Primary localized retroperitoneal sarcomas: report from Slovenian sarcoma referral center.

BACKGROUND: Sarcoma patients should be treated in high volume referral sarcoma centers. Compartmental resection is proposed as the best treatment option in retroperitoneal sarcoma patients. METHODS: Institute of Oncology Ljubljana is the only referral sarcoma center in Slovenia. Having a population of 2.1 million poses a unique situation. We manage all sarcoma patients in the country and operate on patients with soft tissue tumors of extremities, trunk, and abdomen. Data for all consecutive patients surgically treated from January 1999 to December 2018 for primary localized retroperitoneal sarcoma was extracted from a prospective surgical database. Data about the incidence of sarcoma patients in Slovenia was extracted from the Cancer Registry of Republic of Slovenia. Clinicopathologic variables and the outcome were analyzed. RESULTS: In total, 89 patients were included in the study. Median age was 62 years. Dedifferentiated liposarcoma was the most common histology (38.2%). Median tumor size was 21 cm. Compartmental resection was performed in 47.2% (42/89). Postoperative complication grade 3a or higher according to Clavien-Dindo classification had 30.3% (27/89) of patients. The 30-day and 90-day mortality rate was 2.2% and 5.6%. Median follow-up was 62.1 months. Corresponding 5-year overall survival was 67.2%, 5-year disease-specific survival was 72.6%, and 5-year local recurrence-free survival was 81.5%, respectively. CONCLUSION: Results from our institution show that referral sarcoma centers may achieve very good results in management of retroperitoneal sarcoma patients, despite not meeting the criteria for high volume hospitals, as long as they have multidisciplinary team, appropriate facilities, and expertise.

Leiomyosarcoma of the renal vein: analysis of outcome and prognostic factors in the world case series of 67 patients.

BACKGROUND: Leiomyosarcoma is a rare malignant mesenchymal tumour. Some cases of leiomyosarcoma of the renal vein (LRV) have been reported in the literature, but no analysis of data and search for prognostic factors have been done so far. The aim of this review was to describe the LRV, to analyse overall survival (OS), local recurrence free survival (LRFS) and distant metastases free survival (DMFS) in LRV world case series and to identify significant predictors of OS, LRFS and DMFS. METHODS: Cases from the literature based on PubMed search and a case from our institution were included. RESULTS: Sixty-seven patients with a mean age of 56.6 years were identified; 76.1% were women. Mean tumour size was 8.9 cm; in 68.7% located on the left side. Tumour thrombus extended into the inferior vena cava lumen in 13.4%. All patients but one underwent surgery (98.5%). After a median follow up of 24 months, the OS was 79.5%. LRFS was 83.5% after a median follow up of 21.5 months and DMFS was 76.1% after a median follow up of 22 months. Factors predictive of OS in univariate analysis were surgical margins, while factors predictive of LRFS were inferior vena cava luminal extension and grade. No factors predictive of DMFS were identified. In multivariate analysis none of the factors were predictive of OS, LRFS and DMFS. CONCLUSIONS: Based on the literature review and presented case some conclusions can be made. LRV is usually located in the hilum of the kidney. It should be considered in differential diagnosis of renal and retroperitoneal masses, particularly in women over the age 40, on the left side and in the absence of haematuria. Core needle biopsy should be performed. Patients should be managed by sarcoma multidisciplinary team. LRV should be surgically removed, with negative margins.

Aggressive surgical treatment of retroperitoneal sarcoma: long-term experience of a single institution.

Surgery is the main modality in the therapy of retroperitoneal soft-tissue sarcomas (RSTS). A retrospective study was undertaken to evaluate the results of aggressive surgical treatment in a series of patients of primary and recurrent retroperitoneal sarcomas. A review of 166 consecutive patients with RSTS operated on at the Institute of Oncology in Ljubljana from 1975 through 2005 were reviewed. A total of 269 operations were performed on 166 patients. The five- and ten-year survival rates of patients with localized sarcoma were 52% and 38%, respectively. Factors that influenced the survival were distant metastases, tumor grade, and type of resection. The patients with R0 resections had a five-year survival rate of 75% and a ten-year survival rate of 65%; the respective rates for the patients with R1 resections were 25% and 7% (p < 0.00001). When only R0 resection was considered, referral status (primary, residual, recurrent RSTS) influenced survival (p = 0.004). The quality of initial surgery is a crucial prognostic factor to predicting survival in patients with RSTS. Complete surgical resection without microscopic residuum and contamination is likely to offer the best chances for long-term survival. Unless no other treatment modalities are available, aggressive surgery for recurrent sarcoma is recommended.

