Ganguly Subhro, Gawarle Surendra, Keche Prashant
Department of Otorhinolaryngology, Shri Vasantrao Naik Government Medical College, Yavatmal, Maharashtra, India.
Department of Otorhinolaryngology, Aurangabad Government Medical College, Aurangabad, Maharashtra, India.
Iran J Otorhinolaryngol. 2018 Nov;30(101):355-359.
Teratomas are neoplastic tumors derived from totipotent germ cells containing a wide assortment of tissues originating from all three germ cell layers. Teratomas can be mature or immature depending on the presence of immature tissues; typically neuroepithelial tissue. Immature teratomas can be oncologically benign or malignant, and can be divided into three grades with increasingly aggressive biological behavior. The most common site for this tumor is the sacrococcygeal region. The nasal septum is an exceptionally rare site for immature teratomas, with very few cases reported.
We discuss a 14-year-old male patient with a left nasal mass which, on histopathological examination, turned out to be a Grade-3 immature teratoma. Imaging revealed the mass to be confined in the left nasal cavity with erosion of the anterior skull base. During endoscopic excision, the tumor was seen extending intracranially but remaining extradurally. Complete resection was achieved, albeit with mild cerebrospinal fluid (CSF) leakage, which was closed successfully. The patient was subjected to adjuvant chemotherapy. A regular follow-up of 2 years showed no recurrence.
The purpose of this report is to document the first case of a high-grade immature teratoma arising from the nasal septum with intracranial extension, as well as the efficacy of combined endoscopic resection and adjuvant chemotherapy for this pathology.
畸胎瘤是源自全能生殖细胞的肿瘤,包含源自所有三个胚层的多种组织。根据是否存在未成熟组织,畸胎瘤可分为成熟型或未成熟型;典型的未成熟组织为神经上皮组织。未成熟畸胎瘤在肿瘤学上可为良性或恶性,可分为三个级别,其生物学行为的侵袭性逐渐增强。该肿瘤最常见的部位是骶尾部。鼻中隔是未成熟畸胎瘤极为罕见的部位,仅有极少数病例报道。
我们讨论一名14岁男性患者,其左侧鼻腔有肿物,经组织病理学检查证实为3级未成熟畸胎瘤。影像学检查显示肿物局限于左侧鼻腔,伴有前颅底骨质侵蚀。在内镜切除过程中,发现肿瘤向颅内延伸,但仍位于硬膜外。尽管有轻度脑脊液漏,但成功实现了完整切除,脑脊液漏也成功封闭。患者接受了辅助化疗。2年的定期随访显示无复发。
本报告的目的是记录首例起源于鼻中隔且向颅内延伸的高级别未成熟畸胎瘤病例,以及内镜切除联合辅助化疗对该病理情况的疗效。