scholarly journals The Value of Surgery for Retroperitoneal Sarcoma

Sarcoma ◽  
2009 ◽  
Vol 2009 ◽  
pp. 1-6 ◽  
Author(s):  
Sepideh Gholami ◽  
Charlotte D. Jacobs ◽  
Daniel S. Kapp ◽  
Layla M. Parast ◽  
Jeffrey A. Norton

Introduction. Retroperitoneal sarcomas are uncommon large malignant tumors.Methods. Forty-one consecutive patients with localized retroperitoneal sarcoma were retrospectively studied.Results. Median age was 58 years (range 20–91 years). Median tumor size was 17.5 cm (range 4–41 cm). Only 2 tumors were <5 cm. Most were liposarcoma (44%) and high-grade (59%). 59% were stage 3 and the rest was stage 1. Median followup was 10 months (range 1–106 months). Thirty-eight patients had an initial complete resection; 15 (37%) developed recurrent sarcoma and 12 (80%) had a second complete resection. Patients with an initial complete resection had a 5-year survival of 46%. For all patients, tumor grade affected overall survival (). Complete surgical resection improved overall survival for high-grade tumors ().Conclusions. Tumor grade/stage and complete surgical resection for high-grade tumors are important prognostic variables. Radiation therapy or chemotherapy had no significant impact on overall or recurrence-free survival. Complete surgical resection is the treatment of choice for patients with initial and locally recurrent retroperitoneal sarcoma.

2020 ◽  
Vol 16 (1s) ◽  
pp. 5-8 ◽  
Author(s):  
Nastassja Tober ◽  
Marica Gentile ◽  
Giuseppe Iodice

Retroperitoneal sarcomas are extremely rare malignant tumors. The most common type of sarcomas arising in the retroperitoneum are liposarcomas, occurring mostly in the sixth and seventh decades of life. The only potentially curative approach to liposarcomas is the complete surgical resection of the tumor with negative microscopic margins. However, retroperitoneal liposarcomas exhibit a propensity for local recurrence and distant metastasis despite the negative surgical margins, thus requiring additional therapy. Eribulin demonstrated a benefit in terms of overall survival in patients with advanced or metastatic liposarcoma. We report two cases of patients, both submitted to concomitant right nephrectomy, who experienced a long-lasting control of recurrent retroperitoneal liposarcoma before being submitted to eribulin-based therapeutic regimens (23 and 24 treatment cycles completed, respectively).


2017 ◽  
Vol 3 (2) ◽  
pp. 66-73
Author(s):  
Yuyuan Wang ◽  
Kay Kawai Li ◽  
Ji Xiong ◽  
Zhenyu Zhang ◽  
Yang Wang ◽  
...  

Medulloblastoma (MB) is one of the most common pediatrics malignant tumors of the central nervous system. Studies in America and Europe have demonstrated significant differences in the prognostic value of medulloblastoma extent among molecular subgroups of medulloblastoma. However, studies focusing on the Chinese population are still lacking. A total of 113 patients with medulloblastoma who underwent surgical resection in Huashan Hospital between January 2002 and December 2013 were included in this study. Histological diagnoses were confirmed by 2 or more pathologists. Immunohistochemistry and CTNNB1 exon 3 mutation analysis were used to determine the different subgroups. Complete or incomplete resection was defined based on surgeons' reports and confirmed by postoperative computer tomography (CT). In this study, we included 113 patients with medulloblastoma (13 with WNT subgroup, 18 with SHH subgroup, and 82 with non-SHH/WNT subgroups) to assess their event-free and overall survival. We identified event-free survival and overall survival benefit for complete resection over incomplete resection. We found that for patients with NON-SHH/WNT medulloblastoma, incomplete resection was significantly associated with progression and overall survival compared with complete resection. To our best knowledge, the present study is the first to demonstrate the prognostic value of tumor extent of resection among the molecular subgroups of 113 medulloblastoma in the Chinese population. The prognostic benefit of the increased extent of resection for patients with medulloblastoma is attenuated after the molecular subgroups are taken into account. We still need further study to assess the benefit of surgical resection of small residual portions.


