Khera Sanjeev, Singh Vikram, Parikh Badal, Chand Karunesh
Pediatrics, Army Hospital Research and Referral, New Delhi, India
Pathology, Army Hospital Research and Referral, New Delhi, Delhi, India.
BMJ Case Rep. 2021 Mar 17;14(3):e241197. doi: 10.1136/bcr-2020-241197.
Mixed epithelial mesenchymal (MEM) hepatoblastoma with teratoid features is rare histological variant of hepatoblastoma and described in case reports. Growing teratoma syndrome (GTS) is a rare and often unrecognised phenomenon generally associated with less than 5% of germ cell tumour. It is defined by enlarging tumour mass which is generally mature teratoma with normal or significantly decreasing tumour markers during chemotherapy. The treatment outcomes in GTS are dependent on early recognition and complete surgical excision. We describe a rare case of MEM hepatoblastoma with teratoid features with GTS in an infant who had a delay in definitive management due to late diagnosis of GTS.
具有畸胎样特征的混合上皮间质(MEM)型肝母细胞瘤是肝母细胞瘤中一种罕见的组织学变异型,仅有病例报告对其进行过描述。生长性畸胎瘤综合征(GTS)是一种罕见且常未被认识的现象,通常与不到5%的生殖细胞肿瘤相关。它的定义是肿瘤肿块不断增大,通常为成熟畸胎瘤,化疗期间肿瘤标志物正常或显著下降。GTS的治疗效果取决于早期识别和完整的手术切除。我们描述了一例具有畸胎样特征的MEM型肝母细胞瘤合并GTS的罕见病例,该婴儿因GTS诊断延迟而导致确定性治疗延误。