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鞍区和鞍上区颗粒细胞瘤:11例临床病理研究及文献复习

Granular cell tumor of the sellar and suprasellar region: clinicopathologic study of 11 cases and literature review.

作者信息

Cohen-Gadol Aaron A, Pichelmann Mark A, Link Michael J, Scheithauer Bernd W, Krecke Karl N, Young William F, Hardy Jules, Giannini Caterina

机构信息

Department of Neurologic Surgery, Mayo Clinic, Rochester, Minn 55905, USA.

出版信息

Mayo Clin Proc. 2003 May;78(5):567-73. doi: 10.4065/78.5.567.

Abstract

OBJECTIVE

To report a series of cases of surgically resected granular cell tumors in an attempt to better characterize their clinical presentation, imaging features, and treatment outcomes with attention to previously published literature.

PATIENTS AND METHODS

This is a retrospective study of 11 patients with granular cell tumors. Information obtained from patients' medical records was supplemented by direct physician and patient telephone contact.

RESULTS

The study group consisted of 9 women and 2 men, with a mean +/- SD age of 50 +/- 15 years (range, 26-73 years). Seven patients were symptomatic, 3 of whom presented with visual complaints. Four patients presented primarily with endocrine dysfunction (2) or headaches (2). The duration of symptoms varied from 1 to 12 months (mean +/- SD, 6.0 +/- 4.5 months). Formal visual field testing revealed bitemporal hemianopsia in 6 and a right-sided visual field deficit in 2. The tumor appeared as a well-defined, relatively homogeneously (6) or heterogeneously (1) enhancing suprasellar mass on contrast infusion. Tumor size varied from 1.5 to 6.0 cm (mean +/- SD, 3.1 +/- 1.6 cm). At surgery, the mass was noted to be firm and vascular, and in at least 3 patients these features prevented gross total resection. Only 1 patient who had undergone biopsy of his lesion received adjuvant radiation therapy. Follow-up was obtained in all but 1 patient. Nine of these 10 patients are alive and are either free of disease or have had no disease progression. Follow-up in these 9 patients varied from 1 to 16 years (mean +/- SD, 6.5 +/- 53 years).

CONCLUSIONS

A granular cell tumor is generally a surprise finding in that it is seldom considered in the differential diagnosis of contrast-enhancing, demarcated sellar region masses. Granular cell tumors are benign and slow growing with no pronounced tendency for invasion or recurrence. Because of the indolent growth of granular cell tumors, the surgical goal of tumor decompression is a reasonable therapeutic approach.

摘要

目的

报告一系列经手术切除的颗粒细胞瘤病例,以更好地描述其临床表现、影像学特征和治疗结果,并关注既往发表的文献。

患者与方法

这是一项对11例颗粒细胞瘤患者的回顾性研究。从患者病历中获取的信息通过医生与患者直接电话联系进行补充。

结果

研究组包括9名女性和2名男性,平均年龄±标准差为50±15岁(范围26 - 73岁)。7例患者有症状,其中3例有视觉症状。4例患者主要表现为内分泌功能障碍(2例)或头痛(2例)。症状持续时间从1个月至12个月不等(平均±标准差,6.0±4.5个月)。正规视野检查显示6例为双颞侧偏盲,2例为右侧视野缺损。肿瘤在增强扫描时表现为鞍上边界清晰、相对均匀(6例)或不均匀(1例)强化的肿块。肿瘤大小从1.5厘米至6.0厘米不等(平均±标准差,3.1±1.6厘米)。手术中,肿块质地硬且有血管,至少3例患者因这些特征无法进行全切。仅1例曾对病变进行活检的患者接受了辅助放疗。除1例患者外,其余患者均获得随访。这10例患者中的9例存活,无疾病或疾病无进展。这9例患者的随访时间从1年至16年不等(平均±标准差,6.5±5.3年)。

结论

颗粒细胞瘤通常是意外发现,因为在鞍区对比增强、边界清晰肿块的鉴别诊断中很少考虑到它。颗粒细胞瘤是良性的,生长缓慢,无明显侵袭或复发倾向。由于颗粒细胞瘤生长缓慢,手术减压的目标是一种合理的治疗方法。

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