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Sarcoma of tendon sheath
Sarcoma of Tendon Sheath--E. G. Fragiadakis and P. Vatopoulos
SARCOMA OF T E N D O N S H E A T H E. G. F R A G I A D A K I S and P. VATOPOULOS, Athens Sarcoma of the soft tissue of the hand is a rare tumour. Synovial sarcoma is a malignant tumour which may arise from the joint capsules, the bursae or the tendon sheaths. The lesion occurs chiefly in the extremities and the limb girdles but may occur rarely in the trunk and neck. I N C I D E N C E IN T H E H A N D
Cadman, Soule and Kelly (1965) in their analysis of 134 tumours of synovial sarcoma, found that most of the lesions (94.8 per cent) were located on the extremities while hands lay in fourth place (10.5 per cent) in frequency after the thigh, knee and foot. In the series of Tillotson, McDonald and Janes (1951), a total of 67.9 per cent of their cases accounted for the extremities with the hand tying for the fifth place. In the series of Haagensen and Stout (1944), the distribution in the extremities was 94 per cent out of 104 cases, with the hand in the fifth place (3.2 per cent). CASE R E P O R T
A female of twenty-eight years of age presented herself with a large swelling on the radial side of her right hand between the thumb and the index finger. She was complaining of a rapidly enlarging mass on the thenar eminence and the first web space with symptoms of four months' duration. In 1969, two years before admission she had noticed the appearance of a small nodule of a size of a bean on the right thenar eminence, which otherwise was painless and did not interfere with the movements of the fingers and thumb. She was advised by her own doctor to have it excised, but she neglected it until one year later. When it reached the size of an egg she visited her doctor again who recommended excision biopsy. She hesitated to undergo the operation. Ten months later
Fig. 1 Tumour covering the first web space and the thenar eminence. Fig. 2 Dorsal view of the tumour. The H a n d - - V o l . 5
No. 1
1973
71
Sarcoma o[ Tendon Sheath--E. G. Fragiadakis and P. Vatopoulos
she appeared with a huge mass in the first web space and the thenar eminence, slightly painful and rather hard and lobulated on the surface. There was no skin attachment of the tumour (Figs. 1, 2, 3). The mass was easily movable and had no connection with the underlying metacarpals. Finger movements were normal except the abductor pollicis brevis whose action could not be detected. No lymph nodes could be detected in the axilla. The radiographic examination showed the bones of the hand were normal, although the shadow of the tumour was easily seen between the first and the third metacarpals (Fig. 4).
!
1I
Fig. 3 Tumour seen from the radial side. Fig 4a & b. The shadow of the tumour can be easily seen between the first and the third metacarpals. Biopsy was performed. The tumour was noted to be partially encapsulated and extended into the palm through the web space of the index and middle fingers. It appeared to originate from the sheath of the flexor tendons, had no connections to the neurovascular structures and the excision therefore, was done rather easily (Fig. 5). The lesion had been totally removed and the gross specimen weighed 85 gr. and measured 9 cm in its longest dimension. The microscopic examination revealed a synovial sarcoma of tendon sheath (Figs. 6, 7). An amputation was performed at the middle of the forearm. Careful examination for the possibility of metastases was carried out but none was found. One year after the amputation the patient is in good health. DISCUSSION Pathology
Synovial sarcomas are the most commonly observed malignant neoplasms o f somatic tissue involving the hands and feet. (Pack and Ariel, 1964). It is said to account for 10 per cent of all primary malignant tumours of the soft parts of the extremities and is regarded as the commonest sarcoma of the hands and feet. 72
The Hand--Vol. 5
No. 1
1973
Sarcoma of Tendon Sheath--E. G. Fragiadakis and P. Vatopoulos
Fig. 5. The tumour excised.
Fig. 6 and 7. Microscopic appearance of the turnour.
