Clival Chordoma in Childhood: A Report of Two Cases

Abstract

A Jamjoom, Naim-Ur-Rahman, MA Hafeez, ZA Jamjoom, Clival Chordoma in Childhood: A Report of Two Cases. 1993; 13(3): 280-282 Cranial chordomas are rare neoplasms accounting for less than 0.5% of all intracranial tumors [1]. Although they are presumed to arise from remnants of the primitive notochord, it is rare for these tumors to present in childhood. Up to date, only around 37 cases of cranial chordomas in children 16 years and younger [2] have been reported in the literature, while the tumor was reported to occur in children less than five years of age in 11 of these cases only [3]. Considering the worldwide rarity of this pathology in children, we were surprised to find two cases with clival chordomas among the 62 cases of childhood brain tumors treated in our unit. We are unaware of any reports of clival chordomas in children in Saudi Arabia and therefore feel justified in reporting our two cases to emphasize the rarity of this pathology in the pediatric age group and heighten awareness of clinicians about clival chordomas which may prove to be not such a rare tumor in our region. Case ReportsBetween 1984-1991, two children with clival chordomas were treated at King Khalid University Hospital. The clinical data for the cases are summarized in the Table. The diagnosis was made by CT scan which showed a tumor destroying the clivus and petrous bones and invading the sella, sphenoid sinus, and nasopharynx in Case 1 (Figure 1) and a tumor destroying the clivus and extending posteriorly into the cerebellum and displacing the brainstem to the left in Case 2 (Figure 2). The histological diagnosis was undisputed for both cases. The tumor was composed of loosely attached cells arranged in sheets, cords, or lobules ( Figure 3A) and the characteristic multivacuolated or "physaliphorous" cell with its ample vacuolated cytoplasm was evident ( Figure 3B).