Retroperitoneal sarcoma: 25 years of experience with aggressive surgical treatment at the Institute of Oncology, Ljubljana.

BACKGROUND AND OBJECTIVES: Retroperitoneal sarcomas are rare malignant tumors with an aggressive course of disease and high local recurrence rate. Local disease is the main cause of death. A retrospective study was undertaken to evaluate the results of aggressive surgical treatment in a series of patients of primary and recurrent retroperitoneal sarcomas. PATIENTS AND METHODS: From 1975 to 2000, 155 patients with retroperitoneal tumors were operated on at the Institute of Oncology, Ljubljana. Patients with fibromatosis and children less than 16 years were excluded from further calculation. Out of the rest 139 patients, 102 patients had localized retroperitoneal sarcoma, and 37 patients had retroperitoneal sarcoma with distant metastases Leiomyosarcoma was the most common type of histology, followed by liposarcoma and schwannoma. Their referral status was as follow: 56 had primary sarcoma, 20 residual sarcoma after operation elsewhere and 26 already recurrent sarcoma. Our treatment approach was aggressive. We removed surgically primary tumor, recurrent sarcoma and metastases, whenever possible. To this end, we performed, 235 operations for retroperitoneal sarcomas on 139 patients (up to 8 operations on 1 patient). RESULTS: Complete resection was done in 97 of 102 patients with localized sarcoma (resectability rate 95%); in 55 patients, R0 resection was made, in 42 patients, R1 resection, in 3 patients, debulking procedure, and in 2 patients biopsy alone. In 118, patients at least one organ was resected completely or partially en bloc with tumor. The 5-, 10-, and 15-year survivals of patients with localized sarcoma were 52%, 36%, and 22%, respectively. The 5-year survival of patients with metastases was 12% (P = 0.0002), the 5- and 10-year survival rates of patients with R0 resection were 75% and 64%, respectively, and of those with R1 resection, 25% and 8%, respectively (0 < 0.00001). High tumor grade was associated with poor survival. The 5-year local recurrence rate of patients with primary sarcoma and of those with locally recurrent sarcoma was 37% and 71%, respectively (P = 0.04). After the first local recurrence, the 5- and 10-year survival rates were 42% and 26%, after the second local recurrence 45% and 22%, after the third local recurrence 43% and 11%, and after the fourth local recurrence 51% and 17%, respectively. CONCLUSION: Complete surgical resection without microscopic residuum and contamination is likely to offer the best chances for long-term survival. Until there are no other treatment modalities, aggressive surgery for recurrent sarcoma is recommended.

Site of primary tumor has a prognostic role in operable breast cancer: the international breast cancer study group experience.

PURPOSE: Cancer presenting at the medial site of the breast may have a worse prognosis compared with tumors located in external quadrants. For medial tumors, axillary lymph node staging may not accurately reflect the metastatic potential of the disease. PATIENTS AND METHODS: Eight-thousand four-hundred twenty-two patients randomly assigned to International Breast Cancer Study Group clinical trials between 1978 and 1999 were classified as medial site (1,622; 19%) or lateral, central, and other sites (6,800; 81%). Median follow-up was 11 years. RESULTS: A statistically significant difference was observed for patients with medial tumors versus those with nonmedial tumors in disease-free survival (DFS; 10-year DFS, 46% v 48%; HR, 1.10; 95% CI, 1.02 to 1.18; P = .01) and overall survival (10-year OS 59% v 61%; HR, 1.09; 1.01 to 1.19; P = .04). This difference increased after adjustment for other prognostic factors (HR, 1.22; 95% CI, 1.13 to 1.32 for DFS; and HR, 1.24; 95% CI, 1.14 to 1.35 for OS; both P = .0001). The risk of relapse for patients with medial presentation was largest for the node-negative cohort and for patients with tumors larger than 2 cm. In the subgroup of 2,931 patients with negative axillary lymph nodes, 10-year DFS was 61% v 67%, and OS was 73% v 80% for medial versus nonmedial sites, respectively (HR 1.33; 95% CI, 1.15 to 1.54; P = .0001 for DFS; and HR 1.40; 95% CI, 1.17 to 1.67; P = .0003 for OS). CONCLUSION: Tumor site has a significant prognostic utility, especially for axillary lymph node-negative disease, that should be considered in therapeutic algorithms. New staging procedures such as biopsy of the sentinel internal mammary nodes or novel imaging methods should be further studied in patients with medial tumors.