Synovium is a specialised connective tissue structure which lines all open joints, with the exception of the articular surfaces and forms the lining of tendon sheaths. Therefore, tumours that arise from the synovium occur in the joints and the sheaths. Most develop from the tendon sheaths, although Buxton (1922) states that "primary tumours of tendons are extremely rare and probably do not occur at all". They may be benign or malignant. The diagnosis of synovial sarcomas of tendon sheaths histologically is generally easy if one is careful to take a number of sections from different areas for in some of them the characteristic features are sure to appear. Clinical Features
The initial symptom of the synovial sarcoma is usually pain Haagensen and Stout (1944), Lichtenstein (1965). According to Lazarus and Marks (1943) this is preceded in about 25 per cent of cases by a history of trauma. But this is too small to have any aetiological significance. In our case there was neither pain as initial symptom nor any preceding history of trauma. From the study of literature it seems that the location of this malignant tumour in the hand is slightly more common in males than in females, and usually occurs between the ages 2(L40. Characteristic in our case was that the patient sought medical care two years after the first appearance of a small nodule in her right hand. So was the case in most of the synovial sarcomas in the hand reported also by other authors .(Tillotson 1951, Cadman 1965). The fact that the symptoms are mild and worsen only after a prolonged period, accounted for the observation that an average of almost 1.5 years elapsed from the onset of symptoms until the patients sought adequate medical aid. The Hand--Vol. 5
No. 1
1973
73
Sarcoma o[ Tendon Sheath--E. G. Fragiadakis and P. Vatopoulos
Cadman reported that duration of the disease from onset of symptoms to death from this kind of tumour is indeed long (a mean of 6.5 years in 78 cases), in comparison with other comparably lethal sarcomas. Treatment
The diagnosis having been proved histologically amputation should be performed promptly. It is important that it be done at a level high enough to avoid local persistence of the disease, because like certain other types of soft part sarcomas those developing from synovia have a tendency to extend longitudinally between fascial planes at a surprising distance in the affected limb. In generally, the situation of synovial sarcoma in the hand, and its tendency to remain locafised for a long time, are factors that give the surgeon a great advantage in deafing with it. On the other hand Haagensen and Stout (1944) state that since regional lymph node dissection is not a hazardous operation it should be seriously considered as a separate and final stage in the treatment of synovial sarcoma in the hand. In our case we did not excise the regional lymph nodes. Pack and Ariel (1964) suggest that wide local excision be used only for the most superficial lesion of hands. Unfortunately most synovial sarcomas are deep seated and have invaded adjacent structures. Amputation of the involved member at a suitable proximal distance with postoperative irradiation seems to be the treatment of choice. In fact, in the malignant forms early amputation is advisable. The site of amputation in the upper limb can be judged most satisfactorily by the surgeon at the time of operation (Bunnell 1970). R e c u r r e n c e and Survival
The malignant synoviomas in the hand tend to recur locally and metastatise readily through the blood or lymph nodes (Bunnell). Metastases, other than to the regional lymph nodes occur most frequently to the lungs but also involve the bones. A five year survival rate is given for the synovial sarcomas in general, but most authors believe it is not above 15-20 per cent (Weinreich 1963). A review of the literature on this turnout revealed only four cases of five year survivals, among 143 cases (Crampton 1952). Raben (1965) reported a five year survival rate of 50 per cent, using a combination of surgery and radiation therapy. The survival rate in the series of Hand and McFarland (1970) was up to three years. Haagensen and Stout give the information that one patient in which a simple biopsy was done followed shortly by primary amputation has been well for eight years. This is the longest survival on record following any form of treatment for synovial sarcoma. Our case has been followed for only one year at the moment and no metastases have been observed so far. 74
The Itand--Vol. 5
No. 1
1973
Sarcoma o[ Tendon Sheath--E. G. Fragiadakis attd P. Vatopoulos
SUMMARY
A case of synovial sarcoma of tendon sheath in the hand is reported. This has been the only case in the records of "Saint Paul" Orthopaedic and Accident Hospital within the last three years. T h e synovial sarcoma is a rare, highly specialised form of malignant neoplasm which develops in the hand. The tumour generally develops slowly, is more c o m m o n l y found in young adult males and finally metastatises through the blood stream and occasionally to the regional lymph nodes. Diagnosis should be always established by histological examination. Treatment should be radical. High amputation and possibly regional node dissection. R a d i o t h e r a p y has been little used and there is no proof that it is effective.
