Actualización en el manejo de sarcomas retroperitoneales y pélvicos; el papel de la cirugía compartimental

Surgery for retroperitoneal sarcomas should be “en bloc” compartmental, which involves resection of unaffected organs. Its upfront use is key, providing a high percentage of resections with negative margins, resulting in a better local control and increased survival in many patients. Preservation of organs should be done in an individualized manner, especially in the pelvic location, and adapted to the histological aggressiveness of the tumor. Preoperative biopsy is able to establish the diagnosis of sarcoma subtype and consequently an adequate perioperative strategy. These patients should be managed by expert surgeons at referral centers with multidisciplinary units and oncology committees. The use of chemotherapy and radiotherapy is not yet well defined, so it is only recommended at referral centers with clinical trials. Currently, this is the only option to offer the best morbidity and mortality rates, as well as possible improvements in the survival of these patients.     

Distribution and Surgical Management of Visceral Ewing Sarcoma Among Children and Adolescents

IntroductionEwing sarcoma (EWS) is a highly malignant tumor of bone and soft tissue that occasionally arises from viscera. Visceral EWS (V-EWS) is challenging to manage given its varied organ distribution and often late-stage presentation. We aimed to characterize our institutional experience with V-EWS, focusing on its surgical management, and to compare V-EWS outcomes against those with osseous (O-EWS) and soft tissue EWS (ST-EWS).MethodsRetrospective review of all EWS patients ≤21 years presenting to a single institution between 2000 and 2022. Patient- and disease-specific characteristics were compared. Overall and relapse-free survival were estimated using Kaplan Meier methods and log-rank test.Results156 EWS patients were identified: 117 O-EWS, 20 ST-EWS, and 19 V-EWS. V-EWS arose in the kidney (n = 5), lung (n = 5), intestine (n = 2), esophagus (n = 1), liver (n = 1), pancreas (n = 1), adrenal gland (n = 1), vagina (n = 1), brain (n = 1), and spinal cord (n = 1). No significant demographic differences were detected between EWS groups. V-EWS was more frequently metastatic at presentation (63.2%; p = 0.005), yet no significant overall or relapse-free survival differences emerged between EWS groups, with similar follow-up intervals. While V-EWS required multiple unique operative strategies to gain primary control, no significant difference in treatment strategies appeared between groups. Surgery-only primary control was associated with improved overall and relapse-free survival in all groups.ConclusionsV-EWS presents unique management challenges in children and adolescents given its variable sites of origin. This large cohort is the first to describe the surgical management and outcomes of V-EWS, demonstrating more frequent metastatic presentation, while achieving similar survival across groups.Level of evidenceLevel 2 – Cohort Study.     

Malignant peripheral nerve sheath tumors – Outcomes and prognostic factors based on the reference center experience

IntroductionMalignant peripheral nerve sheath tumor (MPNST) accounts for about 5% of soft tissue sarcomas. It can occur as sporadic diseases or can be associated with type 1 neurofibromatosis. MPNST is usually associated with poor prognosis, mostly due to their aggressive behavior, high metastatic potential, and resistance to chemotherapy. Our study aimed to determine treatment outcomes and associated prognostic factors in a large cohort of patients with MPNSTs treated at the reference sarcoma center.Methods239 consecutive patients (114 women and 125 men) diagnosed with MPNST between March 1998 and March 2018 who were treated with surgery with curative intent in the reference sarcoma center were included in the retrospective analysis.ResultsThe mean age at diagnosis was 51 years (range 15–86). 28 (11.7%) patients had neurofibromatosis type 1 associated tumors (NF1 positive). Median OS was 126.5 months and 5-year survival rate was 61.9% in the group treated with curative intent. Median DFS, LRFS and DMFS were 91.6, 126.5 and 126.5 months, respectively. We identified tumor size, high tumor grade and positive surgical margins as independent negative predictors of DFS, LRFS, DMFS and OS.ConclusionsHigh-quality surgery remains a gold standard of MPNST treatment. High grade, size and quality of surgery are significant independent prognostic factors for overall survival. There is an unmet need for improvement, especially regarding the perioperative treatment and treatment of metastatic disease. Future studies on the biology of MPNST would lead to the development of novel treatment options and improvement of treatment outcomes.     

