Functioning Adrenocortical Carcinoma with Superior Vena Cava and Upper Airway Obstructions
Sathapakorn Siriwong MD*, Shanop Shuangshoti MD**, Suthee Saritsiri MD*, Patcharajiree Pak-art MD***, Weerapan Khovidhunkit MD, PhD*, Thiti Snabboon MD*
Affiliation : * Department of Internal Medicine, Faculty of Medicine, Chulalongkorn University ** Department of Pathology, Faculty of Medicine, Chulalongkorn University *** Department of Radiology, Faculty of Medicine, Chulalongkorn University
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.
Materials and 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 (2mg/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.
Conclusion : 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.
Keywords : Adrenocortical carcinoma, Superior vena cava obstruction, Cushing’s syndrome
