Chordomas and cartilaginous tumors at the skull base

TLDR

The study examined clinical and pathologic data of 155 chordomas and all cartilaginous skull‑base tumors at Mayo Clinic. Spheno‑occipital chordomas, comprising a third of skull‑base tumors, show a surprisingly favorable prognosis and are distinct from chondrosarcomas; excluding them leaves only four chondrosarcomas, most of which have an indolent course, and long‑term survival is best with extensive resection plus irradiation.

Abstract

Special attention was given to chordomas and all cartilaginous tumors at the base of the skull during a study of the clinical and pathologic data on 155 chordomas (76 sacrococcygeal, 55 spheno-occipital, and 24 vertebral) seen at the Mayo Clinic. Attention also was drawn to a histologically separate group of spheno-occipital chordolmas which comprised one third of the tumors in this location and was associated with a surprisingly better prognosis. These tumors, which may bear a striking histologic resemblance to chondrosarcomas or chondromas, had clinical and roentgenologic features that convinced us of their kinship to ordinary chordomas and led us to designate them as "chondroid" chordomas. When these were excluded from the cartilaginous tumors of the base of the skull, there remained only four such tumors, all chondrosarcomas, in the files of the Mayo Clinic. Thus most of the chondrosarcoma-like tumors of the base of the skull have a paradoxically indolent course. Assessment of long-term survival patients indicated that extensive surgical resection combined with irradiation offers the best palliation and prolongation of life.