Background/Objectives: Giant Retroperitoneal Liposarcomas (giant RPLs) represent a rare malignant disease of adulthood that does not yet have a univocal definition in the scientific literature. The symptoms may be late, depending on the position and the size reached. The weight may exceed 20 kg, and the diameter 25 cm. The main treatment is the surgical approach. This systematic review aims to collect data from the present literature and to answer some questions on the nature of this pathology. Methods: We performed a search on the PubMed, Cochrane, and Scopus databases using specific search strings. Non-English written articles and abstracts were excluded. Results: Dimensional, histological, and pathological data of giant RPLs were extracted and recorded in an electronic database, and charts were used to synthesize the results. We selected 126 manuscripts, all case reports and case series, and obtained data for 157 giant RPLs. The major axis varied from 15 to 80 cm, and the weight ranged between 2.5 and 98 kg. Sex distribution was homogenous. Age was reported 146 times, and almost 1/3 of the study population was under 50 years old. The most frequent hystotype reported was well-differentiated liposarcomas, while the rarest was pleomorphic liposarcomas. In 139 cases, the symptoms were reported and generally included a mass effect on surrounding organs. The exclusive surgical operation was the most frequent treatment option, and it included both the resection of the tumor and other organs involved. Chemo- and radiotherapy were also performed, in a few cases. In 36 reports, distant metastasis was suspected, but only 6 were effectively positive. Conclusions: Giant RPLs are a clinical entity that shares some common features with normal-size liposarcomas but are different in dimensions, age distribution, histologic prevalence, rate of incidental diagnosis, organ sparing, and R0 resection. More studies are needed to completely characterize these tumors.
背景/目的：巨大腹膜后脂肪肉瘤是一种罕见的成人恶性疾病，目前科学文献中尚未形成统一定义。其症状出现较晚，具体取决于肿瘤位置及生长体积。肿瘤重量可超过20公斤，直径可达25厘米。主要治疗手段为外科手术。本系统综述旨在整合现有文献数据，以解答关于该疾病本质的若干问题。方法：我们在PubMed、Cochrane和Scopus数据库中使用特定检索式进行文献检索，排除非英文撰写的全文及摘要。结果：研究提取了巨大腹膜后脂肪肉瘤的尺寸、组织学和病理学数据并录入电子数据库，通过图表进行结果整合。最终纳入126篇文献（均为病例报告及病例系列），共获得157例巨大腹膜后脂肪肉瘤数据。肿瘤长径范围为15-80厘米，重量介于2.5-98公斤。性别分布均衡。146例报告了年龄数据，近1/3患者年龄低于50岁。最常见的组织学类型为高分化脂肪肉瘤，而多形性脂肪肉瘤最为罕见。139例报告了临床症状，主要表现为对周围器官的占位效应。单纯手术治疗是最常见的治疗方案，包括肿瘤切除及受累器官切除。少数病例联合使用了化疗和放疗。36例报告疑似远处转移，但仅6例经证实为阳性。结论：巨大腹膜后脂肪肉瘤作为独立临床实体，虽与常规尺寸脂肪肉瘤存在共性特征，但在肿瘤尺寸、年龄分布、组织学类型占比、偶发诊断率、器官保留及R0切除率等方面存在显著差异。未来需要更多研究来全面揭示此类肿瘤的特征。
Dark Topics on Giant Retroperitoneal Liposarcoma: A Systematic Review of 157 Cases
肿瘤(癌症)患者之家