Complete Response to Eribulin in a Patient with Unresectable Liposarcoma: A Case Report and Implications of New Biomarkers

We herein report a rare case of unresectable liposarcoma that showed a complete response to eribulin. Furthermore, a low expression of phosphorylated AKT (p-AKT) on an immunohistological evaluation was observed. This result is consistent with our previous preclinical study that demonstrated the significance of p-AKT signaling for eribulin resistance in multiple subtypes of soft tissue sarcoma (STS) cells. This case highlights the potential benefits of eribulin as well as the mechanism underlying resistance to eribulin in patients with unresectable or metastatic STS, especially liposarcoma.     

Dedifferentiated liposarcoma (DDLPS) in the rectum: A case report

Dedifferentiated liposarcoma (DDLPS) is a rare subtype of liposarcoma with a poor prognosis. This current case report describes a rectal DDLPS in a 68-year-old Chinese male that presented with lower abdominal pain and weight loss. Computed tomography and magnetic resonance imaging were undertaken to evaluate the tumour. The patient underwent radical resection of the rectal tumour, sigmoid colostomy and partial ureterectomy. The tumour was positive for mouse double minute 2 by immunohistochemistry. The patient healed well but refused chemotherapy postoperatively for economic reasons. The tumour recurred and metastasized 4 weeks after the operation. After relevant treatment, the patient''s condition deteriorated and he died of shock, metabolic acidosis, hyperlactataemia and acute renal failure. The case report also reviews the literature in terms of the clinical diagnosis, treatment and pathological characteristics of rectal DDLPS with the aim of improving the level of diagnosis and treatment.     

Challenging surgical treatment of giant retroperitoneal liposarcoma: A case report

Liposarcoma is a rare malignant tumor type and surgical resection is the gold standard treatment. The present study reported on the case of a 51-year-old woman who presented with a mass in the left upper abdomen. Computed tomography revealed a 32-cm giant retroperitoneal liposarcoma. Complete tumor resection was performed without the removal of other organs. Postoperative pathological examination indicated retroperitoneal well-differentiated liposarcoma and immunohistochemistry revealed S-100(−), MDM2(+), vimentin(+), CDK4(+), p16(+) and STAT6(+) results. The patient recovered well after the surgery. Complete tumor resection during the first surgery is key to cure liposarcoma. The present case report will be helpful for clinical oncologists to fully understand giant retroperitoneal liposarcoma and treat it accordingly.     

Dedifferentiated liposarcoma of the spermatic cord with a hemangioendothelioma-like component: A case report and review of the literature

Atypical lipomatous tumor or well-differentiated liposarcoma/dedifferentiated liposarcoma (DDLPS) is the most frequent subtype of malignant adipocytic tumor. This tumor typically presents in late adult life, most commonly in the retroperitoneum, extremities, or spermatic cord. It has been reported that the dedifferentiated component consists mainly of high-grade sarcoma, including undifferentiated pleomorphic sarcoma, fibrosarcoma, and myxofibrosarcoma, and it has been recently reported that the dedifferentiated component can be also made up of a low-grade sarcomatous component. Therefore, the dedifferentiated areas exhibit a wide morphological spectrum that commonly includes fibroblastic/myofibroblastic and fibrohistiocytic tumors but very rarely includes vascular tumors. We present here the first reported case of DDLPS with a hemangioendothelioma-like component in the spermatic cord.     

Prognostic significance of the MDM2/HMGA2 ratio and histological tumor grade in dedifferentiated liposarcoma

Dedifferentiated liposarcoma (DDLPS) is a relatively common soft tissue sarcoma that results from the progression of well‐differentiated liposarcoma (WDLPS). This study aimed to investigate the progression process and to clarify the pathological and genetic factors related to poor prognosis in DDLPS. In 32 DDLPS cases and five WDLPS cases, genetic factors were analyzed by custom comparative genomic hybridization (CGH) array, which was designed to densely cover gene regions known to be frequently amplified in WD/DDLPS. The analyses comparing primary and metastatic lesions and those comparing histologically different areas in the same tumor revealed intra‐tumoral genetic heterogeneity and progression. According to a prognostic analysis comparing the good‐prognosis and the poor‐prognosis groups, we selected MDM2 and HMGA2 as candidate genes associated with poor and good prognosis, respectively. The ratios of the amplification or gain levels of MDM2 and HMGA2 expressed in log ratios (log[MDM2/HMGA2] = log[MDM2]‐log[HMGA2]) were significantly associated with prognosis. An amplification or gain level of MDM2 that was more than twice that of HMGA2 (MDM2/HMGA2 > 2, log[MDM2/HMGA2] > 1) was strongly related to poor OS (P < .001) and poor distant metastasis‐free survival (DMFS) (P < .001). In the pathological analysis of 44 cases of DDLPS, histological tumor grade, cellular atypia, and MDM2 immunoreactivity were related to overall survival (OS), while HMGA2 immunoreactivity tended to be associated with OS. Cellular atypia was also associated with DMFS. In conclusion, histological grade and MDM2 expression were determined to be prognostically important, and the MDM2/HMGA2 amplification or gain ratio was found to have significant prognostic value by the custom CGH array analysis.     

