Synovial chondrosarcoma: Report of two cases and literature review

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Synovial chondrosarcoma is a rare soft tissue tumor that can arise from a previous synovial chondromatosis or as de novo tumor. The clinical and radiological findings of this malignancy are very similar to those of aggressive synovial chondromatosis.
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  Please cite this article in press as: Zamora EE, et al. Synovial chondrosarcoma: Report of two cases and literature review. Eur J Radiol(2009), doi:10.1016/j.ejrad.2009.05.029 ARTICLE IN PRESS G Model EURR-4398; No.of Pages6European Journal of Radiology xxx (2009) xxx–xxx Contents lists available atScienceDirect European Journal of Radiology  journal homepage:www.elsevier.com/locate/ejrad Review Synovial chondrosarcoma: Report of two cases and literature review E.E. Zamora a , b , A. Mansor b , c , D. Vanel d , ∗ , C. Errani b , M. Mercuri b , P. Picci d , M. Alberghini e a Department of Orthopaedic Surgery, Hospital Dr. R.A Calderón Guardia, Universidad De Costa Rica, P.O. Box 628-3000, Heredia, Costa Rica b Musculoskeletal Oncology Department, Istituto Ortopedico Rizzoli, Via Pupilli 1, 40136 Bologna, Italy c Department of Orthopaedic Surgery, University of Malaya, Kuala Lumpur 59100, Malaysia d Musculoskeletal Oncology Research Center, Istituto Ortopedico Rizzoli, Via di Barbiano 1/10, 40136 Bologna, Italy e Musculoskeletal Anatomical Pathology Department, Istituto Ortopedico Rizzoli, Via di Barbiano 1/10, 40136 Bologna, Italy a r t i c l e i n f o  Article history: Received 30 April 2009Accepted 4 May 2009 Keywords: Soft tissue tumorSoft tissue sarcomaSynovial chondromatosisMalignant transformationSynovial chondrosarcoma a b s t r a c t Synovial chondrosarcoma is a rare soft tissue tumor that can arise from a previous synovial chondro-matosis or as de novo tumor. The clinical and radiological findings of this malignancy are very similarto those of aggressive synovial chondromatosis. Confusion with other joint pathologies makes the diag-nosis of synovial chondrosarcoma difficult in most of the cases. We present one recently diagnosed andtreated case of synovial chondrosarcoma. The review of our hospital database revealed one more similarcase. In both cases the malignancy arose from a pre-existing synovial chondromatosis. We also present aliterature review emphasizing the clinical and histological findings of this rare entity.© 2009 Published by Elsevier Ireland Ltd. 1. Introduction Synovial chondrosarcoma either primary or secondary to a pre-existing synovial chondromatosis is a very rare entity[1,2].Its aggressiveness and metastatic ability have been well documentedin most of the published cases[1,3–8].Most of them are isolated cases secondary to synovial chondromatosis. Some of them werediagnosed and treated for several years as synovial chondromato-sis previous to the sarcoma appearance[1,3–6,9–16]and othersshowed both synovial chondromatosis and synovial chondrosar-coma features at the time of diagnosis[1,9].We present two cases of synovial chondrosarcoma. Bothcases had a synovial chondromatosis srcin, with longstandingsymptoms, rapid and aggressive evolution at the time of malig-nant transformation. The clinical and histopathology behavior of the synovial chondromatosis with malignant transformation isdescribed. 2. Case 1 A 62-year-old woman presented with history of pain on her lefthip and thigh on November 2007.Pastmedicalhistoryrevealed15-yearsymptomsofmilddiscom-fortinthelefthipwithoutanyfunctionaldisturbance.Radiographs ∗ Corresponding author. Tel.: +39 051 63 66 931. E-mail address: dvanel@ior.it(D. Vanel). 