International
International Orthopaedics (SICOT) (1991 ) 15 : 251 - 255
Orthopaedics © Springer Verlag 1991
Synovial sarcoma: a clinicopathological review S. A. Henderson l, R. Davis 1, and J. R. Nixon 2 Department of Orthopaedics, Musgrave Park Hospital, Belfast BT9 7JB, Northern Ireland 2 Department of Pathology, Institute of Clinical Science, The Royal Victoria Hospital, Grosvenor Road, Belfast BT12 6BA, Northern Ireland
We have reviewed 20 cases o f synovial sarcoma. The main clinical and pathological features of these cases are outlined. Typically these turnouts arise in the leg in young adults with a long history before presentation and diagnosis. Clinical and radiological findings are unhelpful in making an early diagnosis. The five year survival rate in this series was 43%. The best guide to prognosis was assessment of mitotic activity. A high index o f suspicion is required if the diagnosis is not to be missed. Adequate wide excision o f the turnout is the mainstay o f treatment. Summary.
R/~sum6. La r&&ion de 20 cas de sarcome synovial
a permis de dbgager les principales caractbristiques cliniques et anatomopathologiques de cette affection. Dans les cas les plus typiques, ces tumeurs surviennent chez l'adulte jeune, au niveau des membres inf&ieurs. Elles bvoluent longtemps avant d'entrafner une symptomatologie et de pouvoir Otre diagnostiqubes. Les donnbes cliniques et radiologiques ne permettent pas un diagnostic prbcoce. Dans cette skrie le taux de survie d 5 ans est de 43%. Le meilleur blbment du pronostic est l'estimation de l'activitb mitotique. La conclusion est qu'il faut 6tre extrbmement mkfiant pour bviter de passer d c6tb du diagnostic. Une excision large de la tumeur, correetement effectube, reste la base du traitement.
Introduction S a r c o m a a r i s i n g f r o m s y n o v i a l t i s s u e w a s first d e s c r i b e d b y G u s t a v in 1865, b u t it w a s n o t u n t i l
Reprint request to: S. A. Henderson
1944 t h a t H a a g e n s o n a n d S t o u t first u s e d t h e t e r m S y n o v i a l S a r c o m a [13]. H i s t o l o g i c a l , s u r g i c a l a n d oncological concepts have evolved since, but the diagnosis, management, prognosis and pathology o f t h e s e l e s i o n s is still a m a t t e r f o r d e b a t e .
Material and methods The records of all patients with a diagnosis of synovial sarcoma seen in the Northern Ireland teaching hospitals in the period from January 1972 to December 1988 were examined. Tissue blocks were found for all cases and fresh sections were prepared. The diagnosis was confirmed and the tumours were classified as monophasic or biphasic [30] according to the following histological criteria. A monophasic synovial sarcoma (Fig. 1) consisted of sheets of spindle cells arranged in compact interdigitating fascicles with only a small amount of intervening stroma. The spindle cells were thin with scanty basophilic cytoplasm and a central hyperchromatic nucleus. Nuclear pleomorphism and nuclear atypia were not a prominent feature. The biphasic synovial sarcomas (Fig. 2) comprised epithelioid cells arranged as tubular and acinar structures and set within a uniform spindle cell sarcomatous background. The epithelioid cells were cuboidal with clear cytoplasm, a single nucleus and prominent nucleoli. Nuclear atypia was not a feature. Calcification was noted when present. It is a useful distinguishing feature of synovial sarcoma as it is almost never seen in fibrosarcoma. The tumours were also assessed for mitotic activity.
Clinical findings T h e r e w e r e 20 c a s e s o f S y n o v i a l S a r c o m a , 12 m a l e s a n d 8 f e m a l e s . T h e m e a n a g e w a s 35 y r s ( r a n g e 1 5 - 7 3 yrs). T h e m e a n d u r a t i o n o f s y m p t o m s b e f o r e d e f i n i t i v e t r e a t m e n t w a s 21 m o n t h s ( r a n g e 2 m o n t h s to 8 y e a r s ) . A l l t h e p a t i e n t s d e v e l oped a swelling, although several originally presented with pain alone. The preoperative diagn o s e s a r e s h o w n in T a b l e 1. T h e t u m o u r s w e r e s i t e d as i l l u s t r a t e d in T a b l e 2. F o l l o w i n g h i s t o l o g i cal d i a g n o s i s , d e f i n i t i v e t r e a t m e n t w a s p l a n n e d .
