Timofeeva N I, Chernikov R A, Sleptsov I V, Rusakov V F, Rebrova D V, Vorobyev S L, Pridvizhkina T S, Semenov A A, Alexeev M A, Kulikov A U
Saint Petersburg State University Hospital, Saint Petersburg State University.
National Center for Clinical Morphological Diagnostics.
Probl Endokrinol (Mosk). 2025 Jul 22;71(3):25-33. doi: 10.14341/probl13512.
Medullary thyroid carcinoma is a rare aggressive tumor of thyroid gland. Due to its neuroendocrine origine medullary thyroid carcinoma can be the source of ectopic production of different peptides and hormones. We describe a unique case of severe ACTH-dependent Cushing's syndrome due to ectopic corticotropin production by medullary thyroid carcinoma in a male patient of 39 years old with calcitonin level more than 4000 pg/ml (<11,8), thyroid nodule, multiple neck jugular and central lymphnodes. The potassium level was 1,34 mmol/l (3,5-5,1), hypercortisolemia up to 1613,2 nmol/l (185-624) with elevated level of ACTH up to 24,7 pmol/l (1,03-10,74). After the correction of water and electrolytes disorders an operation was performed - thyroidectomy, central and lateral neck dissection. Postoperative calcitonin decreased to 126 pg/ml (<11,8), calcium and parathormone blood levels remained normal. The potassium level didn't decrease without any pharmacological treatment. There was a sharp fall in postoperative blood levels of cortisol and ACTH. The hormonal replacement treatment with hydrocortisone was induced. Morphological examination showed medullary carcinoma with ACTH production in tumor nodule, with metastatic neck lymph nodes. Thus this is a rare case and successful treatment of patient with severe hypercortisolism by thyroidectomy and neck lymphnodes dissection.
甲状腺髓样癌是一种罕见的侵袭性甲状腺肿瘤。由于其神经内分泌起源，甲状腺髓样癌可成为不同肽类和激素异位产生的来源。我们描述了一例独特的严重促肾上腺皮质激素（ACTH）依赖性库欣综合征病例，该病例由一名39岁男性患者的甲状腺髓样癌异位分泌促肾上腺皮质激素所致，患者降钙素水平超过4000 pg/ml（<11.8），有甲状腺结节、颈部多个颈静脉和中央淋巴结。血钾水平为1.34 mmol/l（3.5 - 5.1），皮质醇血症高达1613.2 nmol/l（185 - 624），ACTH水平升高至24.7 pmol/l（1.03 - 10.74）。在纠正水电解质紊乱后进行了手术——甲状腺切除术、颈部中央和外侧淋巴结清扫术。术后降钙素降至126 pg/ml（<11.8），血钙和甲状旁腺激素水平保持正常。未经任何药物治疗，血钾水平未下降。术后皮质醇和ACTH的血液水平急剧下降。遂开始氢化可的松激素替代治疗。形态学检查显示肿瘤结节中有分泌ACTH的髓样癌，并伴有颈部淋巴结转移。因此，这是一例罕见病例，通过甲状腺切除术和颈部淋巴结清扫术成功治疗了严重皮质醇增多症患者。
