1990, The British Journal of Radiology, 63, 804-805

Case reports Progressive systemic sclerosis (diffuse scleroderma) and radiotherapy By Stephen G. Cooper, M B , BS and James W. Denham, M D , FRCR, FRACR Radiotherapy Department, Newcastle Mater Misericordiae Hospital, Waratah, NSW 2298, Australia

(Received November 1989 and in revised form February 1990)

The rheumatological manifestations of cancer are many and varied. Progressive systemic sclerosis has been reported both as an initial manifestation of malignancy (Caldwell & McCallum, 1986) and, of course, as coexisting with malignant disease. Surprisingly, only one case report is to be found in the literature reporting on the interaction between radiotherapy and scleroderma (Ransom & Cameron, 1987), the consequences of which may carry significant morbidity. Case report Our patient was 49 years old in May 1987 when referred for the management of a mass in the right neck. This right cervical node measured 6 x 5 cm, was fungating through the skin, with biopsy confirming metastatic squamous cell carcinoma. No primary site was identified either on direct ear, nose and throat examination or with computed tomography. His past medical history included heavy smoking (50 per day for 25 years) and intermittent heavy alcohol consumption. Otherwise, he had been well, working as a surgical dresser for 19 years. There was no noteworthy family history. Radical irradiation was planned and delivered in a beam direction immobilization shell. This entailed coverage of the nasopharynx and upper neck to a dose of 40 Gy in 20 fractions, given over 26 days with an opposed lateral pair, using 6 M V

(a)

Xrays, measured at the midplane. The nasopharynx was boosted by a further lOGy in five fractions. The lower neck was treated with an anterior split neck field to 50 Gy in 25 fractions over 33 days, measured to a depth of 3 cm. The right and left lower posterior neck was boosted by a further 10 Gy in five fractions using 12 MeV electrons, assessed at 3 cm depth. A final boost of 20 Gy to the neck node in 10 fractions, measured at D max was administered, using 12 MeV electrons. Spinal cord dose was less than 47 Gy. Maximum calculated point dose was 74.5 Gy in 35 fractions. The treatment was well tolerated. In particular, no severe or untoward normal tissue reactions were observed. Initial tumour response was slow, with incomplete regression 2 months after completion. The patient was subsequently lost to follow-up. Two years after initial therapy, the patient was referred back to the clinic. During this time he had lost 8 kg in weight owing to increasing difficulty in swallowing. The patient was unable to extend his neck and there was marked limitation of movement of both shoulders. Questioning elicited symptoms consistent with Raynaud's Syndrome. Additionally, over the last 6-7 months he had noted increasing dyspnoea on exertion. Clinical examination revealed findings typical of scleroderma (Fig. 1). He had widespread skin thickening, beyond thefieldof radiation, apparent in both sets of limbs, trunk and face. The thickening was maximal within the neck, completely limiting

(b)

(c)

Figure 1. Clinical photographs showing a severe flexion deformity.

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The British Journal of Radiology, October 1990

1990, The British Journal of Radiology, 63, 805-808 Case reports any flexion, extension or rotation involving the cervical spine. A severe flexion deformity was present. Similarly, there was limitation of abduction of both shoulders to about 70 or 80 degrees, and he has a flexion deformity in both elbows. Surprisingly, there was very minimal sclerodactylia in comparison to his skin changes elsewhere. There was no evidence of persistent or recurrent tumour. Investigations included a barium swallow examination, which could not be completed satisfactorily owing to regurgitation into the bronchial tree. His immunological profile was consistent with scleroderma, with ANA being highly positive, titre greater than 1 in 2560, with the immunofluorescent pattern being homogenous, nucleolar and speckled. The usual extractable nuclear antigens were not detected. Anti-DNA antibodies were not elevated. Skin biopsy from an unirradiated area was typical of scleroderma, but pulmonary function studies were more compatible with his pre-existing emphysema than with scleroderma. Marked air trapping improved by bronchodilators and a reduced carbon monoxide diffusion were noted. He was referred to a rheumatologist who recommended D-Penicillamine, 125 mgbd, increasing at monthly intervals by 125 mg to a maximum of 500-625 mg daily. Dietary advice was obtained, with some early weight gain. Subsequent follow-up at this stage has been too short to assess the utility of systemic therapy. Discussion The vascular and fibrotic changes of scleroderma are well documented, though their precise aetiology is poorly understood (Gilliland, 1988). Similarly, the late normal tissue effects of radiotherapy and their variations with such factors as total dose, fraction size,

