Background
Retroperitoneal soft tissue sarcomas comprise a heterogeneous group of rare tumors of mesenchymal origin that include several well-defined histologic subtypes. In 2015, the Transatlantic ...Australasian RPS Working Group (TARPSWG) published consensus recommendations for the best management of primary retroperitoneal sarcoma (RPS). Since then, through international collaboration, new evidence and knowledge have been generated, creating the need for an updated consensus document.
Methods
The primary aim of this study was to critically evaluate the current evidence and develop an up-to-date consensus document on the approach to these difficult tumors. The resulting document applies to primary RPS that is non-visceral in origin, with exclusion criteria as previously described. The relevant literature was evaluated and an international group of experts consulted to formulate consensus statements regarding the best management of primary RPS. A level of evidence and grade of recommendation were attributed to each new/updated recommendation.
Results
Management of primary RPS was considered from diagnosis to follow-up. This rare and complex malignancy is best managed by an experienced multidisciplinary team in a specialized referral center. The best chance of cure is at the time of primary presentation, and an individualized management plan should be made based on the 29 consensus statements included in this article, which were agreed upon by all of the authors. Whenever possible, patients should be enrolled in prospective trials and studies.
Conclusions
Ongoing international collaboration is critical to expand upon current knowledge and further improve outcomes of patients with RPS. In addition, prospective data collection and participation in multi-institution trials are strongly encouraged.
Background
The objective of this study was to evaluate the adequate margin in the local treatment of extremity soft tissue sarcomas (ESTS) and understand the relationship between local control and ...overall survival (OS).
Methods
All consecutive patients treated for a primary ESTS at a single center from 1993 to 2012 were reviewed.
Results
In all, 531 patients were included. Twelve (2 %) underwent a first-line amputation. The resections were R0/R1/not available in 434 (82 %), 92 (17 %), and 5 patients (1 %). The median tumor size was 8 cm, and the tumor grades were 1 (
n
= 132), 2 (
n
= 201), and 3 (
n
= 195). The median size of the minimal margin was 2 mm on fixed specimen. Preop or postop chemotherapy was administered to 222 patients, and 414 received radiotherapy. With a median follow-up period of 7 years, the 5-year actuarial local recurrence (LR) rate and OS were 8 % (95 % CI, 6–11 %) and 80 % (95 % CI, 76–83 %). Predictors of worse OS were grade 3, leiomyosarcoma, male gender, and age >60 years, whereas tumor size, margin status, and LR were not. Among patients requiring re-excision (
n
= 252), the presence of residual cells correlated with OS but not LR. After preoperative treatment, a percentage of residual cells ≥10 % correlated with OS but not LR. In the multivariate analysis, specific subtypes (epithelioid sarcoma and myxofibrosarcoma) and margin size <1 mm correlated with LR, whereas grade and the tissue constituting the surgical margins did not.
Conclusions
Specific subtypes and surgical margin size <1 mm were correlated with a higher LR. Neither the margin status nor LR affect OS.
Background This French nationwide NETSARC exhaustive prospective cohort aims to explore the impact of systematic re-excision (RE) as adjuvant care on overall survival (OS), local recurrence free ...survival (LRFS), and local and distant control (RFS) in patients with soft tissue sarcoma (STS) with positive microscopic margins (R1) after initial resection performed outside of a reference center. Methods Eligible patients had experienced STS surgery outside a reference center from 2010 to 2017, and had R1 margins after initial surgery. Characteristics and treatment comparisons used chi-square for categorical variables and Kruskall-Wallis test for continuous data. Survival distributions were compared in patients reexcised (RE) or not (No-RE) using a log-rank test. A Cox proportional hazard model was used for subgroup analysis. Results A total of 1,284 patients had experienced initial STS surgery outside NETSARC with R1 margins, including 1,029 patients with second operation documented. Among the latter, 698 patients experienced re-excision, and 331 were not re-excised. Characteristics were significantly different regarding patient age, tumor site, tumor size, tumor depth, and histotype in the population of patients re-excised (RE) or not (No-RE). The study identified RE as an independent favorable factor for OS (HR 0.36, 95%CI 0.23-0.56, p<0.0001), for LRFS (HR 0.45, 95%CI 0.36-0.56, p<0.0001), and for RFS (HR 0.35, 95%CI 0.26-0.46, p<0.0001). Conclusion This large nationwide series shows that RE improved overall survival in patients with STS of extremities and trunk wall, with prior R1 resection performed outside of a reference center. RE as part of adjuvant care should be systematically considered. Level of evidence II Keywords: Soft tissue sarcoma, Surgery, Relapse, Reference center, Multidisciplinary tumor board resection margins, Survival
Whether re-excision (RE) of a soft tissue sarcoma (STS) of limb or trunk should be systematized as adjuvant care and if it would improve metastatic free survival (MFS) are still debated. The impact ...of resection margins after unplanned macroscopically complete excision (UE) performed out of a NETSARC reference center or after second resection was further investigated.
