Rodriguez Vaca M D, Angel M, Halperin I, Freixenet J, Marti M, Martinez Osaba M J, Sanchez Lloret J, Palacin A, Vilardell E
Endocrinology and Diabetes Unit, Hospital Clinic, School of Medicine, Barcelona, Spain.
J Endocrinol Invest. 1987 Dec;10(6):537-40. doi: 10.1007/BF03346989.
A 26-yr-old male was submitted to bilateral adrenalectomy in 1977 for Cushing's syndrome. Some months later he developed intense skin hyperpigmentation together with increased ACTH levels (149 to 4000 ng/l). The sellar region was always normal in X-ray studies. In April 1985, when the patient complained of chest pain, a chest x-ray showed a polycyclic mass in the upper left lobe of the lung. ACTH ranged from 20,000 to 100,000 ng/l, with no response to CRF or cyproheptadine administration. Urinary 5-OH-indolacetic acid was negative. Thoracotomy was performed in July 1985 with resection of two intrapulmonary masses. Histologic study demonstrated a carcinoid tumor, with positive neuron-specific enolase and ACTH immunochemical stain. ACTH concentration in tumoral tissue was 91 pg/g tissue. After surgery ACTH fell dramatically to 37 ng/l, and has remained at this level since then, associated with resolution of the skin hyperpigmentation.
一名26岁男性于1977年因库欣综合征接受双侧肾上腺切除术。数月后,他出现了严重的皮肤色素沉着,同时促肾上腺皮质激素(ACTH)水平升高(从149至4000 ng/l)。蝶鞍区的X线检查始终正常。1985年4月,患者主诉胸痛,胸部X线显示左肺上叶有一个多环肿块。ACTH水平在20,000至100,000 ng/l之间,对促肾上腺皮质激素释放因子(CRF)或赛庚啶给药无反应。尿5-羟吲哚乙酸检测为阴性。1985年7月进行了开胸手术,切除了两个肺内肿块。组织学研究显示为类癌肿瘤,神经元特异性烯醇化酶和ACTH免疫化学染色呈阳性。肿瘤组织中的ACTH浓度为91 pg/g组织。手术后,ACTH水平急剧降至37 ng/l,此后一直维持在该水平,同时皮肤色素沉着也有所消退。