Rare Tumors Patient & Family Handbook

diagnosis of xeroderma pigmentosum Werner’s syndrome family history of genetic retinoblastoma post-bone marrow or kidney transplant neurocutaneous melanosis.

WHAT IS THE COMMON TREATMENT AND OUTCOME FOR MALIGNANT MELANOMA? The treatment of children with melanoma depends on its location and stage. Children are often treated with similar therapies as adults. The stage of melanoma depends on the depth of the tumor. Surgery is the first line of treatment, and a wide surgical excision is necessary. If lymph nodes are involved, chemotherapy will be used as well. Patients will require close follow-up with skin examinations every 3–6 months.

ADRENOCORTICAL CARCINOMA

WHAT IS ADRENOCORTICAL CARCINOMA? Adrenocortical carcinoma is a rare cancer of the adrenal glands. The adrenal glands are located on top of the kidney and are important to the body’s endocrine (hormonal) system. The cortex—the outer part of the kidney—makes several hormones, including cortisol, aldosterone, estrogen, and testosterone. These hormones regulate metabolism and body characteristics such as body shape and hair growth. Adrenocortical carcinoma begins in the cortex and can be a functioning or nonfunctioning tumor. A functioning tumor produces more of one hormone.

WHAT ARE SOME OF THE SIGNS AND SYMPTOMS OF ADRENOCORTICAL CARCINOMA?

Adrenocortical carcinoma can cause the following symptoms: flushed face with pudgy cheeks (moon face) obesity stunted growth excessive hair growth unusual acne high blood pressure low potassium, thirst, muscle cramps, and weakness.

WHAT CAUSES ADRENOCORTICAL CARCINOMA? The cause of adrenocortical carcinoma is unknown. It is most common in children younger than 5 years. In children, this cancer is most often seen in southern Brazil. Certain hereditary conditions carry a higher risk for this cancer, including Li- Fraumeni syndrome and Carney complex.

WHAT IS THE COMMON TREATMENT AND OUTCOME FOR ADRENOCORTICAL CARCINOMA?

The treatment of children with adrenocortical carcinoma depends on the stage and size of the tumor. Surgical removal of

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Association of Pediatric Hematology/Oncology Nurses (APHON)

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