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Synovial Chondrosarcoma Arising in Synovial Chondromatosis

Synovial Chondrosarcoma Arising in Synovial Chondromatosis Hindawi Publishing Corporation Sarcoma Volume 2014, Article ID 647939, 4 pages http://dx.doi.org/10.1155/2014/647939 Research Article Scott Evans, Michele Boffano, Samena Chaudhry, Lee Jeys, and Robert Grimer Royal Orthopaedic Hospital, Birmingham B31 2AP, UK Correspondence should be addressed to Scott Evans; drscottevans@yahoo.co.uk Received 27 November 2013; Accepted 30 January 2014; Published 5 March 2014 Academic Editor: Jos Bramer Copyright © 2014 Scott Evans et al. This is an open access article distributed under the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited. Primary synovial chondromatosis (SC) is a rare proliferative disorder that causes pain, swelling, and restriction of movement to the joints it aeff cts. The disease frequently runs a protracted course, oeft n requiring multiple surgical procedures to obtain some control. Few reports exist detailing the natural history of SC, although malignant transformation to synovial chondrosarcoma (CHS) is recognized to be a rare event. eTh aim of our study was to review a large orthopaedic oncology database in order to evaluate the incidence of CHS arising from SC. We identified 78 patients who have presented to our centre with primary synovial chondromatosis (SC). Of those patients, 5 went on to develop malignant change. This represents a 6.4% incidence of developing synovial chondrosarcoma (CHS) within preexisting primary synovial chondromatosis. eTh patients had a mean age of 28 years at first diagnosis with synovial chondromatosis with the median time from original diagnosis to malignant transformation being 20 years (range 2.7–39 yrs). 1. Introduction correlating histological and radiological findings to correctly diagnose the benign entity. Synovial chondromatosis is a rare proliferative, metaplastic Malignant transformation of primary synovial chondro- disorder of the synovium [1]. The disease was first described matosis to synovial chondrosarcoma is recognized to be a rare by Leannac in 1813 [2]; however, its current description was event with reports estimating the incidence to be in the range of 1–5% [12–14]. not applied until 1958 by Jaffe [ 3]. The exact prevalence of The aim of our study was to use a large database of primary synovial chondromatosis (SC) is unknown but it patients with orthopaedic oncology to evaluate the incidence usually aeff cts the third to ftfih decades of life [ 4], with of chondrosarcoma arising from primary synovial chon- men being affected two to four times more frequently than drosarcoma and to review the time taken for the benign entity women [5]. The knee is the most commonly aeff cted large to transform into its malignant counterpart. jointfollowedbyhip,shoulder,elbow,ankle,and wrist[6]. Patient’s clinical symptoms typically include pain, swelling, andrestrictedjoint movement [7]. The symptoms are oeft n insidious at disease onset and are gradually progressive, 2. Materials and Methods although rare spontaneous regression has been reported [8]. Patients oen ft have a protracted length of symptoms prior to We conducted a retrospective search of a prospective tumour diagnosis, with an average of 5 years [9, 10]. database to identify all patients treated at our unit with Three phases of SC have been described [ 11]; phase 1 the diagnosis of primary synovial chondromatosis (SC). eTh active intrasynovial disease with nodules but no calcifica- diagnosis was made under the auspices of a supraregional tions/intraarticular bodies; phase 2 synovitis with osteochon- multidisciplinary bone tumour unit. Patient demographics dral nodules in the synovial membrane and loose bodies were recorded along with the site of primary disease. From within the joint; phase 3 multiple loose bodies remain but this initial search, details of those patients who subsequently synovitis is quiescent. This highlights the importance of developed synovial chondrosarcoma (CHS) were collected. 