title: Functioning adrenocortical carcinoma with superior vena cava and upper airway obstructions.
abstract: BACKGROUND
  Adrenocortical carcinoma (ACC) is one of the most aggressive endocrine malignancies with a dismal prognosis. Typically, the tumor is large and has regional invasion or distant metastasis at initial presentation.
  
  
  OBJECTIVE
  To describe an unusual case of functioning ACC presenting with superior vena cava (SVC) and upper airway obstruction.
  
  
  METHODS
  A 23-year-old man with cushingoid appearance was evaluated for a neck mass and SVC syndrome. Hormonal assessment and neck mass biopsy including immunohistochemistry study were performed
  
  
  RESULTS
  Cushing's syndrome was confirmed by elevated 24-hr urinary free cortisol and no suppressible cortisol level after standard low dose (2 mg/day) of dexamethasone suppression test. Computerized tomography (CT) study revealed a huge left suprarenal mass and multiple mediastinal lymph nodes compressing SVC and trachea. Histopathological findings of the neck mass were compatible with metastatic ACC.
  
  
  CONCLUSIONS
  The present report describes a functioning ACC patient with an unusual metastatic site causing SVC and upper airway obstruction. His hospital course was progressively worsened due to peptic perforation and decompensated respiratory failure, which led him to expire.
authors.affiliation: Department of Internal Medicine, Faculty of Medicine, Chulalongkorn University, Bangkok, Thailand.
authors.forename: Sathapakorn
authors.initials: S
authors.lastname: Siriwong
authors.forename: Shanop
authors.initials: S
authors.lastname: Shuangshoti
authors.forename: Suthee
authors.initials: S
authors.lastname: Saritsiri
authors.forename: Patcharajiree
authors.initials: P
authors.lastname: Pak-art
authors.forename: Weerapan
authors.initials: W
authors.lastname: Khovidhunkit
authors.forename: Thiti
authors.initials: T
authors.lastname: Snabboon
mesh_terms: D000306:Adrenal Cortex Neoplasms / Q000150:complications / Q000000981:diagnostic imaging / Q000473:pathology*; D018268:Adrenocortical Carcinoma / Q000150:complications / Q000000981:diagnostic imaging / Q000473:pathology*; D000328:Adult; D000402:Airway Obstruction / Q000000981:diagnostic imaging / Q000209:etiology / Q000473:pathology*; D003480:Cushing Syndrome / Q000175:diagnosis; D017809:Fatal Outcome; D006801:Humans; D008297:Male; D009361:Neoplasm Invasiveness; D009362:Neoplasm Metastasis; D014057:Tomography, X-Ray Computed; D014683:Vena Cava, Superior / Q000000981:diagnostic imaging / Q000473:pathology*