Neurofibrosarcomafollowing radiotherapy for ankylosing ...
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Ann. rheum. Dis. (1975), 34, 536 Case report Neurofibrosarcoma following radiotherapy for ankylosing spondylitis S. J. BENTLEY, P. DAVIS, AND M. I. V. JAYSON From the Department of Medicine, University of Bristol Bentley, S. J., Davis, P., and Jayson, M. I. V. (1975). Annals of the Rheumatic Diseases, 34, 536-538. Neurofibrosarcoma following radiotherapy for ankylosing spondylitis. Twenty-six years after radiotherapy to the spine for ankylosing spondylitis a patient developed a neurofibrosarcoma initially around the left sacroiliac joint. It is likely that this was a long-term complication of the radiotherapy. The long-term hazards of radiotherapy are well known so that it is now little used for ankylosing spondylitis. We here report a new complication-a neurofibrosarcoma developing in a patient treated some 26 years previously. History In 1946 at the age of 19 the patient developed low back pain and polyarthritis, and was diagnosed as having ankylosing spondylitis. In October 1948 he received 20 radiotherapy treatments with a total of 2000rads to each of lumbar spine, dorsal spine, cervical spine, and a 15 x 10 cm area over both sacroiliac joints. Further treatments of 1600 rads and 1400 rads were given to the left shoulder and lumbar spine, respectively, in April 1949 and October 1950. Between 1952 and 1973 he suffered occasional flares of back pain which were treated with physiotherapy and phenylbutazone by his general practitioner. In August 1974 he presented with increasing low back pain of 9 months' duration. He appeared ill with obvious weight loss, but the general examination and the peripheral joints were normal. The dorsal and lumbar spines were rigid and there was marked tenderness of the left sacroiliac joint extending across the midline. Both ankle jerks were absent. X-ray of the pelvis showed an extensive osteolytic lesion around the left sacroiliac joint eroding the sacrum and the ilium. There was also a similar but much less marked change in the right side of the sacrum (Fig. 1). Open biopsy showed an extensive pale brown gelatinous avascular tumour. Microscopy showed this to be a sarcoma with round, spindle, and giant cells, a dense reticulin pattern, marked degeneration, and no mitotic figures (Fig. 2). The appearances were those of a neuro- fibrosarcoma. The patient was treated with palliative radiotherapy and analgesics. He failed to respond and died with ureteric obstruction and renal failure in October 1974. Discussion In the past decade radiotherapy has become less acceptable as treatment for ankylosing spondylitis. It provides only temporary relief to locally inflamed areas of the spine and does not arrest the progress of the disease. In any event radiotherapy appears inferior in analgesic action to phenylbutazone (Mason, 1964). More important, however, are the reports of the long-term complications. Pulmonary fibrosis and transverse myelitis may occur, but more serious is the increased incidence of local and systemic malig- nancies (Court-Brown and Doll, 1965) and parti- cularly leukaemia (Court-Brown and Doll, 1957). Tumours at sites within the beam of radiation are usually carcinomas of the skin or viscera. Sarcomas of bone and soft tissues are rare. Doll (personal communication, 1975) has knowledge of only 5 bone sarcomas, and 7 fibrosarcomas; and Edgar and Robinson (1973) have reported five patients with fibrosarcomas. Our case fulfils the four criteria of Cahan, Woodward, Higinbotham, Stewart, and Coley (1948) for a post-irradiation tumour, and the coincidence of sites of radiotherapy and tumour make it likely that the neurofibrosarcoma is a direct complication of the treatment. Accepted for publication June 11, 1975. Correspondence to Dr. M. I. V. Jayson, Dept. of Medicine, Bristol Royal Infirmary, Bristol BS2 8HW. copyright. on July 22, 2022 by guest. Protected by http://ard.bmj.com/ Ann Rheum Dis: first published as 10.1136/ard.34.6.536 on 1 December 1975. Downloaded from
Transcript of Neurofibrosarcomafollowing radiotherapy for ankylosing ...
Ann. rheum. Dis. (1975), 34, 536
Case report
Neurofibrosarcoma following radiotherapyfor ankylosing spondylitis
S. J. BENTLEY, P. DAVIS, AND M. I. V. JAYSONFrom the Department ofMedicine, University ofBristol
Bentley, S. J., Davis, P., and Jayson, M. I. V. (1975). Annals of the Rheumatic Diseases,34, 536-538. Neurofibrosarcoma following radiotherapy for ankylosing spondylitis.Twenty-six years after radiotherapy to the spine for ankylosing spondylitis a patientdeveloped a neurofibrosarcoma initially around the left sacroiliac joint. It is likely thatthis was a long-term complication of the radiotherapy.
