Roque Catarina, Fonseca Ricardo, Bello Carlos Tavares, Vasconcelos Carlos, Galzerano António, Ramos Sância
Endocrinology Diabetes and Metabolism Clinic.
Anatomopathology Department, Hospital de Egas Moniz C.H.L.O.-E.P.E, LisbonPortugal.
Endocrinol Diabetes Metab Case Rep. 2017 May 16;2017. doi: 10.1530/EDM-17-0002. eCollection 2017.
Primary adrenal lymphoma is a rare malignancy. It frequently presents bilaterally and with symptoms of adrenal insufficiency. Amiodarone may induce secondary organ dysfunction, and thyrotoxicosis develops in 15% of cases. The symptomatology of both conditions is nonspecific, especially in the elderly, and a high suspicion index is necessary for appropriate diagnosis. A 78-year-old female presented to the emergency department with confusion, nausea and vomiting. She had recently been to the emergency department with urinary tract infection, vomiting and acute hypochloremic hyponatremia. Upon re-evaluation, the leukocyturia persisted and because of TSH 0.01 µU/mL and free-T4 68 (10-18) pmol/L, she was admitted to the Endocrinology ward. Further evaluation supported amiodarone-induced thyroiditis type 2. Sepsis ensued, in the setting of nosocomial pneumonia. Hemodynamic instability, hyponatremia, hypoglycemia and vomiting raised the suspicion of adrenocortical insufficiency. Fluid resuscitation and hydrocortisone led to clinical improvement, and adrenal insufficiency was admitted. The thoracoabdominal tomography suggested an endobronchic primary lesion with hepatic and adrenal secondary deposits (6.6 and 7 cm), but this was confirmed neither on pleural effusion nor on bronchofibroscopic fluid analyses. The adrenals were not accessible for biopsy. Despite high-dose hydrocortisone maintenance, the patient died before definite diagnosis. The autopsy confirmed primary non-Hodgkin lymphoma.
Primary adrenal lymphoma is a rare cause of adrenal insufficiency, but progression can be fast and fatal.Hyperpigmentation is frequently absent.The presenting symptoms are nonspecific and might mimic infection. Disproportion of the general state with signs of specific organ symptomatology is a diagnostic clue.Infection may precipitate adrenal crisis and worsen thyroid function with further adrenal insufficiency exacerbation.In the context of thyrotoxicosis, there may be little clinical response to a therapeutic trial with standard dose glucocorticoids.High-dose glucocorticoid substitution may be required to achieve clinical stability in thyrotoxic patients.
原发性肾上腺淋巴瘤是一种罕见的恶性肿瘤。它常双侧发病，并伴有肾上腺功能不全的症状。胺碘酮可能导致继发性器官功能障碍，15%的病例会发生甲状腺毒症。这两种疾病的症状都不具有特异性，尤其是在老年人中，因此需要高度怀疑指数才能做出准确诊断。一名78岁女性因意识模糊、恶心和呕吐被送往急诊科。她最近因尿路感染、呕吐和急性低氯性低钠血症去过急诊科。再次评估时，白细胞尿持续存在，由于促甲状腺激素（TSH）为0.01 μU/mL，游离甲状腺素（free-T4）为68（10 - 18）pmol/L，她被收入内分泌科病房。进一步评估支持2型胺碘酮诱发的甲状腺炎。随后发生脓毒症，伴有医院获得性肺炎。血流动力学不稳定、低钠血症、低血糖和呕吐引发了肾上腺皮质功能不全的怀疑。液体复苏和氢化可的松治疗使临床症状得到改善，确诊为肾上腺功能不全。胸腹部断层扫描显示支气管内原发性病变伴有肝脏和肾上腺继发性病灶（6.6厘米和7厘米），但胸腔积液和支气管纤维镜液体分析均未证实这一点。肾上腺无法进行活检。尽管维持大剂量氢化可的松治疗，患者在明确诊断前死亡。尸检证实为原发性非霍奇金淋巴瘤。
原发性肾上腺淋巴瘤是肾上腺功能不全的罕见病因，但病情进展可能迅速且致命。常无色素沉着。呈现的症状不具特异性，可能类似感染。全身状况与特定器官症状体征不相称是诊断线索。感染可能引发肾上腺危象并使甲状腺功能恶化，进而加重肾上腺功能不全。在甲状腺毒症的情况下，标准剂量糖皮质激素治疗试验可能临床反应甚微。甲状腺毒症患者可能需要大剂量糖皮质激素替代治疗以实现临床稳定。
