Liu RenMing, Chen JianNing, Shao ChunKui, Cheng Na
Department of Pathology, The Third Affiliated Hospital, Sun Yat-Sen University, No. 600 Tianhe Road, Guangzhou, 510630, People's Republic of China.
Diagn Pathol. 2022 Oct 29;17(1):86. doi: 10.1186/s13000-022-01267-8.
Primary liver immature teratoma is extremely rare and only 4 cases have been reported, let alone with growing teratoma syndrome (GTS) and/or gliomatosis peritonei (GP).
Here, we report a case of a 44-year-old female presenting with progressive abdominal distension and elevated serum alpha fetal protein (AFP) level. CT/MRI scans revealed a large cystic-solid mass in the right lobe of the liver, accompanied with implant or metastasis in the abdominal cavity. Pathologic examination at biopsy suggested immature teratoma. After 4 cycles of chemotherapy, an MRI showed a slight increase in tumor size. Therefore, surgical resection of the right lobe of the liver was performed. The final histological diagnosis was a mature teratoma (tumor size 28 cm × 14 cm × 13 cm), with no residual immature component, and the diagnosis of GTS was considered. The patient continued to receive 2 courses of postoperative chemotherapy. An abdominal CT scan revealed innumerable miliary nodules in bilateral adnexal areas 2 months after surgery. Histologically, large numbers of mature glia were observed, supporting the diagnosis of GP.
We report for the first time a case of primary liver immature teratoma with GTS and GP in an adult. Longer follow-up is needed to assess definitive efficacy.
原发性肝脏未成熟畸胎瘤极为罕见，仅报道过4例，更不用说合并生长畸胎瘤综合征（GTS）和/或腹膜胶质瘤病（GP）的情况了。
在此，我们报告一例44岁女性，表现为进行性腹胀和血清甲胎蛋白（AFP）水平升高。CT/MRI扫描显示肝脏右叶有一个大的囊实性肿块，并伴有腹腔内种植或转移。活检病理检查提示为未成熟畸胎瘤。4周期化疗后，MRI显示肿瘤大小略有增加。因此，对肝脏右叶进行了手术切除。最终组织学诊断为成熟畸胎瘤（肿瘤大小为28 cm×14 cm×13 cm），无残留未成熟成分，并考虑GTS诊断。患者继续接受2个疗程的术后化疗。术后2个月腹部CT扫描显示双侧附件区有无数粟粒状结节。组织学上，观察到大量成熟神经胶质细胞，支持GP的诊断。
我们首次报告一例成人原发性肝脏未成熟畸胎瘤合并GTS和GP的病例。需要更长时间的随访来评估确切疗效。
