Pegoraro Francesca, Santangelo Domenico, Santangelo Alfonso, Pelosio Luigi, Jamshidi Akbar, Camera Luigi, Imbriaco Massimo, Mainolfi Ciro Gabriele, Insabato Luigi, Accarino Rossella, Giuliano Mario, Carlomagno Nicola, D'Alessandro Vincenzo, Santangelo Michele L
Operative Unit of Hepato-Biliary-Pancreatic, Minimally Invasive and Robotic Surgery and Kidney Transplantation, Department of Clinical Medicine and Surgery, Federico II University Hospital, I-80131 Naples, Italy.
Department of Radiology, 'Scientific Hospitalization and Treatment Institute' San Raffaele Hospital, I-20132 Milano, Italy.
Oncol Lett. 2023 Aug 4;26(3):410. doi: 10.3892/ol.2023.13996. eCollection 2023 Sep.
Retroperitoneal sarcomas (RPSs) are rare findings that can grow into large masses without eliciting severe symptoms. At present, surgical resection is the only radical therapy, whenever it can be performed with the aim to achieve a complete removal of the tumor. The present report describes two consecutive cases of RPSs that resulted in dedifferentiated liposarcomas (DDLPSs) and these patients underwent R0 surgical resection with and without a nephron-sparing procedure. The diagnostic workup, the surgical approach, the impact of late surgical management due to the COVID pandemic and the latest literature on the topic are discussed and analyzed. The patients, who refused to undergo any medical examination during the prior 2 years due to the COVID pandemic, were admitted to Federico II University Hospital (Naples, Italy) complaining about weight loss and general abdominal discomfort. In the first case, a primitive giant abdominal right neoplasm of retroperitoneal origin enveloping and medializing the right kidney was observed. The second patient had a similar primitive retroperitoneal giant left neoplasm, which did not affect the kidney. Given the characteristics of the masses and the absence of distant metastases, after a multidisciplinary discussion, radical surgical removal was carried out for both patients. The lesions appeared well-defined from the surrounding tissues, and markedly compressed all the adjacent organs, without signs of infiltration. In the first patient, the right kidney was surrounded and undetachable from the tumor and it was removed with the mass. The second patient benefited from a nephron-sparing resection, due to the existence of a clear cleavage plane. The postoperative courses were uneventful. Both the histological examinations were oriented towards a DDLPS and both patients benefited from adjuvant chemotherapy. In conclusion, the treatment of giant RPS is still challenging and requires multidisciplinary treatment as well as, when possible, radical surgical removal. The lack of tissue infiltration and the avoidance of excision or reconstruction of major organs (including the kidney) could lead to an easier postoperative course and an improved prognosis. When possible, surgical management of recurrences or incompletely resected masses must be pursued. Since the COVID pandemic caused limited medicalization of a number of population groups and delayed diagnosis of other oncologic diseases, an increased number of DDLPSs could be expected in the near future.
腹膜后肉瘤(RPSs)是罕见的肿瘤,可生长为巨大肿块而不引起严重症状。目前,手术切除是唯一的根治性治疗方法,只要能够进行手术,目的是实现肿瘤的完全切除。本报告描述了两例连续的RPSs病例,结果均为去分化脂肪肉瘤(DDLPSs),这两名患者分别接受了有或没有保留肾单位手术的R0手术切除。对诊断检查、手术方法、由于新冠疫情导致的延迟手术管理的影响以及该主题的最新文献进行了讨论和分析。这两名患者因新冠疫情在过去两年拒绝接受任何医学检查,因体重减轻和腹部不适入住费德里科二世大学医院(意大利那不勒斯)。在第一例病例中,观察到一个起源于腹膜后的原始巨大腹部右侧肿瘤,包绕并推移右肾向内侧移位。第二例患者有一个类似的原始腹膜后左侧巨大肿瘤,但未累及肾脏。鉴于肿块的特征及无远处转移,经多学科讨论后,对两名患者均进行了根治性手术切除。病变与周围组织界限清楚,明显压迫所有相邻器官,无浸润迹象。在第一例患者中,右肾被肿瘤包绕且无法与肿瘤分离,与肿块一并切除。第二例患者因存在清晰的分离平面,受益于保留肾单位切除术。术后病程顺利。组织学检查均确诊为DDLPSs,两名患者均接受了辅助化疗。总之,巨大RPS的治疗仍然具有挑战性,需要多学科治疗以及尽可能进行根治性手术切除。缺乏组织浸润以及避免切除或重建主要器官(包括肾脏)可能会使术后病程更顺利并改善预后。如果可能,必须对复发或未完全切除的肿块进行手术处理。由于新冠疫情导致一些人群的医疗服务受限以及其他肿瘤疾病的诊断延迟,预计在不久的将来DDLPSs的数量会增加。