Persian BayanGene Research and Training Center, Shiraz University of Medical Sciences, Shiraz, Iran.
Student Research Committee, Shiraz University of Medical Sciences, Shiraz, Iran.
Diagn Pathol. 2021 Jun 24;16(1):55. doi: 10.1186/s13000-021-01117-z.
Undifferentiated embryonal sarcoma of liver (UESL) and hepatic mesenchymal hamartoma (HMH) are two rare entities which mainly affect the pediatric population. The aim of this investigation was to provide a comprehensive overview of the clinicopathologic characteristics of the patients diagnosed with these two conditions in a tertiary referral center in Iran.
In this retrospective study patients diagnosed with UESL or HMH between 2012 and 2020 were studied. A comprehensive histopathologic evaluation of the cases along with immunohistochemistry evaluation using a panel of antibodies was conducted. Furthermore, clinical, paraclinical, and treatment data and follow up information was collected.
A total of 16 patients (8 UESL, 8 HMH) were studied in this investigation. Patients with UESL had a significantly (p = 0.002) higher age at diagnosis compared with those with HMH. Histologically, UESL cases were characterized by anaplastic cells with eosinophilic cytoplasm and bizarre nuclei and frequent atypical mitosis and spindling in a myxoid stroma while disordered arrangement of hepatic parenchyma, bile ducts, and primitive mesenchyme was seen in HMH. Furthermore, small round cells and extramedullary hematopoiesis were seen in 2 UESL and 3 HMH cases, respectively. Concurrent HMH was also seen in two UESL cases. Immunohistochemistry panel showed positive staining for Vimentin, Glypican-3, Desmin, CD56, CD10, and BCL2 in UESL cases and immunoreactivity for Vimentin, HepPar 1, Glypican-3, SMA, CD56, BCL2, and CD34 in various components of HMH.
In this study, the clinicopathologic features of UESL and HMH cases are presented. We also evaluated the utility of an immunohistochemistry panel in the diagnosis of these two rare entities and suggested novel markers. Our study corroborated the findings of previous investigations and expanded the clinicopathologic features of these two rare entities with diagnostic and potential therapeutic implications.
肝未分化胚胎肉瘤（UESL）和肝间质错构瘤（HMH）是两种主要影响儿科人群的罕见实体瘤。本研究的目的是在伊朗的一家三级转诊中心提供对这些两种疾病患者的临床病理特征的全面概述。
在这项回顾性研究中，研究了 2012 年至 2020 年间诊断为 UESL 或 HMH 的患者。对病例进行了全面的组织病理学评估，并使用抗体组合进行了免疫组织化学评估。此外，还收集了临床、辅助检查和治疗数据以及随访信息。
本研究共研究了 16 名患者（8 名 UESL，8 名 HMH）。UESL 患者的诊断年龄明显高于 HMH 患者（p=0.002）。组织学上，UESL 病例的特征是具有嗜酸性细胞质和奇异核的间变性细胞，并且经常出现典型的有丝分裂和梭形细胞在黏液样基质中，而 HMH 则可见肝实质、胆管和原始间充质的排列紊乱。此外，在 2 例 UESL 和 3 例 HMH 中分别可见小圆细胞和髓外造血。在 2 例 UESL 中也发现了并发的 HMH。免疫组织化学检测显示 UESL 病例中 Vimentin、Glypican-3、Desmin、CD56、CD10 和 BCL2 阳性染色，而 HMH 的不同成分中则显示出 Vimentin、HepPar 1、Glypican-3、SMA、CD56、BCL2 和 CD34 免疫反应性。
本研究介绍了 UESL 和 HMH 病例的临床病理特征。我们还评估了免疫组织化学检测在诊断这两种罕见实体瘤中的应用，并提出了新的标志物。我们的研究证实了先前研究的结果，并扩展了这两种罕见实体瘤的临床病理特征，具有诊断和潜在治疗意义。
