Chair of Endocrinology, University of Milan, IRCCS Ospedale San Luca, Istituto Auxologico Italiano, Milan, Italy.
Endocr J. 2010;57(9):833-7. doi: 10.1507/endocrj.k10e-140. Epub 2010 Jun 26.
Double pituitary adenomas are rare occurrences in autoptical, surgical and neuroradiological series and are mostly due to non-functioning pituitary adenomas, GH-secreting and prolactin-secreting adenomas. ACTH secreting tumours are more rare and, to our knowledge, two distinct ACTH-producing adenomas within the same pituitary have never been reported. We herewith describe a 56 year old woman with Cushing' s disease due to two clearly distinct ACTH-secreting pituitary adenomas. She presented with signs and symptoms of hypercortisolism and hormonal testing was indicative for pituitary-dependent Cushing' s syndrome. Sellar MRI visualized an asymmetric pituitary gland with suspect lesions in both the right and the left pituitary lobes. Both lesions were removed during transsphenoidal surgery and remission of hypercortisolism ensued. Pathology confirmed the existence of two distinct adenomas located in different sites in the gland. Both presented ACTH immunoreactivity but displayed distinct morphological features. Our case indicates that double ACTH-secreting pituitary adenomas may occur in patients with Cushing' s disease. Careful radiological, surgical and pathological examination is necessary to recognize this condition and avoid surgical failure.
双重垂体腺瘤在尸检、外科和神经放射学系列中较为罕见，主要由无功能垂体腺瘤、生长激素分泌和泌乳素分泌腺瘤引起。促肾上腺皮质激素（ACTH）分泌肿瘤更为罕见，据我们所知，同一垂体内从未报道过两个不同的 ACTH 分泌性腺瘤。我们在此描述了一位 56 岁女性，因两个明显不同的 ACTH 分泌性垂体腺瘤而患有库欣病。她表现出皮质醇增多症的体征和症状，激素检测提示为垂体依赖性库欣综合征。鞍区 MRI 显示不对称垂体，右侧和左侧垂体叶均有可疑病变。这两个病变都在经蝶窦手术中被切除，皮质醇增多症随之缓解。病理证实存在两个位于不同部位的不同的腺瘤。两个腺瘤均表现出 ACTH 免疫反应性，但具有不同的形态特征。我们的病例表明，双重 ACTH 分泌性垂体腺瘤可能发生在库欣病患者中。需要仔细的影像学、外科和病理学检查来识别这种情况，以避免手术失败。
