Gandhi Gunjan Y, Fung Russell, Natter Patrick E, Makary Raafat, Balaji K C
Division of Endocrinology, University of Florida College of Medicine-Jacksonville, Jacksonville, FL, USA.
Department of Medicine, University of Florida College of Medicine-Jacksonville, Jacksonville, FL, USA.
Case Rep Endocrinol. 2020 Aug 18;2020:8883864. doi: 10.1155/2020/8883864. eCollection 2020.
Metastasis to the pituitary gland is extremely rare (∼2% of sellar masses). Clinical, biochemical, and radiologic characteristics of pituitary metastasis are poorly defined and can be difficult to diagnose before surgery. We present an unusual case with pituitary metastasis as the first manifestation of renal cell carcinoma (RCC). A 70-year-old male presented with acute onset of weakness, dizziness, diplopia, and progressively worsening headache. The initial CT head revealed a heterogeneous sellar mass measuring 2.8 × 1.9 × 1.7 cm. A follow-up MRI showed the sellar mass invading the right cavernous sinus. The presumptive diagnosis was a pituitary macroadenoma. Physical examination revealed bilateral 6th cranial nerve palsy and episodes of intermittent binocular horizontal diplopia. Hormonal testing noted possible secondary adrenal insufficiency (AM serum cortisol: 3.3 mcg/dL, ACTH: 8 pg/mL), secondary hypothyroidism (TSH: <0.01 mIU/L, FT4: 0.7 ng/dL), secondary hypogonadism (testosterone: 47 ng/dL, LH: 1.3 mIU/mL, and FSH: 2.3 mIU/mL), and elevated serum prolactin (prolactin: 56.8 ng/ml, normal: 4.0-15.2 ng/ml). IGF-1 level was normal at 110 ng/mL (47-192 ng/mL). The patient was discharged on levothyroxine and hydrocortisone therapy with plans for close surveillance. However, his condition worsened over the next three months, and he was subsequently readmitted with nausea, vomiting, and hypernatremia secondary to diabetes insipidus. Repeat MRI pituitary showed an interval increase in the size of the sellar mass with suprasellar extension and a new mass effect on the optic chiasm. The sellar mass was urgently resected via a trans-sphenoidal approach. The tumor was negative for neuroendocrine markers and pituitary hormone panel, ruling out the diagnosis of pituitary adenoma and triggered workup for metastatic renal cell carcinoma, clear cell type. The diagnosis of renal cell carcinoma was confirmed by the diffuse and strong staining for renal cell carcinoma markers (Pax-8, RCC-1, and CD10). A follow-up CT scan noted large right renal mass measuring 11 × 10 × 11 cm. The patient underwent a cytoreductive robotic right radical nephrectomy for WHO/ISUP histologic grade II clear cell RCC, stage pT2b pNX pM1. He subsequently received fractionated stereotactic radiotherapy to the pituitary gland. He is presently stable with no radiological evidence of progression or new intracranial disease on subsequent imaging. Pituitary metastasis most commonly occurs from breast, lung, or gastrointestinal tumors but also rarely from renal cell carcinoma. Biochemical findings such as panhypopituitarism, acute clinical signs such as headache, visual symptoms, and diabetes insipidus and interval increase in sellar mass in a short time interval should raise suspicion for sellar metastasis.
垂体转移极为罕见(约占鞍区肿块的2%)。垂体转移瘤的临床、生化及影像学特征尚不明确,术前诊断可能存在困难。我们报告一例以垂体转移为肾细胞癌(RCC)首发表现的罕见病例。一名70岁男性,急性起病,出现乏力、头晕、复视及进行性加重的头痛。最初的头颅CT显示鞍区有一大小为2.8×1.9×1.7 cm的不均匀肿块。后续的MRI显示鞍区肿块侵犯右侧海绵窦。初步诊断为垂体大腺瘤。体格检查发现双侧第6颅神经麻痹及间歇性双眼水平复视发作。激素检测提示可能存在继发性肾上腺功能不全(上午血清皮质醇:3.3μg/dL,促肾上腺皮质激素:8 pg/mL)、继发性甲状腺功能减退(促甲状腺激素:<0.01 mIU/L,游离甲状腺素:0.7 ng/dL)、继发性性腺功能减退(睾酮:47 ng/dL,促黄体生成素:1.3 mIU/mL,卵泡刺激素:2.3 mIU/mL),血清催乳素升高(催乳素:56.8 ng/ml,正常:4.0 - 15.2 ng/ml)。胰岛素样生长因子-1水平正常,为110 ng/mL(47 - 192 ng/mL)。患者出院时接受左甲状腺素和氢化可的松治疗,并计划密切监测。然而,在接下来的三个月里他的病情恶化,随后因尿崩症继发恶心、呕吐和高钠血症再次入院。垂体MRI复查显示鞍区肿块大小增大,向上延伸至鞍上,对视交叉产生新的占位效应。通过经蝶窦入路紧急切除鞍区肿块。肿瘤神经内分泌标志物和垂体激素检测均为阴性,排除垂体腺瘤诊断,进而启动了转移性肾细胞癌(透明细胞型)的检查。肾细胞癌标志物(PAX - 8、RCC - 1和CD10)弥漫性强染色确诊为肾细胞癌。后续CT扫描发现右肾有一大小为11×10×11 cm的巨大肿块。患者接受了机器人辅助下的右肾肿瘤减瘤性根治性切除术,病理为世界卫生组织/国际泌尿病理学会组织学II级透明细胞肾细胞癌,分期为pT2b pNX pM1。随后他接受了垂体的分次立体定向放射治疗。目前患者病情稳定,后续影像学检查未发现进展或新的颅内病变的放射学证据。垂体转移最常见于乳腺癌、肺癌或胃肠道肿瘤,但也很少来源于肾细胞癌。全垂体功能减退等生化表现、头痛、视觉症状和尿崩症等急性临床症状以及短时间内鞍区肿块大小增大应引起对鞍区转移的怀疑。