Department of Neurosurgery, University of Illinois College of Medicine at Peoria, Peoria, Illinois, USA.
Department of Neurosurgery, OSF HealthCare Illinois Neurological Institute, Peoria, Illinois, USA.
Pediatr Neurosurg. 2022;57(1):50-55. doi: 10.1159/000519768. Epub 2021 Nov 2.
Immature teratoma is a known pediatric tumor. However, spinal variants are rare and can present both a diagnostic and therapeutic challenge, particularly regarding aggression as it pertains to extent of resection, likelihood of recurrence and concordant prognosis, and the need and efficacy of adjuvant therapies.
The patient is a 27-day-old female who presented with 10 days of poor feeding, irritability, and progressive hypotonia. Although upon immediate presentation emergency providers' differential diagnoses included meningitis, inborn error of metabolism, and genetic neurodegenerative disease, a subsequent magnetic resonance (MR) imaging of the total spine revealed a large intradural intramedullary mass extending from the medulla to the thoracic cord at T12. The patient underwent multilevel cervical and thoracic laminectomies/laminoplasty for maximal safe resection. Histopathology revealed mostly mature tissue elements originating from all 3 germ layers, interspersed with foci of immature neuroepithelium, consistent with grade 1 immature teratoma. Following surgical intervention, the patient regained strength and spontaneous movement and underwent physical therapy. Follow-up MR imaging of the total spine was obtained every 3 months, and at 9 months, recurrence was demonstrated, which was successfully treated with chemotherapy. Further surveillance MR imaging of the total spine has demonstrated cystic myelomalacia changes without definite tumor recurrence, at 5-year follow-up. Clinically, the patient has developed scoliosis without weakness, pain, or urinary symptoms.
This case demonstrates an exceptionally rare and unusual variant neoplasm in a neonate and highlights the difficulty of diagnosis and the important role of MR imaging. It also illustrates the importance of gross total resection, the risk of recurrence, and the need for close radiographic follow-up of these lesions. It also provides a useful example of the efficacy of adjuvant chemotherapy in treating recurrence.
不成熟畸胎瘤是一种已知的儿科肿瘤。然而，脊柱变体较为罕见，在诊断和治疗方面都极具挑战性，尤其是在涉及切除范围、复发可能性和预后一致性、辅助治疗的必要性和疗效等方面。
患者为一名 27 天大的女性，因 10 天来喂养不良、易激惹和进行性肌无力而就诊。尽管最初的鉴别诊断包括脑膜炎、先天性代谢缺陷和遗传性神经退行性疾病，但随后的全脊柱磁共振成像显示，一个大的硬脊膜内髓内肿块从延髓延伸至 T12 胸段脊髓。患者接受了多节段颈椎和胸椎椎板切除术/椎板成形术，以进行最大程度的安全切除。组织病理学显示，主要是源自三个胚层的成熟组织成分，散布着不成熟神经上皮的病灶，符合 1 级不成熟畸胎瘤。手术干预后，患者恢复了力量和自发性运动，并接受了物理治疗。每 3 个月进行全脊柱磁共振成像随访，9 个月时显示复发，成功接受化疗治疗。进一步的全脊柱磁共振成像随访显示，5 年时囊性脊髓软化改变，但无明确肿瘤复发，患者目前仍处于临床观察中。患者出现脊柱侧凸，但无无力、疼痛或排尿症状。
本病例展示了一例罕见的新生儿罕见且异常的肿瘤变体，强调了诊断的困难和磁共振成像的重要作用。还说明了大体全切除的重要性、复发的风险，以及这些病变密切影像学随访的必要性。同时还提供了辅助化疗治疗复发的有效性的一个有用示例。
