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罕见的漏斗部肿瘤:临床表现、影像学表现及鼻内镜手术在其治疗中的作用

Rare infundibular tumors: clinical presentation, imaging findings, and the role of endoscopic endonasal surgery in their management.

作者信息

Koutourousiou Maria, Gardner Paul A, Kofler Julia K, Fernandez-Miranda Juan C, Snyderman Carl H, Lunsford L Dade

机构信息

Department of Neurological Surgery, University of Pittsburgh School of Medicine, Pittsburgh, Pennsylvania, United States.

Department of Pathology, University of Pittsburgh School of Medicine, Pittsburgh, Pennsylvania, United States.

出版信息

J Neurol Surg B Skull Base. 2013 Feb;74(1):1-11. doi: 10.1055/s-0032-1329619. Epub 2012 Dec 31.

Abstract

Background The spectrum of infundibular lesions is broad and distinct from sellar pathologies. In many cases, histology is needed to establish the correct diagnosis and determine the treatment approach. Methods Medical files of eight patients with distinct infundibular tumors were reviewed. Histopathologically confirmed diagnosis included three pituicytomas, three granular cell tumors, and two pilocytic astrocytomas. Results Patients shared similar imaging findings and clinical symptoms, including visual impairment (n = 5), hypopituitarism (n = 4), and headache (n = 4); one patient presented with disseminated disease and symptoms from spinal metastases. All the pituicytomas, two granular cell tumors, and one infundibular pilocytic astrocytoma case underwent endoscopic endonasal surgery; gross total resection was achieved in five patients, three developed postoperative diabetes insipidus, and two developed hypopituitarism. No recurrences were observed. One granular cell tumor patient was treated with gamma-knife radiosurgery after stereotactic biopsy; the tumor remained stable in size for over 9 years. The infundibular pilocytic astrocytoma patient who presented with spinal metastases received radiotherapy and systemic chemotherapy. The overall mean follow-up period was 25.1 months. Conclusion Infundibular tumors are rare entities that represent a diagnostic challenge. Histopathological examination is essential for definitive diagnosis. Surgery, radiation therapy, and chemotherapy all have a role in the management of these tumors.

摘要

背景

漏斗部病变范围广泛,与鞍区病变不同。在许多情况下,需要组织学检查来确立正确诊断并确定治疗方法。方法:回顾了8例不同类型漏斗部肿瘤患者的病历。组织病理学确诊的诊断包括3例垂体细胞瘤、3例颗粒细胞瘤和2例毛细胞型星形细胞瘤。结果:患者具有相似的影像学表现和临床症状,包括视力障碍(n = 5)、垂体功能减退(n = 4)和头痛(n = 4);1例患者出现播散性疾病及脊柱转移症状。所有垂体细胞瘤、2例颗粒细胞瘤和1例漏斗部毛细胞型星形细胞瘤患者接受了鼻内镜手术;5例患者实现了全切,3例出现术后尿崩症,2例出现垂体功能减退。未观察到复发。1例颗粒细胞瘤患者在立体定向活检后接受了伽玛刀放射外科治疗;肿瘤大小稳定超过9年。出现脊柱转移的漏斗部毛细胞型星形细胞瘤患者接受了放疗和全身化疗。总体平均随访期为25.1个月。结论:漏斗部肿瘤是罕见疾病,诊断具有挑战性。组织病理学检查对明确诊断至关重要。手术、放疗和化疗在这些肿瘤的治疗中均发挥作用。

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