S. A. Henderson et al.: Synovialsarcoma
252
Fig. 1. Classic biphasic pattern showing epithelial and spindle cell components. (H + E x 56) Fig. 2. Monophasic spindle cell synovial sarcoma. (H + E × 85)
Twelve patients underwent amputation of all or part of a limb, 5 had wide local excision of the tumour, 2 had local biopsy and following histological diagnosis refused definitive treatment, and 1 had local excision of the tumour followed by a course of chemotherapy. Twelve patients developed metastases. The chest was the most common site (9 patients); other sites included regional lymph nodes (3), brain (1) and bone (1). Fourteen patients had adjuvant therapy. Ten had one or more courses of chemotherapy usually with a combination of cyclophosphamide, doxorubicin and vincristine, 4 underwent radiotherapy and 6 had no additional treatment.
Pathology Tumours ranged in size from 0.5 cm to 18 cm. Twelve were more than 5 cm in diameter at the time of operation. The larger masses were whitish in colour with varying degrees of haemorrhage and necrosis. They were poorly circumscribed, not encapsulated and growing around nerves, blood vessels and skeletal muscle. In 4 cases the tumour was invading underlying bone. The classic biphasic pattern was found in 6 cases (Fig. 2). In 5 of these the spindle cell component predominated. One case was monophasic epithelioid and 13 were monophasic spindle cell sarcomas (Fig. 1). Mitotic activity within the tumours was variable. Ten cases had low activity (0-2 per high
S. A. Henderson et al.: Synovial sarcoma
253
Table 1. Benign lesions diagnosed in cases of synovial sarcoma Neuroma (2) Lipoma (2) Trauma O s t e o a r t h r i t i s - Knee Muscle Hernia Ganglion Inguinal Hernia Bakers Cyst Gluteal Bursa Gout
Table 2. Anatomical sites of synovial sarcoma
Arm Shoulder Elbow Forearm Wrist Hand
Leg 1 1 1 2 1
Hip/Pelvis Thigh Knee Ankle Foot
3 5 2 2 2
Table 3. 5 year survival rate in synovial sarcoma Haagenson and Stout Pack and Ariel Wright C a d m a n et al. Mackenzie Cameron and Kostuik Buck et al. Wright et al. Zito et al. H e d e r s o n et al.
(1944) (1950) (1952) (1964) (1966) (1974) (1981) (1982) (1984) (1990)
104 60 47 134 49 39 33 185 48 20
cases cases cases cases cases cases cases cases cases cases
3% 13 % 13 % 25 % 51% 45% 36% 55 % 50% 43 %
Table 4. Prognosis following a diagnosis of synovial sarcoma
Dead - average survival 28 raonths Long term survivors ( > 5 years) Short term survivors ( < 5 years)
:8 :6 :6
power field) and 7 had high activity (75). Necrosis was present in 7 tumours and it was not related to size, site or mitotic activity. Calcification was found in 6 tumours including 3 of the 4 present in the thigh.
Prognosis The 5 year survival rate was 43%, which compares favourably with other published results (Table 3). The patients were split into three groups according to clinical outcome (Table 4). Age, sex, duration of symptoms, tumour size and site, the use of adjuvant chemotherapy or radiotherapy and his-
tological differentiation into mono or biphasic types had no influence on outcome. Radical surgery was associated with prolonged survival. Fourteen patients had their tumours diagnosed 5 or more years prior to this review. Six survived for more than five years, four of whom had a major proximal limb amputation, but of the 8 who died only 2 had a similar operation. Eleven of the 12patients with metastases died from their disease. The mean survival of this group was 10 months from the presentation of the metastases. There was a strong relationship between mitotic rate and survival. Five of the long term survivor group had tumours with a low mitotic activity. In comparison, 4 out of 8 patients who died had tumours which demonstrated a high mitotic rate.
