Practice Pattern Variability in the Management of Regional Lymph Node Metastasis in Extremity and Trunk Soft Tissue Sarcoma: A Survey of the Society of Surgical Oncology and Musculoskeletal Tumor Society Membership.

Background: Regional lymph node metastasis in extremity and trunk soft tissue sarcoma (ETSTS) is rare with no standardized management. We sought to determine management patterns for regional lymph node metastasis in ETSTS.

Methods: A survey regarding the management of ETSTS lymph node metastasis was distributed to the membership of the Musculoskeletal Tumor Society (MSTS) and the Society of Surgical Oncology (SSO) in January 2022.

Retrospective evaluation of the role of gemcitabine-docetaxel in well-differentiated and dedifferentiated liposarcoma.

Objective: Well-differentiated (WDLPS) and dedifferentiated liposarcoma (DDLPS) account for the majority of liposarcomas. Although gemcitabine-docetaxel is used as second-line treatment in soft tissue sarcomas, its efficacy in WDLPS/DDLPS is not established. This study retrospectively analyzed the efficacy of gemcitabine regimens in WDLPS/DDLPS.

Clinical Impact of External Beam Radiotherapy for Surgically Resected Primary Retroperitoneal Liposarcoma.

Introduction: EORTC-62092 (STRASS) was a phase 3, randomized study that compared surgery alone versus surgery plus neoadjuvant radiotherapy (RT) for retroperitoneal sarcomas. RT was not associated with improved abdominal recurrence-free survival, the primary outcome measure, although on subanalysis, there may have been benefit for well-differentiated (WD) liposarcoma. This study investigated the real-world use and outcomes of RT (neoadjuvant and adjuvant) for the management of retroperitoneal liposarcoma.

Re-excision After Unplanned Excision of Soft Tissue Sarcoma is Associated with High Morbidity and Limited Pathologic Identification of Residual Disease.

Background: Patients with unplanned excision (UPE) of trunk and extremity soft tissue sarcoma (STS) present a significant management challenge for sarcoma specialists. Oncologic re-resection has been considered standard practice after UPE with positive or uncertain margins. A strategy of active surveillance or "watch and wait" has been suggested as a safe alternative to routine re-excision.

Sentinel Lymph Node Biopsy and Formal Lymphadenectomy for Soft Tissue Sarcoma: A Single Center Experience of 86 Consecutive Cases.

Background: Lymph node metastases (LNMs) are rare in patients with soft tissue sarcoma (STS), and there is limited evidence to guide clinical management. We describe our experience with sentinel lymph node biopsy (SLNB) and lymphadenectomy in STS patients.

Methods: A single-center, retrospective review was performed for patients with STS treated with SLNB and/or lymphadenectomy from 1994 to 2018.

Sarculator is a Good Model to Predict Survival in Resected Extremity and Trunk Sarcomas in US Patients.

Background: Sarculator is an online validated nomogram that predicts overall survival of patients with resected, primary extremity sarcomas. However, its ability to accurately predict outcomes in US patients with sarcoma is unknown.

Patients And Methods: Patients from the National Cancer Data Base (NCDB) (2006-2016) with resected stage I-III primary extremity or trunk sarcoma were included.

Real-world use of palbociclib monotherapy in retroperitoneal liposarcomas at a large volume sarcoma center.

Palbociclib has been evaluated in early phase trials for well-differentiated liposarcoma (WDLPS) and dedifferentiated liposarcoma (DDLPS) patients, with reported median progression-free survival (PFS) of 18 weeks. Here, we report on real-world use and surgical outcomes associated with palbociclib treatment. We retrospectively reviewed 61 consecutive patients with retroperitoneal WDLPS (n = 14) or DDLPS (n = 47) treated with palbociclib monotherapy between 1 March 2016 and 28 February 2021 at The University of Texas MD Anderson Cancer Center.

Enhancer reprogramming in PRC2-deficient malignant peripheral nerve sheath tumors induces a targetable de-differentiated state.

Malignant peripheral nerve sheath tumors (MPNSTs) are soft tissue sarcomas that frequently harbor genetic alterations in polycomb repressor complex 2 (PRC2) components-SUZ12 and EED. Here, we show that PRC2 loss confers a dedifferentiated early neural-crest phenotype which is exclusive to PRC2-mutant MPNSTs and not a feature of neurofibromas. Neural crest phenotype in PRC2 mutant MPNSTs was validated via cross-species comparative analysis using spontaneous and transgenic MPNST models.

National Utilization of Imatinib in the Management of Resected Gastrointestinal Stromal Tumors.

Background: Imatinib decreases recurrence risk and improves overall survival (OS) in localized gastrointestinal stromal tumors (GISTs); however, the extent to which patients receive appropriate treatment in the US has not been well characterized.

Methods: Patients with non-metastatic, resectable GIST were included in this study (National Cancer Database, 2010-2015). Those with a low-risk of recurrence were classified as receiving overtreatment or guideline-concordant treatment, while those with a high-risk of recurrence were classified as receiving undertreatment or guideline-concordant treatment.

Comparison of Cancer Prevalence in Patients With Neurofibromatosis Type 1 at an Academic Cancer Center vs in the General Population From 1985 to 2020.

Importance: Neurofibromatosis type 1 (NF1) is a complex genetic disorder that is associated with not only neurofibromas, but also an increased susceptibility to other neoplasms.

