Dorian Yarih Garcia-Ortega, Claudia Hs Caro-Sánchez, Alethia Alvarez-Cano, Mario Alvarez-Bojorquez, Emmanuel Melgarejo-Estefan, Ernesto Rodríguez-Ayala
Surgical Oncologist, National Cancer Institute (Instituto Nacional de Cancerología), Mexico City, Mexico.
Oncologic Pathology, National Cancer Institute (Instituto Nacional de Cancerología), Mexico City, Mexico.
Rare Tumors. 2021 Feb 26;13:2036361320986655. doi: 10.1177/2036361320986655. eCollection 2021.
Sarcomas are a heterogenous group of malignant tumors with origin or mesenchymal differentiation, they comprise 1-2% of all solid tumors. Retroperitoneum is the second most frequent site affected. Prognosis is worse compared to the limbs, with a 5y OS of 36-58%, and 50-60% patients will relapse. Dedifferentiated liposarcomas (ddLPS) are more aggressive, it is known that presence of a de-differentiated component increases the probability of distant recurrence and lowers OS. There is little information about the specific impact of each type of de-differentiation. To determine if the presence of myogenic differentiation markers in DDLPS is an adverse prognostic factor. A retrospective, observational, analytic cohort study was performed. Cases identified from the electronic clinical files from the National Cancer Institute in Mexico City, we included cases from January 1st 2005 to December 31st 2016. We correlated the presence of expression of myogenic markers (Smooth muscle actin, Calponin, H-caldesmon, Desmin and Myogenin) in the dedifferentiated component of DDLPS with overall survival and surgical outcomes. One hundred and forty-three cases were analyzed. Eighty-two were liposarcomas, and 38 had a dedifferentiated component. Of these 38 cases, 21(55.3%) were males and, 17(44.7%) were females. Median age was 54.1(27-79) years, median tumor size was 28 cm (13-56). Most patients had locally advanced disease: 32(84.2%) were in stage IIIB. 2.6% had metastatic disease and 5(13.2%) had stage Ib at diagnosis. Myogenic marker expression was found in 18.4% of cases; these patients had a worse median survival than cases with no myogenic expression: 18 months (95% CI 15.4-20.5) vs 32 months (95% CI 21.8-42.1) = 0.01, we also found a relation with higher postoperative morbidity in these cases ( = 0.045). The presence of myogenic differentiation markers might be associated with a worse prognosis, in our series it corelated with worse OS, however it is not a common event. Relation with surgical morbidity is to be analyzed in further studies.
肉瘤是一组起源于间充质或具有间充质分化的异质性恶性肿瘤，占所有实体瘤的1%-2%。腹膜后是第二常见的受累部位。与肢体肉瘤相比，其预后更差，5年总生存率为36%-58%，50%-60%的患者会复发。去分化脂肪肉瘤（ddLPS）侵袭性更强，已知去分化成分的存在会增加远处复发的可能性并降低总生存率。关于每种去分化类型的具体影响的信息很少。为了确定ddLPS中肌源性分化标志物的存在是否是一个不良预后因素。进行了一项回顾性、观察性、分析性队列研究。从墨西哥城国家癌症研究所的电子临床档案中识别出病例，我们纳入了2005年1月1日至2016年12月31日的病例。我们将ddLPS去分化成分中肌源性标志物（平滑肌肌动蛋白、钙调蛋白、H-钙调蛋白、结蛋白和肌细胞生成素）的表达情况与总生存率和手术结果进行关联分析。共分析了143例病例。其中82例为脂肪肉瘤，38例有去分化成分。在这38例病例中，21例（55.3%）为男性，17例（44.7%）为女性。中位年龄为54.1（27-79）岁，中位肿瘤大小为28cm（13-56）。大多数患者患有局部晚期疾病：32例（84.2%）处于IIIB期。2.6%有转移性疾病，5例（13.2%）在诊断时为Ib期。18.4%的病例发现有肌源性标志物表达；这些患者的中位生存期比无肌源性表达的病例更差：18个月（95%CI 15.4-20.5）对32个月（95%CI 21.8-42.1），P=0.01，我们还发现这些病例术后发病率更高（P=0.045）。肌源性分化标志物的存在可能与更差的预后相关，在我们的系列研究中它与更差的总生存率相关，然而这并非常见情况。手术发病率之间的关系有待进一步研究分析。
