Endocrine Abstracts

Introduction: Adrenocortical carcinoma (ACC) is a rare tumor with an aggressive prognosis of 1–2 people per million per year. They are functional tumors which mostly occur with virilization and/or Cushing syndrome. Metastatic ACC has a dismal prognosis and the most common sites of metastases are the lung, liver, lymph nodes and bones. Herein, we present metastatic ACC patient without Cushing clinic at the time of diagnosis.

Case presentation: A 48-year-old male patient was admitted to the outpatient clinic due to abdominal swelling, right side pain, and a growing mass on the left side of the neck. The patient denied relevant pathological medical history including smoking, alcohol use or drug consumption and had no previous hospital admissions and was on no regular medication. On examination, patient had abdominal fullness in the left upper quadrant with a palpable mass. There were no cushingoid appearance and hiperandrojenism. CT and MRI scan of abdomen showed malignant mass with approximately 19×10×19 cm, invading the renal cortex in the right adrenal gland region. There were also pulmonary and multiple retrocrural lymph node metastasis and significant compression findings to the inferior vena cava and posterior right lobe of the liver. The excisional biopsy of cervical D5 lymph node was consistent with ACC metastasis. Laboratory studies showed that serum ACTH <5 pg/ml (N:0–46), serum cortisol 20.1 ug/dL (N:4.6–22.8), 1-mg dexamethasone-suppressed morning serum cortisol 17.8 μg/dl (N: <1.8), 2-mg dexamethasone-suppressed morning serum cortisol 20.73 ug/dl (N: <1.8), aldosterone 363 pg/ml, renin 0.3 ng/ml/h. 24-hr-urinary cortisol, vanilmandelic acid, metanephrine and normetanephrin measurements were respectively 1742 nmol/24hr (N:38–208), 8.7 mg/24hr (N:1.6–7.3), 29 μg/24hr (N: 276–341) and 110g/24hr (N:88–444). The patient was considered as inoperable and chemotherapy was started.

Discussion: ACC are aggressive tumors with a very poor prognosis and 70% of patients have stage III or IV disease at the time of diagnosis. Early diagnosis and treatment determine the life expectancy of ACC. Our patient presented with multiple organ metastasis as an ACC diagnosed with biopsy from the cervical lymph node. Although laboratory findings were compatible with Cushing’s syndrome, it was not reflected in clinical findings.

Keywords: Adrenal cortical carcinoma, metastasis