Abstract

Background: Kaposi sarcoma (KS) is a malignant disease most commonly diagnosed in the setting of a human immunodeficiency virus (HIV) infection and in patients receiving immunosuppressive treatment. Pulmonary KS has never been reported in association with endogenous Cushing’s syndrome (CS). Case presentation: A 60-year-old woman presented with symptoms and signs of CS. Adrenal CS was confirmed by standard biochemical evaluation. Imaging revealed a right adrenal lesion (diameter 3.5 cm) and multiple pulmonary nodules, suggesting a cortisol-secreting adrenal carcinoma with pulmonary metastases. The patient underwent right adrenalectomy with a pathohistological diagnosis of an adrenal adenoma. Subsequent thoracoscopic wedge resection of one lung lesion revealed pulmonary KS with positive immunostaining for human herpes virus 8 (HHV-8). HIV-serology was negative. Hydrocortisone replacement was initiated for secondary adrenal insufficiency after surgery. Post-operative follow up imaging showed complete remission of all KS-related pulmonary nodules solely after resolution of hypercortisolism. Conclusion: KS may occur in the setting of endogenous CS and may go into remission after cure of hypercortisolism without further specific treatment.

Highlights

  • Kaposi sarcoma (KS) is a malignant disease most commonly diagnosed in the setting of a human immunodeficiency virus (HIV) infection and in patients receiving immunosuppressive treatment

  • In the case of epidemic (HIV-related) or iatrogenic KS, treatment primarily aims at immune reconstitution by means of highly active antiretroviral therapy (HAART) or reduction of immunosuppressive therapy, respectively [11,12]

  • Restaging was performed with chest and abdominal computed tomography (CT) three months (Figure 5) and with fluorodeoxyglucose positron emission tomography (FDG-PET)/CT six months after the adrenalectomy and showed complete disappearance of all KS-induced pulmonary nodules

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Summary

Background

Endogenous Cushing’s syndrome (CS) is a rare disorder with an incidence of ca. 1/100.000 population [1,2]. Case presentation A 60-year-old woman was admitted to our endocrine department with a diagnosis of Cushing’s syndrome (CS) presumably caused by a cortisol-secreting adrenal carcinoma with pulmonary metastases. The patient reported a progressive course of central obesity, moon-shaped face, muscle atrophy and weakness, asthenia and emotional disturbance over the last six years. During this period she had developed arterial hypertension, which was poorly controlled despite five antihypertensive drugs. Five years ago abdominal magnetic resonance (MR) imaging had been performed in the local hospital for further evaluation of a suspected liver hemangioma, and revealed an incidental finding of a 2.5 cm lesion in the right adrenal. Restaging was performed with chest and abdominal CT three months (Figure 5) and with FDG-PET/CT six months after the adrenalectomy and showed complete disappearance of all KS-induced pulmonary nodules

Discussion
Conclusions
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