Chuang Huei Chieh, Kang Chung-Jan, Lee Li-yu
1 Department of Pathology, Chang Gung Memorial Hospital , Kwei Shan, Taoyuan County , Taiwan.
Fetal Pediatr Pathol. 2014 Jun;33(3):127-34. doi: 10.3109/15513815.2013.839013. Epub 2014 Feb 18.
Extragonadal pure yolk sac tumor of sinonasal origin is very rare. We report herein a case with sinonasal yolk sac tumor in a 1 year and 3 months old girl. The initial complaint was persistent nasal bleeding for about 2 months. Computed tomography (CT) and magnetic resonance imaging (MRI) revealed a lobulated soft tissue mass in paranasal sinus that extended to oral cavity, nasopharynx, and oropharynx. The histology showed typical features of yolk sac tumor and the positive immunohistochemical staining of SALL4 and α-fetoprotein. After tumor excision, adjuvant chemotherapy of JEB regimen was prescribed. After the follow-up for 13-months, α-fetoprotein was normal and neither tumor progression nor metastasis was found. We review the previous literature and discuss the etiology, histology, treatment, and the prognosis of the rare sinonasal yolk sac tumor.
鼻窦原发的性腺外纯卵黄囊瘤非常罕见。我们在此报告一例1岁3个月女童的鼻窦卵黄囊瘤病例。最初的症状是持续鼻出血约2个月。计算机断层扫描(CT)和磁共振成像(MRI)显示鼻窦内有一个分叶状软组织肿块,延伸至口腔、鼻咽和口咽。组织学显示卵黄囊瘤的典型特征,以及SALL4和甲胎蛋白免疫组化染色阳性。肿瘤切除后,采用JEB方案进行辅助化疗。随访13个月后,甲胎蛋白正常,未发现肿瘤进展或转移。我们回顾了既往文献,并讨论了这种罕见的鼻窦卵黄囊瘤的病因、组织学、治疗及预后。