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腹膜后或肠系膜原发性脂肪肉瘤:5例临床及预后评估

Retroperitoneal or mesenteric primary liposarcoma: clinical and prognostic evaluations on five cases.

作者信息

Rosato L, Panier Suffat L, Bertotti L, Perino P, Comello E, Mondini G

出版信息

G Chir. 2018 Jan-Feb;39(1):57-62. doi: 10.11138/gchir/2018.39.1.057.

Abstract

BACKGROUND

Retroperitoneal or mesenteric primary liposarcoma is a malignant neoplasia whose prognosis depends on the biological variants, the radical resection surgery and the histological subtypes, as well as on local and remote recurrences. Liposarcoma is the most common variant of this tumour among tumours of soft retroperitoneal tissue. The purpose of this study is to evaluate whether the treatment of this disease, carried out at the Ivrea Hospital for urgent reasons, may be considered in line with the experience of high-volume Centres.

PATIENTS AND METHODS

Between 1982 and 2016 five patients were hospitalized with a diagnosis of acute abdomen due to retroperitoneal or mesenteric primary liposarcoma: one myxoid of the mesentery, one well-differentiated of lesser epiploon, one well-differentiated of mesentery, and two dedifferentiates of the retroperitoneum.

RESULTS

The five patients, all males, had a average age of 59 years (range 48-86) and were all subjected to general anaesthesia and open technique for total tumour removal. Only the patient with the myxoid liposarcoma had two recurrences. All are alive and healthy after a follow-up of 177 months (range 9-420).

CONCLUSION

These tumours should be removed radically, if necessary in conjunction with contiguous organs. Rarity and therapeutic complexity require a treatment, sometimes even multimodal, in highvolume centres. Patients admitted in General Surgery Unit for unpostponable urgency should be operated by surgeons experienced in the treatment of abdominal disease to achieve survival results in line to those achievable in Hub Centres.

摘要

背景

腹膜后或肠系膜原发性脂肪肉瘤是一种恶性肿瘤,其预后取决于生物学变异、根治性切除手术、组织学亚型以及局部和远处复发情况。脂肪肉瘤是腹膜后软组织肿瘤中最常见的这种肿瘤变体。本研究的目的是评估因紧急原因在伊夫雷亚医院进行的这种疾病的治疗是否可被认为与大型中心的经验相符。

患者与方法

1982年至2016年间,有5例患者因腹膜后或肠系膜原发性脂肪肉瘤诊断为急腹症而住院:1例肠系膜黏液样型,1例小网膜高分化型,1例肠系膜高分化型,2例腹膜后去分化型。

结果

这5例患者均为男性,平均年龄59岁(范围48 - 86岁),均接受全身麻醉和开放手术以完全切除肿瘤。只有黏液样脂肪肉瘤患者有两次复发。经过177个月(范围9 - 420个月)的随访,所有患者均存活且健康。

结论

这些肿瘤应进行根治性切除,必要时可连同相邻器官一并切除。由于其罕见性和治疗复杂性,需要在大型中心进行有时甚至是多模式的治疗。因不可推迟的紧急情况入住普通外科病房的患者应由有腹部疾病治疗经验的外科医生进行手术,以获得与中心医院可达到的生存结果相当的疗效。

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