Rodríguez-Zubieta M, Albarenque K, Lagues C, San Roman A, Varela M, Russo D, Podesta G, Steinberg D, Schauvinhold C, Etchegaray A, de Dávila M T G
Department of Pathology, Hospital Universitario Austral, Buenos Aires, Argentina.
Department of Oncology, Hospital Universitario Austral, Buenos Aires, Argentina.
Case Rep Pathol. 2021 Apr 19;2021:6674372. doi: 10.1155/2021/6674372. eCollection 2021.
We report a case of a newborn with two synchronous tumors-sialoblastoma and hepatoblastoma-diagnosed at 20 weeks of gestation by magnetic resonance imaging (MRI) and ultrasonography (US). The aim of this study was to describe the management of this case together with a review of the literature. Our patient had a large facial tumor associated with extremely high alpha-fetoprotein levels. Diagnosis of the tumors was made by surgical biopsy, showing typical features in both. Sialoblastoma is a potentially aggressive tumor. In our case, the Ki67 index in the sialoblastoma was between 20 and 30%, indicating a possibly unfavorable behavior. The infant underwent surgery and chemotherapy in different steps. Complete surgical resection with clean margins is considered to be the best treatment option for sialoblastoma. Only four similar cases were previously reported. Timely management by a multidisciplinary team is essential in these difficult cases. In our patient, outcome was good at the time of this report.
我们报告一例新生儿，通过磁共振成像（MRI）和超声检查（US）在妊娠20周时诊断出患有两种同步肿瘤——涎母细胞瘤和成肝细胞瘤。本研究的目的是描述该病例的治疗过程并对文献进行综述。我们的患者面部有一个大肿瘤，且甲胎蛋白水平极高。通过手术活检对肿瘤进行了诊断，两者均显示出典型特征。涎母细胞瘤是一种具有潜在侵袭性的肿瘤。在我们的病例中，涎母细胞瘤的Ki67指数在20%至30%之间，表明其行为可能不佳。该婴儿在不同阶段接受了手术和化疗。完整切除且切缘阴性的手术被认为是涎母细胞瘤的最佳治疗选择。此前仅报告过4例类似病例。在这些疑难病例中，多学科团队的及时管理至关重要。在撰写本报告时，我们患者的预后良好。
