Virilizing Adrenocortical Tumors Flashcards
What is the most common presenting symptom seen with adrenocortical tumors in children?
Virilization
What laboratory tests are elevated with adrenocortical tumors?
Urinary 17-ketosteroids, serum DHEA, serum DHEAS, serum androstenedione, and testosterone.
In which age range are adrenocortical tumors most common in children?
They are rare, but most commonly found in children <10 years of age.
What are signs of virilization in girls?
Masculinization, clitoral enlargement, increased growth, acne, deep voice, premature pubic hair, and premature axillary hair.
What are signs of virilization in boys?
Accelerated growth and muscle development, acne, increased penis size, and early development of pubic and axillary hair without testicular enlargement.
Which other endocrine disorder commonly occurs with adrenocortical tumors?
Cushing syndrome occurs in 20-40% of children with adrenocortical tumors.
Which syndrome is associated with an increased risk for developing adrenocortical tumors?
Beckwith-Wiedemann syndrome
Which imaging modalities can be used to identify the presence of an adrenocortical tumor?
MRI and CT can both detect adrenal tumors
What is the treatment for adrenocortical tumors?
Surgical resection. Radiation is not helpful and chemotherapy has not been shown to improve survival.
When an adrenocortical tumor is identified, what is the likelihood that it will be carcinoma (malignant) vs adenoma (benign)?
Carcinoma is 3x more likely than adenoma, and metastases are common.
What is a common complication of surgical resection of an adrenocortical tumor? How should it be managed?
Adrenal insufficiency is a common complication and should be managed with administration of hydrocortisone and careful postoperative management of water and sodium balance.
