Nga Vu Bich, Thom Mac-Thi, Hai Tran-Thi, Huong Nguyen-Thi Thanh, Thang Nguyen Duy, Minh Tran Ngoc, Duc Nguyen Minh
Department Endocrinology & Respirology, Hanoi Medical University Hospital, Hanoi, Vietnam.
Department of Cardiovascular and Thoracic Surgery, Hanoi Medical University Hospital, Hanoi, Vietnam.
Radiol Case Rep. 2023 Jan 5;18(3):963-967. doi: 10.1016/j.radcr.2022.12.002. eCollection 2023 Mar.
Ectopic Cushing's syndrome, caused by a neuroendocrine tumor (NET), is a rare finding. NETs of the mediastinum are extremely rare. NETs arising from the anterior mediastinum are generally aggressive. They are widely characterized at clinical presentations, and may be asymptomatic or present with atypical symptoms. Prognosis is often poor due to their local recurrence and distant metastasis despite a multimodal approach. A 33-year-old male patient was admitted to our department with a femoral soft tissue abscess, diabetes, and hypokalemia. He had no typical features of Cushing's syndrome. However, with a few simple tests, that is, a basal hormone profile, and low-dose and high-dose dexamethasone suppression tests, we diagnosed this complicated condition of ectopic adrenocorticotrophic hormone (ACTH) secretion. Thoracic computed tomography revealed an anterior mediastinal mass of 35 × 22 mm. A surgical excision of the tumor was proposed, and intra-operative pathology consultation returned positive for the suspected NET. Immunohistochemically, the tumor cells were positive for CK, CD56, Chromogranin, Synaptophysin, S100, and CD117. No thymic tissue was found. The Ki-67 was 4%. A diagnosis of primary NETs of the mediastinum, intermediate grade (G2), of atypical carcinoids according to WHO 2015 was established. This patient survived with no sequelae, no distant metastasis, no recurrence, and without adjuvant radiotherapy or chemotherapy 2 years after surgery thanks to earlier diagnosis and prompt surgical intervention. Mediastinum ectopic ACTH-secreting tumors are a rare type of cancer. According to recent research, these tumors frequently display more aggressive behavior and are linked to endocrinopathies. It is noted that patient might have a better outcome and a longer survival time due to earlier detection and complete resection of malignancies.
由神经内分泌肿瘤（NET）引起的异位库欣综合征是一种罕见病症。纵隔NET极为罕见。起源于前纵隔的NET通常具有侵袭性。它们在临床表现上具有广泛特征，可能无症状或表现为非典型症状。尽管采用了多模式治疗方法，但由于其局部复发和远处转移，预后往往较差。一名33岁男性患者因股部软组织脓肿、糖尿病和低钾血症入院。他没有库欣综合征的典型特征。然而，通过一些简单检查，即基础激素谱以及低剂量和高剂量地塞米松抑制试验，我们诊断出了这种复杂的异位促肾上腺皮质激素（ACTH）分泌病症。胸部计算机断层扫描显示前纵隔有一个35×22毫米的肿块。建议对肿瘤进行手术切除，术中病理会诊显示疑似NET呈阳性。免疫组化结果显示，肿瘤细胞对细胞角蛋白（CK）、CD56、嗜铬粒蛋白、突触素、S100和CD117呈阳性。未发现胸腺组织。Ki-67为4%。根据世界卫生组织2015年的标准，确诊为纵隔原发性NET，中级（G2），非典型类癌。由于早期诊断和及时的手术干预，该患者术后2年存活，无后遗症，无远处转移，无复发，也未接受辅助放疗或化疗。纵隔异位分泌ACTH的肿瘤是一种罕见的癌症类型。根据最近的研究，这些肿瘤通常表现出更具侵袭性的行为，并与内分泌病变有关。值得注意的是，由于早期发现和恶性肿瘤的完全切除，患者可能会有更好的预后和更长的生存时间。
