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 the tumor is usually the main treatment. Chemotherapy or radiation therapy may be used if the cancer has spread to other areas. Hormone regulation and replacement therapy will be an important part of treatment, along with follow-up care with an endocrinologist.
n PLEUROPULMONARY BLASTOMA
What Is Pleuropulmonary Blastoma? Pleuropulmonary blastoma (PPB) is a rare type of childhood lung cancer. PPB begins in the tissues of the pleura (a thin layer of tissue that covers the lungs and lines the interior wall of the chest cavity) or in the lung tissue itself (pulmonary). There are four main types of PPB: • Type I is made up of cysts (air pockets) with evidence that the cysts are cancerous. This usually occurs in very young children, usually around 10 months old. • Type Ir (the “r” stands for regressing) is similar to type I but does not have cancerous cells. • Types II and III generally occur in children ages 3–4 years. They are cancerous and require intensive chemotherapy.
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