Habibi Zohreh, Nejat Farideh, Naeini Parisa Emami, Mahjoub Fatemeh
Department of Neurosurgery, Imam Khomeini Hospital, Tehran University of Medical Sciences, Iran.
J Neurosurg. 2007 Jun;106(6 Suppl):467-71. doi: 10.3171/ped.2007.106.6.467.
A myelomeningocele (MMC) can be associated with paraplegia, bladder and bowel incontinence, Chiari malformation Type II, and hydrocephalus. The coincidence of an MMC and a neoplasm is rare, and only limited reports on the concurrence of a teratoma within an MMC have been published.
A retrospective study was performed using the records of 330 children who underwent operations to correct an MMC at the Children's Hospital Medical Center in Tehran between January 2001 and June 2005. The postoperative histopathological assessments in 15 (4.5%) of these patients revealed evidence of a teratoma inside the MMC. The age of these patients at admission ranged from 2 days to 2 years (median 30 days). Neurological findings were normal in all patients except for three with lower-extremity weakness or paralysis. Hydrocephalus was detected in six patients. Physical appearance of the teratoma in all cases included cystic or solid soft-tissue masses in the dorsal midline area, covered with abnormal rudimentary skin. Pedunculated skin tags, a primitive genitalia-like appendage, dermal sinus, a human tail, and cutaneous stigmata such as color changes, hemangioma, dysplastic skin, and tufts of hair around the mass were occasionally observed.
The special feature of a protruding fingerlike appendage or intraoperative detection of a cystic portion of an MMC without direct connection to the spinal canal can be possible signs of teratoma concurrent with an MMC. Teratoma inside an MMC is a benign neoplasm, without any recurrence after standard surgery for an MMC.
脊髓脊膜膨出(MMC)可伴有截瘫、膀胱和肠道失禁、II型Chiari畸形以及脑积水。MMC与肿瘤同时存在的情况罕见,仅有关于MMC内合并畸胎瘤的有限报道。
对2001年1月至2005年6月在德黑兰儿童医院接受MMC矫正手术的330例儿童的记录进行回顾性研究。其中15例(4.5%)患者术后组织病理学评估显示MMC内存在畸胎瘤证据。这些患者入院时年龄从2天至2岁不等(中位年龄30天)。除3例有下肢无力或瘫痪外,所有患者神经学检查结果均正常。6例患者检测出脑积水。所有病例中畸胎瘤的外观表现为背侧中线区域的囊性或实性软组织肿块,表面覆盖异常的原始皮肤。偶尔可见带蒂皮肤赘生物、类似原始生殖器的附属物、皮样窦、人尾以及肿块周围的皮肤体征,如颜色改变、血管瘤、发育异常的皮肤和毛发簇。
突出的指状附属物或术中发现MMC的囊性部分且与椎管无直接连接的特殊表现可能是MMC合并畸胎瘤的迹象。MMC内的畸胎瘤是一种良性肿瘤,在接受MMC标准手术后无任何复发。