The long-term hazards of radiotherapy are wellknown so that it is now little used for ankylosingspondylitis. We here report a new complication-aneurofibrosarcoma developing in a patient treatedsome 26 years previously.
History
In 1946 at the age of 19 the patient developed low backpain and polyarthritis, and was diagnosed as havingankylosing spondylitis. In October 1948 he received 20radiotherapy treatments with a total of2000rads to each oflumbar spine, dorsal spine, cervical spine, and a 15 x 10cm area over both sacroiliac joints. Further treatments of1600 rads and 1400 rads were given to the left shoulder andlumbar spine, respectively, in April 1949 and October 1950.Between 1952 and 1973 he suffered occasional flares ofback pain which were treated with physiotherapy andphenylbutazone by his general practitioner. In August1974 he presented with increasing low back pain of 9months' duration. He appeared ill with obvious weightloss, but the general examination and the peripheral jointswere normal. The dorsal and lumbar spines were rigidand there was marked tenderness of the left sacroiliacjoint extending across the midline. Both ankle jerks wereabsent.X-ray of the pelvis showed an extensive osteolytic lesion
around the left sacroiliac joint eroding the sacrum and theilium. There was also a similar but much less markedchange in the right side of the sacrum (Fig. 1). Openbiopsy showed an extensive pale brown gelatinousavascular tumour. Microscopy showed this to be asarcoma with round, spindle, and giant cells, a densereticulin pattern, marked degeneration, and no mitotic
figures (Fig. 2). The appearances were those of a neuro-fibrosarcoma.The patient was treated with palliative radiotherapy
and analgesics. He failed to respond and died withureteric obstruction and renal failure in October 1974.
Discussion
In the past decade radiotherapy has become lessacceptable as treatment for ankylosing spondylitis.It provides only temporary relief to locally inflamedareas of the spine and does not arrest the progress ofthe disease. In any event radiotherapy appears inferiorin analgesic action to phenylbutazone (Mason, 1964).More important, however, are the reports of the
long-term complications. Pulmonary fibrosis andtransverse myelitis may occur, but more serious is theincreased incidence of local and systemic malig-nancies (Court-Brown and Doll, 1965) and parti-cularly leukaemia (Court-Brown and Doll, 1957).Tumours at sites within the beam of radiation areusually carcinomas of the skin or viscera. Sarcomasof bone and soft tissues are rare. Doll (personalcommunication, 1975) has knowledge of only 5 bonesarcomas, and 7 fibrosarcomas; and Edgar andRobinson (1973) have reported five patients withfibrosarcomas. Our case fulfils the four criteria ofCahan, Woodward, Higinbotham, Stewart, andColey (1948) for a post-irradiation tumour, and thecoincidence of sites of radiotherapy and tumourmake it likely that the neurofibrosarcoma is a directcomplication of the treatment.
Accepted for publication June 11, 1975.Correspondence to Dr. M. I. V. Jayson, Dept. of Medicine, Bristol Royal Infirmary, Bristol BS2 8HW.
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Neurofibrosarcoma followving radiotherapyfor ankylosing spondylitis 537
FIG. 1 X-ray showingextensive destructivechanges markedly in-volving the left sacroiliacjoint, but also the rightside of the sacrum
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FIG. 2 Biopsy oflesion39
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538 Annals ofthe Rheumatic Diseases
References
CAHAN, W. G., WOODWORD, H. Q., HIGINBOTHAM, N. L., STEWART, F. W., AND COLEY, B. L. (1948) Cancer, 1, 3(Sarcoma arising in irradiated bone)
COURT- BROWN, W. M., AND DOLL, R. (1957) 'Leukaemia and aplastic anaemia in patients irradiated for ankylosingspondylitis', in 'Special Report Series, Medical Research Council', No. 295. H.M.S.O., London
-, , 1965 Brit. med. J., 2, 1327. (Mortality from cancer and other causes after radiotherapy for ankylosingspondylitis)
EDGAR, M. A., AND ROBINSON, M. P. (1973) J. Bone Jt Surg. 55B, 183, (Post radiation sarcoma in ankylosingspondylitis)
Mason, R. M. (1964) Proc. roy. Soc. Med., 57, 533 (Spondylitis)
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