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

Abstract

Chordomas are rare, locally aggressive tumours that probably originate from embryonic remnants of the notochord. The sacrum is the most common site followed by the base of the skull/clival region. We present three cases with different sites of presentation of supra and parasellar, vertebral and clival chordomas that are managed at department of Neurosurgery Government General Hospital, Kurnool, Andhra Pradesh, India. Histopathologically chordomas are divided into 3 subtypes: conventional, chondroid and dedifferentiated. Of these three cases one is chondroid type hitopathologically. The chondroid chordoma is having a significantly favorable outlook than the typical physaliphorous chordoma and the pertinent literature is reviewed. KEYWORDS: Chordomas; Presentation of three case reports of para sellar, Clival and vertebral region and the literature reviewed. INTRODUCTION:Chordomas are generally slow growing malignant neoplasms of presumed notochordal origin. 1 Cranial chordomas account for less than 1% of all intracranial neoplasms 2 and 4% of all primary bone tumours. Muller (1858) was the first to recognize its origin from notochordal cells, and Ribbert (1894) first introduced the term chordoma. Nearly all chordomas arising in the skull are related to the clivus. Those tumours related to the most rostral extension of the notochord in the dorsum sellae will present as sellar and parasellar chordomas. 3 The term chondroid chordoma was introduced in 1973 by Heffelfinger etal. 1 It represented a sub-group of chordoma that straddles the bridge between conventional chordoma and chondrosarcoma. It has a better prognosis than the conventional type. 4,5