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

AbstractBACKGROUND:
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.
AuthorsMargarita Bala, Cristina L Ronchi, Josef Pichl, Vanessa Wild, Stefan Kircher, Bruno Allolio, Stefanie Hahner
JournalBMC endocrine disorders (BMC Endocr Disord) Vol. 14 Pg. 63 (Jul 30 2014) ISSN: 1472-6823 [Print] England
PMID25077599 (Publication Type: Case Reports, Journal Article, Research Support, Non-U.S. Gov't)
Chemical References
  • Hydrocortisone
Topics
  • Adrenal Cortex Neoplasms (complications, pathology, surgery)
  • Adrenalectomy
  • Adrenocortical Carcinoma (complications, pathology, surgery)
  • Cushing Syndrome (complications, pathology, surgery)
  • Diagnosis, Differential
  • Female
  • Humans
  • Hydrocortisone (administration & dosage)
  • Lung Neoplasms (complications, secondary, surgery)
  • Middle Aged
  • Prognosis
  • Sarcoma, Kaposi (etiology, pathology, surgery)

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