Feng Jeffrey J, Cannon Sophie M, Cheok Stephanie K, Shiroishi Mark S, Hurth Kyle M, Mathew Anna J, Zada Gabriel, Carmichael John D
Department of Neurological Surgery, Keck School of Medicine of the University of Southern California, Los Angeles, California.
Western Michigan University Homer Stryker M.D. School of Medicine, Kalamazoo, Michigan.
AACE Clin Case Rep. 2024 Sep 12;11(1):14-17. doi: 10.1016/j.aace.2024.09.003. eCollection 2025 Jan-Feb.
BACKGROUND/OBJECTIVE: Although most gonadotroph cell-derived pituitary adenomas (PAs) give rise to nonfunctional PAs, hormonally active functional gonadotroph adenomas (FGAs) are exceedingly rare. We present a case of a giant and invasive functional gonadotropic pituitary macroadenoma treated with endoscopic transsphenoidal surgery and subsequent postoperative radiotherapy.
A 54-year-old man presented with gradually worsening vision over 1 year. Magnetic resonance imaging demonstrated a 5.2-cm sellar and suprasellar mass with cavernous sinus invasion, mass effect on the optic chiasm, and extension into the sphenoid sinus, nasal cavity, and clivus. Preoperative workup was remarkable for erythrocytosis without sleep apnea and increased levels of follicle-stimulating hormone (FSH), luteinizing hormone (LH), prolactin, and testosterone. Immunohistochemistry results following endoscopic transsphenoidal resection confirmed dominant staining for steroidogenic factor-1, FSH, and LH. Postoperatively, the patient's FSH level decreased, whereas the LH level normalized within 1 week. The free testosterone level normalized at 9 months. The patient underwent radiotherapy for a small amount of residual tumor in the right cavernous sinus and has demonstrated no evidence of disease or hormonal progression.
There is no consensus on FGA-specific management that differs from the management of nonfunctional PAs; surgery is recommended when vision is impacted. The invasive nature of the tumor presented in this case is rare and limited safe gross total resection, requiring adjuvant radiotherapy.
FGAs are rare, and those of similar size and extent of invasion as in our case are even more so. In addition to surgical resection, consideration of adjunct therapies including radiation and multidisciplinary physician involvement are vital in achieving clinical improvement and remission while preventing possible progression and recurrence.
背景/目的：尽管大多数促性腺激素细胞来源的垂体腺瘤（PA）会发展为无功能垂体腺瘤，但具有激素活性的功能性促性腺激素腺瘤（FGA）极为罕见。我们报告一例巨大侵袭性功能性促性腺激素垂体大腺瘤，采用内镜经蝶窦手术及术后放疗进行治疗。
一名54岁男性，视力逐渐下降1年。磁共振成像显示鞍内及鞍上有一个5.2厘米的肿块，侵犯海绵窦，对视交叉产生占位效应，并延伸至蝶窦、鼻腔和斜坡。术前检查发现红细胞增多症但无睡眠呼吸暂停，促卵泡生成素（FSH）、促黄体生成素（LH）、催乳素和睾酮水平升高。内镜经蝶窦切除术后的免疫组织化学结果证实，类固醇生成因子-1、FSH和LH呈主要染色。术后，患者的FSH水平下降，而LH水平在1周内恢复正常。游离睾酮水平在9个月时恢复正常。患者因右侧海绵窦少量残留肿瘤接受了放疗，目前未发现疾病或激素进展的证据。
对于FGA的特异性治疗，目前尚无不同于无功能PA治疗的共识；当视力受到影响时，建议进行手术。本例肿瘤的侵袭性罕见，安全的全切除范围有限，需要辅助放疗。
FGA罕见，像我们病例中大小和侵袭范围相似的更是如此。除手术切除外，考虑包括放疗和多学科医生参与的辅助治疗对于实现临床改善和缓解、预防可能的进展和复发至关重要。
