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是可怕的“畸胎瘤”还是单纯的脊髓脊膜膨出:我们的“组织学意外”经历。

Is it the Monster "Teratoma" or Simply Meningomyelocele: Our Experience of "Histological Surprise".

作者信息

Singh Suyash, Sardhara Jayesh, Sharma Pradeep, Srivastava Arun Kumar, Das Kuntal Kanti, Bhaisora Kamlesh S, Mehrotra Anant, Jaiswal Awadhesh Kumar, Behari Sanjay, Kumar Raj

机构信息

Department of Neurosurgery, Sanjay Gandhi Postgraduate Institute of Medical Sciences, Lucknow, Uttar Pradesh, India.

出版信息

J Pediatr Neurosci. 2017 Apr-Jun;12(2):192-195. doi: 10.4103/jpn.JPN_188_16.

Abstract

Teratomas are one of the most common tumors in newborn with excellent prognosis arises from totipotent primordial germ cells harboring two or three germ cell layers. The tumor has been titled "Great masquerade." The teratomas of sacrococcygeal region present with lower limb weakness, urinary or bowel obstruction, and swelling at lower back or intrauterine mass in ultrasound or complicated delivery. A 2-month-old male child presented with complaints of swelling over lumbosacral region with discharging punctum since birth. Sagittal T2-weighted magnetic resonance imaging (MRI) of the spine showed myelocele at L5 level forming placode with central defect at L4-S1 and low-lying tethered cord up to L4-L5. The patient was operated, and histopathology surprisingly came to be mature teratoma. We followed the patient with serum beta human chorionic gonadotropin and alpha-fetoprotein markers and MRI. Literature supports complete surgical removal, including coccyx and tumor base. Mature teratoma is considered as benign disease thus even subtotal excision is appropriate but with aggressive follow-up. The difference in recurrence following total compared to subtotal resection is considered insignificant. In this article, we have discussed the management of teratoma in detail. Teratoma with meningomyelocele is a rare entity. There is still dilemma in managing cases and prognosticating parents in such patients. The provisional diagnosis of teratoma should also be considered when child presents as midline sacrococcygeal mass.

摘要

畸胎瘤是新生儿最常见的肿瘤之一,预后良好,起源于具有两到三个胚层的全能原始生殖细胞。该肿瘤被称为“伟大的伪装者”。骶尾部畸胎瘤表现为下肢无力、尿路或肠道梗阻,以及下背部肿胀或超声检查发现的子宫内肿块或分娩复杂。一名2个月大的男婴自出生以来就有腰骶部肿胀并有排脓小孔的症状。脊柱矢状面T2加权磁共振成像(MRI)显示L5水平的脊髓脊膜膨出形成基板,L4-S1有中央缺损,低位脊髓栓系至L4-L5。该患者接受了手术,组织病理学结果令人惊讶地显示为成熟畸胎瘤。我们通过血清β人绒毛膜促性腺激素和甲胎蛋白标志物以及MRI对患者进行随访。文献支持完整切除手术,包括尾骨和肿瘤基底部。成熟畸胎瘤被认为是良性疾病,因此即使是次全切除也是合适的,但需要积极随访。全切除与次全切除后的复发差异被认为不显著。在本文中,我们详细讨论了畸胎瘤的治疗。伴有脊髓脊膜膨出的畸胎瘤是一种罕见的实体。在处理此类病例和向家长预测预后方面仍然存在困境。当儿童表现为骶尾部中线肿块时,也应考虑畸胎瘤的初步诊断。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/1aee/5588652/a3d2de56798d/JPN-12-192-g001.jpg

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