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Two patients with primary sellar leiomyomas, a rare entity.

机译:2例原发性蝶鞍平滑肌瘤,罕见。

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摘要

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.
机译:平滑肌瘤是在泌尿生殖道或胃肠道中常见的良性平滑肌肿瘤。很少,它们表现为原发性颅内轴外脑肿瘤。这些损伤中的大多数已在免疫功能低下的患者中描述,但在免疫功能正常的患者中很少发现。我们目前有两名散发性鞍肌平滑肌瘤患者。第一名患者是一名25岁的女性,她有2年的闭经史和异质性病灶。第二个是一名53岁的男子,他出现头痛和进行性泛垂体功能减退,并有一个巨大的囊性病变扩大蝶鞍。在这两名患者中,均进行了经鼻经蝶窦手术切除肿瘤。我们回顾了术中发现,神经病理学和免疫组化以及临床随访。文献检索仅揭示了先前报道的两个散发性鞍状肌瘤病例,随后的综述使我们得出结论:鞍状肌瘤的自然病史与患者的免疫状态有关,并且这些肿瘤可能通过浸润性腺瘤导致垂体功能障碍。腺体,肿块效应和压迫,甚至可能是肿瘤本身固有的催乳激素分泌的副产物。当垂体功能完好时,不建议对这些浸润性肿瘤进行完整的手术切除。建议对这些患者进行长期内分泌随访。

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