Late House Surgeon to the Department of Plastic Surgery, Surgical Professorial Unit, Queen Elizabeth Hospital, Birmingham

MALIGNANT SYNOVIOMA OF THE HAND By R. F. CRAMI'TON, B.Sc., M.B., Ch.B. Late House Surgeon to the Department of Plastic Surgery, Surgical Professorial Unit, Queen Elizabeth Hospital, Birmingham MALIGNANT synovioma is a rare neoplasm. One hundred and forty-three cases have been reported, and of these in only one (Coley and Pierson, 1937) was it situated on the dorsum of the hand. The present case is therefore worth recording, and in addition the opportunity has been taken to review briefly the reported cases. CASE HISTORY History.--The patient, an Irishman aged 32 years, was a house painter by trade. He was first seen at Birmingham in January 195 o, complaining of a swelling on the back of his right hand. This first appeared in 1945 and slowly enlarged. In October 1947 it was excised. One year later the swelling recurred at the site of operation, and in February 1949 was again excised. In August 1949 it again recurred. The patient had had no pain, and until February 1949 no disability of the hand, when, at operation, portions of the extensor tendons to the first and second fingers were excised. I am indebted to Mr F. F. McKee, Dublin, for this detailed report :-- " I first saw him in October 1947, when he had an ulcerating cyst on the dorsum of his right hand. He said that he had had this for two years, when it commenced as a small lump and gradually enlarged. On examination the lump was cystic, about the size of a small tangerine orange, not attached to the skin, but attached to the tendon, and gave me the impression that it was a malignant tumour of the tendon sheath. It was not attached to the bone. It appeared to be bursting through the skin rather than ulcerating. It was decided to remove the mass, including the surrounding skin. This was done and the tumour removed. At one point it was attached to the extensor tendon of the index finger, but as the attachment was of a small nature I decided not to remove the tendon. I grafted the area and it healed satisfactorily. " About ten days after that the patient noticed a swelling on the inner side of the right elbow. The epitrochlear gland was enlarged, and fearing a metastasis it was removed. " He was admitted again on 3rd February 1949 with a recurrence. On his admission he had one large swelling, and a smaller one on the right side of it. They were similar in nature to the first, and I decided that the only thing to do was to sacrifice the tendon. This was done and all was grafted again. He was in hospital for four weeks and was discharged healed." The following pathology report on the tumour after first excision was kindly forwarded by Dr J. H. Pollock of Dublin :-- " The sections showed that this tumour is not apparently derived from the squamous epithelium. " The marginal areas show a structure which I fear to be sarcomatous, the tumour cells in places having a radial distribution relative to the small blood-vessels, causing the picture to resemble that attributed to a " perithelioma." The bulk of the tumour has undergone a myxomatous change. Sections through the minute lymph nodes fail to show any evidence of metastatic tumour deposit." 56

MALIGNANT SYNOVIOMA OF THE HAND 57 Examination.raThe patient was admitted to the Queen Elizabeth Hospital, Birmingham, on 7th January I95O. A cystic swelling was present on the dorsum of the right hand (Fig. I). It was oval in shape, measuring x by ~ in. The surrounding skin consisted mainly of the previous grafts. The swelling was attached to its overlying skin, and only slightly movable over the third metacarpal. Small lymph nodes were palpable in both axilla:. No supraclavicular nodes were palpable. Radiological examination of the chest showed no abnormality, and of the hand, failed to reveal any involvement of the bone. FIG. I The appearance of the tumour when first seen in January 195o. FIG. 2 The appearance of the hand eight weeks after operation. Operation.--On I3th January I95o operation was performed by Mr O. T. Mansfield. The tumour and a wide area of surrounding skin were removed, together with the middle finger (including its metacarpal) and the shaft of the fourth metacarpal. The skin of the flexor surface of the middle finger was retained. Cancellous bone grafts from the right iliac crest were used to replace the shaft of the fourth metacarpal, and to construct a strut between the second and fourth metacarpals. The strut was inserted to maintain the width of the palm. The flap from the middle finger was turned back to cover the defect on the dorsum of the hand. The wound was dressed, and a plaster case applied from the distal interphalangeal joints to the middle of the forearm. Post-operative Course.--This was uneventful, and after seven weeks the plaster was removed. The wound had healed by first intention (Fig. 2) and the bone grafts were clinically stable. Wax baths and active movements were then begun, and recovery of function was rapid. Ten weeks after operation the patient was discharged to a Rehabilitation Centre. On discharge, full flexion and 5 per cent. extension of the fingers, with full opposition of the thumb, were present.

