胸腺类癌表现为库欣综合征

A. Deodhar, D. Kulkarni, Kishor D. Kharche, T. George
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摘要

原发性胸腺神经内分泌肿瘤(NETTs)是一种罕见的肿瘤,具有生物侵袭性。这种情况在30岁到50岁之间更为常见。内分泌疾病,如库欣综合征、肢端肥大症或多发性内分泌肿瘤-1在50%的病例中相关。这些肿瘤通常表现为侵犯周围纵隔结构。由于复发或转移风险高,NETT的长期预后较差。预后取决于分期、侵袭、切除和可能与内分泌疾病的关联。我们报告一例17岁男性临床诊断为库欣综合征伴甲状腺功能减退,无肾上腺肿块,但有前纵隔肿块,组织学结果为胸腺类癌。虽然胸腺类癌已知会出现库欣综合征,但我们还没有发现它与甲状腺功能减退症的关联。对该病例进行了全胸腺切除术。患者在过去3年定期随访,完全无症状,未服用任何药物。
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Thymic carcinoid presenting as cushing's syndrome
Primary neuroendocrine tumors of the thymus (NETTs) are rare neoplasms and are biologically aggressive. These are more frequently seen in the third to fifth decade of life. Endocrinopathies such as Cushing's syndrome, acromegaly, or multiple endocrine neoplasia-1 are associated in 50% of cases. These tumors usually present with the invasion of surrounding mediastinal structures. The long-term outcome of NETT is poor due to high risk of recurrence or metastasis. Prognosis depends on the stage, invasion, resection, and possible association of endocrinopathies. We present this case of a 17-year-old male clinically diagnosed as Cushing's syndrome with hypothyroidism, without adrenal mass but with anterior mediastinal mass, and it histologically turned out to be carcinoid of the thymus. Although thymic carcinoids are known to present with Cushing's syndrome, we have not come across with its association of hypothyroidism. Total thymectomy was performed for the case. The patient is on regular follow-up for the past 3 years, he is totally asymptomatic and not on any medication.
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