2 Sarcoma Table 1: Details of the patients, their tumours, and the outcome. Age at Time to Surgical diagnosis malignant Case Site Treatment(s) for SC Malignancy management of Mets Outcome with SC change chondrosarcoma (years) (years) Hindquarter Alive 13 yrs 23 Hip 3 debridements 2.7 Grade I CHS — amputation postoperatively Dedifferentiated (i) Initially symptomatic At chondrosar- Died 3 yrs 48 Hip (ii) THR 11 yrs aer ft initial 11.3 Debulking presentation coma postoperatively diagnosis (lung) (osteosarcoma) (i) 2 arthroscopies At Died 4 yrs (ii)1hip resurfacing 17yrs 25 Hip 20 Grade II CHS Debulking presentation postoperatively aer ft diagnosis (lung) (i) Multiple debridements (ii) THR 2005 Hindquarter Alive 9 months 25 Hip 39 Grade I CHS — (iii) Large recurrence 5 amputation postoperatively years after THR Lung—34 (i) Multiple arthroscopies Above knee Died 4 yrs 5 19 Knee 38.5 Grade II CHS months aer ft (ii) TKR aged 55 amputation postoperatively amputation 3. Results an abovekneeamputationaslimbsalvage wasnot possible. Histology revealed a grade II synovial chondrosarcoma. He A search of our database, which holds prospectively gathered developed lung metastases 34 months postoperatively and data on over 30,000 patients including over 3800 primary subsequently died 4 years aeft r the amputation. bone sarcomas,identiefi d78patientswithSC. Allhad As with other chondrsarcomas, radio/chemotherapy are been diagnosed following analysis of their radiological and not typically effective treatments and, therefore, our cases did histological findings between 1980 and 2011. There were 33 not receive adjuvant therapy. In summary, 78 patients were females (42.3%) and 45 males (57.7%) with a mean age at identified with SC, with 5 developing CHS. This represents presentation with SC of 28 years. The primary site of SC was transformation to malignancy in 6.4% of cases. Furthermore, the knee in 30 patients, hip in 22, hand in 7, shoulder in 6, it is apparent from performing Kaplan-Meier survival analy- elbowin5,footin5,wrist in 2, andthe anklein1. sis that the risk of malignant transformation increases as time The median time from original diagnosis with SC to elapses (see Figure 3). malignant transformation was 20 years (range 2.5–39 years). All patients had undergone multiple procedures in an attempt to control their SC prior to the diagnosis of malignancy. 4. Discussion All cases of CHS were diagnosed aer ft careful discussion at a multidisciplinary team meeting involving orthopaedic Primarysynovialchondromatosisisarare disease. Itstrue oncologist, histopathologist, and radiologist (Table 1). incidence is unknown. The incidence of chondrosarcoma in One of the main problems in diagnosing malignancy England is however known and is reported to be 1.8/million in SC is that biopsies are oeft n not helpful and clinical population per year [15]. Our unit has treated 800 patients features oen ft suggest malignancy more than histology. In two with a chondrosarcoma in the same time period as we patients (case 1 + 4), repeated biopsy demonstrated benign treated these 78 primary synovial chondromatosis patients. disease, whilst their imaging studies revealed likely extensive This would suggest that for every 800 chondrosarcomas primary synovial chondromatosis. However, it was clear from diagnosed, 5originatedinsynovialchondromatosis; that is, the patients’ level of nonmechanical pain in combination with 0.6% of chondrosarcomas are linked to primary synovial the rapid and repeated progression of their tumours aer ft chondromatosis. As the incidence of chondrosarcoma in debulking that they had aggressive disease. The patients were England is known, we would expect to see approximately included in a pragmatic discussion and the decision was made 95 new cases of chondrosarcoma every year. Therefore, to perform hind-quarter amputation. In both cases, the actual every year, 0.57 cases of chondrosarcomas are associated diagnosis of malignancy was only confirmed when the whole with preexistingsynovialchondromatosis; that is,every 1.7 tumour had been sampled (see Figures 1 and 2). years (20.4 months), 1 new case of chondrosarcoma can be Two cases (patients 2 and 3) had large tumours with lung attributed to primary synovial chondromatosis. metastasesbythetimeofdiagnosisandthereforeweretreated Even though our unit is a supra-regional referral unit, it symptomatically. Both patients subsequently died with chest is unlikely that we were referred all patients with synovial metastases 3 and 4 years, respectively, aer ft diagnosis. Our chondromatosis over this time period. If we had been then the one patient with CHS aeff cting the knee (case 5) underwent incidence of primary synovial chondromatosis would be 78/5 Sarcoma 3 1.0 0.8 0.6 0.4 Figure 1: Plain X-ray of case 4 preoperatively. 