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Background: A multi-institutional nomogram for predicting disease-free survival (DFS) and overall survival (OS) in patients with primary retroperitoneal sarcoma (RPS) incorporating relevant prognostic factors not included in the American Joint Committee on Cancer staging system for soft tissue sarcoma has been reported. The authors validated this nomogram with an independent, transatlantic cohort., Methods: Data from patients with RPS who were undergoing definitive resection at 1 of 6 sarcoma centers in Europe and North America ("validation set") were used to validate a RPS nomogram developed from 3 other centers ("development set"). The nomogram incorporated 6 variables: age, tumor size, grade, histologic subtype, multifocality, and quality of surgery. Nomogram-predicted probabilities were stratified into 6 subgroups and compared with observed outcomes. Discriminative ability was quantified by Harrell C statistics., Results: The validation and development sets included 631 and 523 patients, respectively, all of whom underwent surgical resection at the institutions represented. The 7-year DFS and OS rates for the validation set were 38% (95% confidence interval, 34%-43%) and 58% (95% confidence interval, 53%-63%), respectively. All 6 nomogram variables were found to be independently prognostic. The corrected Harrell C statistics concordance index values for the validation set were 0.69 for DFS and 0.73 for OS, which were similar to those for the development set, suggesting good calibration of the nomogram in the validation cohort., Conclusions: The RPS nomogram was externally validated using a larger, independent cohort. The nomogram can be generalized to patients undergoing surgery for RPS by specialized sarcoma surgeons at sarcoma centers. The nomogram provides a more individualized and disease-relevant estimation of OS compared with the American Joint Committee on Cancer classification. Cancer 2016;122:1417-1424. © 2016 American Cancer Society., (© 2015 American Cancer Society.)

Background: Extragonadal germ cell tumors (EGCT) represent only 2-5% of adult germ cell malignancies. Because they are rare and biologically distinct from testis cancer, their natural history and optimal management continue to be defined. The clinical characteristics, treatment, and outcome of 40 patients are presented here., Methods: Patients were identified through the medical records of four University of Toronto teaching hospitals. All patients were treated in specialized oncology units between 1978 and 1993., Results: Thirty-seven males and three females age 16-54 years (median, 24 years) with primary mediastinal (n = 24), retroperitoneal (n = 7), CNS (n = 7), and widespread (n = 2) EGCT were identified. Eight of nine patients (88%) with mediastinal seminoma are alive with no evidence of disease (NED) at 4-132 months (median, 45 months). After combined modality therapy, only 8 of 15 patients (53%) with mediastinal nonseminomas achieved complete remission (CR); 1 experienced relapse and died, resulting in 7 of 15 patients (47%) with NED at 45-86 months (median, 70 months). All three patients with retroperitoneal seminomas achieved CR and all have NED at 77, 103, and 120 months, respectively. Two of four patients with retroperitoneal nonseminomas have died, and the other two are alive at 36 and 54 months. Seven patients with CNS germinomas (seminoma) achieved CR after craniospinal radiation therapy, but one subsequently died after local relapse. The overall survival rate was 87% (median, 74 months). One patient with widespread choriocarcinoma died and the other achieved CR., Conclusions: Regardless of site of presentation, extragonadal seminomas have a greater than 80% 5-year disease-free survival rate. Mediastinal nonseminomas are biologically distinct, with a poorer prognosis. Treated with cisplatin-based chemotherapy followed by aggressive resection, approximately 50% of patients survive. CNS seminomas have a good prognosis. Nonseminomas of the CNS are extremely rare and were not represented in the current series. These findings concur with other reported series.

Metastatic disease adjacent to major vascular structures in the retroperitoneum sometimes necessitates planned removal of portions of these vessels, or the vessels may inadvertently be injured when retroperitoneal lymphadenectomy is performed. In 78 patients who underwent retroperitoneal lymphadenectomy, 17 (22%) required vascular repair intraoperatively. The vena cava was most frequently involved (eight cases) followed by inadvertent injury to the renal arteries (five cases) or the infrarenal aorta (three cases). Resection of the infrarenal aorta was planned in two cases without postoperative complication. In six cases a resection of either the whole inferior vena cava or a portion of it was needed to remove all of the tumour. There were nine cases of inadvertent injury to the aorta, vena cava or a renal artery. The outcome after arterial repair was satisfactory with the exception of the inadvertent renal artery injuries, which required nephrectomy in three cases. Leg swelling was not a long-term sequela of either caval repair or resection.