Patient Education

Adrenocortical carcinoma (ACC)

Adrenocortical carcinoma (ACC) is a rare cancer with an estimated 500 new cases annually is US.
The majority of ACC are sporadic (non-hereditary and  there are few recently hereditary predisposition.

Symptoms:

  • Initially the symptoms can be very subtle and adrenal cancer is often identified when tumor is large and causing back pain or abdominal pain by compressing structures in abdominal cavity.
  • Some tumors secrete hormones and cause patients to have associated symptoms:
    • Cortisol : weight gain especially abdomen, round face, increased blood pressure, increased blood glucose, insomnia, easy bruising
    • Aldosterone: increased blood pressure, decreased potassium
    • DHEA-S: increased weight and body hair in women

Diagnosis:

  • Based on symptoms the evaluation includes:
    • Blood test to check the adrenal hormone levels.
    • Imaging to evaluate the tumor size and presence of metastasis.

Treatment:

  • Resection by the expert surgeon remains the first line of treatment, however some tumors are unresectable. 
  • Mitotane, a medicine that destroys the normal adrenal as well as cancer cells, is the main chemotherapeutic agent.
  • When many metastases are present, chemotherapy and occasionally radiation therapy are used.

Pheochromocytoma and paraganglioma

Pheochromocytoma and paraganglioma are adrenal and extra-adrenal tumors, respectively, that secrets high amount of adrenaline.

  • Approximately 35-40% of pheochromocytomas/paragangliomas are hereditary, and about 20-25% are malignant.

Symptoms:

  • Classics symptoms including headaches, sweating and racing heart beats as well as high blood pressure
  • Patients can also have anxiety, high blood sugar, dizziness or even no symptoms at all

Diagnosis:

  • Based on symptoms, the evaluation includes:
    • 24-hr urine collection and blood tests
    • CT scan or MRI and sometimes nuclear medicine imaging

Treatment:

  • Prior to surgical removal of a pheochromocytoma or paraganglioma, medical management with special blood pressure medications for 1-2 weeks is needed
  • Surgical removal of the tumor by an expert surgeon is curative in the majority of patients
  • Metastatic or recurrent pheochromocytomas are managed by a multidisciplinary team of oncologists, endocrinologists, endocrine surgeons and radiation oncologists.

Functional Adrenal Tumors

Symptoms:

  • Usually are associated with the type of hormone that is overproduced:
    • Cortisol (Cushing’s syndrome): weight gain especially in the abdomen, round face, increased blood pressure, increased blood glucose, insomnia, easy bruising and purple stretch marks
    • Aldosterone (Conn’s syndrome): water retention and swelling, increased blood pressure, decreased potassium
    • Testosterone: increased hair growth, voice deepening and male pattern baldness in women; weight gain

Diagnosis:

  • Based on symptoms, the evaluation includes:
    • 24-hr urine collection and blood tests
    • CT scan of the abdomen

Treatment:

  • All patients with functional adrenal tumors should undergo prompt surgical removal of the tumor by an expert surgeon, as their disease associated with hormone overproduction may progress rapidly
  • In the pre-surgical period, patients with functional adrenal tumors are managed by an endocrinologist using medical therapies to suppress the abnormal hormone levels.