive malignancy, usually presenting at advanced stage, with a high incidence of recurrence and metastasis. Complete resection and selective combination of chemotherapy and radiotherapy might constitute the optimal treatment for this disease.
OBJECTIVE:To summarize the clinical and spiral computed tomography (CT) features of undifferentiated embryonic liver sarcoma (UELS) in adults.
METHODS:Clinical and CT findings of four adult patients with pathology-approved UELS were retrospectively analyzed with a literature review. All patients were examined using a plain and enhanced spiral CT scan.
RESULTS:The participants were two men and two women with a mean age of 36.5 years. The clinical manifestations were the right upper quadrant mass, pain (n = four) and intermittent fever (n = three). Laboratory tests showed a mild elevation of alphafetoprotein in one patient and mildly abnormal liver function in two. Liver cirrhosis was present in one patient coexistent with hepatocellular carcinoma. CT findings indicated a huge well-defined cystic-solid mass with varying degrees of enhancement of the solid component. Irregular high-density lesions showing hemorrhage occurred in three patients and the inferior vena cava was compressed in one.CONCLUSION: UELS showed a solitary and predominantly cystic or multicystic appearance on CT imaging compared with ultrasound (US) from the medical literature, which shows a predominantly hypoechoic mass. The laboratory tests were normal or mildly abnormal despite the presence of the large mass. The combination of these clinical features and CT findings together with those of US are helpful in making a prospective diagnosis.
Pulmonary sequestration is a rare congenital malformation mostly located in the thorax, while intra-abdominal pulmonary sequestration is an extremely rare type of pulmonary sequestration usually diagnosed during the first 6 months of life. Only 1 case of intra-abdominal pulmonary sequestration in a patient older than 60 years has been reported in the current literature. It is difficult to differentiate an intra-abdominal pulmonary sequestration from other retroperitoneal tumors. A definitive diagnosis is always made by histologi- cal examination. Intra-abdominal pulmonary sequestration commonly responds well to surgical resection and is associated with excellent results and prognosis. The authors present the case of a 74-year-old asymptomatic man with a retroperitoneal mass which was completely excised and revealed by histopathological study to be an intra-abdominal pulmonary sequestration.
Renal vascular deformity (RVD) happens during the embryonic period when kidneys ascend into the lumber region. However, the exact etiology of this deformity remains unknown. RVD is not a rare congenital anomaly in urology as many cases with vascular deformity have been reported.However, no case has been reported with unilateral kidney almost in the normal position, but with several different branches of ectopic arteries-one even receiving the arterial supply from the contralateral iliac artery-and ectopic veins, one of which forms a collateral circulation with the portal vein. Moreover, it is quite interesting that this case of RVD is combined with another abnormality-duplex pelvis and renal malrotation. We will present this extremely rare case of multiple malformation of the right renal vascular deformity combined with duplex pelvis and malrotation.
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