58 BRITISH JOURNAL OF PLASTIC SURGERY In September 195 the patient was in very good health, working, and showed no signs of either local recurrence or metastases. Pathology Report (by Dr J. G. Jackson).--" This tumour consists of elements of two types. Firstly, a framework of dense collagenous fibroblastic tissue, and secondly, a loose meshwork of cells which vary in size and shape to a considerable degree. These are predominantly of stellate or fusiform type, but in places they D FIG. 3 Radiograph of the hand eight months after operation. appear as cells approaching the cuboidal epithelial type. In many places mitoses are present together with hyperchromatic nuclei, and the tumour must be looked upon as malignant. There is in this tumour no structure approaching the spaces lined by a papillary layer of epithelioid-like cells as described by Haagenson and Stout (I944), but, however, this is possibly due to degree of dedifferentiation in this tumour. The myxomatous appearances in many parts of this tumour is not inconsistent with synovioma, and the appearance has been described by Willis (1948). The intercellular material is stained red with mucicarmine. "It would thus appear that this turnout approaches the criteria presented by Haagenson and Stout (1944) in that it consists of a fibromatous or fibrosarcomatous element, together with a second element of cells of variable morphology which are mucicarminophilic, and thus it may be considered to be a synovial sarcoma even if rather atypical in some of its appearances."

MALIGNANT SYNOVIOMA OF THE HAND 59 FIG. 4 Photomicrographs of sections of the tumour after excision in January I95 o. x I00. x 400.

60 BRITISH JOURNAL OF PLASTIC SURGERY DISCUSSION Review of Reported Cases.--Previous reviews of cases up to I946 have been made by Haagenson and Stout (1944) and Bennett (I947). Since that time only six cases have been recorded, but the papers on three of these are not available at present. The remaining three cases, all reported by Harris (I948), are summarised in Table I. TABLE I Author Harris. i Harris. Harris. t Age. Sex Site. Duration Primary amputation Local excision Local recurrence. Secondary amputation Metastases. Result Total duration 61 1 Female, Ankle ] 8 months i Living 2½ years 33 Male Calf I week Living year 37 Male Thigh 4 months Lungs Dead I year J I Age and Sex Incidence.--Males are more frequently affected than females, in the ratio of 3 to 2. Any age group is affected, ranging from birth (Bennett, r947) to 88 years of age (Haagenson and Stout, I944) ; 8o per cent. of reported cases, however, occur between the ages of 20 and 40 years, with a mean age of 32. Site.--Fifty per cent. of the tumours occur around the knee joint, 25 per cent. around the ankle joint, and the rest scattered over the body. A few tumours have been reported where normally synovial membrane is not found, e.g., in the thigh (Bennett, I947) and the forearm (Briggs, I942). Clinical Features.--The presenting symptoms are swelling, localised pain, or both. The mean duration of symptoms of I43 cases, before seeking advice, was 1.9 years. On examination a swelling is always found, which is usually cystic. Briggs (i942) reported a series of nine cases where a clinical diagnosis of" benign bursal cyst "was made in every case. At this stage the turnout may be misdiagnosed as a simple synovial cyst, hence the necessity of sending all cystic swellings of synovial membrane for histological examination. In the early stages the tumour is mobile and localised. It grows slowly, and after a period varying from a few months to several years becomes attached to surrounding structures. At this stage it behaves as a locally malignant neoplasm. Metastases occur late, most frequently in the lungs, but the regional lymph nodes are involved in about 2o per cent. of cases. Prognosis.--In spite of the slow growth and accessibility of this tumour, cure is very rare. In only four of the reported cases have metastases been detected before operation. The follow up of r43 cases is summarised in Table II.

