Compartmental resection of a retroperitoneal sarcoma

Abstract 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 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. 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.

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Primary localized retroperitoneal sarcomas: report from Slovenian sarcoma referral center

World Journal of Surgical Oncology ◽

10.1186/s12957-020-02038-9 ◽

2020 ◽

Vol 18 (1) ◽

Author(s):

Marko Novak ◽

Andraž Perhavec ◽

Milena Kerin Povšič ◽

Matej Arnuš ◽

Darja Eržen

Keyword(s):

High Volume ◽

Soft Tissue Tumors ◽

Retroperitoneal Sarcoma ◽

Free Survival ◽

Median Tumor Size ◽

Compartmental Resection ◽

Retroperitoneal Sarcomas ◽

Local Recurrence Free Survival ◽

Disease Specific

Abstract 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.

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Primary Localized Retroperitoneal Sarcomas: Report from Slovenian Sarcoma Referral Center.

10.21203/rs.3.rs-42717/v1 ◽

2020 ◽

Author(s):

Marko Novak ◽

Andraz Perhavec ◽

Milena Kerin Povsic ◽

Matej Arnus ◽

Darja Erzen

Keyword(s):

High Volume ◽

Soft Tissue Tumors ◽

Retroperitoneal Sarcoma ◽

Recurrence Free Survival ◽

Free Survival ◽

Compartmental Resection ◽

Retroperitoneal Sarcomas ◽

Local Recurrence Free Survival ◽

Disease Specific

Abstract Background: Sarcoma patients should be treated in high volume referral sarcoma centers. Compartmental resection is proposed as best treatment option in retroperitoneal sarcoma patients. Methods: Institute of Oncology Ljubljana is the only referral sarcoma center in Slovenia. Having 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 prospective surgical database. Clinicopathologic variables and the outcome were analyzed.Results: In total 89 patients were included. Mean age was 59.2 years. Dedifferentiated liposarcoma was the most common histology (38.2 %). Mean tumor size was 23.5 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.

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Surgical outcome and patterns of recurrence for retroperitoneal sarcoma at a single centre

Annals of The Royal College of Surgeons of England ◽

10.1308/rcsann.2016.0057 ◽

2016 ◽

Vol 98 (03) ◽

pp. 192-197 ◽

Cited By ~ 5

Author(s):

HDJ Hogg ◽

DM Manas ◽

D Lee ◽

P Dildey ◽

J Scott ◽

...

Keyword(s):

Systemic Disease ◽

Survival Rates ◽

Disease Free Survival ◽

Disease Recurrence ◽

Retroperitoneal Sarcoma ◽

Neoadjuvant Radiotherapy ◽

Invasive Tumour ◽

Compartmental Resection ◽

Low Incidence ◽

Disease Free

Introduction Retroperitoneal sarcoma is a surgically managed condition that can recur locally following macroscopically complete resection. Owing to the low incidence of the condition, advances in treatment are reported infrequently but complete compartmental resection and adjuvant or neoadjuvant radiotherapy are areas under investigation. Given the practical difficulty of randomised trials, observational data can highlight advantages from progressive treatment approaches. Methods A retrospective database of consecutive retroperitoneal sarcoma resections performed at a single referral centre between March 1997 and March 2013 was interrogated. Histological, radiological and clinical data were collected. Univariate and multivariate analyses for disease free and overall survival were performed to establish independent predictors of disease recurrence and patient survival. Results A total of 79 patients underwent 90 resections (63 primary). The mean five-year overall and disease free survival rates were 55.3% and 24.8% respectively. Higher patient age, high tumour grade, presence of extraretroperitoneal disease and invasive tumour phenotype were found to significantly predict survival following multivariate analysis. Half (50%) of the tumours displayed invasive behaviour on histopathology and 42% of locoregional recurrence was intraperitoneal. Conclusions Retroperitoneal sarcoma is commonly an infiltrative tumour and often recurs outside of the retroperitoneum. These features limit the therapeutic impact of interventions that focus on gaining local control such as complete compartmental resection and radiotherapy. It seems likely that future advances in the management of this cancer will involve new systemic agents to treat this frequently systemic disease.

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Featured Clinical Trial: Preoperative Radiotherapy for Retroperitoneal Sarcoma

PsycEXTRA Dataset ◽

10.1037/e481372006-006 ◽

2005 ◽

Author(s):

Keyword(s):

Clinical Trial ◽

Preoperative Radiotherapy ◽

Retroperitoneal Sarcoma

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Comparison of comprehensive complication index and Clavien–Dindo classification in patients with retroperitoneal sarcoma

Journal of Surgical Oncology ◽

10.1002/jso.26612 ◽

2021 ◽

Author(s):

Fabio Tirotta ◽

Alessandro Parente ◽

Thomas Richardson ◽

Ahmed Almonib ◽

Caroline Evenden ◽

...

Keyword(s):

Retroperitoneal Sarcoma ◽

Comprehensive Complication Index

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PO-1233: Perioperative Radiotherapy with a Moderate Dose-Escalation for Retroperitoneal Sarcoma (RPS).

Radiotherapy and Oncology ◽

10.1016/s0167-8140(21)01251-2 ◽

2020 ◽

Vol 152 ◽

pp. S649

Author(s):

M. Nuñez Baez ◽

A. Montero ◽

X. Chen-Zhao ◽

A. Acosta ◽

B. Alvarez ◽

...

Keyword(s):

Dose Escalation ◽

Retroperitoneal Sarcoma ◽

Moderate Dose

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Doege-Potter syndrome presenting as ‘end-stage renal disease-associated hypoglycaemia’: a primary presentation of retroperitoneal sarcoma

BMJ Case Reports ◽

10.1136/bcr-2020-235549 ◽

2020 ◽

Vol 13 (8) ◽

pp. e235549

Author(s):

Skand Shekhar ◽

Julie Chen ◽

Kaniksha Desai

Keyword(s):

Renal Disease ◽

End Stage Renal Disease ◽

Haemorrhagic Shock ◽

Retroperitoneal Sarcoma ◽

Obstructive Nephropathy ◽

Aged Woman ◽

Surgical Debulking ◽

Middle Aged Woman ◽

Stage Renal Disease ◽

End Stage

A middle-aged woman with end-stage renal disease (ESRD) due to obstructive nephropathy presented to the hospital for an episode of unresponsiveness and hypoglycaemia. Initially, she was diagnosed with hypoglycaemia associated with ESRD and was discharged. However, she returned to the hospital after experiencing tonic–clonic seizures and recurrent hypoglycaemia. Her hypoglycaemia workup revealed an elevated insulin-like growth factor 2 (IGF2) to IGF1 ratio consistent with paraneoplastic IGF2 secretion. Subsequently, a CT abdomen revealed a retroperitoneal mass, found to be a retroperitoneal sarcoma. Her hypoglycaemia was treated with glucocorticoids and growth hormone. Surgical debulking of her tumour was attempted, but she expired due to postoperative haemorrhagic shock. Doege-Potter syndrome is a rare cause of hypoglycaemia which should be suspected in any new-onset, worsening, inexplicable or refractory hypoglycaemia, particularly in non-diabetic ESRD. Here we present a report of retroperitoneal sarcoma presenting with hypoglycaemia in a patient with ESRD without diabetes.

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