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.
- 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
- Aldosterone: increased blood pressure, decreased potassium
- DHEA-S: increased weight and body hair in women
- Based on symptoms the evaluation includes:
- Blood test to check the adrenal hormone levels.
- Imaging to evaluate the tumor size and presence of metastasis.
- 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.
- 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
- Based on symptoms, the evaluation includes:
- 24-hr urine collection and blood tests
- CT scan or MRI and sometimes nuclear medicine imaging
- 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
- 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
- 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