Discussion This series of 20 cases of synovial sarcoma presented over a 16 year period. It remains an aggressive malignant tumour with a poor prognosis and difficulty in making the diagnosis before operation. The delay from onset of symptoms to accurate tissue diagnosis was 21 months, an improvement from the 2.8 years reported previously [27]. As in other studies [2, 15] many of these tumours were initially diagnosed and treated as clinically benign swellings (Table 1). The low level of awareness of this type of soft tissue tumour was confirmed by the significant proportion of large tumours (~5 cm max diameter) treated. None of our patients was diagnosed as having a potentially malignant soft tissue sarcoma before operation. Plain x-ray [7], bone scintigraphy [20], CT scanning [3, 21], and magnetic resonance imaging [26] may be of help in making the diagnosis. However, the main role of these investigations is in preoperative planning of the optimal surgical approach to remove the tumour [27, 30].
Pathology tn 1965 Cadman described a monophasic variant consisting of spindle cells alone and these are now recognised as being quite common [14]. Recently, ultrastructural and histochemical techniques [1] have l e d to a reappraisal of the histogenesis of synovial sarcoma. It has been suggested that rather than being a mesenchymal tumour it may be a true carcinosarcoma of the connective tissue [11, 18]. Clinical and pathological characteristics of prognostic importance have been identified. Large tumours have a poor prog-
254
nosis [30, 31]. The importance of adequate initial surgery [9] has been emphasised [4, 8, 22, 30]. Other important variables include age [30], sex, site [4, 5], duration of symptoms [7] and the presence of metastases [30]. The relationship between the histological features and the ultimate outcome is less clear. Some studies have shown that monophasic tumours have a poor prognosis [4, 6, 22, 31] while others have reported series in which biphasic lesions do less well [28]. We found no difference in outcome between the two types. It is generally agreed that lesions with a high mitotic rate have a worse outcome than those with a low rate [6, 8, 31], and our series confirms this.
S. A. Henderson et al.: Synovial sarcoma
2.
3.
4. 5. 6.
7. 8.
Treatment
As the management of synovial sarcoma has become more sophisticated the prognosis has improved. In the absence of metastases local control determines the final outcome [24]. Radical surgical intervention in the form of amputation or wide en bloc compartmental excision remains the main principle of treatment [9, 19, 29]. Radiotherapy has given indifferent results [4, 5, 7, 10, 16]. Adjuvant chemotherapy for patients with high grade soft tissue malignancies, such as synovial sarcoma, remains to be fully assessed, but promising results have been reported [8, 25]. Metastases are common [19, 30]. The majority occur in the lung. The mean time from diagnosis of metastases to death is 10 months [19]. Shiu advocates surgical resection of isolated pulmonary metastases. In our series 12 had evidence of metastatic disease, 9 of these were pulmonary and all died soon after diagnosis.
9.
10.
11. 12.
13. 14.
15.
16.
Conclusions
This small series emphasises the important features of synovial sarcoma. It continues to be an aggressive malignant tumour with a poor prognosis. It typically affects the leg in young adults, with a long history before presentation and definitive diagnosis. A high index of suspicion is required if the diagnosis is not to be missed. The clinical findings and radiographs are generally unhelpful. The best guide to prognosis is assessment of mitotic activity. Adequate wide excision of the tumour remains the mainstay of treatment. Large tumours treated with adequate surgery have the potential for long term survival.
17.
18. 19.
20.
21.
22.
References 1. Abenoza P, Manivel JC, Swanson PE, Wick MR (1986) Synovial Sarcoma: ultrastructural study and immunohistochemical analysis by a combined peroxidase - antiper-
23.
