Background/Objectives: Pituitary tumors account for over 90% of all sellar region masses. However, a spectrum of rare neoplastic, inflammatory, infectious, and vascular lesions—benign and malignant—can arise in the intra- and parasellar compartments and clinically and radiologically mimic PitNETs. We report a cohort of 47 such rare and cystic midline intracranial lesions, emphasizing their distinctive morphological, clinical, and imaging features and the personalized treatment strategies applied.Methods: In this retrospective single-center study, we reviewed all patients treated for suspected PitNETs via transsphenoidal approach between 2015 and 2024. Of 529 surgical cases, we excluded confirmed PitNETs, meningiomas, and classical intradural craniopharyngiomas. Collected data encompassed patient demographics, tumor characteristics, presenting symptoms, extent of resection or medical therapy, endocrine outcomes, and follow-up information.Results: Among all 529 patients who underwent surgical treatment for sellar lesions from 2015 to 2024, 47 cases (8.9%) were identified as rare or cystic masses. Forty-six underwent transsphenoidal resection; one patient with hypophysitis received corticosteroid therapy alone. Presenting symptoms included headache (n = 16), dizziness (n = 5), oculomotor disturbances (n = 2), and visual impairment (n = 17). Endocrine dysfunction was found in 30 patients, 27 of whom required hydrocortisone replacement. Histopathological diagnoses were led by colloid cysts (n = 14) and Rathke’s cleft cysts (n = 11). The remaining 22 cases comprised plasmacytoma, germinoma, lymphoma, pituicytoma, inverted papilloma, metastatic carcinoma, chordoma, nasopharyngeal carcinoma, chloroma, and other rare entities. Preoperative imaging diagnosis proved incorrect in 38% (18/47) of cases, with several lesions initially misidentified as PitNETs.Conclusions:Nearly 9% of presumed PitNETs were rare, often benign or inflammatory lesions requiring distinct management. Most could be safely resected and demonstrated excellent long-term outcomes. Yet, despite advanced imaging techniques, accurate preoperative differentiation remains challenging, with over one-third misdiagnosed. Clinical red flags—such as early hormone deficits, rapid progression or atypical imaging findings—should prompt early interdisciplinary evaluation and, when indicated, image-guided biopsy to avoid unnecessary surgery and ensure tailored therapy.
**背景/目的：** 垂体肿瘤占所有鞍区占位性病变的90%以上。然而，一系列罕见的肿瘤性、炎症性、感染性和血管性病变——包括良性和恶性——可发生于鞍内及鞍旁区域，并在临床和影像学上模仿垂体神经内分泌肿瘤。我们报告了一组47例此类罕见且囊性的中线颅内病变，重点阐述其独特的形态学、临床及影像学特征，以及所应用的个体化治疗策略。 **方法：** 在这项回顾性单中心研究中，我们回顾了2015年至2024年间所有因疑似垂体神经内分泌肿瘤而经蝶窦入路接受治疗的患者。在529例手术病例中，我们排除了确诊的垂体神经内分泌肿瘤、脑膜瘤和典型的硬膜内颅咽管瘤。收集的数据包括患者人口统计学特征、肿瘤特征、临床表现、切除范围或药物治疗情况、内分泌结局以及随访信息。 **结果：** 在2015年至2024年间因鞍区病变接受手术治疗的所有529例患者中，47例（8.9%）被确认为罕见或囊性占位。其中46例接受了经蝶窦切除术；1例垂体炎患者仅接受了皮质类固醇治疗。临床表现包括头痛（n=16）、头晕（n=5）、动眼神经障碍（n=2）和视力障碍（n=17）。30例患者存在内分泌功能障碍，其中27例需要氢化可的松替代治疗。组织病理学诊断以胶样囊肿（n=14）和拉克氏囊肿（n=11）为主。其余22例包括浆细胞瘤、生殖细胞瘤、淋巴瘤、垂体细胞瘤、内翻性乳头状瘤、转移癌、脊索瘤、鼻咽癌、绿色瘤及其他罕见病变。术前影像学诊断在38%（18/47）的病例中被证明是错误的，其中多个病变最初被误判为垂体神经内分泌肿瘤。 **结论：** 近9%的疑似垂体神经内分泌肿瘤实为罕见病变，通常是需要特殊处理的良性或炎性病变。大多数病变可安全切除，并显示出良好的远期预后。然而，尽管影像技术先进，准确的术前鉴别仍然具有挑战性，超过三分之一的病例被误诊。临床警示征象——如早期激素缺乏、快速进展或不典型影像学表现——应促使早期进行多学科评估，并在必要时进行影像引导下活检，以避免不必要的手术并确保个体化治疗。
肿瘤(癌症)患者之家