■原发性颅内神经内分泌肿瘤(NETs)极为罕见,经常带来诊断挑战,特别是在非分泌变体中。这些肿瘤最初可能存在非特异性症状,导致诊断延迟和潜在的神经系统并发症。
方法:我们介绍了一例33岁男性入院,有一年的逐渐恶化的头痛并伴有急性左上下垂和复视的病史。初步检查显示左眼下垂和下斜视,指示第三和第四脑神经麻痹。肠镜检查显示蝶骨轻度前壁隆起,粘膜正常。MRI成像揭示了一个向鞍区和左海绵窦延伸的广阔的斜坡过程,完全占据蝶窦并对垂体柄施加肿块效应。激素测定在参考范围内,排除了荷尔蒙活跃的肿瘤.内镜鼻内手术活检显示低度神经内分泌肿瘤细胞角蛋白AE1/AE2,嗜铬粒蛋白A阳性,突触素,和β-连环蛋白,Ki-67标记指数<2%。生长抑素受体闪烁显像证实了示踪剂在蝶骨肿瘤中的强烈过固定,支持它的神经内分泌起源。患者在知情同意后拒绝手术干预,放疗前选择C1生长抑素类似物。一年的随访显示症状稳定,无肿瘤进展。
■时至今日,关于这些案件的最佳管理的报告之间没有达成共识。成像评估对于验证肿瘤的原发性性质和排除任何远处定位至关重要。各种治疗方式,比如手术,放射治疗,和生长抑素类似物,应根据肿瘤的具体特征和程度来考虑。
结论:我们的病例清楚地提示神经内分泌肿瘤应被视为颅底肿瘤的鉴别诊断。
UNASSIGNED: Primary intracranial neuroendocrine tumors (NETs) are exceedingly rare, often posing diagnostic challenges, particularly in non-secreting variants. These tumors may initially present with nonspecific symptoms, leading to delayed diagnosis and potential neurological complications.
METHODS: We present the
case of a 33-year-old male admitted with a one-year history of progressively worsening headache accompanied by acute left ptosis and diplopia. Initial examination revealed left eye ptosis and hypotropia, indicative of third and fourth cranial nerve paralysis. Cavoscopy revealed a mild anterior wall bulge of the sphenoid with normal mucosa. MRI imaging unveiled an expansive clival process extending towards the sellar region and left cavernous sinus, completely occupying the sphenoid sinus and exerting mass effect on the pituitary stalk. Hormonal assays were within reference ranges, ruling out a hormonally-active tumor. Endoscopic endonasal surgery for biopsy revealed a low-grade neuroendocrine tumor positive for cytokeratin AE1/AE2, chromogranin A, synaptophysin, and beta-catenin, with a Ki-67-labeling index <2 %. Somatostatin receptor scintigraphy confirmed intense hyper fixation of the tracer in the sphenoidal tumor, supporting its neuroendocrine origin. The patient declined surgical intervention after informed consent, opting for C1 somatostatin analogs prior to radiotherapy. One-year follow-up demonstrated symptom stability with no tumor progression.
UNASSIGNED: To this day, no consensus among
reports concerning the optimal management of these cases. Imaging assessment is crucial to validate the primary nature of the tumor and to exclude any distant localization. Various therapeutic modalities, such as surgery, radiotherapy, and somatostatin analogs, should be considered based on the specific characteristics and extent of the tumor.
CONCLUSIONS: Our
case is a clear reminder that neuroendocrine tumors should be considered as a differential diagnosis for skull base neoplasms.