This large nationwide series used data from patients having experienced UE outside of a reference center from 2010 to 2019, collected in a French nationwide exhaustive prospective cohort NETSARC. Patient characteristics and survival distributions in patients reexcised (RE) or not (No-RE) are reported. Multivariate Cox proportional hazard model was conducted to adjust for classical prognosis factors. Subgroup analysis were performed to identify which patients may benefit from RE.
Out of 2371 patients with UE for STS performed outside NETSARC reference centers, 1692 patients were not reviewed by multidisciplinary board before treatment decision and had a second operation documented. Among them, 913 patients experienced re-excision, and 779 were not re-excised. Characteristics were significantly different regarding patient age, tumor site, size, depth, grade and histotype in patients re-excised (RE) or not (No-RE). In univariate analysis, final R0 margins are associated with a better MFS, patients with R1 margins documented at first surgery had a better MFS as compared to patients with first R0 resection. The study identified RE as an independent favorable factor for MFS (HR 0.7, 95% CI 0.53-0.93;
= 0.013). All subgroups except older patients (>70 years) and patients with large tumors (>10 cm) had superior MFS with RE.
RE might be considered in patients with STS of limb or trunk, with UE with macroscopic complete resection performed out of a reference center, and also in originally defined R0 margin resections, to improve LRFS and MFS. Systematic RE should not be advocated for patients older than 70 years, or with tumors greater than 10 cm.
A sporadic desmoid tumor (DT) is a rare type of tumor of the mesenchymal connective tissues is now considered an intermediate disease or locally aggressive. They may develop on scars or ...after traumatism, favored by growth factors released during the initial phase of wound healing. Most of the abdominal DT arising on a scar is described on the wall incision. In this report, we describe two cases of DT arising on the intraperitoneal surgical scar, shortly after the resection of a low-grade retroperitoneal liposarcoma and a low-risk gastric gastrointestinal stromal tumor (GIST). Inconsistency between low risk according to the classification of the primary sarcoma and early local recurrence (LR) should raise the possibility of DT. Core needle biopsy (CNB) should be performed when it is feasible, including on local recurrences (LR). Surveillance has become the first-line treatment for DT. In case of progression between two imaging during the surveillance phase, surgery, when it's not mutilating, is indicated for selected cases as second-line treatment.
Monobloc resection of soft tissue sarcomas (STSs) has a major impact on overall survival and local recurrence. Anatomical boundaries, such as the sciatic notch, increase the risk of fragmentation of ...the lesion. To date there are few papers describing the optimal surgical technique to remove such STSs. The objective of this study is to describe a simultaneous anterior and posterior approach for resection of sciatic notch dumbbell tumours.
We present the surgical management of two patients diagnosed with well-differentiated liposarcomas of the sciatic notch with a retroperitoneal and gluteal extension in the two cases. Pre-operative diagnosis was made with a percutaneous biopsy including molecular analysis which demonstrated MDM2 amplification. We describe a simultaneous anterior and posterior approach, including the ligation of the posterior trunk of the internal iliac artery, to reduce intra-operative blood loss and devascularise the tumour. The anterior approach allows the evaluation of the tumour's retroperitoneal extension, release from its pelvic attachments and control of the surrounding neurovascular structures. During the posterior approach, bleeding is reduced by the devascularisation of the gluteal musculature achieved with internal iliac artery ligation. Clear margins were achieved in both cases. No vascular, skeletal or soft tissue reconstructions were required.