MALIGNANT SYNOVIOMA OF THE HAND 6I TABLE II Follow Up of I43 Cases Died with metastases Living with metastases Alive within 5 years with no metastases Alive after 5 years with no metastases. Unknown 59 I7 35 4 ~8 Total number of cases Treatment.--All forms of treatment have been disappointing. Excision combined with post-operative irradiation has yielded the best results, but the numbers are too few to have statistical significance. The treatment of the four cases of five-year survivals is shown in Table III. TABLE III The Treatment of the Four Five-year Survivals ~43,i Author. Coley and Pierson (i937) Coley and Pierson (i937) Briggs (i942) Briggs (i942) Treatment. Primary amputation. Primary amputation with irradiation. Local excision with irradiation. Local excision with irradiation. Pathology.--In an attempt to define malignant synovioma on a histological basis, studies in both histology and tissue culture have been made by Haagenson and Stout (I944). These demonstrated the following histological characteristics :-- I. Areas of mesothelial cells containing centres into which a mucicarminophilic substance is secreted. 2. Strands of fibrosarcomatous cells with intermingling reticular fibres. 3. These two characteristics are invariably present, but in widely varying proportions. However, any other serous membrane developed from mesodermal tissue secretes mucicarminophilic substances. This characteristic, therefore, indicates only the origin of the turnout and has no bearing on its malignancy. Willis (I948) has described how the lining cells of the cavities in this tumour may assume an epitheliamike appearance. He also points out that myxomatous degeneration is a frequent finding. SUMMARY i. A case of malignant synovioma of the hand is described. 2. The typical clinical and pathological characteristics of this tumour are discussed after a brief review of I43 reported cases.

62 BRITISH JOURNAL OF PLASTIC SURGERY 3" The necessity of sending all cystic tumours of synovial membrane for examination is emphasised. 4. An operation is described, using a bone graft to maintain the width of the palm after excision of a finger and metacarpal. 5. Treatment is reviewed. Neither local excision nor amputation is successful. Irradiation after excision has yielded better results, but the numbers are too few to be statistically significant. Only four five-year survivals are reported from I43 cases. I am indebted to Mr O. T. MansfieM, Director of the Department of Plastic Surgery, for his unfailing assistance and encouragement. I wish to record my grateful thanks to Professor E. B. Manley, Dr.7. G. ffackson, Mr E. B. Brain, and Mr ft. Dee. REFERENCES ADmR, F. E. (I935). Ann. Surg., xox, 8Io. BENNETT, G. A. (I947). ft. Bone fit. Surg., 29, 259. BERGER, L. (I938). Amer. ft. Cancer, 34, 5oi. BLACK, W. C. (r936).,4rner. ft. Cancer, 28, 48I. BRIGGS, C. D. (I942). Ann. Surg., xzs, 4r3. COLEY, B. L., and PIERSON, J. C. (I937). Surgery, i, Ir 3. I4-aAGENSON, C. D., and STOUT, A. P. (I944). Ann. Surg., x2o, 826. HARRIS, V. C. J. (i948). Brit. reed. ft., x, 447. KNox, L. C. (I936). Amer. ft. Cancer, 28, 46I. KNUTSSON, F. (I948). Acta Radiol., 29, 4, 48. LAUCHE, A. (I947). Frankfurt. Ztschr. Path., 59, 2. MAROTTOLI, O. R. (I946). An. de circ., xi, 82. (I946). Rev. Assoc. reed. argent., 60, 505. MURRAY, M. R., STOUT, A. P., and POGOGEFF, I. A. (t944). Ann. Surg., r2o, 843. SMrTH, W. L. (I927). Arner. ft. Path., 3, 355. SNYDER, C. H. (I942). Amer. ft. Surg., 55, 67. WILLIS, R. A. (I948). " The Pathology of Tumours." London : Butterworth & Co.