胰母细胞瘤（PB）是一种源自胰腺滤泡细胞的肿瘤，主要影响儿童群体。尽管在成人中很少见，但它与相当差的预后相关。大约三分之一的患者被诊断患有转移性疾病，其中肝转移最为常见。诊断依赖于组织病理学改变，包括鳞状囊泡、CK8/CK18/CK19 阳性染色以及 β-连环蛋白的核移位。此外，肝转移在对比增强计算机断层扫描 (CT) 扫描的动脉期显示出显着增强。手术切除是解决原发性病变和肝转移性 PB 的主要治疗方法。在手术干预不可行的情况下，患者可能会从全身治疗和放射治疗中获益。这份特殊病例报告介绍了一名被诊断患有 PB 的 27 岁女性患者的临床细节，该患者随后在胰十二指肠切除术后出现多发性肝转移。基因组检查显示患者存在 ERBB2 扩增、RAD54L 缺失、TMB-L 低和 MSS。尽管患者接受化疗和 Her-2 靶向治疗与免疫治疗相结合，但在服用索凡替尼之前，并未观察到病灶大小的缩小。随后，出现了显着的结果，通过手术干预有效切除了转移灶。索凡替尼已证明无进展生存期 (PFS) 不少于 14 个月，并且患者的生存期持续了 33 个月。这表明索凡替尼作为 PB 成年患者的可行治疗替代方案的潜在功效。版权所有 © 2024 Leng、Lv、Yang、Li、Wang、Cheng、Chang 和 Cao。

Pancreatoblastoma (PB), a neoplasm derived from pancreatic follicular cells, primarily affects the pediatric population. Although infrequent in adults, it is associated with a considerably worse prognosis. Approximately one-third of patients are diagnosed with metastatic disease, with liver metastases being the most prevalent. Diagnosis relies on histopathological alterations including squamous vesicles, positive staining for CK8/CK18/CK19, and nuclear displacement of β-catenin. Additionally, liver metastases demonstrate substantial enhancement during the arterial phase of a contrast-enhanced computed tomography (CT) scan. Surgical resection serves as the principal therapeutic approach for addressing primary lesions and liver metastatic PB. In instances where surgical intervention is not viable, patients may derive benefits from systemic therapy and radiotherapy. This particular case report presents the clinical details of a 27-year-old female patient diagnosed with PB, who subsequently developed multiple liver metastases following a pancreaticoduodenectomy. Genomic examinations revealed the presence of ERBB2 amplification, RAD54L deletion, low TMB-L, and MSS in the patient. Despite the patient undergoing chemotherapy and Her-2 targeted therapy in conjunction with immunotherapy, no reduction in lesion size was observed until the administration of surufatinib. Subsequently, a notable outcome ensued, where the metastatic lesions were effectively excised via surgical intervention. Surufatinib has demonstrated a progression-free survival (PFS) of no less than 14 months, and the patient's survival has endured for a duration of 33 months. This indicates the potential efficacy of surufatinib as a viable therapeutic alternative for adult patients afflicted with PB.Copyright © 2024 Leng, Lv, Yang, Li, Wang, Cheng, Chang and Cao.