The differential diagnosis and treatment of Cushing's syndrome

肺肿瘤异位促肾上腺皮质激素分泌引起库欣综合征与高发病率相关。最佳管理仍然是模糊的，因为知识是基于罕见的报告和少数患者。对1982年至2020年的观察性病例系列进行了回顾。参与者接受了肺神经内分泌肿瘤引起库欣综合征的治疗目的手术。68例患者中位年龄41岁（范围17 ~ 80岁），男性占42.6%（68例中29例），白人占81.8%（66例中54例），术后平均随访16个月（范围0.1 ~ 341个月）。肺叶切除术是最常见的手术（68例中48例为70.6%），其次是楔形切除术（68例中16例为23.5%）和节段切除术（68例中3例为4.4%）。68例患者中有19例（27.9%）接受了电视胸腔镜手术。手术发病率为19.1%（68例中13例），围手术期死亡率为1.5%（68例中1例）。淋巴结阳性率为37%（22 / 59）。持续/复发的总发生率为16.2%（68例中的11例），中位复发时间为55个月（范围18-152个月）。中位无病生存期为12.7年（0.1-334个月）。基于肿瘤大小、分期（第8版TNM）、是否进行全系统淋巴结切除术、淋巴结状态或手术方式，无病生存率无统计学差异。在本病例系列研究中，与静止的支气管肺类癌肿瘤相比，与库欣综合征相关的神经内分泌肺肿瘤淋巴结转移增加，复发率高，DFS较低，但许多患者预后良好。tnm分期分类与预后的不一致强调了这一观察结果。观察到手术技术没有差异，这可能意味着肺保留入路就足够了。这些结果可能反映了激素生理学的内在重要性，而不是类癌生物学因素。

Ectopic adrenocorticotropic hormone secretion from lung tumors causing Cushing syndrome is associated with high rates of morbidity. Optimal management remains obscure because knowledge is based on rare reports with few patients. An observational case series review from 1982 to 2020 was conducted. Participants underwent curative-intent surgery for a lung neuroendocrine tumor causing Cushing syndrome. Of the 68 patients, the median age was 41 years (range, 17-80 years), 42.6% (29 of 68) were male, 81.8% (54 of 66) were White, with a mean follow-up after surgery was 16 months (range, 0.1-341 months). Lobectomy was the most common procedure (48 of 68 70.6%), followed by wedge resection (16 of 68 23.5%) and segmentectomy (3 of 68 4.4%). Video-assisted thoracoscopic surgery was performed in 19 of 68 (27.9%) of patients. Surgical morbidity was 19.1% (13 of 68), and perioperative mortality was 1.5% (1 of 68). Lymph node positivity was 37% (22 of 59) when evaluable. The overall incidence of persistence/recurrence was 16.2% (11 of 68) with a median time to recurrence of 55 months (range, 18-152 months). The median disease-free survival was reached in 12.7 years (0.1-334 months). There were no statistical differences in disease-free survival based on tumor size, stage (8th edition TNM), whether full systematic lymphadenectomy was performed or not, nodal status, or surgical approach. In this case series study, neuroendocrine pulmonary tumors associated with Cushing syndrome had increased nodal metastasis, higher recurrence, and lower DFS than quiescent bronchopulmonary carcinoid tumors, but many patients experienced favorable outcomes. This observation is underscored by the discordance of TNM-stage classifications vs prognosis. Observing no difference in surgical techniques, the implication may be that a lung-sparing approach could suffice. These results may reflect the intrinsic importance of the hormone physiology instead of the carcinoid biologic factors. Confirming a diagnosis of Cushing disease (CD) remains challenging, yet is critically important before recommending transsphenoidal surgery for adenoma resection. We retrospectively reviewed the preoperative biochemical and imaging data in 105 patients with presumed CD to evaluate whether they could predict post-operative remission. Patients were classified into 3 groups: group A (n = 84) pathology-proven ACTH adenoma; group B (n = 6) pathology-unproven but with postoperative hypocortisolemia consistent with CD; and group C (n = 15) pathology-unproven, without postoperative hypocortisolemia. Group A + B were combined as confirmed CD and group C as unconfirmed CD. All groups had a similar clinical phenotype. Compared with group C, group A + B had higher mean UFC (P < 0.001), LNSC (P = 0.003), DST (P = 0.06), and ACTH (P = 0.03) and larger MRI-defined lesions (P < 0.001). Early (3-month) biochemical remission was achieved in 76/105 (72%) patients: 76/90(84%) and 0/15(0%) of group A + B vs group C, respectively, P < 0.0001. In group A + B, nonremission was strongly associated with adenoma cavernous sinus invasion. Use of strict biochemical thresholds may help avoid offering transsphenoidal surgery to presumed CD patients with equivocal data and improve surgical remission rates. Patients with Cushingoid phenotype but equivocal biochemical data warrant additional rigorous testing.