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一名年轻成年人颅内生长性畸胎瘤综合征的罕见病例。

A Rare Case of Intracranial Growing Teratoma Syndrome in a Young Adult.

作者信息

Gwak Young Gook, Yang Seung Ho, Yoo Yeun Ji, Kim Hyun Ho, Lee Yujin, Kim Young Il

机构信息

Department of Neurosurgery, Incheon St. Mary's Hospital, College of Medicine, The Catholic University of Korea, Seoul, Korea.

Department of Neurosurgery, St. Vincent's Hospital, College of Medicine, The Catholic University of Korea, Seoul, Korea.

出版信息

Brain Tumor Res Treat. 2024 Jul;12(3):200-203. doi: 10.14791/btrt.2024.0025.

Abstract

Intracranial growing teratoma syndrome (iGTS) is a rare phenomenon in patients with non-germinomatous germ cell tumor (NGGCT) after chemotherapy or radiotherapy. It manifests as paradoxical growth of teratomatous components, with multiple cystic lesions on cranial imaging despite normalized tumor markers. This paper presents a 22-year-old male with iGTS, diagnosed one month after chemotherapy against NGGCT. Initially diagnosed with presumptive pineal NGGCT causing obstructive hydrocephalus, the patient underwent endoscopic third ventriculostomy and extraventricular drainage with tumor biopsy followed by two chemotherapy cycles. Despite normalization of tumor markers, follow-up MRI showed increased tumor size with honeycomb-like cystic patterns. The patient underwent suboccipital craniotomy for tumor removal via combined telovelar and infratentorial supracerebellar approaches. The final pathology confirmed mature teratoma. However, postoperative bleeding and left thalamic infarction occurred, resulting in severe neurological deficits. Despite challenges, the patient eventually regained the ability to follow simple commands. To understand iGTS pathophysiology, several hypotheses, including the differentiation of immature components and the uninhibited growth of mature components induced by chemotherapy or radiotherapy, were explored. Surgical intervention remains as an ideal treatment, while clinical trials investigate chemotherapy options. Frequent imaging follow-ups are crucial for early detection in iGTS for NGGCT patients.

摘要

颅内生长性畸胎瘤综合征(iGTS)是化疗或放疗后非生殖细胞性生殖细胞肿瘤(NGGCT)患者中罕见的现象。它表现为畸胎瘤成分的反常生长,尽管肿瘤标志物已恢复正常,但头颅影像学检查显示有多个囊性病变。本文介绍了一名患有iGTS的22岁男性,在针对NGGCT进行化疗后1个月被诊断出来。该患者最初被诊断为疑似松果体NGGCT导致梗阻性脑积水,接受了内镜下第三脑室造瘘术和脑室外引流并进行了肿瘤活检,随后进行了两个周期的化疗。尽管肿瘤标志物恢复正常,但后续的MRI显示肿瘤大小增加,呈蜂窝状囊性形态。患者接受了枕下开颅手术,通过联合经小脑幕和幕下小脑上入路切除肿瘤。最终病理证实为成熟畸胎瘤。然而,术后发生了出血和左侧丘脑梗死,导致严重的神经功能缺损。尽管面临挑战,患者最终恢复了执行简单指令的能力。为了了解iGTS的病理生理学,探讨了几种假说,包括未成熟成分的分化以及化疗或放疗诱导的成熟成分不受抑制的生长。手术干预仍然是理想的治疗方法,同时临床试验正在研究化疗方案。对于NGGCT患者的iGTS,频繁的影像学随访对于早期检测至关重要。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/43b9/11306839/35f7b29115e7/btrt-12-200-g001.jpg

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