Alternate Names: Sipple's syndrome

Causes and Risks: The cause of multiple endocrine neoplasia II (MEN II) is genetic, but the mechanism is unknown. Tumors of various glands appear in the same person, but not necessarily at the same time. The disorder may occur at any age, and affects men and women equally. The adrenal tumor is a pheochromocytoma and the thyroid tumor is a medullary carcinoma of the thyroid. Risk factors are a family history of MEN II. The incidence is 3 out of 100,000 people.

Prevention: Screening of close relatives of a person with MEN II may lead to early detection.

Symptoms:

• severe headache
• palpitations
• rapid heart rate
• sweating
• chest pain
• abdominal pain
• nervousness
• irritability
• increased appetite
• loss of weight
• diarrhea
• cough
• cough with blood
• fatigue
• back pain
• joint pain
• fractures of long bones
• decreased height
• increased urine output
• increased thirst
• loss of appetite
• nausea
• muscular weakness
• depression
• personality changes
• stupor and possibly coma
• itching of the skin
• cataract

Signs and Tests: Diagnostic tests are used to evaluate the function of each endocrine gland.

A physical examination reveals enlarged cervical lymph nodes. An examination of the thyroid reveals a single or multiple thyroid nodules. Vital signs (temperature, pulse, rate of breathing, blood pressure) reveal high blood pressure, which may be sustained or episodic, rapid heart rate and elevated temperature.

These tests help confirm the diagnosis:

• adrenal biopsy showing pheochromocytoma
• MIBG scintiscan showing tumor
• MRI of abdomen showing adrenal mass
• Abdominal CT scan showing mass
• elevated urine metanephrine
• elevated urine catecholamines
• thyroid biopsy showing medullary carcinoma cells
• ultrasound of the thyroid revealing nodule
• thyroid scan showing cold nodule
• elevated calcitonin
• parathyroid biopsy showing tumor or hyperplasia
• radioimmune assay of parathyroid hormone showing increased level
• increased serum calcium
• possibly increased serum chloride
• decreased serum phosphorus
• possibly increased serum alkaline phosphatase
• bone X-ray showing bone reabsorption or fractures
• imaging of the kidneys or ureters showing calcification or obstruction
• ECG possibly showing abnormalities

Treatment: Surgical removal is indicated for both medullary carcinoma of the thyroid and for pheochromocytoma. Hormone replacement therapy is given after surgery.

Prognosis: Pheochromocytoma is usually benign. People with metastatic medullary carcinoma of the thyroid have had prolonged survival.

Complications: A complication is the metastasis of cancerous cells.

Call your health care provide if you notice symptoms of MEN II.