Chondroid Chordoma of the Lateral Skull Base

Abstract

Clinical, radiological, and pathologic features of an intracranial chondroid chordoma in a 9-year-old boy are described. This is the first reported case of a chordoma, the center of which was laterally situated in the cranial base, lying in or near jugular foramen and carotid canal, but without midline involvement. Although cranial chordomas in childhood are extremely rare, and all previously reported cases appeared to have arisen in the clivus, this location should not be considered ectopic. Forking at the rostral end of the notochord has been demonstrated in embryos, and would be the presumed embryological source for this tumor. This is also the first reported case of a chondroid chordoma in a child with immunohistochemical documentation distinguishing it from a chondrosarcoma. This chondroid chordoma contained two populations of cells: neoplastic cartilage and chordoid tissue in a myxoid stroma. The distinction between chordoma and chondrosarcoma and the implications on treatment will be discussed.

Cordoma en la articulación facetaria de C4: reporte de caso

“…Se ha observado que nidos de células notocordales corresponden topográficamente con los lugares de ocurrencia del cordoma, lo que explica su localización típica en la línea media [2,7,8,9,10,11]. Sin embargo, el reporte de casos de cordomas extraxiales [12] y como masas paravertebrales sin afectación ósea [8,9] permiten pensar en la existencia de restos ectópicos de la notocorda que expliquen su aparición.…”

“…Adicionalmente, la aparición de cordomas en la parte lateral de la base de cráneo se ha explicado por la división en la terminación rostral de la notocorda embrionaria [10]. La revisión de las bases de datos no permitió encontrar ningún artículo donde se presente afectación ósea exclusivamente de la articulación facetaria, cuyo origen sea un cordoma.…”

Chordomas; Presentation of Three Cases of Para Sellar, Clival, and Vertebral Region

“…3 Forking at the rostral end of the notochord has been demonstrated in embryo and would be presumed as embryological source of the lateral skull base tumours. 7 Cranial chordomas are most frequent in men than in women 2:1 and may present at any age with mean age at diagnosis being 38 years. These lesions present clinically as destructive bony masses with soft tissue involvement.…”

Chondroid Chordoma of the Lateral Skull Base

“…The importance of distinguishing these three neoplasms lies in their different prognoses: low-grade chondrosarcoma has a better survival rate than chordoid tumors [1,2,11]. The survival rate does not differ significantly between chordoma and CC [2,16], though some studies report a better prognosis for CC than for classical chordoma [1].The advent of immunochemistry has provided a valuable tool for validating the diagnosis of CC, because immunohistochemical profiles of chordoma, CC and low-grade chondrosarcoma can be well established and may represent useful diagnostic criteria [2,4,9,11]. Nevertheless, only few immunohistochemical data on CC are reported in the literature.…”

“…They account for less than 1% of all intracranial neoplasms [1][2][3], the clivus and spheno-occipital synchondrosis being their most common site of origin; only few cases presenting at the lateral skull base have been reported [4][5][6]. Chondroid chordoma (CC) is a controversial clinicopathological entity, first described by Falconer et al [7] in 1968 as a biphasic neoplasm composed of cartilaginous and chordoid elements in variable amounts.…”