Horowitz Joel, Singhal Mayank, Marrero Dana, Bashjawish Fuad, Leto Dezarae, Winters Maryselle, Jeberaeel Julian
Department of Oncological Surgery, Cape Fear Valley Hospital, Fayetteville, NC, USA.
Department of Internal Medicine, Cape Fear Valley Hospital, Fayetteville, NC, USA.
Am J Case Rep. 2020 Mar 2;21:e919245. doi: 10.12659/AJCR.919245.
BACKGROUND Retroperitoneal sarcomas are rare tumors, only affecting 2 to 5 people per million population and accounting for 0.1% of all malignancies. Liposarcoma is the most common of all retroperitoneal sarcomas, responsible for approximately 20% of all sarcomas in adults. The most important prognostic factors are tumor grade, the presence of positive margins, tumor integrity, and degree of resection. CASE REPORT Our patient was a 73-year-old man with abdominal pain whose CT scan of the abdomen and pelvis demonstrated a 15×15 cm heterogeneous, left-sided intra-abdominal mass. He underwent resection of the retroperitoneal tumor, left colectomy, and left nephrectomy. Final pathology demonstrated a high-grade, de-differentiated liposarcoma with a rhabdosarcomatous component. The postoperative course was complicated by a small intra-abdominal abscess and abdominal dehiscence. a CT scan after surgery showed a residual tumor of the retroperitoneal posterior margin. Re-exploration to resect the residual tumor and repair the fascial dehiscence were performed. The patient underwent an initial chemotherapy regimen with doxorubicin, then moved to targeted therapy with Palbociclib, and is now on chemotherapy using Eribulin. CONCLUSIONS Achieving complete resection and the grade of the tumor at diagnosis are the 2 most important prognostic factors for patient survival in retroperitoneal liposarcoma, as survival rates are inversely proportional to the grade of the tumor. Even with the best resection attempts, there is always a risk of residual tumor cells within the tumor bed, which contribute to recurrence and need for additional surgical interventions. It is important to approach this disease process with a multidisciplinary team that includes surgical, medical, and radiation oncology to ensure the best survival outcomes. Retroperitoneal sarcoma recurrence and survival are directly related to the ability to achieve negative margins of resection, as well as the grade and size of the primary tumor. Adjuvant therapies that include radiation and immunotherapy may be effective in treating recurrent disease.
腹膜后肉瘤是罕见肿瘤,每百万人口中仅2至5人受影响,占所有恶性肿瘤的0.1%。脂肪肉瘤是所有腹膜后肉瘤中最常见的,约占成人所有肉瘤的20%。最重要的预后因素是肿瘤分级、切缘阳性情况、肿瘤完整性和切除程度。
我们的患者是一名73岁男性,有腹痛症状,腹部和盆腔CT扫描显示左侧腹腔内有一个15×15 cm的异质性肿块。他接受了腹膜后肿瘤切除术、左半结肠切除术和左肾切除术。最终病理显示为高级别、去分化脂肪肉瘤,伴有横纹肌肉瘤成分。术后过程出现了一个小的腹腔内脓肿和腹部裂开并发症。术后CT扫描显示腹膜后后缘有残留肿瘤。进行了再次探查以切除残留肿瘤并修复筋膜裂开。患者最初接受了阿霉素化疗方案,然后转为使用帕博西尼的靶向治疗,现在正在使用艾瑞布林进行化疗。
实现完全切除和诊断时肿瘤的分级是腹膜后脂肪肉瘤患者生存的两个最重要的预后因素,因为生存率与肿瘤分级成反比。即使进行了最佳的切除尝试,肿瘤床内始终存在残留肿瘤细胞的风险,这会导致复发并需要额外的手术干预。重要的是要由包括外科、医学和放射肿瘤学在内的多学科团队来处理这个疾病过程,以确保最佳的生存结果。腹膜后肉瘤的复发和生存与实现阴性切缘的能力以及原发肿瘤的分级和大小直接相关。包括放疗和免疫治疗在内的辅助治疗可能对治疗复发性疾病有效。