oxidase/avidin - biotin - peroxidase complex procedure. Hum Pathol 17 (11): 1107-1115 Bogumil GP, Bruno PD, Barrick EF (1981) Malignant lesions masquerading as popliteal cysts. J Bone Joint Surg [Am] 63:474-477 Boriani S, Torricelli P, Ruggieri P, Biagini R (1985) Valore e limiti della tomografia assiale computerizzata nello studio diagnostico del sarcoma sinoviale. Chir Org Mov LXX: 149-156 Buck P, Mickelson MR, Bonfiglio M (1981) Synovial Sarcoma - A review of 33 cases. Clin Orthop 156:211-215 Cadman NL, Soule EH, Kelly PJ (1965) Synovial Sarcoma. An analysis of 134 tumours. Cancer 18:613-617 Cagle LA, Mirra LM, Storm K, Roe DJ, Eilber FR (1987) Histologic features relating to prognosis in synovial sarcoma. Cancer 59: 1810-1814 Cameron HU, Kostuik JP (1974) A long term followup of Synovial sarcoma. J Bone Joint Surg [Br] 56:613-617 Collin CF, Friedrich C, Godbold J, Hajdu S, Brennan MF (1988) Prognostic factors for local recurrence and survival in patients with localized extremity soft tissue sarcoma. Semin Surgical Oncol 4:30-37 Enneking WF, Spanier SS, Goodman MA (1980) A system for the Surgical Staging of musculoskeletal sarcomas. Clin Orthop 153: 106-120 Fletcher GH, Lindberg RD, Tapley N duV, Guillamondegui OM, Byers RM, Perez CA, Gunderson LL (1976) Emerging roles of radiation therapy in four selected areas: soft tissue sarcoma, salivary glands, prostate, rectal and sigmoid carcinoma. Curr Probl Cancer 1: 4-13 Ghadially FN (1987) Is Synovial Sarcoma a Carcinosarcoma of connective tissue. Ult Path 11 : 147-151 Gustav S (1865) Extirpation einer sehr grogen, mit dickem Stiele angewachsenen Kniegelenkmaus mit gliicklichem Erfolge. Arch Klin Chir 6:573-576 Haagenson CD, Stout AP (1944) Synovial sarcoma. Ann Surg 120:826-842 Hajdu SI, Shiu MH, Fortner JG (1977) Tendosynovial sarcoma, a clinicopathological study of 136 cases. Cancer 39:1201-1217 Ichinose H, Wickstrom JK, Hoerner HE, Derbes VL (1979) The early clinical presentation of synovial sarcoma. Clin Orthop 142: 185-189 Kagan AR, Friedman NB, Jaffe HL (1971) Synovial sarcoma: an analysis of its response to radiation. J Surg Oncol 3:379-385 Kindblom LG, Walaas L, Widehn S (1987) Ultrastructural studies in the preoperative cytologic diagnosis of soft tissue tumours. Semin Diagnost Pathol 3:317-344 Leader M, Patel J, Collins M, Kristin H (1987) Synovial sarcomas- true Carcinosarcomas ? Cancer 59:2096-2098 Ryan JR, Baker LH, Benjamin RS (1982) The natural history of metastatic synovial sarcoma. Clin Orthop 164: 257-260 Schaub T, Schild H, Werner B, Hahn K, Thelen M (1986) Diagnostik und Verlauf des Synovialsarkoms. Ein Vergleich yon Roentgendiagnostik und Skelettszintigraphie. Fortschr Roentgenstr 144:453-459 Schauer A (1987) Morphologische Diagnostik maligner Weichgewebstumoren - Aspekte der RadikalitS.t aus der Sicht des Pathologen. Langenbecks Arch Chir 372: 281-287 Shiu MH, McCormack PM, Hajdu S, Fortner JG (1979) Surgical treatment of tendosynovial sarcoma. Cancer 43: 889-897 Sim FH, Pritchard D J, Reimann HM, Edmonson JH, Schray MF (1988) Soft tissue sarcoma: Mayo clinic experience. Semin Surg Oncol 4:38-44
S. A. Henderson et al.: Synovial sarcoma 24. Simon MA, Enneking WF (1976) The management of soft tissue sarcomas of the extremities. J Bone Joint Surg 58 [Am]: 317-327 25. Stephens FO, Tattersall MHN, Marsden W, Waugh RC, Green D, McCarthy SW (1987) Regional chemotherapy with the use of cisplatin and doxorubicin as primary treatment for advanced sarcomas in shoulder, pelvis, and thigh. Cancer 60:724-735 26. Sundaram M, McGuire MH, Fletcher J, Wolverson MK, Heiberg E, Sheilds JB (1986) Magnetic resonance imaging of lesions of synovial origen. Skeletal Radiol 15 : 110-116
