RESUMO
OBJECTIVE: We aim to report a case of Kaposi sarcoma (KS) with Cushing's syndrome caused by endogenic glucocorticoid-induced immunosuppression. CLINICAL PRESENTATION: A 43-year-old woman presented with delirium, hirsutism, fatigue, and hypertension. At the time of presentation, physical findings showed a Cushingoid appearance, with moon-like facies, hirsutism, and hyperpigmentation. Laboratory findings showed the following: adrenocorticotropic hormone, 86.7 pg/mL (normal range, 0-46 pg/mL); baseline cortisol level, 50 microg/dL (normal range, 6.2-19 microg/dL); potassium, 2.2 mEq/L (normal range, 3.5-5 mEq/L); and midnight cortisol level, 33 microg/dL. Serum cortisol levels failed to suppress after low and high doses of dexamethasone; these findings confirmed the diagnosis of ectopic adrenocorticotropic hormone production. Magnetic resonance imaging revealed a 12 x 15-mm, round, hypothalamic mass lesion in the center of the median eminence. INTERVENTION: Endoscopic biopsy from the floor of the third ventricle was performed, and pathological examination of the lesion showed a diffuse adrenocorticotropic hormone-secreting adenoma. The patient developed diffuse skin lesions that were proven to be a KS by skin biopsy while she was prepared for transcranial surgery. After surgical removal of the adenoma, she became hypocortisolemic and required cortisol replacement. Within 1 month after surgery, all KS lesions disappeared spontaneously. CONCLUSION: Excessive cortisol may induce immunosuppression. KS is one of the most common malignant tumors of patients with immunosuppression. To the best of our knowledge, this is the first case of Cushing's syndrome with KS caused by endogenous glucocorticoid-induced immunosuppression.
Assuntos
Síndrome de ACTH Ectópico/imunologia , Adenoma/metabolismo , Hormônio Adrenocorticotrópico/metabolismo , Neoplasias Hipotalâmicas/metabolismo , Neoplasias Hipofisárias/metabolismo , Sarcoma de Kaposi/imunologia , Síndrome de ACTH Ectópico/patologia , Síndrome de ACTH Ectópico/fisiopatologia , Adenoma/patologia , Adenoma/cirurgia , Hormônio Adrenocorticotrópico/sangue , Adulto , Coristoma/patologia , Coristoma/fisiopatologia , Coristoma/cirurgia , Síndrome de Cushing/complicações , Síndrome de Cushing/imunologia , Síndrome de Cushing/fisiopatologia , Feminino , Humanos , Hidrocortisona/sangue , Neoplasias Hipotalâmicas/patologia , Neoplasias Hipotalâmicas/cirurgia , Hipotálamo/metabolismo , Hipotálamo/patologia , Hipotálamo/cirurgia , Tolerância Imunológica/imunologia , Hospedeiro Imunocomprometido/imunologia , Imageamento por Ressonância Magnética , Eminência Mediana/metabolismo , Eminência Mediana/patologia , Eminência Mediana/cirurgia , Procedimentos Neurocirúrgicos , Neoplasias Hipofisárias/patologia , Neoplasias Hipofisárias/cirurgia , Sarcoma de Kaposi/patologia , Sarcoma de Kaposi/fisiopatologia , Resultado do TratamentoRESUMO
Hormones of the hypothalamic-pituitary-adrenal (HPA) axis are connected closely with immune measures. To investigate whether Cushing's syndrome (CS) is associated with immune dysregulation, we compared the percentage of specific lymphocyte subsets as well as natural cell activity (NKCA) in 48 patients with Cushing's syndrome and 48 age- and sex-matched normal controls. Lymphocyte subset analysis included the percentage of lymphocytes expressing CD3 (total T), CD4 (T helper/inducer), CD8 (T suppressor/cytotoxic) and CD56 (NK cell) antigens. Baseline plasma concentrations of cortisol, ACTH and beta-endorphin as well as 24 h urinary-free cortisol (UFC) levels also were determined. Results indicated a decrease in the percentage of CD4+ cells (p < 0.05), an increase in percentage of CD8+ cells (p < 0.05), a decrease in CD4/CD8 ratios (p < 0.01), and a reduction in NKCA (p < 0.05) in patients with CS compared to matched controls. We also found significant negative correlations between NKCA on the one hand and 24 h UFC (p < 0.05) and plasma beta-endorphin (p < 0.05) on the other. These results indicate there is immune dysregulation in CS patients which can be explained in part by an increase in HPA-axis hormones.