Department of Internal Medicine and Endocrine Unit, Medical School and Clementino Fraga Filho University Hospital, Federal University of Rio de Janeiro Endocrinology Section, Federal Hospital of Lagoa.
Endocr Pract. 2013 Nov-Dec;19(6):e145-9. doi: 10.4158/EP12400.CR.
To describe a case of a pure silent somatotroph pituitary carcinoma.
We describe a 54-year-old female with a clinically nonfunctioning pituitary macroadenoma diagnosed 15 years earlier.
The patient underwent transsphenoidal surgery and no visible tumor remnant was observed for 6 years. A magnetic resonance imaging (MRI) detected the recurrence of a 1.2 × 1.5 cm macroadenoma. The patient was submitted to conventional radiotherapy (4500 cGy), and the tumor volume remained stable for 7 years. Then, an MRI revealed a slight increase in tumor size, and 2 years later, a subsequent MRI detected a very large, invasive pituitary mass. The patient was resubmitted to transsphenoidal surgery, and the histopathological examination showed diffuse positivity for growth hormone (GH). The nadir GH level during an oral glucose tolerance test was 0.06 ng/mL, and the pre- and postoperative insulin like growth factor type I (IGF-I) levels were within the normal range. Abdominal, chest, brain, and spine MRI showed multiple small and hypervascular liver and bone lesions suggestive of metastases. Liver biopsy confirmed metastasis of GH-producing pituitary carcinoma. The patient has been treated with temozolomide and zoledronic acid for 7 months and with octreotide long-acting release (LAR) for 4 months. The primary tumor and metastases are stable.
Despite being an extremely rare event, pituitary carcinoma may develop several years after the successful treatment of even a silent GH-producing pituitary adenoma, which suggests that close long-term follow-up is necessary.
描述一例单纯性无功能性垂体生长激素腺瘤。
我们描述了一位 54 岁女性,15 年前诊断为临床无功能性垂体大腺瘤。
患者接受了经蝶窦手术,6 年内未见可见肿瘤残留。磁共振成像(MRI)发现复发了 1.2×1.5cm 的大腺瘤。患者接受了常规放疗(4500cGy),肿瘤体积稳定了 7 年。随后 MRI 显示肿瘤体积略有增加,2 年后,再次 MRI 检测到一个非常大的侵袭性垂体肿块。患者再次接受经蝶窦手术,组织病理学检查显示生长激素(GH)弥漫阳性。口服葡萄糖耐量试验时 GH 水平最低为 0.06ng/ml,术前和术后胰岛素样生长因子 I(IGF-I)水平均在正常范围内。腹部、胸部、脑部和脊柱 MRI 显示多个小而富血管的肝和骨病变,提示转移。肝活检证实为 GH 分泌性垂体癌转移。患者已接受替莫唑胺和唑来膦酸治疗 7 个月,奥曲肽长效释放(LAR)治疗 4 个月。原发肿瘤和转移灶稳定。
尽管是一种极为罕见的事件,但即使是成功治疗后的无功能性 GH 分泌性垂体腺瘤,也可能在数年后发展为垂体癌,这表明需要密切的长期随访。