背景:腹膜后分化脂肪肉瘤(RPDDL)是一种罕见的恶性肿瘤,由于在腹膜后腔中具有足够的空间并且在疾病的早期阶段缺乏临床表现,因此通常多年未被发现。外科手术通常作为治疗的首选。然而,手术后容易局部复发,导致不良预后。我们的目的是从新病例中吸取有益的教训,为疾病的管理提供一些经验。
方法:我们描述了一名55岁的男性患者,他因3周的左腰持续隐痛而入院。在体格检查中触诊了左上腹部的大肿块。此外,影像学检查显示肿块直径约21厘米,一些邻近的重要器官被侵入,这给完成手术切除带来了很大的挑战。
方法:术后病理证实肿块为RPDDL,浸润胰腺等周围重要结构,脾,脾左肾上腺,左肾,和有肿瘤栓子的脉管系统。
方法:我们的多学科团队进行了肿块的手术切除。患者术后1个月接受化疗。
结果:化疗的效果似乎不令人满意。手术后约2个月考虑肿瘤的局部多灶性复发。最后,他放弃了任何治疗,死于这种疾病。
结论:定期体检和超声筛查可以尽早发现疾病,特别是对于60-70岁的高危人群,应该推广。不完全切除,血管浸润,中断术后治疗可能导致不良预后。因此,我们认为这种疾病的患者可能受益于完整的手术切除和不间断的辅助治疗.
BACKGROUND: Retroperitoneal dedifferentiated
liposarcoma (RPDDL) is an uncommon malignancy, which often remains undetected for many years due to having adequate space in the retroperitoneal cavity and lacking clinical manifestations in the early stage of the disease. Surgical procedure is usually used as the first choice for treatment. However, it is prone to local recurrence after the operation, resulting in an unfavorable prognosis. Our aim is to draw useful lessons from the new case and provide some experience for management of the disease.
METHODS: We describe a 55-year-old male patient who was admitted for a 3-week history of persistent dull ache of the left waist. A large mass of the left upper abdomen was palpated in physical examination. Moreover, the imaging examination revealed that the diameter of the mass was about 21 cm, and some adjacent vital organs were invaded, which brought great challenges to complete surgical resection.
METHODS: The postoperative pathological results confirmed that the mass was RPDDL with invasion of the surrounding vital structures including pancreas, spleen, left adrenal gland, left kidney, and vasculature with tumor emboli.
METHODS: Surgical resection of the mass was performed by our multidisciplinary team. The patient received chemotherapy 1 month after surgery.
RESULTS: The effect of chemotherapy seemed to be unsatisfactory. Local multifocal recurrence of the tumor was considered about 2 months after surgery. Finally, he gave up any treatments and died of the disease.
CONCLUSIONS: Regular physical examination and ultrasound screening may detect the disease as early as possible, especially for high-risk group aged 60 to 70, which should be popularized. Incomplete resection, vascular invasion, and interruption of postoperative treatment may lead to an unfavorable prognosis. Therefore, we think that patients with the disease may benefit from complete surgical resection and uninterrupted adjuvant therapy.