Wang Jinchao, Yao Zhigang, Xu Shangchen, Liu Bin
Department of Neurosurgery, Shandong Provincial Hospital Affiliated to Shandong First Medical University, Shandong First Medical University, Jinan, China.
Graduated School of Medicine, Shandong First Medical University, Jinan, China.
Front Surg. 2023 Jan 13;9:1080286. doi: 10.3389/fsurg.2022.1080286. eCollection 2022.
Malignant triton tumor (MTT) is a rare kind of malignant peripheral nerve sheath tumors, histologically characterized by rhabdomyoblastic differentiation. There are limited reports of MTT occurring in the intracranial area. The treatment modality consisting of total surgical resection plus post-operative radiotherapy is generally accepted. However, even with optimal treatment, most patients will die within a few months. We report a 71-year-old man with a history of pituitary adenoma, who underwent surgical treatment and postoperative gamma knife therapy. Magnetic resonance imaging (MRI) of the brain revealed a mass with two distinctive components in the sellar area. Postoperative pathology found that the lesion consisted of a MTT and a relapsed pituitary adenoma. The present case is the first report of MTT that occurred in the sellar area. It is also the first case of intracranial MTT with other concurrent tumors (relapsed pituitary tumors). Meanwhile, this case has a clear history of radiation therapy, suggesting that the occurrence of MTT may be related to radiation.
恶性蝾螈瘤（MTT）是一种罕见的恶性周围神经鞘瘤，组织学特征为横纹肌母细胞分化。关于MTT发生在颅内区域的报道有限。由手术全切加术后放疗组成的治疗方式普遍被接受。然而，即使采用最佳治疗，大多数患者也会在几个月内死亡。我们报告一名71岁有垂体腺瘤病史的男性，他接受了手术治疗和术后伽玛刀治疗。脑部磁共振成像（MRI）显示蝶鞍区有一个具有两个不同成分的肿块。术后病理发现病变由一个MTT和一个复发性垂体腺瘤组成。本病例是MTT发生在蝶鞍区的首例报道。也是颅内MTT合并其他并发肿瘤（复发性垂体肿瘤）的首例病例。同时，该病例有明确的放疗史，提示MTT的发生可能与放疗有关。