15 years ago were interpretated as synovial chondromatosis andshe was not operated.Limited range of motion with disturbance of some daily activ-ities such as climbing stairs was present for the past year prior topresentation. The symptoms became worse in the last few monthsandpaindidnotdisappearwithrest.Shealsonoticedahardmassinher left thigh, and diffuse parasthesia. For the last two months thepatient had to use the cane due to increasing difficulty in walking.She also required intramuscular injections of non-steroidal anti-inflammatory drugs at least twice a week. Physical examinationrevealedahardtendermassthatcouldbepalpatedintheproximalanteromedial part of the left thigh, fixed to deep planes. She hadfixed flexion hip contracture at 30 ◦ with very limited internal andexternal rotations. In September 2007 an incisional biopsy of thismass was performed in another center. This biopsy showed typicalsynovial chondromatosis features.Present radiographs of the left hip showed a diffuse soft tissuemass, with multiple rounded and ovoid calcifications, destructionand permeation of the femoral head and acetabulum. This massextended towards the proximal medial side of left femur (Fig. 1a). CT scan of pelvis confirmed the mass in the hip joint with fluidmasses and numerous calcifications, with permeation of the prox-imal femur, pelvic bones and irregularity of the ischiopubic ramus.The iliacus, gluteus maximus and the adductor muscles were alsoinvolved (Fig. 1b). Thoracic CT scan revealed a nodule in the anterior segment of the superior lobe of the left lung (Fig. 1c). The decision for frozen biopsy was made. This biopsy revealeda grade 2 chondrosarcoma. Wide extra-articular hip resection 0720-048X/$ – see front matter © 2009 Published by Elsevier Ireland Ltd.doi:10.1016/j.ejrad.2009.05.029  Please cite this article in press as: Zamora EE, et al. Synovial chondrosarcoma: Report of two cases and literature review. Eur J Radiol(2009), doi:10.1016/j.ejrad.2009.05.029 ARTICLE IN PRESS G Model EURR-4398; No.of Pages62 E.E. Zamora et al. / European Journal of Radiology xxx (2009) xxx–xxx Figure 1. Case 1. (a) Radiograph of the left hip, demonstrating a diffuse soft tissue mass, with multiple rounded and ovoid calcifications, destruction and permeation of thefemoral head and acetabulum, extending to the proximal medial side of the femur. (b) Axial CT image of the left hemipelvis, showing an intra-articular extraosseous mass,containing multiple hyperdense nodules, infiltration of the periacetabular pelvic bones and proximal femur, and involving of the periarticular muscles. (c) Chest CT imagedemonstrating one nodule in the anterior segment of superior lobe of the left lung (arrow). (d) Post-operative radiograph showing reconstruction with massive pelvic bone  Please cite this article in press as: Zamora EE, et al. Synovial chondrosarcoma: Report of two cases and literature review. Eur J Radiol(2009), doi:10.1016/j.ejrad.2009.05.029 ARTICLE IN PRESS G Model EURR-4398; No.of Pages6 E.E. Zamora et al. / European Journal of Radiology xxx (2009) xxx–xxx 3 and reconstruction with allograft and prosthesis was performed(Fig. 1d). Complete histopathology report revealed two different tissues,almost side by side. One area showed typical synovial chondro-matosis appearance, a hypercellular hyaline cartilage tissue withclusterpatternarrangementofthecellsanddiscretenuclearatypia.Theotherareashowedsynovialchondrosarcoma:mixoiddegener-ation, loss of the cellular cluster pattern and sheets arrangementsofthecells.Therewasnoobvioustransitionalplaneinbetweenthetwoareas(Fig.1e–g).Thesefindingsmadethediagnosisofsynovial chondrosarcoma arising from synovial chondromatosis.In March 2008 resection of four lung nodules was made.Histopathologic examination confirmed the metastatic srcin inthree of these lesions, showing chondrosarcoma grades 1–2 withmixoid aspect. 3. Case 2 In August 1994, a 61-year-old male was referred for right hiprecurrent synovial chondromatosis.Fourteen months prior, synovectomy had been performed inanother hospital but symptoms recurred 10 months later.Atphysicalexaminationrighthipflexioncontracturewasfound.Rangeofmotionwasdiminishedofaboutone-thirdofnormalinallplanes and pain radiated to right thigh and knee, it worsened withweight loading and gait.Radiographs, CT and MRI revealed a multinodular rounded softtissue mass in the right hip between ischiopubic ramus and lessertrochanter with proximal femur erosions (Fig. 2a–c). Synovectomy and cemented total hip replacement were done.Histopathology