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巨大的腹膜后脂肪肉瘤包裹右肾:来自尼泊尔的一例病例报告。

Huge retroperitoneal liposarcoma encasing right kidney: A case report from Nepal.

作者信息

Suryabanshi Anil, Timilsina Binita, Khadka Namrata, Rijal Ashes, Bhandari Ramesh Singh

机构信息

Maharajgunj Medical Campus, Institute of Medicine, 44600 Kathmandu, Nepal.

Maharajgunj Medical Campus, Institute of Medicine, 44600 Kathmandu, Nepal.

出版信息

Int J Surg Case Rep. 2022 Oct;99:107647. doi: 10.1016/j.ijscr.2022.107647. Epub 2022 Sep 13.

Abstract

INTRODUCTION AND IMPORTANCE

Retroperitoneal liposarcoma is a rare entity originating from adipocytes. Before showing any symptoms, it can grow hugely and envelop nearby structures. Surgery is the optimum course of treatment, however, well-differentiated liposarcomas make it challenging to discern between malignant and normal adipocytes during surgery.

CASE PRESENTATION

We report a case of a 62-year-old male referred to evaluate his abdominal distention presented for four years. Recently he had developed loin pain for six months. A computerized tomography scan showed extensive tumor encompassing the right kidney and ureter, colon, and duodenum, however, exploratory laparotomy revealed free colon and duodenum. Complete resection of the mass with a right nephrectomy was performed. Subsequently, a histopathological assessment of the resected specimen confirmed the diagnosis of well-differentiated liposarcoma. Adjuvant chemotherapy was initiated as the tumor was a high-risk sarcoma but local recurrence was observed after 2 years despite surgery and chemotherapy.

CLINICAL DISCUSSION

Imaging modalities are the mainstay of preoperative diagnosis. Preferably, surgical resection with a tumor-free margin is recommended to avoid tumor recurrence which remains the primary challenge. This, along with the grade of the tumor, multifocal disease, and invasion of adjacent structure dictate the prognosis of the disease. Adjuvant chemotherapy and radiotherapy are not regarded as standard therapies for resectable retroperitoneal liposarcoma, although further research is still needed to determine their value in the case of high-risk sarcoma.

CONCLUSION

Retroperitoneal liposarcoma has the potential to present as huge asymptomatic masses which with an added predilection for recurrence poses a huge challenge to any surgeon.

摘要

引言与重要性

腹膜后脂肪肉瘤是一种起源于脂肪细胞的罕见疾病。在出现任何症状之前,它可能会大幅生长并包裹附近的结构。手术是最佳的治疗方法,然而,高分化脂肪肉瘤使得在手术过程中难以区分恶性和正常脂肪细胞。

病例报告

我们报告一例62岁男性患者,因腹部膨隆4年前来评估。最近他出现腰痛6个月。计算机断层扫描显示广泛的肿瘤包绕右肾、输尿管、结肠和十二指肠,然而,剖腹探查发现结肠和十二指肠未受侵犯。遂行肿块完整切除并右肾切除术。随后,对切除标本的组织病理学评估证实为高分化脂肪肉瘤。由于该肿瘤为高危肉瘤,故开始辅助化疗,但尽管进行了手术和化疗,2年后仍观察到局部复发。

临床讨论

影像学检查是术前诊断的主要手段。最好推荐进行切缘无肿瘤的手术切除以避免肿瘤复发,而肿瘤复发仍是主要挑战。这一点,连同肿瘤分级、多灶性病变以及对相邻结构的侵犯决定了疾病的预后。辅助化疗和放疗不被视为可切除腹膜后脂肪肉瘤的标准治疗方法,尽管仍需要进一步研究以确定它们在高危肉瘤病例中的价值。

结论

腹膜后脂肪肉瘤有可能表现为巨大的无症状肿块,且复发倾向增加,这给任何外科医生都带来了巨大挑战。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/8216/9568774/18adf89e86bd/gr1.jpg

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