REFERENCES
BUNNELL, S. (1970). Surgery of the Hand. Revised by J. H. Boyes. Fifth Edition. Philadelphia, J. B. Lippincott Company 703-704. BUXTON, St. J. D. (1922). Tumours of Tendon and Tendon Sheaths. British Journal of Surgery 10: 469-474. CADMAN, N. L., SOULE, E. H., and KELLY, P. J. (1965). Synovial Sarcoma. An Analysis of 134 Tumors. Cancer, 18: 613427. CRAMPTON, R. F. (1952). Malignant Synovioma of the Hand. British Journal of Plastic Surgery 4: 56-62. HAAGENSEN, C. D. and STOUT, A. P. (1944). Synovial Sarcoma. Annals of Surgery. 120: 826-842. HAND, C. R. and McFARLAND, G. B. (1970). Synovial Sarcoma of the Hand. Journal of the Louisiana State Medical Society. Vol. 122: I-5. LAZARUS, J. A. and MARKS, M. S. (1943). Synovial Sarcoma: With Report of Two Cases. Surgery: 13, 290-308. LICHTENSTEIN, L. (1965). Bone Tumors. Third Edition. Saint Louis, The C. V. Mosby Company. 397--404. PACK, G. T. and ARIEL, I. M. (1964). Treatment of Cancer and Allied Diseases. Second Edition, New York, Harper and Row Publishers Inc. Vol. 8: 182-193. RABEN, M., CALABRESE, A., I-IIGINBOTHAM, N. L. and PHILIPS, R. (1965). Malignant Synovioma. American Journal of Roentgenology. 93: 145-153. TILLOTSON, J. F., McDONALD, J. R. and JANES, J. M. (1951). Synovial Sarcomata. Journal of Bone and Joint Surgery. 33-A: 459--473. WEINREICH, M. (1963). Clinical Picture of Malignant Synovioma. Zeitschrift fiir Orthopadie und ihre Grenzgebiete 97: 353. The H a n d - - V o l . 5
No. 1
1973
75
SARCOMA OF T E N D O N S H E A T H E. G. F R A G I A D A K I S and P. VATOPOULOS, Athens Sarcoma of the soft tissue of the hand is a rare tumour. Synovial sarcoma is a malignant tumour which may arise from the joint capsules, the bursae or the tendon sheaths. The lesion occurs chiefly in the extremities and the limb girdles but may occur rarely in the trunk and neck. I N C I D E N C E IN T H E H A N D
Cadman, Soule and Kelly (1965) in their analysis of 134 tumours of synovial sarcoma, found that most of the lesions (94.8 per cent) were located on the extremities while hands lay in fourth place (10.5 per cent) in frequency after the thigh, knee and foot. In the series of Tillotson, McDonald and Janes (1951), a total of 67.9 per cent of their cases accounted for the extremities with the hand tying for the fifth place. In the series of Haagensen and Stout (1944), the distribution in the extremities was 94 per cent out of 104 cases, with the hand in the fifth place (3.2 per cent). CASE R E P O R T
A female of twenty-eight years of age presented herself with a large swelling on the radial side of her right hand between the thumb and the index finger. She was complaining of a rapidly enlarging mass on the thenar eminence and the first web space with symptoms of four months' duration. In 1969, two years before admission she had noticed the appearance of a small nodule of a size of a bean on the right thenar eminence, which otherwise was painless and did not interfere with the movements of the fingers and thumb. She was advised by her own doctor to have it excised, but she neglected it until one year later. When it reached the size of an egg she visited her doctor again who recommended excision biopsy. She hesitated to undergo the operation. Ten months later
Fig. 1 Tumour covering the first web space and the thenar eminence. Fig. 2 Dorsal view of the tumour. The H a n d - - V o l . 5
No. 1
1973
71
Sarcoma o[ Tendon Sheath--E. G. Fragiadakis and P. Vatopoulos
she appeared with a huge mass in the first web space and the thenar eminence, slightly painful and rather hard and lobulated on the surface. There was no skin attachment of the tumour (Figs. 1, 2, 3). The mass was easily movable and had no connection with the underlying metacarpals. Finger movements were normal except the abductor pollicis brevis whose action could not be detected. No lymph nodes could be detected in the axilla. The radiographic examination showed the bones of the hand were normal, although the shadow of the tumour was easily seen between the first and the third metacarpals (Fig. 4).