examination revealed synovial chondromatosis.The same was found in the review of histology sample of 1993surgery.Twoyearslater,abiopsyofanewrecurrenceshowedaggressivesynovial chondromatosis.Eventually, 3 years after the first presentation, externalhemipelvectomy was performed when the biopsy of the thirdrecurrenceshowedchondrosarcomagrade3dedifferentiationfromsynovial chondromatosis (Fig. 2d–f). FlowcytometryanalysisshowedaneuploidDNAandDNAindexof 1.52. These analyses were done after the malignant transforma-tion was found.At last follow-up on May 2000 (69 months after presentation)he was free of disease.The review of the histopathology confirmed synovial chon-drosarcoma from synovial chondromatosis. 4. Discussion Synovial chondromatosis is a rare benign disorder srcinatingfromthesynovium,asresultofself-limitingproliferativeandmeta-plastic changes in the synovial membrane of the joints, tendonsheaths, or bursae, leading to formation of intrasynovial cartilagi-nousnodules[2,17,18].Thistumorlikeprocessismoreprevalentin malesandtheusualageofpresentationisbetween20and50years,beingrareinchildren[2].About50%ofcasesinvolvethekneejoint, 10%ofwhicharebilateral[18],f ollowedinfrequencybyhip,shoul- der, elbow, ankle, and wrist joints. The symptoms in most casesare vague, which usually contrast with the radiological findings atmoment of diagnosis[2].Thetreatmentofteninvolvesremovalofloosebodiesandsubto-tal synovectomy, which can be difficult in some aggressive forms.Insomecasestotaljointreplacementshavetobeconsidered.Localrecurrence is possible after incomplete synovectomy, inadequateremoval of the loose bodies or diseased synovium at the time of initial surgery[2,4].The self-limiting course of synovial chondromatosis was pro-posedbyMilgramin1977,basedonahistopathologicalstudyof30cases of synovial chondromatosis. In this series the affected siteswere the knee in 13 cases (43%), elbow 5 cases (16%), shoulder 4cases(13%),hip3cases(10%),otherjoints5cases(16%).Heobserved3 cases (10%) of recurrence; however the follow-up period in mostof cases was too short to establish a conclusion of real recurrence.The author described three recognizable phases and their clinicaland histopathological findings[19].Similar results were obtained by Davis et al. in a clinico-pathologic review of 53 cases of primary synovial chondromatosiscovering a period of 30 years. The average age at presentation was41 years ranging from 17 to 64, with a male to female ratio of 1.8:1.Thekneejointwasinvolvedin68%ofcasesfollowedbyhipin20%of casesandrecurrenceoccurredin9patients(15%ofcases)including3 that became malignant[4].Radiological findings of synovial chondromatosis are variabledepending upon the level of calcification of the cartilaginousnodules[17],but in most cases imaging is fairly diagnostic[2]. Radiographs, CT and MRI show evidence of an intra-articular softtissue mass with multiple mineralized osteocartilaginous nodulesconstituting the lesion, and well-defined bone erosions on bothsidesofthejoint[18].Innon-mineralizedlesionstheMRIshowsan intra-articular conglomerate mass isointense relative to muscle onT1-weighted images and hyperintense on T2-weighted images. Inlesions with calcified cartilaginous nodules small areas of low sig-nalintensityareobservedonallpulsesequences[20].CTaswellas MRImaybeextremelyusefulforconfirmingtheextraosseoussrcinof the lesion. The non-mineralized loose bodies are well shown onarthrography as multiple filling defects in an enlarged joint space[18].Histological examination of synovial chondromatosis demon-strates coalescent and well circumscribed hypercellular lobules of hyaline cartilage attached to the synovium. Typically, the cartilagecells are arranged in clustered patterns. In most cases the lesionsshow high hypercellularity and nuclear atypia. Myxoid change israre and when present it is only focal; necrosis is absent[2,18].Synovial chondrosarcoma either primary or secondary to a pre-existing synovial chondromatosis is a very rare entity. The clinicalandradiologicalfindingsareverysimilartothoseofaggressivesyn-ovial chondromatosis; it is often recurrent, steadily growing