This paper reports cases of a twin brother and sister with a peculiar systemic disease of the skeleton, which notwithstanding accurate clinical observation over a number of years and, in one of the cases, extensive post-mortem examination, has not fitted into any classification of diseases of the skeleton so far described. It is true that signs which also occur in known bone diseases were found, although in these there was also a considerable number of dissimilarities. C a s e 1. Male, aged 48 years, was admitted with meningococcal meningitis; recovery following treatment with penicillin. He had attended school till his eleventh year and remained two years in one class. Worked as a farm labourer up to his 32nd year, since when, unable to continue his occupation, he became a hawker. He regularly went out on a bicycle with a fairly large load on his back up to half a year previously. According to photographs, he had a normal appearance when 10 years of age but, at the age of 25, his chin was already large and broad, suggesting acromegalia. At the age of 37, he was no longer able to see well. An examination showed papilloedema with atrophy of the optic nerves, as well as hyperostosis of the skull bones. At that time he tired rapidly and since then had had occasional vertigo. During the last few years walking, as well as speech, became difficult. Gradually he became more deaf, and was now and then troubled by stabbing head pains. Health otherwise good.Clinical examination: Well-built man; good nutritional condition. The skin was dry and slightly flaky but not thickened or hardened. Height: 1 . 8 0 m, weight: 64 kg. Conspicuously high forehead and large prominent chin, caused by the greatly thickened lower jaw, not painful t o pressure. The teeth were ground off and in a normal position in the lower jaw. Kormal bite. In contrast to acromegalia there was no progn6thism and the teeth occupied normal space in the mandible. Mouth-opening rather small; tongue in normal size. Slight exophthalmus with a wide eye-fissure. The pupillae were equally round, reacted well to light and convergence. Discs: atrophy of the optic nerves on both -
Sacrococcygeal chordoma is a rare malignant neoplasm situated in a location adjacent to important structures. Distant metastases are usually rare and occur late. The treatment of choice usually consists of radical surgery, sometimes followed by radiotherapy. Extensive surgical resection is difficult and often causes bladder and/or bowel dysfunction, and the local recurrence rate remains high. In an attempt to diminish both risks, the authors introduced cryosurgery in situ as a new treatment modality for chordoma in the sacrococcygeal region. From 1974 to 1980, four patients (two male, two female) with sacrococcygeal chordoma were treated with cryosurgery without resection. Two patients had extensive tumors (greater than 10 cm) and could be treated only palliatively. Two other patients with smaller tumors (less than 10 cm) had radical cryosurgical treatment. Both patients are disease-free 10 and 7 years after cryosurgical treatment. One of the palliatively treated patients is alive with local recurrence 4 years after cryosurgery, the other died of tumor after 5 years. In a cryosurgical lesion, the tissue is completely devitalized; however, the architecture of the tissue in peripheral nerves, large vessels, and bone is preserved and remains as a perfect autograft. Frozen tissue is very susceptible to the hematogenous spread of infection. Therefore, infection prevention is of utmost importance. The authors believe that cryosurgery should have a place in the treatment of sacrococcygeal chordoma.
THE development of a tumour can be taken as an expression of a disturbed equilibrium between the number of undifferentiated and differentiated cells of a tissue, in favour of the number of undifferentiated cells. Irrespective of the cause of this disorder, the derailed cells can show morphological, biochemical and immunological deviations. The purpose of this investigation was to compare the histochemical and enzyme histochemical pattern of cells of regenerating bone tissue and of cells of induced bone tumours. To determine the enzyme pattern of cells in regenerating bone tissue healing fractures have been studied. Several methods of inducing bone tumours are known: Radioactive compounds such as 45Ca, 89Sr, 90Sr, 239Pu and 226Ra (Anderson,
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