Objective: To evaluate the prevalence of neoplasia and outcomes among patients with NF1.

Design, Setting, And Participants: This cohort study was conducted among patients with NF1 at a single academic cancer center from 1985 to 2020 with median (range) follow-up of 2.

PET/CT Imaging as a Diagnostic Tool in Distinguishing Well-Differentiated versus Dedifferentiated Liposarcoma.

Distinguishing well-differentiated liposarcoma (WDLPS) from dedifferentiated liposarcoma (DDLPS) is essential given distinct treatment paradigms and chemosensitivity. Percutaneous biopsy has a low sensitivity for detecting DDLPS. We sought to identify the diagnostic utility of positron emission tomography/computed tomography (PET/CT) in identifying WDLPS versus DDLPS.

Evaluating the Impact of Surveillance Follow-Up Intervals in Patients Following Resection of Primary Well-Differentiated Liposarcoma of the Retroperitoneum.

Background: Resection of recurrent retroperitoneal well-differentiated liposarcoma (RP-WDLPS) is unlikely to result in cure. Thus, most clinicians delay surgery after recurrence until symptom intolerance or increasing rate of disease progression. The aim of this study was to determine whether longer surveillance intervals in this population would impact outcomes or delay treatment in those who recur.

ACR Appropriateness Criteria® Radiologic Management of Gastric Varices.

Hemorrhage, resulting from gastric varies, can be challenging to treat, given the various precipitating etiologies. A wide variety of treatment options exist for managing the diverse range of the underlying disease processes. While cirrhosis is the most common cause for gastric variceal bleeding, occlusion of the portal or splenic vein in noncirrhotic states results in a markedly different treatment paradigm.

A reconstructive algorithm of oncologic defects of the upper trunk and shoulder girdle: Factors predicting complexity and outcomes.

Background: Locally advanced malignancies of the upper torso and shoulder girdle (UT-SG) necessitate extensive resection and complex reconstruction. Due to the infrequent nature of these operations, a global reconstructive algorithm has not been defined.

Methods: A retrospective review of all patients who received reconstructive surgery following malignant tumor extirpation in the UT-SG from 2008 to 2018 at the University of Texas MD Anderson Cancer Center.

Certain risk factors for patients with desmoid tumors warrant reconsideration of local therapy strategies.

Background: The objective of this study was to evaluate treatment outcomes for patients with desmoid tumors (DTs) receiving local therapy with surgery alone, radiation therapy (RT) alone, or combined modality therapy (RT and surgery).

Methods: This was a cross-sectional cohort study of 412 patients with nonmesenteric DTs who received local therapy at the authors' institution between 1965 and 2018.

Results: The median follow-up time was 95 months (range, 1-509 months).

Management and outcomes of ruptured, perforated or fistulized tumors of mesenchymal origin.

Background: Patients with ruptured, perforated or fistulized (RPF) sarcomas commonly have issues such as sepsis and malnutrition and are usually unsuitable for oncologic resection in the emergency setting. We present our approach for managing a series of patients and the outcomes which were achieved with multidisciplinary care.

Methods: We reviewed records of patients referred to the section of sarcoma surgical oncology.

Postoperative pancreatic fistula after distal pancreatectomy for non-pancreas retroperitoneal tumor resection.

Introduction: Short-term outcomes after distal pancreatectomy (DP) for retroperitoneal (RP) tumors are unknown. We sought to identify rates of postoperative pancreatic fistula (POPF) and morbidity after en bloc DP with RP tumor resection.

Methods: A retrospective review of 43 patients who underwent DP with RP tumor resection (1/2011-12/2017) was performed.

ACR Appropriateness Criteria® Palpable Abdominal Mass-Suspected Neoplasm.

Palpable abdominal masses may arise from the abdominal cavity or the abdominal wall. The differential diagnosis is broad for each variant ranging from benign lipomas, inflammatory processes, to malignant tumors. The imaging approach to diagnosis varies by location.

The degree of sclerosis is associated with prognosis in well-differentiated liposarcoma of the retroperitoneum.

Background And Objectives: Well-differentiated liposarcomas (WDL) are often partly composed of sclerotic tissue, however, the amount varies widely between tumors, and its prognostic significance is unknown. We hypothesized that tumors with more sclerosis would behave more aggressively.

Methods: Primary retroperitoneal WDL from 29 patients resected at our institution with follow-up were histologically evaluated by soft tissue pathologists blinded to outcome.

ACR Appropriateness Criteria Left Lower Quadrant Pain-Suspected Diverticulitis.

This review summarizes the relevant literature regarding imaging of suspected diverticulitis as an etiology for left lower quadrant pain, and imaging of complications of acute diverticulitis. The most common cause of left lower quadrant pain in adults is acute sigmoid or descending colonic diverticulitis. Appropriate imaging triage for patients with suspected diverticulitis should address the differential diagnostic possibilities and what information is necessary to make a definitive management decision.

ACR Appropriateness Criteria Dysphagia.

This review summarizes the relevant literature for the initial imaging of patients with symptoms of dysphagia. For patients with oropharyngeal dysphagia who have an underlying attributable cause, a modified barium swallow is usually appropriate for initial imaging but for those who have unexplained dysphagia a fluoroscopic biphasic esophagram is usually appropriate. Fluoroscopic biphasic esophagram is usually appropriate for initial imaging in both immunocompetent and immunocompromised patients who have retrosternal dysphagia.