0.2 0.0 010 20 30 40 Time (years) Malignant transformation Figure 3: Kaplan-Meier survival (malignant transformation = end- point). actually they are typical of benign primary synovial chondro- matosis [22–24]. Immunohistochemical markers to reliably Figure 2: STIR MRI of case 4 preoperatively. differentiate between primary synovial chondromatosis and low grade chondrosarcoma would be of great value but, to date, only preliminary studies have been performed [25]with no definitive immunostaining technique being identified as of that of chondrosarcoma in primary synovial chondromato- the gold-standard. sis; that is, 0.06/million per yr, suggesting that there may be The local recurrence of primary synovial chondromatosis 10 new cases of primary synovial chondromatosis diagnosed hasbeenreportedtobeashighas23% [14] despite adequate ayearinEngland.Weknownoway of conrfi mingthis. surgical debridement. The difficulty lies with identifying Although primary synovial chondromatosis can be those recurrences that are benign. locallyaggressivewithatendency to recur, it hasno In conclusion, we view multiple recurrences with the metastatic potential. eTh treatment for symptomatic primary development of marrow invasion as highly suspicious of synovial chondromatosis consists of removal of the cartilagi- malignant transformation. From our study, it is clear that not nous bodies with or without partial synovectomy. In contrast, all patients with primary synovial chondromatosis require chondrosarcoma is a malignant condition requiring more long-term surveillance to monitor for the development of invasive surgery in the form of wide or radical resection or malignant change; however, we recommend that any rapid amputation [16] and has a reported incidence of metastases deterioration in the patient’s clinical course, including wors- of up to 29% [17, 18]. ening pain or aggressive recurrence, should be regarded as Clearly because of the differences in prognosis and treat- suspicious and treated at, or referred appropriately to, a ment, it is vitally important to distinguish the benign primary tertiary hospital familiar with managing these often complex synovial chondromatosis from the malignant chondrosar- cases. coma. In practice, this can be difficult as both conditions have similar patterns of disease presentation with pain, swelling, and restricted joint movement. Numerous authors Conflict of Interests have found clinical and radiological criteria unhelpful to differentiate between the two disease processes as there eTh authors declare that there is no conflict of interests is oen ft significant overlap with no definitive features to regarding the publication of this paper. separate the two [1, 14, 18–20]. Histologically primary synovial chondromatosis reveals References lobules of hyaline cartilage that are oen ft hypercellular with atypical histological features, including multinucleation, [1] B. Wittkop, A. M. Davies, and D. C. Mangham, “Primary syn- nuclear crowding, nuclear enlargement, and hyperchromasia ovial chondromatosis and synovial chondrosarcoma,” European with mild myxoid changes often with a variable degree Radiology,vol.12, no.8,pp. 2112–2119, 2002. of synovial proliferation or hyperplasia [21]. These atypical [2] J.M.Crotty, J. U. V. Monu,and T. L. Pope Jr., “Synovial features could suggest a malignant neoplasm (grade I or II osteochondromatosis,” Radiologic Clinics of North America,vol. chondrosarcoma) to the less experienced pathologist when 34,no. 2, pp.327–342,1996. Malignant transformation 4 Sarcoma [3] H. L. Jaeff , Tumours and Tumourous Conditions of the Bones and [22] D. Resnik, “Tumours and tumour-like lesions of soft tissues,” in Joints, Kimpton, London, UK, 1958. Diagnosis of Bone and Joint Disorders, pp. 4204–4273, Saunders, Philadelphia, Pa, USA, 4th edition, 2002. [4] “Synovial chondromatosis,” in