Kivelä T, Tarkkanen A
Department of Ophthalmology, Helsinki University Central Hospital, Finland.
Surv Ophthalmol. 1994 May-Jun;38(6):541-54. doi: 10.1016/0039-6257(94)90147-3.
Orbital teratomas are unilateral, congenital germ cell tumors, which present at birth with moderate to massive proptosis, leading to exposure keratopathy. They enlarge the orbit diffusely. Primary orbital teratomas cause axial or vertical proptosis, are usually unassociated with bony defects, and do not extend outside the orbit. Recurrence is unusual after an early excision without enucleation or exenteration. Combined orbital teratomas additionally involve periorbital and intracranial spaces, cause lesser degrees of proptosis, and typically are associated with defects of orbital bones. Their intracranial part is small as compared to the orbital one. A significant risk of recurrence exists even after a combined craniotomy, orbitotomy and plastic reconstructive surgery. Intracranial teratomas with secondary orbital spread present prenatally with polyhydramnois and hydrocephalus, due to a massive primary tumor replacing parts of the brain, and typically lead to stillbirth. One orbital teratoma with malignant change has been well documented. Included twins, monsters rather than neoplasms, represent parts of a second fetus in the orbit. Orbital yolk sac carcinomas or endodermal sinus tumors are malignant neoplasms affecting children, which frequently invade periorbital and intracranial spaces, and which may respond to chemotherapy and irradiation. Finally, a teratoma or a yolk sac carcinoma may rarely present as an intraocular tumor.
眼眶畸胎瘤是单侧先天性生殖细胞肿瘤，出生时即出现，伴有中度至重度眼球突出，可导致暴露性角膜病变。它们会使眼眶弥漫性增大。原发性眼眶畸胎瘤可导致轴向或垂直性眼球突出，通常与骨缺损无关，且不会延伸至眼眶外。早期切除且未行眼球摘除术或眶内容剜出术时，复发情况不常见。复合型眼眶畸胎瘤还累及眶周和颅内间隙，导致眼球突出程度较轻，且通常与眼眶骨缺损有关。与眼眶部分相比，其颅内部分较小。即使进行了联合开颅术、眼眶切开术和整形重建手术，仍存在显著的复发风险。伴有继发性眼眶扩散的颅内畸胎瘤在产前表现为羊水过多和脑积水，这是由于巨大的原发性肿瘤取代了部分脑组织，通常会导致死产。有一例眼眶畸胎瘤发生恶变的病例记录详实。包含双胎、怪胎而非肿瘤，代表眼眶内第二个胎儿的部分组织。眼眶卵黄囊癌或内胚窦瘤是影响儿童的恶性肿瘤，常侵犯眶周和颅内间隙，可能对化疗和放疗有反应。最后，畸胎瘤或卵黄囊癌很少会表现为眼内肿瘤。