!
1I
Fig. 3 Tumour seen from the radial side. Fig 4a & b. The shadow of the tumour can be easily seen between the first and the third metacarpals. Biopsy was performed. The tumour was noted to be partially encapsulated and extended into the palm through the web space of the index and middle fingers. It appeared to originate from the sheath of the flexor tendons, had no connections to the neurovascular structures and the excision therefore, was done rather easily (Fig. 5). The lesion had been totally removed and the gross specimen weighed 85 gr. and measured 9 cm in its longest dimension. The microscopic examination revealed a synovial sarcoma of tendon sheath (Figs. 6, 7). An amputation was performed at the middle of the forearm. Careful examination for the possibility of metastases was carried out but none was found. One year after the amputation the patient is in good health. DISCUSSION Pathology
Synovial sarcomas are the most commonly observed malignant neoplasms o f somatic tissue involving the hands and feet. (Pack and Ariel, 1964). It is said to account for 10 per cent of all primary malignant tumours of the soft parts of the extremities and is regarded as the commonest sarcoma of the hands and feet. 72
The Hand--Vol. 5
No. 1
1973
Sarcoma of Tendon Sheath--E. G. Fragiadakis and P. Vatopoulos
Fig. 5. The tumour excised.
Fig. 6 and 7. Microscopic appearance of the turnour.
Synovium is a specialised connective tissue structure which lines all open joints, with the exception of the articular surfaces and forms the lining of tendon sheaths. Therefore, tumours that arise from the synovium occur in the joints and the sheaths. Most develop from the tendon sheaths, although Buxton (1922) states that "primary tumours of tendons are extremely rare and probably do not occur at all". They may be benign or malignant. The diagnosis of synovial sarcomas of tendon sheaths histologically is generally easy if one is careful to take a number of sections from different areas for in some of them the characteristic features are sure to appear. Clinical Features
The initial symptom of the synovial sarcoma is usually pain Haagensen and Stout (1944), Lichtenstein (1965). According to Lazarus and Marks (1943) this is preceded in about 25 per cent of cases by a history of trauma. But this is too small to have any aetiological significance. In our case there was neither pain as initial symptom nor any preceding history of trauma. From the study of literature it seems that the location of this malignant tumour in the hand is slightly more common in males than in females, and usually occurs between the ages 2(L40. Characteristic in our case was that the patient sought medical care two years after the first appearance of a small nodule in her right hand. So was the case in most of the synovial sarcomas in the hand reported also by other authors .(Tillotson 1951, Cadman 1965). The fact that the symptoms are mild and worsen only after a prolonged period, accounted for the observation that an average of almost 1.5 years elapsed from the onset of symptoms until the patients sought adequate medical aid. The Hand--Vol. 5
No. 1
1973
73
Sarcoma o[ Tendon Sheath--E. G. Fragiadakis and P. Vatopoulos
Cadman reported that duration of the disease from onset of symptoms to death from this kind of tumour is indeed long (a mean of 6.5 years in 78 cases), in comparison with other comparably lethal sarcomas. Treatment
The diagnosis having been proved histologically amputation should be performed promptly. It is important that it be done at a level high enough to avoid local persistence of the disease, because like certain other types of soft part sarcomas those developing from synovia have a tendency to extend longitudinally between fascial planes at a surprising distance in the affected limb. In generally, the situation of synovial sarcoma in the hand, and its tendency to remain locafised for a long time, are factors that give the surgeon a great advantage in deafing with it. On the other hand Haagensen and Stout (1944) state that since regional lymph node dissection is not a hazardous