255 27. Tillotson JF, McDonald JR, Janes JM (1951) Synovial sarcomata. J Bone Joint Surg [Am] 33:459-473 28. Tsuneyoshi M, Yokoyama K, Enjoji M (1983) Synovial sarcoma. A clinicopathologic and ultrastructural study of 42 cases. Acta Pathol Jpn 33:23-36 29. Watts HG (1980) Introduction to resection of musculoskeletal sarcomas. Clin Orthop 153:31-38 30. Wright PH, Sire FH, Soule EH, Taylor WF (1982) Synovial Sarcoma. J Bone Joint Surg 64 [Am]: 112-122 31. Zito RA (1984) Synovial sarcoma: An Australian series of 48 cases. Pathology 16:45-52
International Orthopaedics (SICOT) (1991 ) 15 : 251 - 255
Orthopaedics © Springer Verlag 1991
Synovial sarcoma: a clinicopathological review S. A. Henderson l, R. Davis 1, and J. R. Nixon 2 Department of Orthopaedics, Musgrave Park Hospital, Belfast BT9 7JB, Northern Ireland 2 Department of Pathology, Institute of Clinical Science, The Royal Victoria Hospital, Grosvenor Road, Belfast BT12 6BA, Northern Ireland
We have reviewed 20 cases o f synovial sarcoma. The main clinical and pathological features of these cases are outlined. Typically these turnouts arise in the leg in young adults with a long history before presentation and diagnosis. Clinical and radiological findings are unhelpful in making an early diagnosis. The five year survival rate in this series was 43%. The best guide to prognosis was assessment of mitotic activity. A high index o f suspicion is required if the diagnosis is not to be missed. Adequate wide excision o f the turnout is the mainstay o f treatment. Summary.
R/~sum6. La r&&ion de 20 cas de sarcome synovial
a permis de dbgager les principales caractbristiques cliniques et anatomopathologiques de cette affection. Dans les cas les plus typiques, ces tumeurs surviennent chez l'adulte jeune, au niveau des membres inf&ieurs. Elles bvoluent longtemps avant d'entrafner une symptomatologie et de pouvoir Otre diagnostiqubes. Les donnbes cliniques et radiologiques ne permettent pas un diagnostic prbcoce. Dans cette skrie le taux de survie d 5 ans est de 43%. Le meilleur blbment du pronostic est l'estimation de l'activitb mitotique. La conclusion est qu'il faut 6tre extrbmement mkfiant pour bviter de passer d c6tb du diagnostic. Une excision large de la tumeur, correetement effectube, reste la base du traitement.
Introduction S a r c o m a a r i s i n g f r o m s y n o v i a l t i s s u e w a s first d e s c r i b e d b y G u s t a v in 1865, b u t it w a s n o t u n t i l
Reprint request to: S. A. Henderson
1944 t h a t H a a g e n s o n a n d S t o u t first u s e d t h e t e r m S y n o v i a l S a r c o m a [13]. H i s t o l o g i c a l , s u r g i c a l a n d oncological concepts have evolved since, but the diagnosis, management, prognosis and pathology o f t h e s e l e s i o n s is still a m a t t e r f o r d e b a t e .
Material and methods The records of all patients with a diagnosis of synovial sarcoma seen in the Northern Ireland teaching hospitals in the period from January 1972 to December 1988 were examined. Tissue blocks were found for all cases and fresh sections were prepared. The diagnosis was confirmed and the tumours were classified as monophasic or biphasic [30] according to the following histological criteria. A monophasic synovial sarcoma (Fig. 1) consisted of sheets of spindle cells arranged in compact interdigitating fascicles with only a small amount of intervening stroma. The spindle cells were thin with scanty basophilic cytoplasm and a central hyperchromatic nucleus. Nuclear pleomorphism and nuclear atypia were not a prominent feature. The biphasic synovial sarcomas (Fig. 2) comprised epithelioid cells arranged as tubular and acinar structures and set within a uniform spindle cell sarcomatous background. The epithelioid cells were cuboidal with clear cytoplasm, a single nucleus and prominent nucleoli. Nuclear atypia was not a feature. Calcification was noted when present. It is a useful distinguishing feature of synovial sarcoma as it is almost never seen in fibrosarcoma. The tumours were also assessed for mitotic activity.
