Extragonadal endodermal sinus tumors in children: a review of 24 cases

Endodermal sinus tumors (EST) are highly malignant lesions with a tendency to recur locally and with a high incidence of metastatic disease at the time of diagnosis. The clinical and radiographic spectra of 24 patients with extragonadal EST are reported. There were 17 females and seven males and the mean age of presentation was 21 months. The primary lesion occurred in the sacrococcygeal region in 16 patients; in the vagina in two patients; in the anterior mediastinum in two patients; and in the bladder, liver, nasopharynx and posterior cranial fossa in one patient each. Computed tomography (CT) is the most accurate study in delineating the extent of primary lesions particularly in the pelvis and head and neck and also for demonstrating metastatic disease in the lungs. Significant improvement in survival rates is obtained with a combination of surgery, chemotherapy and radiotherapy.