Poflee Sandhya V, Pawar Prajkta S, Gaddewar Nilesh S, Raut Waman K
Department of Pathology, Government Medical College, Nagpur, Maharashtra, India.
Department of Opthalmology, Government Medical College, Nagpur, Maharashtra, India.
Indian J Cancer. 2020 Jul-Sep;57(3):337-339. doi: 10.4103/ijc.IJC_595_18.
Extragonadal germ cell tumors (GCTs) of head and neck region account for 5% of all benign and malignant GCTs. Orbit is an uncommon site for occurrence of extragonadal GCTs. Pure yolk sac tumor (YST) of orbit is a rare neoplasm and only a few cases are reported in the literature. An 18-month-old boy presented with right eye proptosis of 2 months duration. Because of rapid clinical course and magnetic resonance imaging (MRI) findings, neoplastic lesion was suspected. Histopathological examination of the biopsy revealed neoplasm with possibility of GCT. Raised serum alpha-feto-protein levels suggested YST component. Positivity for immunohistochemical markers Glypican-3 and SALL4 confirmed pure YST nature of the tumor. The child received six cycles of cisplatin-based chemotherapy with significant reduction in size of the tumor, followed by exenteration of the orbit. This case is reported for its rarity as well as for highlighting diagnostic characteristics and management of orbital YST.
头颈部性腺外生殖细胞肿瘤（GCTs）占所有良性和恶性GCTs的5%。眼眶是性腺外GCTs的一个不常见发病部位。眼眶纯卵黄囊瘤（YST）是一种罕见肿瘤，文献中仅报道了少数病例。一名18个月大男孩出现右眼突出2个月。由于临床病程进展迅速及磁共振成像（MRI）表现，怀疑为肿瘤性病变。活检组织病理检查显示肿瘤有可能为GCT。血清甲胎蛋白水平升高提示存在YST成分。免疫组化标志物Glypican-3和SALL4呈阳性证实肿瘤为纯YST性质。该患儿接受了六个周期以顺铂为基础的化疗，肿瘤大小显著缩小，随后进行了眼眶内容剜除术。报道此病例是因其罕见性以及突出眼眶YST的诊断特征和治疗方法。