Pathophysiology of Orthopaedic Diseases,H.J.Mankin, Ed., pp.53–58,TheAmericanAcademy [23] K. K. Unni, C. Y. Inwards, J. A. Bridge, L. G. Kindbolm, and L. of Orthopaedic Surgeons, Rosemont, Ill, USA, 2006. E. Wold, “Synovial tumours,” in Tumours of the Bone and Joints, pp. 386–432, ARP Press, Silver Spring, Md, USA, 4th edition, [5] M.D.Darfamand B. Czerniak,“Synovial lesions,”in Bone Tumours, pp. 1041–1086, Mosby, St. Louis, Mo, USA, 1998. [24] S. W. Weiss and J. R. Goldblum, “Cartilagenous soft tissue [6] G. Hermann, M. J. Klein, I. F. Abdelwahab, and S. Kenan, tumours,” in Enzinger and Weiss’ Soft Tissue Tumours ,pp. 1368– 1388, Mosby, Philadelphia, Pa, USA, 4th edition, 2001. of the right hip,” Skeletal Radiology,vol.26, no.6,pp. 366–369, [25] B. L. Sperling,S.Angel,G.Stoneham, V. Chow,A.Mcfadden, and R. Chibbar, “Synovial chondromatosis and chondrosar- [7] F.P.Murphy, D. C. Dahlin, andC.R.Sullivan,“Articular coma: a diagnostic dilemma,” Sarcoma,vol.7,no. 2, pp.69–73, synovial chondromatosis,” The Journal of Bone and Joint Surgery. American,vol.44, pp.77–86,1962. [8] S. H. Butt, T. Muthukumar, V. N. Cassar-Pullicino, and D. C. Mangham, “Primary synovial osteochondromatosis presenting as constrictive capsulitis,” Skeletal Radiology,vol.34, no.11, pp. 707–713, 2005. [9] B. Tins and V. Cassar-Pullicino, “Synovial osteochondromatosis in hereditary arthro-ophthalmopathy (Wagner-Stickler syn- drome),” Skeletal Radiology, vol. 32, no. 5, pp. 302–305, 2003. [10] A. Trias and O. Quintana, “Synovial chondrometaplasia: review of world literature and a study of 18 Canadian cases,” Canadian Journal of Surgery,vol.19, no.2,pp. 151–158, 1976. [11] J. W. Milgram, “Synovial osteochondromatosis: a histopatho- logical study of thirty cases,” The Journal of Bone and Joint Surgery. American,vol.59, no.6,pp. 792–801, 1977. [12] A. K. Bhadra, R. Pollock, R. P. Tirabosco et al., “Primary tumours of the synovium: a report of four cases of malignant tumour,” The Journal of Bone and Joint Surgery. British ,vol.89, no. 11, pp. 1504–1508, 2007. [13] R.I.Davis,A.Hamilton, andJ.D.Biggart,“Primarysynovial chondromatosis: a clinicopathologic review and assessment of malignant potential,” Human Pathology,vol.29, no.7,pp. 683– 688, 1998. [14] M. D. Murphy,J.A.Vidal,J.C.Famburg-Smith,and D. A. Gajenski, “Imaging of synovial chondromatosis with radiologic-pathologic correlation,” Radiographics,vol.27, pp. 1465–1468, 2007. [15] http://www.ncin.org.uk/publications/data briefings/bone sar- comas incidence and survival. [16] L. D. Rybak, L. Khaldi, J. Wittig, and G. C. Steiner, “Primary synovial chondrosarcoma of the hip joint in a 45-year-old male: case report and literature review,” Skeletal Radiology,vol.40, no. 10, pp. 1375–1381, 2011. [17] D. A. Campanacci, D. Matera, A. Franchi, and R. Capanna, “Synovial chondrosarcoma of the hip: report of two cases and literature review,” La Chirurgia Degli Organi di Movimento,vol. 92,no. 3, pp.139–144,2008. [18] E. E. Zamora, A. Mansor, D. Vanel et al., “Synovial chondrosar- coma: a report of two cases and literature review,” European Journal of Radiology,vol.72, no.1,pp. 38–43, 2009. [19] F. Bertoni, K. K. Unni,J.W.Beabout,and F. H. Sim, “Chon- drosarcoma of the synovium,” Cancer,vol.67, pp.155–162,1991. [20] W. K. Taconis, R. O. van der Heul, and A. M. M. Taminiau, “Synovial chondrosarcoma: report of a case and review of the literature,” Skeletal Radiology,vol.26, no.11, pp.682–685,1997. [21] M. V. Miller, A. Kind, and F. Mertens, “Synovial chondromato- sis,” in Pathology and Genetics of Tumours of Soft Tissue and Bone,C.D.M.Fletcher, K. K. Unni,and F. Mertens, Eds.,p. 246, IARC Press, Lyon, France, 2002. 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Synovial Chondrosarcoma Arising in Synovial Chondromatosis

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Copyright © 2014 Scott Evans et al. This is an open access article distributed under the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.