Hemoglobin,alkalic phosphatase,and C‐reactive protein predict the outcome in patients with liposarcoma

Data on prognostic biomarkers in soft tissue sarcomas are scarce. The aim of the study was to define prognostic markers in patients with a liposarcoma, a subtype of sarcoma derived from adipose tissue. We restrospectively reviewed 85 patients with liposarcoma treated at our department from May 1994 to October 2011. Kaplan–Meier curves, uni‐, and multivariable Cox proportional hazard models and competing risk analysis were performed to evaluate the association between putative biomarkers with disease‐specific and overall survival. We observed a significant association between both alkalic phosphatase (ALP; subhazard ratio [SHR] per 1 unit increase: 1.35; 95%CI 1.10–1.65; p = 0.005) and C‐reactive protein (CRP; SHR per 1 mg/dl increase: 2,57; 95%CI 1.36–4,86; p = 0.004) with disease‐specific survival. Hemoglobin (Hb) (HR per 1 g/dl increase: 065; 95%CI 0.48–0.87; p = 0.003) was associated with overall survival. These associations prevailed after multivariable adjustment for AJCC tumor stage. This study identifies CRP and ALP as novel independent predictors of disease‐specific survival in patients with liposarcoma. © 2015 Orthopaedic Research Society. Published by Wiley Periodicals, Inc. J Orthop Res 33:765–770, 2015.     

三维可视化技术在原发性腹膜后脂肪肉瘤切除术中的应用

目的 探讨三维可视化技术在原发性腹膜后脂肪肉瘤(PRPLS)切除术中的应用价值.方法 回顾性分析2014年7月厦门大学附属成功医院肝胆胰血管外科收治的1例64岁PRPLS男性患者的临床资料,应用三维可视化技术将患者术前腹部CT图像进行三维重建,通过观察肿瘤与腹腔器官、腹部大血管之间的关系及测量肿瘤体积、手术模拟进行术前评估.术前手术方案拟采用分步减瘤逐步完全切除肿瘤的方法.术中沿着脂肪肉瘤的包膜分离粘连,精细操作,依次将肿瘤从右肾、下腔静脉、肝脏剥离,最终通过分步减瘤的方法将肿瘤完全切除.术后对患者行CT检查随访.随访时间截至2014年9月.结果 三维可视化重建后图像清晰立体地显示肿瘤组织、腹腔实质器官、腹腔大血管的解剖结构及毗邻关系,术中证实三维重建肿瘤的解剖关系与实际基本一致.患者手术时间为5.5h,术中出血量为1 500 mL,切除的肿瘤质量为7.512 kg.患者术后2周痊愈出院,术后2个月复查CT未见肿瘤复发.结论 三维可视化技术在PRPLS切除术前可行精确评估及手术方案的制订,减少术中的盲目性,使患者获得良好的手术疗效.     

Liposarcoma of the larynx: report of a case and review of literature

Liposarcomas of the larynx are extremely rare tumors, with only 37 cases reported in the English or French literature to date. The first two cases of laryngeal liposarcomas were reported respectively by Kapur and Dockerty in 1968 [1, 2]. Liposarcoma of the larynx is at high risk of local recurrence and seldom has metastatic potential. Prognosis for this tumor is better than that of non laryngeal liposarcoma. The present case is the first patient of primary liposarcoma of the larynx reported from China. A review of the literature was performed, and the presentation, position, pathological diagnosis, treatment and prognosis of the patients with liposarcoma of the larynx of the reported cases before are analyzed.