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).
BOTH these patients were young women and their case histories present certain features which may have a bearing on the atiology, treatment and post-operative complications of this distressing condition.Case L-Girl aged 18, admitted to the London Hospital in January 1948. In 1942 Mr. D. W. C. Northfield had removed an ependymoma from the cauda equina. There had been no local recurrence. Following the operation the girl showed signs of a partial lesion of sacral nerves 1-4. There was a saddle area of anesthesia over the buttocks and in 1948 one of her ankles had been arthrodesed because of a flail joint. Since her operation she had suffered from urinary incontinence and for the past five months her rectum had prolapsed.On admission her general condition was good but she was in considerable distress from double incontinence. Despite her large prolapse voluntary contraction of the anal sphincters was quite good.18.2.48: Rectosigmoidectomy was carried out from below and about 6 in. of bowel was removed. 26.2.48: Severe secondary hTemorrhage occurred from the suture line; this was treated by irrigation of the rectum, packing the bowel and an emergency left inguinal colostomy. 7.4.48: The colostomy was closed. 5.3.50: Admitted with a large recurrent prolapse; in other respects her condition was unchanged. 7.3.50: Lithotomy Trendelenburg position. Laparotomy showed the pelvic colon running down to the distal rectum surrounded by a deep pouch of peritoneum which extended between the colon and vagina and also part way behind the colon on the right side.
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