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Hindawi Publishing Corporation Sarcoma Volume 2014, Article ID 647939, 4 pages http://dx.doi.org/10.1155/2014/647939 Research Article Scott Evans, Michele Boffano, Samena Chaudhry, Lee Jeys, and Robert Grimer Royal Orthopaedic Hospital, Birmingham B31 2AP, UK Correspondence should be addressed to Scott Evans; drscottevans@yahoo.co.uk Received 27 November 2013; Accepted 30 January 2014; Published 5 March 2014 Academic Editor: Jos Bramer Copyright © 2014 Scott Evans et al. This is an open access article distributed under the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited. Primary synovial chondromatosis (SC) is a rare proliferative disorder that causes pain, swelling, and restriction of movement to the joints it aeff cts. The disease frequently runs a protracted course, oeft n requiring multiple surgical procedures to obtain some control. Few reports exist detailing the natural history of SC, although malignant transformation to synovial chondrosarcoma (CHS) is recognized to be a rare event. eTh aim of our study was to review a large orthopaedic oncology database in order to evaluate the incidence of CHS arising from SC. We identified 78 patients who have presented to our centre with primary synovial chondromatosis (SC). Of those patients, 5 went on to develop malignant change. This represents a 6.4% incidence of developing synovial chondrosarcoma (CHS) within preexisting primary synovial chondromatosis. eTh patients had a mean age of 28 years at first diagnosis with synovial chondromatosis with the median time from original diagnosis to malignant transformation being 20 years (range 2.7–39 yrs). 1. Introduction correlating histological and radiological findings to correctly diagnose the benign entity. Synovial chondromatosis is a rare proliferative, metaplastic Malignant transformation of primary synovial chondro- disorder of the synovium [1]. The disease was first described matosis to synovial chondrosarcoma is recognized to be a rare by Leannac in 1813 [2]; however, its current description was event with reports estimating the incidence to be in the range of 1–5% [12–14]. not applied until 1958 by Jaffe [ 3]. The exact prevalence of The aim of our study was to use a large database of primary synovial chondromatosis (SC) is unknown but it patients with orthopaedic oncology to evaluate the incidence usually aeff cts the third to ftfih decades of life [ 4], with of chondrosarcoma arising from primary synovial chon- men being affected two to four times more frequently than drosarcoma and to review the time taken for the benign entity women [5]. The knee is the most commonly aeff cted large to transform into its malignant counterpart. jointfollowedbyhip,shoulder,elbow,ankle,and wrist[6]. Patient’s clinical symptoms typically include pain, swelling, andrestrictedjoint movement [7]. The symptoms are oeft n insidious at disease onset and are gradually progressive, 2. Materials and Methods although rare spontaneous regression has been reported [8]. Patients oen ft have a protracted length of symptoms prior to We conducted a retrospective search of a prospective tumour diagnosis, with an average of 5 years [9, 10]. database to identify all patients treated at our unit with Three phases of SC have been described [ 11]; phase 1 the diagnosis of primary synovial chondromatosis (SC). eTh active intrasynovial disease with nodules but no calcifica- diagnosis was made under the auspices of a supraregional tions/intraarticular bodies; phase 2 synovitis with osteochon- multidisciplinary bone tumour unit. Patient demographics dral nodules in the synovial membrane and loose bodies were recorded along with the site of primary disease. From within the joint; phase 3 multiple loose bodies remain but this initial search, details of those patients who subsequently synovitis is quiescent. This highlights the importance of developed synovial chondrosarcoma (CHS) were collected. 