Clival Chordoma and Sellar-Suprasellar Germinoma in a Pediatric Patient: A Review and 1 Case Report
Introduction: Associated multiple intracranial neoplasms have always been of special interest. Unlike well known genetic syndromes, like Neurofibromatosis, an association between 2 congenital or vestigial tumors is an extremely rare occurrence.
Case Report: A 10 year old female, was diagnosed with central diabetes insipidus, after presenting with polyuria, polydipsia and hypernatremia. On work up, she was also found to have panhypopituitarism. No visual field defects or optic disc edema/pallor. Her MRI showed the presence of a large sellar and suprasellar mass as well as a non enhancing component in the dorsal clivus. She underwent an endoscopic transsphenoidal resection and was confirmed to have clival chordoma and sellar germinoma.
Discussion: Chordomas are thought to originate from the Notochord. Less than 5% of them occur in the pediatric population. The reported incidence of chordomas in kids is estimated at 1 per million per year.
Germinomas are thought to originate from undifferentiated embryonal cells (meso-endo-ectoderm). They have been described intracranially in pre pubescent children with an incidence of 0.7 per million, and represent only 0.4–3.5% of all primary intracranial tumors.
Both Germinomas and Chordomas are regarded as congenital or vestigial tumors. They both rarely occur in the pediatric population. No prior connection was made in literature. To the best of our knowledge, this is the first reported case of association between these two tumors. We suspect that because of the rarity of the two tumors, it would be unlikely that this is a pure coincidence. We believe that reporting these cases is important in our continuous effort to understand these lesions. Further cytoanalysis is certainly needed to clarify the link between these 2 tumors.