volume are well known and documented (Hall, 1988). When occurring together, significant morbidity may develop secondary to dense subcutaneous and softtissue fibrosis. In this case, diffuse scleroderma manifested some 2 years after the completion of radical irradiation to the head and neck region. Whilst small areas received greater than 70 Gy, the bulk of the neck received only 50 Gy in 25 fractions. Nevertheless, major morbidity developed because of severe bilateral fibrosis involving all structures in the neck. Importantly, however, no evidence of tumour recurrence is to be found. Response to D-Penicillamine therapy will be monitored. This is only the second case report we have been able to identify regarding the adverse interaction between radiation therapy and scleroderma. Acknowledgment Mrs Kim Lusis is thanked for preparing the manuscript. References CALDWELL,

D.

S.

&

MCCALLUM,

R.

M.,

1986.

Rheumatological manifestations of cancer. Medical Clinics of North America, 70, 385-417. GILLILAND, B. C , 1988. Progressive systemic sclerosis (Diffuse scleroderma). In Harrisons Principles of Internal Medicine, 11th edn (McGraw Hill, Maidenhead), pp. 1428-1432. HALL, E. J., 1988. Radiobiology for the Radiobiologist, 3rd edn (J. B. Lippincott, Philadelphia), pp. 57-58; 239-261. RANSOM, D. T. & CAMERON, F. G.,

1987. Scleroderma—a

possible contraindication to lumpectomy and radiotherapy in breast carcinoma. Australasian Radiology, 31, 317-318.

Spinal cord sarcoidosis with intramedullary cyst formation By A. G. Clifton, MA, MRCP, FRCR, J. M. Stevens, DRACR, FRCR, *R. Kapoor, MA, MRCP and *P. Rudge, FRCP Departments of Radiology and *Neurology, The National Hospitals for Nervous Diseases, Maida Vale, London W9 (Received November 1989 and in revised form February 1990)

We report a case of spinal cord sarcoidosis with surgically confirmed localized cyst formation and probably syringomyelia involving almost the entire spinal cord, an association which has not been documented previously. The magnetic resonance (MR) findings before and after gadolinium diethylenetriamine-pentaacetic acid (Gd-DTPA) are described. There has been only one previous report of the MR findings in histologically confirmed spinal cord sarcoidosis, and in this case Gd-DTPA was not given. Case report A 49-year-old man presented with a 3-week history of progressive urinary hesitancy and weakness of both legs. Three Vol. 63, No. 754

years previously he was diagnosed as having sarcoidosis on the basis of bilateral hilar adenopathy on plain chest radiography, and a positive Kveim test. Ten months before this admission he had developed granulomatous uveitis. Clinical findings On initial examination there was moderate reduction of muscle power in all four limbs, whilst tone and co-ordination were normal. Reflexes were generally brisk, abdominal reflexes were absent, and there were bilateral extensor plantar responses. Vibration and joint position sensation were absent bilaterally in his feet, and there was a spinothalamic sensory level at C8 on the right and D6 on the left. Routine blood tests were normal. The erythrocyte sedimentation rate was 70 mm/h. Serum angiotensin converting enzyme

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Progressive systemic sclerosis (diffuse scleroderma) and radiotherapy.

1990, The British Journal of Radiology, 63, 804-805 Case reports Progressive systemic sclerosis (diffuse scleroderma) and radiotherapy By Stephen G...
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