2016 ◽  
Vol 2016 ◽  
pp. 1-2 ◽  
Author(s):  
Aleksandr A. Reznichenko

Background. The concomitant occurrence of a renal cell carcinoma and retroperitoneal sarcoma is extremely rare with only few cases being reported.Methods. We present a case of simultaneous renal cell carcinoma and exceptionally large size retroperitoneal sarcoma involving small intestine. Surgical resection of retroperitoneal sarcoma and simultaneous right nephrectomy were performed.Results. Patient developed recurrent and metastatic disease and underwent debulking surgery following by chemotherapy. Despite aggressive behavior of the retroperitoneal sarcomas, patient is currently (7 years after simultaneous resection and nephrectomy) recurrence-free.Conclusions. Complete surgical resection is the mainstay of therapy for both renal cell carcinoma and retroperitoneal sarcoma. We present a case of simultaneous renal cell carcinoma and exceptionally large size retroperitoneal sarcoma. Debulking surgery and chemotherapy were helpful in our case.


2017 ◽  
Author(s):  
Armen Parsyan ◽  
Abha Gupta ◽  
Charles Catton ◽  
Rebecca Gladdy

Retroperitoneal sarcomas (RPSs) are a heterogeneous group of mesenchymal tumors. The mainstay of treatment of RPS is curative surgical resection, which often involves a multivisceral resection. The predominant pattern of failure in RPS is local recurrence, which poses significant management challenges and limits survival. There is a paucity of high-level evidence to guide the management of primary and recurrent RPS, mainly due to the rarity of the disease. This review highlights the challenges and decision making in the diagnosis and management of recurrent RPS.  This review contains 6 figures, 5 tables and 50 references Key words: chemotherapy, distant recurrence, leiomyosarcoma, liposarcoma, local recurrence, radiation therapy, retroperitoneal sarcoma, surgical resection 


Author(s):  
Maria Paola Ciliberti ◽  
Rosa D'Agostino ◽  
Laura Gabrieli ◽  
Anna Nikolaou ◽  
Angela Sardaro

Hemangiopericytoma (HPC) is an extremely rare hypervascular tumor of mesenchymal lineage. It tends to recur and to develop distant metastases even many years after primary surgical resection. The management of recurrent and metastatic disease is not always so well defined. A complete surgical resection does not eliminate the high risk of local recurrences that occur in the central nervous system, often in the same surgical bed. However, treatment with adjuvant radiotherapy even in cases of complete resection remains controversial. Because of its rarity, there is no standard for treatment. We focused on radiotherapy treatment options, analyzing the literature and making a base on conduct further studies to establish the standard radiation dose to be used for locoregional control of such a complex and extremely rare disease and to help specialists to take the most appropriate therapeutic option.


2013 ◽  
Vol 31 (15_suppl) ◽  
pp. TPS10591-TPS10591 ◽  
Author(s):  
Kristin Baird ◽  
Denise K. Reinke ◽  
Joseph Gerald Pressey ◽  
Leo Mascarenhas ◽  
Noah Federman ◽  
...  

TPS10591 Background: Osteosarcoma is a rare cancer and 33% of patients who have completed primary treatment will recur. The Src pathway has been implicated in the metastatic behavior of several tumors including osteosarcoma where 95% of samples express Src or have evidence of downstream activation of this pathway. Saracatinib (AZD0530) is a potent and selective Src kinase inhibitor. The recommended phase II dose in adults was found to be 175mg daily. The primary goal of this study is to determine if treatment with Saracatinib can increase progression free survival (PFS) for patients who have undergone complete resection of metastatic osteosarcoma nodules in the lung. Secondary goals are evaluation of overall survival, time to treatment failure, and evaluation of several biological correlatives. Methods: This is a multi-institutional, phase II.5, placebo-controlled study with an accrual goals of 88 randomized patients. Patients between 15 and 75 years, with histological confirmation of recurrent osteosarcoma, localized to the lung, who have potential for complete surgical resection, are eligible for enrollment. After complete resection, patients are randomized to treatment with saracatinib or placebo, of a daily oral dose of 175 mg, continuously for up to 1 year or until progression. Patients who recur in the lung while on-study and who are amenable to complete surgical resection will be un-blinded. Those patients who received placebo may have the option to undergo surgical resection. If fully resected, they will be offered therapy with saracatinib under the same treatment guidelines as above. As of January 2013, 38 patients have enrolled and 32 patients met the criteria to be randomized and began oral therapy with either saracatinib or placebo. An interim analysis is planned after 40 patients have been randomized. Clinical trial information: NCT00752206.