2 Sarcoma Table 1: Details of the patients, their tumours, and the outcome. Age at Time to Surgical diagnosis malignant Case Site Treatment(s) for SC Malignancy management of Mets Outcome with SC change chondrosarcoma (years) (years) Hindquarter Alive 13 yrs 23 Hip 3 debridements 2.7 Grade I CHS — amputation postoperatively Dedifferentiated (i) Initially symptomatic At chondrosar- Died 3 yrs 48 Hip (ii) THR 11 yrs aer ft initial 11.3 Debulking presentation coma postoperatively diagnosis (lung) (osteosarcoma) (i) 2 arthroscopies At Died 4 yrs (ii)1hip resurfacing 17yrs 25 Hip 20 Grade II CHS Debulking presentation postoperatively aer ft diagnosis (lung) (i) Multiple debridements (ii) THR 2005 Hindquarter Alive 9 months 25 Hip 39 Grade I CHS — (iii) Large recurrence 5 amputation postoperatively years after THR Lung—34 (i) Multiple arthroscopies Above knee Died 4 yrs 5 19 Knee 38.5 Grade II CHS months aer ft (ii) TKR aged 55 amputation postoperatively amputation 3. Results an abovekneeamputationaslimbsalvage wasnot possible. Histology revealed a grade II synovial chondrosarcoma. He A search of our database, which holds prospectively gathered developed lung metastases 34 months postoperatively and data on over 30,000 patients including over 3800 primary subsequently died 4 years aeft r the amputation. bone sarcomas,identiefi d78patientswithSC. Allhad As with other chondrsarcomas, radio/chemotherapy are been diagnosed following analysis of their radiological and not typically effective treatments and, therefore, our cases did histological findings between 1980 and 2011. There were 33 not receive adjuvant therapy. In summary, 78 patients were females (42.3%) and 45 males (57.7%) with a mean age at identified with SC, with 5 developing CHS. This represents presentation with SC of 28 years. The primary site of SC was transformation to malignancy in 6.4% of cases. Furthermore, the knee in 30 patients, hip in 22, hand in 7, shoulder in 6, it is apparent from performing Kaplan-Meier survival analy- elbowin5,footin5,wrist in 2, andthe anklein1. sis that the risk of malignant transformation increases as time The median time from original diagnosis with SC to elapses (see Figure 3). malignant transformation was 20 years (range 2.5–39 years). All patients had undergone multiple procedures in an attempt to control their SC prior to the diagnosis of malignancy. 4. Discussion All cases of CHS were diagnosed aer ft careful discussion at a multidisciplinary team meeting involving orthopaedic Primarysynovialchondromatosisisarare disease. Itstrue oncologist, histopathologist, and radiologist (Table 1). incidence is unknown. The incidence of chondrosarcoma in One of the main problems in diagnosing malignancy England is however known and is reported to be 1.8/million in SC is that biopsies are oeft n not helpful and clinical population per year [15]. Our unit has treated 800 patients features oen ft suggest malignancy more than histology. In two with a chondrosarcoma in the same time period as we patients (case 1 + 4), repeated biopsy demonstrated benign treated these 78 primary synovial chondromatosis patients. disease, whilst their imaging studies revealed likely extensive This would suggest that for every 800 chondrosarcomas primary synovial chondromatosis. However, it was clear from diagnosed, 5originatedinsynovialchondromatosis; that is, the patients’ level of nonmechanical pain in combination with 0.6% of chondrosarcomas are linked to primary synovial the rapid and repeated progression of their tumours aer ft chondromatosis. As the incidence of chondrosarcoma in debulking that they had aggressive disease. The patients were England is known, we would expect to see approximately included in a pragmatic discussion and the decision was made 95 new cases of chondrosarcoma every year. Therefore, to perform hind-quarter amputation. In both cases, the actual every year, 0.57 cases of chondrosarcomas are associated diagnosis of malignancy was only confirmed when the whole with preexistingsynovialchondromatosis; that is,every 1.7 tumour had been sampled (see Figures 1 and 2). years (20.4 months), 1 new case of chondrosarcoma can be Two cases (patients 2 and 3) had large tumours with lung attributed to primary synovial chondromatosis. metastasesbythetimeofdiagnosisandthereforeweretreated Even though our unit is a supra-regional referral unit, it symptomatically. Both patients subsequently died with chest is unlikely that we were referred all patients with synovial metastases 3 and 4 years, respectively, aer ft diagnosis. Our chondromatosis over this time period. If we had been then the one patient with CHS aeff cting the knee (case 5) underwent incidence of primary synovial chondromatosis would be 78/5 Sarcoma 3 1.0 0.8 0.6 0.4 Figure 1: Plain X-ray of case 4 preoperatively. 