andinvading bone and soft tissue[2].Bertoni et al. studied 10 cases of synovial chondrosarcomas. They described 6 women and 4 menranging in age from 30 to 70 years. Five lesions involved the knee,threethehip,onetheelbowandonetheankle.The9patientswithavailable follow-up developed recurrences, 5 patients (55%) devel-oped metastasis and only 4 patients (45%) were alive and free of disease at the time of the study, 4 months to 11 years after surgery[1].The treatment of synovial chondrosarcoma is similar to thatof skeletal chondrosarcomas. Wide margin surgical removal is thetreatment of choice, and occasionally requires amputation[2].In the literature review we found 29 cases reported as synovialchondrosarcoma arising from a synovial chondromatosis. Somecases have a proven biopsy with synovial chondromatosis diag- allograft, McMinn acetabular prosthesis and modular proximal femur megaprosthesis. (e) Low-power photomicrograph (20 × , H&E) demonstrating two different tissues,one well-formed hyaline cartilage with cluster pattern arrangement of the cells, typical of synovial chondromatosis (thin arrows), second with loss of the cluster patternarrangement of the cells and mixoid tissue degeneration (thick arrows). (f) High-power photomicrograph (40 × , H&E) with the typical aspect of synovial chondromatosis. (g)High-power photomicrograph (40 × , H&E) demonstrating the aspect of synovial chondrosarcoma with crowding and spindling of the nuclei at the periphery.  Please cite this article in press as: Zamora EE, et al. Synovial chondrosarcoma: Report of two cases and literature review. Eur J Radiol(2009), doi:10.1016/j.ejrad.2009.05.029 ARTICLE IN PRESS G Model EURR-4398; No.of Pages64 E.E. Zamora et al. / European Journal of Radiology xxx (2009) xxx–xxx Figure 2. Case 2. (a) Axial CT image of the pelvis with a rounded intra-articular mass in the right hip with extraosseous srcin (arrows). (b) Pelvic coronal T1-weighted MRimage (TR: 420) showing the mass in the right hip (arrow) constituted by multiples nodules isointense relative to muscle. (c) Pelvic axial T2-weighted MR image (TR: 2140)demonstrating the lobulate mass in the right hip hyperintense relative to muscle (arrows), with erosions of the proximal femoral. (d) Low-power photomicrograph (20 × ,H&E) demonstrating the coexistence of synovial chondromatosis (black arrows) and the synovial chondrosarcoma (arrow heads), with an island of synovial chondromatosis(whitearrows)surroundedbysynovialchondrosarcoma.(e)Low-powerphotomicrograph(20 × ,H&E)showingtheabrupthistologicalchangebetweenthewelldifferentiatedhyalinecartilagetissueofthesynovialchondromatosis(arrows)andthemixoidtissuewithlossoftheclusterarrangementofthecellsinthesynovialchondrosarcoma(arrowheads). nosis prior to the diagnosis of chondrosarcoma, like our secondcase[1,3–5,9–15,21].Others shown synovial chondrosarcoma in synovial chondromatosis in the same histologic evaluation[1,9],similar to our first case, or have a previous biopsy with diagnosisofsynovialchondromatosiswhichwasre-evaluatedretrospectivelyshowingevidenceofmalignancy[1,6,16].Theprincipalclinicaldata of these studies are summarized inTable 1.As the clinical and radiological findings are very similarbetween both pathologies, their differentiation is often difficult.Thehistopathologicalfeaturesmostusefultodifferentiatesynovialchondrosarcoma from synovial chondromatosis are as follows:1. The arrangement of the cartilaginous tumor cells in sheets andnot in cluster pattern.2. Myxoid changes in the matrix.3. Hypercellularitywithcrowdingandspindlingofthenucleiattheperiphery.4. Necrosis not related to calcifications and in the absence of apathologic fracture.5. The synovial chondrosarcoma shows permeation of trabecularbonewith“filling-up”ofmarrowspaces,thatmustbedifferenti-atedfromtheerosionsofboneandsofttissuescausedbysynovialchondromatosis due to tumor pushing margins, contrary to thepermeativegrowthpatternevidentintheperipheraledgeofthesynovial chondrosarcoma[1,2,4].Some clinical and radiological findings could alert the clinicianto the possibility of malignant transformation of synovial