2017 ◽  
Vol 35 (15_suppl) ◽  
pp. e15688-e15688
Author(s):  
Nicholas Manguso ◽  
Attiya Harit ◽  
Nicholas N. Nissen ◽  
James Mirocha ◽  
Andrew Eugene Hendifar ◽  
...  

e15688 Background: Management of liver metastasis in patients with small bowel neuroendocrine tumors (SBNET) remains unclear. Complete surgical resection improves long term survival however factors that influence overall prognosis are not clear. Methods: Database review identified 301 patients diagnosed with SBNET from 1990 to 2013. Only patients with known liver metastasis who underwent resection of the primary tumor were included. Outcomes among patients who underwent complete surgical resection, incomplete debulking of liver metastasis, and resection of the primary tumor alone were compared. The Kaplan-Meier method was used for survival estimates and Cox regression was used to identify predictors of death. Results: 111 patients met study criteria. Median age was 59 years (range 16-80); 49% were male. The terminal ileum (47/111, 42%) was the most common primary tumor location. The median number of liver lesions was 8.5 (range 1-31) and median lesions resected was 1 (range 0-31). In addition to resection of the primary tumor, 36 patients (32%) had no liver resection (NR), 41 (36.9%) had complete resection of liver disease (R0) and 34 (30%) had incomplete resection of liver metastasis (R1). 58 patients (36%) had one or more wedge resections, 12 (10.8%) underwent segmentectomy and 5 (4.5%) had a lobectomy. 33 (29.7%) patients underwent post-operative chemoembolization, 25 (22.5%) had radioembolization and 23 (20.7%) had radiofrequency ablation. The R1 group differed from the R0 group in median size of primary tumor (2.5 cm R1 vs 1.6 cm R0, p = 0.05) and median number of positive lymph nodes (5.0 R1 vs 3.0 R0, p = 0.05). The 5-year OS was 80.9%, 81.1% and 100% for NR, R1 and R0 groups respectively (p = 0.01). 10-year OS did not differ between groups (72.8% NR vs 81.1% R1vs 82.5% NR, p = 0.31). Cox regression showed post-operative administration of chemotherapy (HR = 3.68, p < 0.01) and higher tumor grade (HR = 18.4, p = 0.02) increased risk of death. Conclusions: In patients with SBNET with liver metastasis, higher tumor grade and post-operative chemotherapy increased risk of death. However, resection of the primary tumor along with liver metastasis improves the 5-year OS with complete cytoreduction providing the most benefit.


2017 ◽  
Author(s):  
Armen Parsyan ◽  
Abha Gupta ◽  
Charles Catton ◽  
Rebecca Gladdy

Retroperitoneal sarcomas (RPSs) are a heterogeneous group of mesenchymal tumors. The mainstay of treatment of RPS is curative surgical resection, which often involves a multivisceral resection. The predominant pattern of failure in RPS is local recurrence, which poses significant management challenges and limits survival. There is a paucity of high-level evidence to guide the management of primary and recurrent RPS, mainly due to the rarity of the disease. This review highlights the challenges and decision making in the diagnosis and management of recurrent RPS.  Key words: chemotherapy, distant recurrence, leiomyosarcoma, liposarcoma, local recurrence, radiation therapy, retroperitoneal sarcoma, surgical resection 


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