Sacrococcygeal chordoma

Abstract

THE LONDON HOSPITAL, LONDON CHORDOMAS are malignant tumours which arise in remnants of the embryonic notochord. The notochord develops in the 4th week of embryonic life (Fig. I) and regresses by the 7th week to form the backbone from basi-occiput to coccyx. Forty-one occurred in the sacrococcygeal region. A similar proportion occurred in the basi-occipital region and the remainder were scattered in the intervening vertebral column. Chordomas occur about three times more commonly in men than in women B FIG. 1.-Sagittal sections of two embryos showing the notochord Nc developing into the vertebral column. A, 4th week (3.5-4 mm.). 6, 10th week (50-55 mm.). FIG. 3.--Smear preparation of tumour cells obtained by aspiration H. and E. ( x 214.)vertebral bodies are originally formed and the number subsequently falls to about thirty-four. During this regrouping, rests of notochordal tissue may remain (Fig. 2). One view holds that chordomas arise from these rests (Horwitz, I94I), but, on the other hand, ectopic remnants of notochord (ecchondrosis physaliphora) which are found on the clivis in 2 per cent of autopsy examinations are not thought to become neoplastic (Russell and Rubinstein, 1963). The notochord is normally manifest in the adult as the nucleus pulposus of the intervertebral disks.Six hundred and forty-one cases of chordoma have becn reported in the literature, of which 41 per cent FIG. 2.--Sagirtal sections of the coccyx of two full-term foetuses showing rests of notochord tissue in the vertebrae. FIG. 4.--Section showing masses of tumour cells separated by connective-tissue trabeculae. The characteristic physaliferous cells are filled with abundant cytoplasm. A single, large, cytoplasmic vacuole displaces the nucleus in many cells. There are few mitoses. H. and E. ( x SS.)during an age spectrum from the second to the eighth decades. The incidence is less than I per cent of all central nervous system tumours (Poppen and King, 1952). These tumours are soft, mucinous, slow growing but infiltrative and malignant; they are often quite large at the time of diagnosis. The histological picture is of an epithelioid or sarcomatous general architecture with a characteristic large vacuolated physaliferous cell (Figs. 3, 4) containing an abundance of glycogen (Anderson, 1966).

Combined Anterior and Posterior En Bloc Vertebrectomy for Lumbar Chordoma

“…When adequate excision is not possible, radiation therapy may provide at least a short-term benefit. Radiation therapy is also used for recurrence 2,10) . So, it is gener-ally agreed that complete surgical excision of the tumor is the only curative procedure 8) .…”

MRI in the Evaluation Spinal Cord Tumors with Histopathological Correlation

“…The lesion was suppressed on fat sat and STIR images. Lee et al (16) (20).50% arise in the sacrum, 35% in the clivus, and 15% in the vertebrae. (21,22) On MRI 75% of chordomas are isointense to cord on T1-weighted images and 25% are hypointense.…”

Sacrococcygeal Chordoma: Clinicoradiological Study

“…The notochord is normally represented in the adult as the nucleus pulposus of the invertebral disks [8]. There are two theories about the origin of chordomas: (1) chor domas arise from the persistence of aberrant notochor dal tissue at the surface or outside of vertebral bodies; (2) chordomas arise from the notochordal tissue nor mally present in the adult in the nuclei pulposi, which has been expelled as a result of trauma [9], A previous trauma was reported in about 20% of patients [4] and the medium interval between trauma and the development of the tumor was about 4 years.…”

“…Presacral tumors can also mechanically obstruct the bladder and/or rectum, causing difficulty in defeca tion, constipation, dysuria, frequency or urinary reten tion [7,9]. Diagnosis may be established by a careful physical and neurological examination and with appropriate ra diological study, but it has to be confirmed by a histo pathologic examination of biopsy specimens [7][8][9], Also needle aspiration cytology can be useful in the diagnosis [ …”