Salunke Pravin, Bhansali Anil, Dutta Pinnaki, Bansal Arun, Gupta Kirti, Vasishta Rakesh K, Singh Paramjeet, Mukherjee Kanchan K
Department of Neurosurgery, Postgraduate Institute of Medical Education and Research, Chandigarh, India.
Pediatr Neurosurg. 2010;46(1):46-50. doi: 10.1159/000315002. Epub 2010 May 27.
Cushing's disease in a neonate is rare and has been reported secondary to pituitary macroadenoma. A case of Cushing's syndrome due to congenital immature teratoma in the region of the pituitary has never been reported. We discuss a case of a neonate who presented with Cushing's syndrome secondary to a congenital immature teratoma in sellar, suprasellar and parasellar regions with ectopic ACTH secretion, thereby mimicking Cushing's disease. The management issues and prognosis of congenital teratomas and neonatal Cushing's syndrome have been discussed. We describe the first case of intracranial ectopic ACTH secreting teratoma in a young infant. The prognosis is usually bad unless total excision is achieved. In the preoperative period and in case of subtotal excision, chemotherapy to take care of hypercortisolemia may be given.
新生儿库欣病罕见,曾有继发于垂体大腺瘤的报道。垂体区域先天性未成熟畸胎瘤导致库欣综合征的病例从未见报道。我们讨论了一例新生儿,其因鞍区、鞍上区和鞍旁区先天性未成熟畸胎瘤伴异位促肾上腺皮质激素(ACTH)分泌而出现库欣综合征,从而酷似库欣病。文中讨论了先天性畸胎瘤及新生儿库欣综合征的处理问题和预后。我们描述了首例婴幼儿颅内异位分泌ACTH的畸胎瘤。除非实现全切,否则预后通常较差。在术前阶段以及次全切除的情况下,可给予化疗以控制高皮质醇血症。