Clinical findings T h e r e w e r e 20 c a s e s o f S y n o v i a l S a r c o m a , 12 m a l e s a n d 8 f e m a l e s . T h e m e a n a g e w a s 35 y r s ( r a n g e 1 5 - 7 3 yrs). T h e m e a n d u r a t i o n o f s y m p t o m s b e f o r e d e f i n i t i v e t r e a t m e n t w a s 21 m o n t h s ( r a n g e 2 m o n t h s to 8 y e a r s ) . A l l t h e p a t i e n t s d e v e l oped a swelling, although several originally presented with pain alone. The preoperative diagn o s e s a r e s h o w n in T a b l e 1. T h e t u m o u r s w e r e s i t e d as i l l u s t r a t e d in T a b l e 2. F o l l o w i n g h i s t o l o g i cal d i a g n o s i s , d e f i n i t i v e t r e a t m e n t w a s p l a n n e d .
S. A. Henderson et al.: Synovialsarcoma
252
Fig. 1. Classic biphasic pattern showing epithelial and spindle cell components. (H + E x 56) Fig. 2. Monophasic spindle cell synovial sarcoma. (H + E × 85)
Twelve patients underwent amputation of all or part of a limb, 5 had wide local excision of the tumour, 2 had local biopsy and following histological diagnosis refused definitive treatment, and 1 had local excision of the tumour followed by a course of chemotherapy. Twelve patients developed metastases. The chest was the most common site (9 patients); other sites included regional lymph nodes (3), brain (1) and bone (1). Fourteen patients had adjuvant therapy. Ten had one or more courses of chemotherapy usually with a combination of cyclophosphamide, doxorubicin and vincristine, 4 underwent radiotherapy and 6 had no additional treatment.
Pathology Tumours ranged in size from 0.5 cm to 18 cm. Twelve were more than 5 cm in diameter at the time of operation. The larger masses were whitish in colour with varying degrees of haemorrhage and necrosis. They were poorly circumscribed, not encapsulated and growing around nerves, blood vessels and skeletal muscle. In 4 cases the tumour was invading underlying bone. The classic biphasic pattern was found in 6 cases (Fig. 2). In 5 of these the spindle cell component predominated. One case was monophasic epithelioid and 13 were monophasic spindle cell sarcomas (Fig. 1). Mitotic activity within the tumours was variable. Ten cases had low activity (0-2 per high
S. A. Henderson et al.: Synovial sarcoma
253
Table 1. Benign lesions diagnosed in cases of synovial sarcoma Neuroma (2) Lipoma (2) Trauma O s t e o a r t h r i t i s - Knee Muscle Hernia Ganglion Inguinal Hernia Bakers Cyst Gluteal Bursa Gout
Table 2. Anatomical sites of synovial sarcoma
Arm Shoulder Elbow Forearm Wrist Hand
Leg 1 1 1 2 1
Hip/Pelvis Thigh Knee Ankle Foot
3 5 2 2 2
Table 3. 5 year survival rate in synovial sarcoma Haagenson and Stout Pack and Ariel Wright C a d m a n et al. Mackenzie Cameron and Kostuik Buck et al. Wright et al. Zito et al. H e d e r s o n et al.
(1944) (1950) (1952) (1964) (1966) (1974) (1981) (1982) (1984) (1990)
104 60 47 134 49 39 33 185 48 20
cases cases cases cases cases cases cases cases cases cases
3% 13 % 13 % 25 % 51% 45% 36% 55 % 50% 43 %
Table 4. Prognosis following a diagnosis of synovial sarcoma
Dead - average survival 28 raonths Long term survivors ( > 5 years) Short term survivors ( < 5 years)
:8 :6 :6
power field) and 7 had high activity (75). Necrosis was present in 7 tumours and it was not related to size, site or mitotic activity. Calcification was found in 6 tumours including 3 of the 4 present in the thigh.
