Multidisciplinary Management of Retroperitoneal Sarcoma: Diagnosis, Prognostic Factors and Treatment

Carbone, Fabio; Pizzolorusso, Antonio; Lorenzo, Giuseppe Di; Marzo, Massimiliano Di; Cannella, Lucia; Barretta, Maria Luisa; Delrio, Paolo; Tafuto, Salvatore

doi:10.3390/cancers13164016

Cited by 19 publications

(27 citation statements)

References 103 publications

(131 reference statements)

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“…Multifocal disease has profound effects on the oncological outcomes of RPLS patients (see Table S4). In a recent study, 20% of patients presented with multifocal disease, and the 5-year OS rate was significantly lower in the multifocal group than the unifocal group (8,(22)(23)(24)(25). In another study, 25% of RPLS patients presented with multifocal disease with curtailed OS (24).…”

Section: Discussionmentioning

confidence: 99%

A better overall survival (OS) for total (ipsilateral) retroperitoneal lipectomy than standard complete resection in patients with retroperitoneal liposarcoma: a comparative multi-institutional study

Miao¹,

Zhang²,

Tseng³

et al. 2022

Ann Transl Med

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Background: Complete resection (CR) serves as the standard of surgical treatment for retroperitoneal liposarcoma (RPLS). Unfortunately, even at referral centers, recurrence rates are high, and CR may not address multifocal diseases, which are a common phenomenon in RPLS. We sought to retrospectively compare the clinical outcomes of RPLS patients treated with total (ipsilateral) retroperitoneal lipectomy (TRL) and CR. Because TRL remove potentially multifocal tumors in the fat, patients may have a better prognosis than CR.Methods: Patients with primary/first-recurrent RPLS who had been treated at 5 referral centers were recruited from December 2014 to June 2018. Multivariable Cox regression analyses were conducted to determine the effects of demographic, operative, and clinicopathological variables on the following primary endpoints: local recurrence (LR), local recurrence-free survival (LRFS), and overall survival (OS).Results: A total of 134 patients were enrolled in this retrospective study, 53 of whom underwent TRL, and 81 of whom underwent CR. The 2 groups were comparable in terms of age, gender, presentation (primary vs. first-recurrent RPLS), number of tumors (unifocal vs. multifocal) at presentation, and Fédération Nationale des Centres de Lutte Contre le Cancer (FNCLCC) grade. The TRL group had higher levels of preoperative hemoglobin (Hb) (13 vs. 12.5 g/dL; P=0.008) and a lower amount of intraoperative blood loss (400 vs. 500 mL; P=0.034), but there were no significant differences in the length of hospital stay (23 vs. 22 d; P=0.47) or complications (32 vs. 30; P=0.82) between the 2 groups. In a subset of patients with multifocal tumors at initial presentation, OS was more prolonged in those treated with TRL than those treated with CR (P=0.0272). Based on the multivariable analysis, primary liposarcoma and a low FNCLCC grade were associated with decreased LR and improved OS.Conclusions: TRL is a safe procedure that positively affects the OS of patients with multifocal RPLS. This novel strategy deserves further investigation in prospective studies.

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Section: Discussionmentioning

confidence: 99%

A better overall survival (OS) for total (ipsilateral) retroperitoneal lipectomy than standard complete resection in patients with retroperitoneal liposarcoma: a comparative multi-institutional study

Miao¹,

Zhang²,

Tseng³

et al. 2022

Ann Transl Med

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“…Treatment methods for LMSs other than surgery have not yet shown any clear benefits; therefore, complete resection is the only established therapy. However, there are some trials for this [ 18 , 19 ]. Determining the treatment for LMSs is complex and cases should be discussed among multidisciplinary team members.…”

Section: Discussionmentioning

confidence: 99%

Abnormal growth of a pleomorphic leiomyosarcoma originating from the mesenteric vein associated with poor outcome after curative-intent resection: a case report

et al. 2022

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Background A leiomyosarcoma (LMS) is a rare tumor that mainly originates from the urinary tract and digestive system; however, non-visceral organ-derived patterns are rare. Herein, we report that a vessel-derived pleomorphic LMS (PLMS) originating from the mesenteric vein has a poor prognosis even after curative-intent surgery. Case presentation The patient was a 41-year-old woman with no relevant medical history. The patient presented with abdominal pain and an abnormal bulge on the left lower abdomen. Magnetic resonance imaging revealed a large tumor occupying the left abdomen. Enhanced computed tomography revealed a bulky tumor with a maximum size of 13 × 13 cm with impending rupture, and a small amount of ascites was detected around the tumor. As the tumor directly invaded the small intestine and descending colon, left hemicolectomy and partial resection of the small intestine were performed. The patient was discharged on postoperative day 10, without any complications. On histopathological analysis, the tumor was diagnosed as a vessel-derived LMS with a pleomorphic pattern. The patient died due to disseminated intravascular coagulation because generalized peritonitis was induced by the super-early recurrence of the tumor 2 months after the surgery. Conclusions Regardless of curative-intent surgery for a vessel-derived PLMS, super-early local recurrence and distant metastasis were observed. A vessel-derived PLMS requires further investigation to determine its characteristics and therapeutic strategies to improve long-term prognosis.

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“…Malignant fibrous histiocytomas were historically the most common entity within the retroperitoneum; however with advances in histopathology, such tumors have been re-evaluated to represent leiomyosarcomas or dedifferentiated sarcomas [ 10 ]. The average prevalence of each subtype was estimated by Carbone et al after reviewing 8 of the largest case series [ 11 ] and illustrated in Figure 2 . To add to their complexity, each subtype displays different behavior, making these tumors exceedingly difficult to treat [ 6 , 9 , 12 , 13 , 14 ].…”

Section: Histologic Subtypesmentioning

confidence: 99%

Retroperitoneal Sarcoma Care in 2021

Schmitz

Nessim

2022

Cancers

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Soft-tissue sarcomas are biologically heterogenous tumors arising from connective tissues with over 100 subtypes. Although sarcomas account for <1% of all adult malignancies, retroperitoneal sarcomas are a distinct subgroup accounting for <10% of all sarcomatous tumors. There have been considerable advancements in the understanding and treatment of retroperitoneal sarcoma in the last decade, with standard treatment consisting of upfront primary surgical resection. The evidence surrounding the addition of radiation therapy remains controversial. There remains no standard with regards to systemic therapy, including immunotherapy. Adjunctive therapy remains largely dictated by expert consensus and preferences at individual centers or participation in clinical trials. In this 2021 review, we detail the anatomical boundaries of the retroperitoneum, clinical characteristics, contemporary standard of care and well as recent advancements in retroperitoneal sarcoma care. Ongoing international collaborations are encouraged to advance our understanding of this complex disease.

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Multidisciplinary Management of Retroperitoneal Sarcoma: Diagnosis, Prognostic Factors and Treatment

Cited by 19 publications

References 103 publications

A better overall survival (OS) for total (ipsilateral) retroperitoneal lipectomy than standard complete resection in patients with retroperitoneal liposarcoma: a comparative multi-institutional study

A better overall survival (OS) for total (ipsilateral) retroperitoneal lipectomy than standard complete resection in patients with retroperitoneal liposarcoma: a comparative multi-institutional study

Abnormal growth of a pleomorphic leiomyosarcoma originating from the mesenteric vein associated with poor outcome after curative-intent resection: a case report

Retroperitoneal Sarcoma Care in 2021

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