PDF(Pubmed)
Abstract：
Ectopic ACTH syndrome (EAS) remains one of the most demanding diagnostic and therapeutic challenges for endocrinologists. Thymic neuroendocrine tumors account for 5%-10% of all EAS cases. We report a unique case of a 31-year-old woman with severe EAS caused by primary metastatic combined large-cell neuroendocrine carcinoma and atypical carcinoid of the thymus. The patient presented with severe hypercortisolemia, which was successfully controlled with continuous etomidate infusion. Complex imaging initially failed to detect thymic lesion; however, it revealed a large, inhomogeneous, metabolically active left adrenal mass infiltrating the diaphragm, suspected of primary disease origin. The patient underwent unilateral adrenalectomy, which resulted in hypercortisolemia resolve. The pathology report showed an adenoma with adrenal infarction and necrosis. The thymic tumor was eventually revealed a few weeks later on follow-up imaging studies. Due to local invasion and rapid progression, only partial resection of the thymic tumor was possible, and the patient was started on radio- and chemotherapy.
摘要：
异位ACTH综合征（EAS）仍然是内分泌学家最苛刻的诊断和治疗挑战之一。胸腺神经内分泌肿瘤占所有EAS病例的5%-10%。我们报告了一例31岁女性的独特病例，该女性患有由原发性转移性大细胞神经内分泌癌和胸腺非典型类癌引起的严重EAS。病人出现严重的高皮质醇血症,通过连续输注依托咪酯成功控制。复杂成像最初未能检测到胸腺病变；然而，它揭示了一个很大的，不均匀，代谢活跃的左肾上腺肿块浸润隔膜，怀疑是原发疾病。患者接受了单侧肾上腺切除术，导致皮质醇血症消退。病理报告示腺瘤伴肾上腺梗死坏死。几周后，在后续的影像学研究中最终发现了胸腺肿瘤。由于当地入侵和快速发展，仅部分切除胸腺肿瘤是可能的，患者开始接受放疗和化疗。
