Giant hypothalamic hamartomas (GHH) are rare neonatal intracerebral congenital malformations responsible for gelastic epilepsy and/or endocrine disturbances. Sacrococcygeal teratomas (SCT) are fetal neoplasms associated with perinatal morbidity and mortality, especially hemorrhagic complications in giant examples (GSCT). Here, we describe an immature ruptured GSCT complicated by hemorrhagic shock at 32-week gestation boy requiring an emergency delivery, followed immediately by urgent surgical removal. A brain lesion resembling a GHH was also present on the antenatal MRI. In order to exclude metastatic immature teratoma or glioma, a biopsy was performed by a retro-sigmoidal approach, which confirmed the nature of the hamartoma. Here, we describe for the first time the association of a ruptured immature GSCT associated with a GHH.

巨大下丘脑错构瘤 (GHH) 是罕见的新生儿脑内先天性畸形，可导致凝胶性癫痫和/或内分泌紊乱。骶尾部畸胎瘤 (SCT) 是与围产期发病率和死亡率相关的胎儿肿瘤，尤其是巨例出血并发症 (GSCT)。在这里，我们描述了一个未成熟破裂的 GSCT 并发失血性休克的 32 周妊娠男孩，需要紧急分娩，然后立即进行紧急手术切除。产前 MRI 上也存在类似于 GHH 的脑部病变。为了排除转移性未成熟畸胎瘤或胶质瘤，通过乙状结肠后入路进行活检，证实了错构瘤的性质。在这里，我们首次描述了与 GHH 相关的未成熟 GSCT 破裂的关联。