Clinical behavior and a contemporary management algorithm for prepubertal testis tumors: a summary of the Prepubertal Testis Tumor Registry.
OBJECTIVE
The Prepubertal Testis Tumor Registry was established by the Urologic Section of the American Academy of Pediatrics in 1980 to record data on a large number of prepubertal testis tumors regarding presentation, treatment and outcome to define appropriate management better. We reviewed the registry data in the context of other modern studies to elucidate the appropriate management of these rare tumors.


METHODS
Relevant data in the prepubertal testis tumor registry were tabulated and analyzed.


RESULTS
There were 395 prepubertal patients who had a primary testis tumor. Generally benign tumors accounted for 38% of cases. A significant proportion of tumors were benign regardless of patient age. alpha-Fetoprotein levels for patients with benign and malignant tumors overlapped in children younger than 6 months. Of the patients with yolk sac tumor 80% presented with stage 1 disease and overall survival was excellent. There were no metastases or deaths among the patients with teratoma. Of all patients with stromal tumors metastases developed in only 1.


CONCLUSIONS
We recommend initial excisional biopsy for all amenable prepubertal testis tumors, except those with an alpha-fetoprotein level that is clearly increased for patient age. Patients with benign tumors may be released from oncological followup. Patients with stage I yolk sac tumor should be monitored closely, and those with recurrent or metastatic yolk sac tumor should be treated with chemotherapy. Retroperitoneal lymph node dissection is reserved for patients with recurrent retroperitoneal masses following chemotherapy. Aggressive treatment of metastatic Sertoli cell or undifferentiated stromal tumors is warranted.
D000465:Algorithms; D002648:Child; D002675:Child, Preschool; D003131:Combined Modality Therapy; D018240:Endodermal Sinus Tumor / Q000151:congenital / Q000401:mortality / Q000473:pathology / Q000628:therapy; D006801:Humans; D007223:Infant; D008297:Male; D009367:Neoplasm Staging; D011379:Prognosis; D012042:Registries / Q000706:statistics & numerical data*; D012707:Sertoli Cell Tumor / Q000151:congenital / Q000401:mortality / Q000473:pathology / Q000628:therapy; D018312:Sex Cord-Gonadal Stromal Tumors / Q000151:congenital / Q000401:mortality / Q000473:pathology / Q000628:therapy; D015996:Survival Rate; D013736:Testicular Neoplasms / Q000151:congenital* / Q000401:mortality / Q000473:pathology / Q000628:therapy; D014481:United States; D000509:alpha-Fetoproteins / Q000378:metabolism
D000509:alpha-Fetoproteins