chon-dromatosis. However definitive clinical or radiological criteria of malignancy do not exist. In fact for some authors the clinical andradiographic findings are the same on both entities[1,5,10].Recur- rence is not a reliable sign of malignancy because it is usual bothin synovial chondromatosis and synovial chondrosarcoma[1,5,11].However; sudden clinical deterioration in longstanding cases or inrecurrent synovial chondromatosis should raise the suspicion of chondrosarcoma[11,12];especially if it is present in an unusual locationasshoulder[3].Radiographs,CTandMRIcouldshowaper- meative, destructive pattern rather than an erosive pattern. Thesefindings should be considered as suspicious for malignancy[11].Studies in flow cytometry have been useful in assessing thedegree of malignancy of numerous neoplasms; distinguishingbetweenbenignandmalignantprocessandalsopredictingwhethera tumor is likely to metastasize or cause patient’s death[22].Syn- ovialchondromatosishasshownanintermediatepositionbetweenenchondroma and chondrosarcoma in flow cytometry, making thedifferentiation of synovial chondromatosis from chondrosarcoma  P l    e  a  s  e  c i    t   e  t  h i    s  a r  t  i    c l    e i   n  pr  e  s  s  a  s  : Z  a m or  a E E  , e  t   a l    . S   yn ovi    a l    c h  on d r  o s  a r  c  om a  : R  e   p or  t   of    t  w o c  a  s  e  s  a n d l   i    t   e r  a  t   ur  e r  e vi    e w .E  ur   J   R  a  d i    ol     (   2  0  0  9   )    , d  oi    : 1  0  .1  0 1  6   /     j    . e   j   r  a  d  .2  0  0  9  . 0  5  . 0 2  9  ART I   C L E I  N P RE  S  S   GM o d  e l   E  UR R -4  3  9  8  ;  N o . of   P  a   g e  s  6  E  .E  .Z  a m or  a e  t   a l    .  /   E  ur  o  p e  a n  J    o ur  n a l    o  f   R  a d   i    o l    o  g   y  x  x  x   (   2  0  0  9   )    x  x  x – x  x  x   5   Table 1 Literature review of synovial chondrosarcoma arising from synovial chondromatosis.Author Age Sex Location Treatment SC Treatment chondrosarcoma RS METS FUKing et al.[16]43 M Right knee Synovectomy AKA No No NED 13moDunn et al.[7]67 M Right knee – Hip desarticulation No Scalp NED 2 years 6 monthsHamiltom et al.[15]25 M Left knee Synovectomy Hip desarticulation No No NED 12 monthsHamiltom et al.[14]58 M Left knee Arthrotomy+synovectomy AKA Yes Lung UnknownPerry et al.[8]51 F Right knee Synovectomy AKA+hemipelvectomy Yes Lung+brain DOD 2 yearsBertoni et al.[1]47 F Right hip Synovectomy Hemipelvectomy Yes No NED 1 year 7 months57 F Left elbow Elbow replacement Shoulder disarticulation Yes Lung DOD 1 year47 F Left ankle Intralesional excision BKA Yes No NED 11 years64 F Right knee Intralesional excision AKA Yes Lung+sternum DOD 3 years66 F Hip Capsule and femoral head removed Lost Lost Lost Lost30 M Left knee Synovectomy AKA Yes Lung NED 1 year40 M Right knee Synovectomy Intralesional excision Yes No Alive 4 monthsKenan et al.[13]74 M Right knee Arthroscopy Arthrotomy+synovectomy Yes No NED 18 monthsAnract et al.[9]36 M Right hip – Resection+THR No No –62 M Right knee – AKA No No –40 F Shoulder – Shoulder replacement No No –51 F Knee Synovectomy AKA+desarticulation Yes No –58 F Knee Synovectomy AKA No No –44 M Knee Synovectomy AKA No No –Bascoulerge et al.[21]40 M Knee Synovectomy En-bloc resection Yes NoHermann[6]55 M Right hip THR – Yes CS+lungs DODWuisman et al.[11]50 F Left hip Synovectomy En-bloc resection+endoprosthesis No No NED 3 years30 M Right hip Synovectomy En-bloc resection+endoprosthesis No No NED 2 yearsTaconis et al.[5]59 M Right hip Synovectomy – No No DODDavis et al.[4]25 M Knee – AKA – Lost –58 M Knee – AKA Yes Lung DODBlokx et al.[10]64 M Right hip Synovectomy Nothing – – DOD 10 yearsHallam et al.[12]45 M Left knee Synovectomy TKR No No NEDSah et al.[3]47 M Right shoulder Synovectomy+hemiarthroplasty Scapulectomy+forequarter amputation Yes Lung –AKA: above knee amputation; BKA: below knee amputation; NED: not evidence disease; DOD: dead of disease; THR: total hip replacement; TKR: total knee replacement; SC: synovial chondromatosis; RS: recurrence of sarcoma;FU: follow-up; METS: metastasis.
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