Brignardello E, Manti R, Papotti M, Allìa E, Campra D, Isolato G, Cassinis M C, Fronda G, Boccuzzi G
Division of Endocrine Oncology, COES, University of Turin, Turin, Italy.
J Endocrinol Invest. 2004 Apr;27(4):361-5. doi: 10.1007/BF03351063.
Ectopic production of biologically active glycoprotein hormones other than hCG has been reported in exceptional cases. A 61-yr-old man came to our Unit complaining of weakness, fatigue and reduced libido with erectile dysfunction. There was also a history of polycythemia, known for about 10 yr and never further investigated. The physical examination showed acne and redness of facial skin and upper chest; no other significant abnormalities were detected. Serum levels of LH were very high, whereas alpha-subunit and hCG were only slightly increased. Testosterone and 17beta-estradiol levels were increased too. Abdominal computed tomography (CT) scan revealed a large hypervascularized mass within the pancreatic tail, which was surgically removed by distal splenopancreatectomy. Diffuse immunoreactivity for LH was detected in more than 70% of the tumor cells. The alpha-subunit was also positive, while chorionic gonadotropin had only a focal reactivity. Reverse transcriptase-polymerase chain reaction (RT-PCR) and Southern Blot analysis confirmed the synthesis of LH by the tumor. Four weeks after surgery, serum levels of LH, alpha-subunit, testosterone, hCG and 17beta-estradiol were all undetectable. The redness of facial skin and upper chest had disappeared, but libido was still reduced. At a further control, 3 months after surgery, serum levels of LH, FSH, hCG, alpha-subunit and 17beta-estradiol were all within the normal range, as well as hemoglobin concentration and the red blood cells count. Testosterone was slightly below normal, but the patient reported an increase of libido. This is an unusual case of ectopic secretion of LH from an endocrine tumor of the pancreas.
据报道,在极少数情况下会出现除hCG以外的生物活性糖蛋白激素的异位产生。一名61岁男性因虚弱、疲劳、性欲减退伴勃起功能障碍前来我院就诊。患者还有真性红细胞增多症病史,已知约10年,从未进一步检查。体格检查发现面部皮肤和上胸部痤疮及发红;未发现其他明显异常。血清LH水平非常高,而α亚基和hCG仅略有升高。睾酮和17β-雌二醇水平也升高。腹部计算机断层扫描(CT)显示胰尾有一个大的血管丰富的肿块,通过远端脾胰切除术将其手术切除。在超过70%的肿瘤细胞中检测到LH的弥漫性免疫反应。α亚基也呈阳性,而绒毛膜促性腺激素仅有局灶性反应。逆转录聚合酶链反应(RT-PCR)和Southern印迹分析证实肿瘤合成了LH。手术后四周,血清LH、α亚基、睾酮、hCG和17β-雌二醇水平均无法检测到。面部皮肤和上胸部的发红消失,但性欲仍减退。在术后3个月的进一步复查中,血清LH、FSH、hCG、α亚基和17β-雌二醇水平均在正常范围内,血红蛋白浓度和红细胞计数也正常。睾酮略低于正常,但患者报告性欲有所增强。这是一例罕见的胰腺内分泌肿瘤异位分泌LH的病例。