0.2 0.0 010 20 30 40 Time (years) Malignant transformation Figure 3: Kaplan-Meier survival (malignant transformation = end- point). actually they are typical of benign primary synovial chondro- matosis [22–24]. Immunohistochemical markers to reliably Figure 2: STIR MRI of case 4 preoperatively. differentiate between primary synovial chondromatosis and low grade chondrosarcoma would be of great value but, to date, only preliminary studies have been performed [25]with no definitive immunostaining technique being identified as of that of chondrosarcoma in primary synovial chondromato- the gold-standard. sis; that is, 0.06/million per yr, suggesting that there may be The local recurrence of primary synovial chondromatosis 10 new cases of primary synovial chondromatosis diagnosed hasbeenreportedtobeashighas23% [14] despite adequate ayearinEngland.Weknownoway of conrfi mingthis. surgical debridement. The difficulty lies with identifying Although primary synovial chondromatosis can be those recurrences that are benign. locallyaggressivewithatendency to recur, it hasno In conclusion, we view multiple recurrences with the metastatic potential. eTh treatment for symptomatic primary development of marrow invasion as highly suspicious of synovial chondromatosis consists of removal of the cartilagi- malignant transformation. From our study, it is clear that not nous bodies with or without partial synovectomy. In contrast, all patients with primary synovial chondromatosis require chondrosarcoma is a malignant condition requiring more long-term surveillance to monitor for the development of invasive surgery in the form of wide or radical resection or malignant change; however, we recommend that any rapid amputation [16] and has a reported incidence of metastases deterioration in the patient’s clinical course, including wors- of up to 29% [17, 18]. ening pain or aggressive recurrence, should be regarded as Clearly because of the differences in prognosis and treat- suspicious and treated at, or referred appropriately to, a ment, it is vitally important to distinguish the benign primary tertiary hospital familiar with managing these often complex synovial chondromatosis from the malignant chondrosar- cases. coma. In practice, this can be difficult as both conditions have similar patterns of disease presentation with pain, swelling, and restricted joint movement. Numerous authors Conflict of Interests have found clinical and radiological criteria unhelpful to differentiate between the two disease processes as there eTh authors declare that there is no conflict of interests is oen ft significant overlap with no definitive features to regarding the publication of this paper. separate the two [1, 14, 18–20]. Histologically primary synovial chondromatosis reveals References lobules of hyaline cartilage that are oen ft hypercellular with atypical histological features, including multinucleation, [1] B. Wittkop, A. M. Davies, and D. C. Mangham, “Primary syn- nuclear crowding, nuclear enlargement, and hyperchromasia ovial chondromatosis and synovial chondrosarcoma,” European with mild myxoid changes often with a variable degree Radiology,vol.12, no.8,pp. 2112–2119, 2002. of synovial proliferation or hyperplasia [21]. These atypical [2] J.M.Crotty, J. U. V. Monu,and T. L. Pope Jr., “Synovial features could suggest a malignant neoplasm (grade I or II osteochondromatosis,” Radiologic Clinics of North America,vol. chondrosarcoma) to the less experienced pathologist when 34,no. 2, pp.327–342,1996. Malignant transformation 4 Sarcoma [3] H. L. Jaeff , Tumours and Tumourous Conditions of the Bones and [22] D. Resnik, “Tumours and tumour-like lesions of soft tissues,” in Joints, Kimpton, London, UK, 1958. Diagnosis of Bone and Joint Disorders, pp. 4204–4273, Saunders, Philadelphia, Pa, USA, 4th edition, 2002. [4] “Synovial chondromatosis,” in Pathophysiology of Orthopaedic Diseases,H.J.Mankin, Ed., pp.53–58,TheAmericanAcademy [23] K. K. Unni, C. Y. Inwards, J. A. Bridge, L. G. Kindbolm, and L. of Orthopaedic Surgeons, Rosemont, Ill, USA, 2006. E. Wold, “Synovial tumours,” in Tumours of the Bone and Joints, pp. 386–432, ARP Press, Silver Spring, Md, USA, 4th edition, [5] M.D.Darfamand B. 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