The diagnostic significance of repeat ultrasound-guided biopsy of musculoskeletal soft-tissue lesions with initially inconclusive biopsy results

IntroductionTo determine the diagnostic yield of repeat ultrasound (US)-guided biopsy of musculoskeletal soft-tissue lesions with initially inconclusive biopsy results, and to explore predictive factors for success of repeat biopsy.Materials and methodsThis retrospective study included 42 patients who underwent a repeat (second) US-guided biopsy session to target a musculoskeletal soft-tissue lesion because an initial US-guided biopsy session provided inconclusive results. Both biopsy sessions were performed in a tertiary referral center for soft-tissue sarcomas.ResultsThe diagnostic yield of repeat US-guided biopsy was 47.6%. Malignant nature of the lesion (P = 0.031), sharp lesion borders on US (P = 0.011), and good to very good lesion visibility on US (P = 0.017) were significantly associated with a diagnostic repeat US-guided biopsy. There was also a trend towards significance (P = 0.073) for a higher number of biopsy passes through the lesion. Other patient characteristics (age and gender), magnetic resonance imaging features (lesion homogeneity on T1-weighted, T2-weighted, and gadolinium chelate enhanced sequences, borders, enhancement pattern, depth and size), US features (lesion appearance, vascular flow, and depth), biopsy-related factors (days between initial and repeat US-guided biopsy, needle diameter, maximum length of acquired samples), and operator-related factors (same or different radiologists/pathologists for initial and repeat biopsies), were not associated with the diagnostic success of the repeat US-guided biopsy.ConclusionsRepeat US-guided biopsy of a musculoskeletal soft-tissue lesion with initially inconclusive biopsy results can be useful to establish a final diagnosis. Lesion features on US (borders and visibility) may be used to prospectively determine the utility of a repeat US-guided biopsy.     

静脉瓣功能障碍致假性Kaposi肉瘤1例报告并文献回顾

背景与目的 假性Kaposi肉瘤，又称肢端血管性皮炎，是一种罕见的血管外科疾病。本文通过报告1例静脉瓣功能障碍致假性Kaposi肉瘤病例，结合文献回顾探讨其临床病理特征及诊治。方法 回顾性分析1例假性Kaposi肉瘤患者的临床病例资料，结合国内外文献总结假性Kaposi肉瘤的病因、分型、临床表现、病理、诊断及治疗。结果 患者男性，37岁。长期从事站立性体力劳动，两踝关节内侧下方数个暗紫红色瘀斑，近1个月久站后皮损处疼痛。结合病史、病理切片及辅助检查，诊断为“假性Kaposi肉瘤”。经穿弹力袜、促进静脉回流、抗凝、改善循环后，双下肢皮损处无明显疼痛，皮损较前无明显扩大或增多。结论 静脉瓣功能障碍与假性Kaposi肉瘤发生发展相关，较为罕见且鉴别诊断存在一定困难，需结合皮损特点、病理及血管影像特点综合考虑，目前无特异性治疗。     

子宫肉瘤的术前诊断

子宫肉瘤（uterine sarcoma，US）是一种罕见的恶性肿瘤，具有早期诊断困难、恶性程度高、易远处转移、易复发、预后差等特点。早期诊断和手术治疗是改善预后的关键因素，因此术前对子宫肉瘤的诊断和分期有重大的临床意义。子宫肉瘤无特异的临床表现，单一的术前检查，如血清CA125或乳酸脱氢酶（LDH）值升高，对子宫肉瘤有提示意义，但无法明确诊断。当子宫肉瘤凸向宫腔时，子宫内膜细胞学检查可有阳性发现。影像学检查如磁共振成像与弥散加权成像相结合有助于区分子宫肉瘤与子宫良性肿瘤，但仍需大样本研究进一步验证。超声引导下穿刺活检可以获得较为可靠的病理结果，但由于相关风险及并发症并不作为常规术前检查手段。通过将临床体征、生物学标记物及影像学检查相结合构建的综合术前预测系统可以显著提高子宫肉瘤诊断的准确性。