operation it should be seriously considered as a separate and final stage in the treatment of synovial sarcoma in the hand. In our case we did not excise the regional lymph nodes. Pack and Ariel (1964) suggest that wide local excision be used only for the most superficial lesion of hands. Unfortunately most synovial sarcomas are deep seated and have invaded adjacent structures. Amputation of the involved member at a suitable proximal distance with postoperative irradiation seems to be the treatment of choice. In fact, in the malignant forms early amputation is advisable. The site of amputation in the upper limb can be judged most satisfactorily by the surgeon at the time of operation (Bunnell 1970). R e c u r r e n c e and Survival
The malignant synoviomas in the hand tend to recur locally and metastatise readily through the blood or lymph nodes (Bunnell). Metastases, other than to the regional lymph nodes occur most frequently to the lungs but also involve the bones. A five year survival rate is given for the synovial sarcomas in general, but most authors believe it is not above 15-20 per cent (Weinreich 1963). A review of the literature on this turnout revealed only four cases of five year survivals, among 143 cases (Crampton 1952). Raben (1965) reported a five year survival rate of 50 per cent, using a combination of surgery and radiation therapy. The survival rate in the series of Hand and McFarland (1970) was up to three years. Haagensen and Stout give the information that one patient in which a simple biopsy was done followed shortly by primary amputation has been well for eight years. This is the longest survival on record following any form of treatment for synovial sarcoma. Our case has been followed for only one year at the moment and no metastases have been observed so far. 74
The Itand--Vol. 5
No. 1
1973
Sarcoma o[ Tendon Sheath--E. G. Fragiadakis attd P. Vatopoulos
SUMMARY
A case of synovial sarcoma of tendon sheath in the hand is reported. This has been the only case in the records of "Saint Paul" Orthopaedic and Accident Hospital within the last three years. T h e synovial sarcoma is a rare, highly specialised form of malignant neoplasm which develops in the hand. The tumour generally develops slowly, is more c o m m o n l y found in young adult males and finally metastatises through the blood stream and occasionally to the regional lymph nodes. Diagnosis should be always established by histological examination. Treatment should be radical. High amputation and possibly regional node dissection. R a d i o t h e r a p y has been little used and there is no proof that it is effective.
REFERENCES
BUNNELL, S. (1970). Surgery of the Hand. Revised by J. H. Boyes. Fifth Edition. Philadelphia, J. B. Lippincott Company 703-704. BUXTON, St. J. D. (1922). Tumours of Tendon and Tendon Sheaths. British Journal of Surgery 10: 469-474. CADMAN, N. L., SOULE, E. H., and KELLY, P. J. (1965). Synovial Sarcoma. An Analysis of 134 Tumors. Cancer, 18: 613427. CRAMPTON, R. F. (1952). Malignant Synovioma of the Hand. British Journal of Plastic Surgery 4: 56-62. HAAGENSEN, C. D. and STOUT, A. P. (1944). Synovial Sarcoma. Annals of Surgery. 120: 826-842. HAND, C. R. and McFARLAND, G. B. (1970). Synovial Sarcoma of the Hand. Journal of the Louisiana State Medical Society. Vol. 122: I-5. LAZARUS, J. A. and MARKS, M. S. (1943). Synovial Sarcoma: With Report of Two Cases. Surgery: 13, 290-308. LICHTENSTEIN, L. (1965). Bone Tumors. Third Edition. Saint Louis, The C. V. Mosby Company. 397--404. PACK, G. T. and ARIEL, I. M. (1964). Treatment of Cancer and Allied Diseases. Second Edition, New York, Harper and Row Publishers Inc. Vol. 8: 182-193. RABEN, M., CALABRESE, A., I-IIGINBOTHAM, N. L. and PHILIPS, R. (1965). Malignant Synovioma. American Journal of Roentgenology. 93: 145-153. TILLOTSON, J. F., McDONALD, J. R. and JANES, J. M. (1951). Synovial Sarcomata. Journal of Bone and Joint Surgery. 33-A: 459--473. WEINREICH, M. (1963). Clinical Picture of Malignant Synovioma. Zeitschrift fiir Orthopadie und ihre Grenzgebiete 97: 353. The H a n d - - V o l . 5
No. 1
1973
75