Prognosis The 5 year survival rate was 43%, which compares favourably with other published results (Table 3). The patients were split into three groups according to clinical outcome (Table 4). Age, sex, duration of symptoms, tumour size and site, the use of adjuvant chemotherapy or radiotherapy and his-
tological differentiation into mono or biphasic types had no influence on outcome. Radical surgery was associated with prolonged survival. Fourteen patients had their tumours diagnosed 5 or more years prior to this review. Six survived for more than five years, four of whom had a major proximal limb amputation, but of the 8 who died only 2 had a similar operation. Eleven of the 12patients with metastases died from their disease. The mean survival of this group was 10 months from the presentation of the metastases. There was a strong relationship between mitotic rate and survival. Five of the long term survivor group had tumours with a low mitotic activity. In comparison, 4 out of 8 patients who died had tumours which demonstrated a high mitotic rate.
Discussion This series of 20 cases of synovial sarcoma presented over a 16 year period. It remains an aggressive malignant tumour with a poor prognosis and difficulty in making the diagnosis before operation. The delay from onset of symptoms to accurate tissue diagnosis was 21 months, an improvement from the 2.8 years reported previously [27]. As in other studies [2, 15] many of these tumours were initially diagnosed and treated as clinically benign swellings (Table 1). The low level of awareness of this type of soft tissue tumour was confirmed by the significant proportion of large tumours (~5 cm max diameter) treated. None of our patients was diagnosed as having a potentially malignant soft tissue sarcoma before operation. Plain x-ray [7], bone scintigraphy [20], CT scanning [3, 21], and magnetic resonance imaging [26] may be of help in making the diagnosis. However, the main role of these investigations is in preoperative planning of the optimal surgical approach to remove the tumour [27, 30].
Pathology tn 1965 Cadman described a monophasic variant consisting of spindle cells alone and these are now recognised as being quite common [14]. Recently, ultrastructural and histochemical techniques [1] have l e d to a reappraisal of the histogenesis of synovial sarcoma. It has been suggested that rather than being a mesenchymal tumour it may be a true carcinosarcoma of the connective tissue [11, 18]. Clinical and pathological characteristics of prognostic importance have been identified. Large tumours have a poor prog-
254
nosis [30, 31]. The importance of adequate initial surgery [9] has been emphasised [4, 8, 22, 30]. Other important variables include age [30], sex, site [4, 5], duration of symptoms [7] and the presence of metastases [30]. The relationship between the histological features and the ultimate outcome is less clear. Some studies have shown that monophasic tumours have a poor prognosis [4, 6, 22, 31] while others have reported series in which biphasic lesions do less well [28]. We found no difference in outcome between the two types. It is generally agreed that lesions with a high mitotic rate have a worse outcome than those with a low rate [6, 8, 31], and our series confirms this.
S. A. Henderson et al.: Synovial sarcoma
2.
3.
4. 5. 6.
7. 8.
Treatment
As the management of synovial sarcoma has become more sophisticated the prognosis has improved. In the absence of metastases local control determines the final outcome [24]. Radical surgical intervention in the form of amputation or wide en bloc compartmental excision remains the main principle of treatment [9, 19, 29]. Radiotherapy has given indifferent results [4, 5, 7, 10, 16]. Adjuvant chemotherapy for patients with high grade soft tissue malignancies, such as synovial sarcoma, remains to be fully assessed, but promising results have been reported [8, 25]. Metastases are common [19, 30]. The majority occur in the lung. The mean time from diagnosis of metastases to death is 10 months [19]. Shiu advocates surgical resection of isolated pulmonary metastases. In our series 12 had evidence of metastatic disease, 9 of these were pulmonary and all died soon after diagnosis.
9.
10.
11. 12.
13. 14.
15.
16.
Conclusions
This small series emphasises the important features of synovial sarcoma. It continues to be an aggressive malignant tumour with a poor prognosis. It typically affects the leg in young adults, with a long history before presentation and definitive diagnosis. A high index of suspicion is required if the diagnosis is not to be missed. The clinical findings and radiographs are generally unhelpful. The best guide to prognosis is assessment of mitotic activity. Adequate wide excision of the tumour remains the mainstay of treatment. Large tumours treated with adequate surgery have the potential for long term survival.
17.
18. 19.
20.
21.
22.
References 1. Abenoza P, Manivel JC, Swanson PE, Wick MR (1986) Synovial Sarcoma: ultrastructural study and immunohistochemical analysis by a combined peroxidase - antiper-
23.
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