Extraneural recurrence of an intracranial nongerminomatous germ cell tumor to cervical lymph nodes in a pediatric patient: Case report

Abstract

Background Intracranial germ cell tumors (GCTs) comprise 3%–5% of pediatric primary central nervous system (CNS) tumors in Western countries. Though they are related in embryonic origin to gonadal GCTs, which are considered highly treatable with cisplatin‐based chemotherapy regimens, intracranial GCTs vary in malignant potential and sensitivity to radiation and chemotherapy, generally carrying a worse prognosis. Metastases of intracranial GCTs outside of the CNS are rare, indicate a poor prognosis, and their salvage treatment is not well established. Case A 15‐year‐old boy presented with bifocal (suprasellar and pineal) intracranial nongerminomatous germ cell tumors of mixed origin. The tumors were treated to full response with a multimodal approach of neoadjuvant chemotherapy, surgical resection, and adjuvant craniospinal proton radiation. Nine months following treatment completion, the patient presented with an enlarged cervical lymph node determined on excisional biopsy to be a recurrence of pure germinoma from the primary tumors. Salvage treatment involved high‐dose chemotherapy and autologous stem cell transplantation; however, the patient denied further treatment prior to planned focal radiotherapy. Thirty months post‐treatment, the patient is well with no evidence of recurrence. Conclusion This case demonstrated the successful salvage treatment of an extraneural recurrence of an intracranial GCT using surgical resection and a high‐dose chemotherapy and autologous stem‐cell transplantation regimen, highlighting the unique factors which led to the selection of this regimen.

Bifocal germ cell tumor of pineal germinoma and neurohypophyseal embryonal carcinoma: illustrative case

“…In some cases, even when the histology appeared identical, a closer examination revealed that the two tissues were mixed germ cell tumors. 9,14,15 To our knowledge, this is the first report documenting a simultaneous biopsy of a lesion from the pineal and neurohypophysis areas, each exhibiting different histological characteristics, specifically germinoma and embryonal carcinoma. It is not rare for CNS GCTs to have a mixed histology.…”