Kamijo K, Sato M, Saito T, Yachi A, Minase T, Noro H, Kawahara T
Department of Internal Medicine (Section 1), Sapporo Medical College, Japan.
Pathol Res Pract. 1991 Jun;187(5):637-41. doi: 10.1016/S0344-0338(11)80162-7.
We report on a patient with ACTH and FSH producing invasive pituitary adenoma complaining of cutaneous pigmentation. Elevations in plasma ACTH, beta-endorphin and cortisol levels as well as urinary 17-OHCS and cortisol excretion were found. Serum FSH concentration was just within the upper limit of the normal range, whereas serum LH level was reduced and alpha-subunit level was normal. Roentogenographic examination showed an almost complete loss of sellar floor and destruction of the posterior clinoids and dorsum sella. CT scan and MRI demonstrated an enlarged tumor invasion of the clivus and its extension to the sphenoid sinus. After subtotal removal of the large pituitary tumor, serum cortisol and plasma beta-endorphin levels as well as plasma ACTH concentrations returned to normal and serum FSH levels also remarkably decreased. Histologically, the tumor corresponded to a chromophobe, slightly PAS positive adenoma. These tumor cells exhibited positive immunostaining with antibody to ACTH (1-24), beta-LPH, beta-endorphin and FSH, while immunostaining of the adenoma cells was negative for LH, TSH, GH and prolactin. The immunogold technique also demonstrated ACTH and FSH particles in the secretory granules in the cytoplasm of the adenoma cells. Some of the tumor cells disclosed Crooke's hyalinization and type I microfilament occupied most of the cytoplasm. In the present study, a very rare case of ACTH and FSH producing invasive pituitary adenoma is reported.
我们报告了一例患有促肾上腺皮质激素(ACTH)和促卵泡生成素(FSH)分泌性侵袭性垂体腺瘤且伴有皮肤色素沉着的患者。发现血浆ACTH、β-内啡肽和皮质醇水平升高,以及尿17-羟皮质类固醇(17-OHCS)和皮质醇排泄增加。血清FSH浓度刚好在正常范围上限,而血清促黄体生成素(LH)水平降低,α亚基水平正常。X线检查显示蝶鞍底几乎完全缺失,后床突和鞍背破坏。CT扫描和磁共振成像(MRI)显示肿瘤增大并侵犯斜坡,延伸至蝶窦。在大部分切除巨大垂体肿瘤后,血清皮质醇、血浆β-内啡肽水平以及血浆ACTH浓度恢复正常,血清FSH水平也显著下降。组织学上,肿瘤符合嫌色性、轻度过碘酸雪夫(PAS)阳性腺瘤。这些肿瘤细胞对ACTH(1-24)、β-促脂素(β-LPH)、β-内啡肽和FSH抗体呈阳性免疫染色,而腺瘤细胞对LH、促甲状腺激素(TSH)、生长激素(GH)和催乳素的免疫染色为阴性。免疫金技术也在腺瘤细胞胞质的分泌颗粒中显示出ACTH和FSH颗粒。部分肿瘤细胞显示出克鲁克透明变性,I型微丝占据了大部分胞质。在本研究中,报告了一例非常罕见的ACTH和FSH分泌性侵袭性垂体腺瘤病例。