Suspected metastatic adrenocortical carcinoma revealing as pulmonary Kaposi sarcoma in adrenal Cushing’s syndrome

Abstract

BackgroundKaposi 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 presentationA 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.ConclusionKS may occur in the setting of endogenous CS and may go into remission after cure of hypercortisolism without further specific treatment.

Kaposi sarcoma secondary to endogenous adrenocorticotropic hormone‐dependent Cushing syndrome

“…We conducted a search through the Medline and PubMed databases and found only three cases that had KS associated with ECS; these are summarized in Table . These cases showed either intrathoracic or cutaneous KS lesions, but none of them showed both simultaneously, as in our patient.…”

“…Epidemiological studies reporting the incidence of KS in CS are needed. If more cases were reported, a fifth class of KS, as proposed by Bala et al ., could be established. Cutaneous and intrathoracic KS manifestations may appear in HHV‐8‐positive patients with ECS, probably facilitated by cortisol‐induced immunosuppression; these can improve spontaneously after hypercortisolism control without further specific treatment.…”