Simultaneous combined anterior and posterior approaches to remove a malignant sciatic notch tumour optimises the chance of complete en bloc resection. This surgical strategy allows oncologic en bloc resection with minimal blood loss.
In front of a primary retroperitoneal tumour, it is necessary to have in mind all possible diagnoses in order to specify the diagnostic strategy and the treatment. According to the World Health ...Organization (WHO) classification of tumours, mesenchymal benign and malignant tumours (including sarcomas and, currently, neurogenic tumours), parasympathetic tumours, extragonadal germ cell tumours, and lymphoid tumours have been identified. By definition, primary retroperitoneal tumours start independently from the retroperitoneal organs. Secondary lesions, carcinoma metastasis, and adenopathy are excluded from this definition, but they can also develop in the retroperitoneal space and lead to misdiagnoses. In the absence of positive tumour markers or an evocative biology, percutaneous biopsy is necessary. Pathological diagnosis is necessary to decide whether surgery must be done, its timing among the other treatments, and its extension. This paper summarizes all the diagnostic possibilities.
-Retro- and infraperitoneal tumours: Percutaneous biopsy is the standard of care. In fact, the indications for and extent of surgery depends on the diagnosis.-Both the early complication rate and the incidence of needle tract seeding of per cutaneous biopsy of an RP mass are very low.-The well-differentiated components of liposarcomas are often underestimated, leading to incomplete surgery, which definitely worsens prognosis.-The surgical standard of care for sarcoma is a complete, non-fragmented, en bloc resection with clear margins on the larger surface. Typical resection is a compartmental surgery, with en bloc resection of the kidney, colon, and psoas fascia. Adjustments are made according to the histological subtype.-Sarcoma surgery must be performed in a referral sarcoma centre.
The treatment of desmoid tumours (DTs) has greatly evolved in recent years, and surgery is no longer considered a first-line treatment. Percutaneous biopsy with molecular analysis for beta-catenin or ...APC gene mutation provides a certain diagnosis. After imaging, a specialized multidisciplinary tumour board (MDT) addresses the following therapeutic strategy. As more than half of patients stabilize or regress, despite initial progression, active surveillance is offered to most patients as the first option. Surgery is proposed for complications such as perforation and occlusion, which sometimes are the first manifestations of the disease. In these cases, limiting surgery to the treatment of complications and leaving the tumour in place is possible if significant bowel sacrifice is needed, especially in patients with previous colectomy for polyposis. Medical therapy is discussed by the MDT in cases of functional or life-threatening masses and is preferred to local treatments that could be mutilating and often incomplete. First-line surgery is now contraindicated in cases of incomplete unplanned surgery, recurrence, pregnancy or DTs occurring in familial adenomatous polyposis (FAP). The best indications of second-line surgery are significantly progressing disease when morbidity is acceptable, such as parietal locations. Medical and other locoregional treatments (radiotherapy, isolated limb perfusion and cryotherapy) should be considered by the MTB when surgery might cause sequelae.
Major vascular resections in retroperitoneal sarcoma Tzanis, Dimitri; Bouhadiba, Toufik; Gaignard, Elodie ...
Journal of surgical oncology,
January 1, 2018, 2018-Jan, 2018-01-00, 20180101, Letnik:
117, Številka:
1
Journal Article
Recenzirano
Retroperitoneal sarcomas (RPS) frequently involve major vessels, which either originate from them or secondarily encase or invade them. In this field, major vascular resections result in increased ...morbidity. However, survival does not seem to be affected by the need for vascular resection or by this higher morbidity. This paper aims to provide descriptions of the surgical strategy and outcomes for retroperitoneal sarcomas involving major vessels.
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