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两位原发性鞍部平滑肌瘤患者,一种罕见的实体。

Two patients with primary sellar leiomyomas, a rare entity.

机构信息

Department of Neurosurgery, University of Washington School of Medicine, Seattle, WA 98195, USA.

出版信息

J Clin Neurosci. 2013 Jun;20(6):897-901. doi: 10.1016/j.jocn.2012.07.001. Epub 2012 Dec 6.

DOI:10.1016/j.jocn.2012.07.001
PMID:23219820
Abstract

Leiomyomas are benign smooth muscle tumors commonly found in the genitourinary or gastrointestinal tracts. Rarely, they present as primary intracranial extra-axial brain tumors. Most of these lesions have been described in immunocompromised patients, but have been found very rarely in the immunocompetent patient. We present two patients with sporadic sellar leiomyomas. The first patient is a 25-year-old woman who presented with a 2-year history of amenorrhea and a heterogeneous lesion. The second is a 53-year-old man who presented with headaches and progressive panhypopituitarism, and a large cystic lesion expanding the sella. In both patients, transnasal transphenoidal surgery was performed for resection of the tumor. We review the intraoperative findings, neuropathology and immunohistochemistry and the clinical follow-up. A literature search, which revealed only two prior reported cases of sporadic sellar leiomyomas, and subsequent review led us to conclude that the natural history of sellar leiomyomas relates to the immune status of the patient and that these tumors may cause pituitary dysfunction through infiltration of the gland, mass effect and compression, or even potentially as a byproduct of prolactin secretion intrinsic to the tumor itself. Complete surgical resection of these infiltrating tumors may not be advisable when pituitary function is intact. Long-term endocrine follow-up in these patients is advised.

摘要

平滑肌瘤是常见于泌尿生殖道或胃肠道的良性平滑肌肿瘤。它们很少作为原发性颅内轴外脑肿瘤出现。这些病变大多数发生在免疫功能低下的患者中,但在免疫功能正常的患者中很少发现。我们介绍了两名患有散发性鞍部平滑肌瘤的患者。第一例患者是一名 25 岁女性,因 2 年闭经和不均匀病变就诊。第二例是一名 53 岁男性,因头痛和进行性垂体功能减退症以及扩大鞍部的大囊性病变就诊。在这两名患者中,均通过经鼻蝶窦手术进行肿瘤切除。我们回顾了手术中的发现、神经病理学和免疫组织化学以及临床随访。文献检索仅显示了两例先前报道的散发性鞍部平滑肌瘤病例,随后的回顾使我们得出结论,鞍部平滑肌瘤的自然病程与患者的免疫状态有关,这些肿瘤可能通过浸润腺体、占位效应和压迫导致垂体功能障碍,甚至可能是肿瘤本身内在的催乳素分泌的副产物。当垂体功能完好时,不建议对这些浸润性肿瘤进行完全手术切除。建议对这些患者进行长期内分泌随访。

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Two patients with primary sellar leiomyomas, a rare entity.两位原发性鞍部平滑肌瘤患者,一种罕见的实体。
J Clin Neurosci. 2013 Jun;20(6):897-901. doi: 10.1016/j.jocn.2012.07.001. Epub 2012 Dec 6.
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Intraoperative magnetic resonance imaging and early prognosis for vision after transsphenoidal surgery for sellar lesions.经蝶窦手术治疗鞍区病变的术中磁共振成像与术后视力的早期预后
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Magnetic resonance imaging after transsphenoidal surgery of clinically non-functional pituitary macroadenomas and its impact on detecting residual adenoma.临床无功能垂体大腺瘤经蝶窦手术后的磁共振成像及其对检测残留腺瘤的影响。
Acta Neurochir (Wien). 2002 May;144(5):433-43. doi: 10.1007/s007010200064.
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Benign brain tumors: sellar/parasellar tumors.良性脑肿瘤:鞍区/鞍旁肿瘤。
Neurol Clin. 2007 Nov;25(4):1231-49, xi. doi: 10.1016/j.ncl.2007.07.003.
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Primary fibrosarcoma of the sella unrelated to previous radiation therapy.
Mod Pathol. 1998 Jun;11(6):579-84.
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Pituitary adenoma coexisting with a suprasellar arachnoid cyst.垂体腺瘤合并鞍上蛛网膜囊肿。
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2
A Rare Case of a Primary Leiomyoma of the Clivus in an Immunocompetent Patient and a Review of the Literature Regarding Clival Lesions.免疫功能正常患者中斜坡原发性平滑肌瘤的罕见病例及关于斜坡病变的文献综述
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