Intracranial Teratomas

Head and Neck Teratomas

Treatment

Diagnosis

Mediastinal Teratomas

Retroperitoneal Teratomas

Retroperitoneal teratomas occur with equal frequency between the genders. Children usually present with gastrointestinal symptoms or abdominal mass. In addition to chest x-ray, abdominal plain films, and ultrasound, a CT scan may be useful to determine the relationship of the tumor to other retroperitoneal structures and distinguish it from a primary renal or adrenal tumor.

The differential diagnosis includes those listed for ovarian teratomas as well as Wilms’ tumor, neuroblastoma, and sarcoma.

Retroperitoneal teratomas should be removed. The vast majorities are benign and require no further treatment. Patients with malignant lesions and those with high-grade immature elements should be treated with cisplatin-based chemotherapy.

Mediastinal teratomas may arise in the mediastinum, the pericardium, or the heart. The latter two are mentioned only for completeness. Mediastinal teratomas occur with equal frequency between the genders. They usually present with respiratory symptoms or chest pain. A small portion of boys with mediastinal teratomas may present with precocious puberty as a result of a beta-hCG secreting tumor.

The differential diagnosis includes thymoma, parathyroid adenoma, bronchogenic cysts, cystic hygroma, duplications, aneurysms, lymphoma, lipoma, myxoma, and thyroid goiter.

Chest radiograph and CT scan are necessary to confirm the presence of a mass and define its relationship with other intrathoracic structures. AFT and beta-hCG levels should be drawn preoperatively and followed in the postoperative period. Boys with beta-hCG secreting mediastinal teratomas should have chromosomal analysis as there is an association between these lesions and Klinefelter’s syndrome.

The treatment is surgical resection. Children with malignant lesions and those with tumors containing high-grade immature elements also receive cisplatin-based chemotherapy.

Intracranial teratomas have a bimodal distribution occurring in infants < 2 months and children 12-16 years. They usually originate from the pineal gland and increase the intracranial pressure. In the newborn this is manifest as obstructive hydrocephalus and in the child as headaches, visual changes, seizures, and vomiting. On CT or MRI, teratomas are typically calcified, supratentorial, midline lesions. In newborns, most of these lesions are benign. In older children, the majority are malignant.

Complete resection, although rarely possible, is the only chance for long-term survival. Partial resection will provide palliation of symptoms in some cases.