o Knee > wrist, hip o Slow, painless swelling o Recurrence very rare o Does not erode bone o MRI increased nodularity and fat signal in synovium

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1 Disclsures Neplastic jint disease (and tumr-like cnditins) Andrew Hrvai, MD, PhD Clinical Prfessr, Pathlgy I have nthing t disclse. Intrductin Virtually any neplasm f bne can secndarily invlve jint imaging essential t exclude Primary neplasms f jints Tend t invlve cavitated jints (synvium) Mst are benign Masses f nnsynvial jints are usually degenerative Synvial neplasms Lipma(tsis) Chndrma(tsis) Hemangima Tensynvial giant cell tumr Synvial sarcma Synvial lipma(tsis) (Lipma arbrescens) Clinical Knee > wrist, hip Slw, painless swelling Recurrence very rare Imaging Des nt erde bne MRI increased ndularity and fat signal in synvium Pathlgy Ndular, bright yellw synvium Replacement f subsynvial cnnective tissue with mature adipse tissue, villus architecture May cntain lipblasts Lined by single layer f synvicytes Differential diagnsis Nrmal synvial fat (has mre cllagen between fat cells) Atypical lipmatus tumr (very rare in synvium) Hffa s disease (prbably same lesin, lcated in infrapatellar fat pad) 1

2 Synvial lipma Synvial lipmatsis T2 FS Des nt erde bne 1 cm Synvial lipmatsis Synvial lipmatsis 2

3 Synvial chndrma(tsis) Clinical 3 rd -5 th decade Knee, shulder, large jints Rare recurrence Malignant transfrmatin ~1% Imaging If calcified, plain film shws multiple cludy r ring calcs Can erde crtex MRI, lbulated, bright n T2 Pathlgy Multiple ndules f blue cartilage, + lined by synvium Clustering f chndrcytes Nuclemegaly and binucleatin cmmn, mitses absent Necrsis OK Differential diagnsis Chndr-sseus lse bdy Sft tissue chndrma (clsely related if nt same) Synvial chndrsarcma (secndary chndrsarcma) Synvial chndrmatsis Synvial chndrmatsis: multiple ndules, erding articular cartilage Synvial chndrmatsis: clustering + endchndral ssificatin 3

4 Synvial chndrmatsis Synvial chndrmatsis Synvial chndrmatsis Synvial chndrmatsis, secndary chndrsarcma 4

5 Synvial chndrmatsis, secndary chndrsarcma Chndr-sseus lse bdy Chndr-sseus lse bdy: Hyaline and fibrcartilage, irregular calcificatins Chndr-sseus lse bdy 5

6 Synvial hemangima Clinical Vascular malfrmatin nt a true vascular neplasm Children > Adults, knee mst cmmn Mst asymptmatic, r slw swelling, decreased range f mtin Cured by excisin Imaging Plain film may suggest effusin (nnspecific) MRI T2 bright serpentine structures, fluid-fluid levels Pathlgy Vascular channels f varying caliber, increased density beneath nrmal r hyperplastic synvium Increased fat N atypia r mitses Differential diagnsis Chrnic hemarthrsis (grssly) Diffuse tensynvial giant cell tumr (imaging) Synvial hemangima T2 FS Synvial hemangima Synvial hemangima 6

7 Synvial hemangima Synvial hemangima Tensynvial giant cell tumr Tensynvial giant cell tumr Clinical Adults, knee, wrist, digits, shulder Slw grwing jint swelling, lcking Recurrence risk ~ grwth pattern Imaging Sft tissue mass, may erde bne Blm n MRI, T2 bright, smetimes T1 bright als Pathlgy Plymrphic: Histicytes, multinucleated giant cells, spindly fibrblasts, siderphages, fam cells, chrnic inflammatin Central fibrsis in lder lesins Mitses may be brisk nt atypical Differential diagnsis Giant cell tumr f lw malignant ptential (sft tissue analg f GCT f bne) Chrnic hemarthrsis Malignant tensynvial giant cell tumr Lcalized Diffuse Gender M > F F > M Jints Small, hand Large, knee Size < 5 cm >10 cm Recurrence 4-30% 30-50% IHC CD68, desmin CD68, desmin 7

8 Tensynvial giant cell tumr Tensynvial giant cell tumr: Diffuse Diffuse, T2 FS Lcalized, T2 FS Hrvai A, Rbbins Basic Pathlgy, Elsevier, 2017 Tensynvial giant cell tumr: Diffuse Tensynvial giant cell tumr: Diffuse 8

9 Tensynvial giant cell tumr: diffuse Tensynvial giant cell tumr: diffuse Tensynvial Tensynvial giant cell tumr: cell diffuse tumr: lcalized Tensynvial giant cell tumr: lcalized 9

10 Tensynvial giant cell tumr: lcalized Tensynvial giant cell tumr: lcalized Tensynvial giant cell tumr: lcalized Tensynvial giant cell tumr: lcalized 10

11 Tensynvial giant cell tumr: lcalized Tensynvial giant cell tumr: lcalized Tensynvial giant cell tumr: desmin Tensynvial giant cell tumr Same genetic abnrmality in bth subtypes (COL6A3:CSF1) Diffuse and lcalized type related t anatmic cmpartment rather than bilgy Prvides mechanism f pathgenesis analgus t giant cell tumr f bne West RB et al. PNAS, (3)

12 Jnes SF, Whitman RC. Ann Surg. 1914;60(4): Synvial sarcma Synvial sarcma Clinical Yung adults (peak), wide age range 80% extremity, knee cmmn but nt intra-articular, can arise anywhere 5 year survival 36-76%, chemtherapy + surgery Pathlgy Mnphasic (70%): unifrm spindle cells, shrt fascicles, herringbne Biphasic (30%): unifrm spindle cells + pseudglands Prly differentiated (<1%): small rund blue cell tumr Hyperchrmatic nuclei, paradxically lw mitses Branching vessels, calcificatin IHC Keratin, EMA usually fcal and patchy S100 in ~30%, SOX10 <10% (usually intraneural synvial sarcma) CD34 negative Genetics t(x;18), SS18-SSX1 SSX2, -SSX4 fusin Mnphasic synvial sarcma Mnphasic synvial sarcma 12

13 Mnphasic synvial sarcma Mnphasic synvial sarcma Biphasic synvial sarcma Biphasic synvial sarcma 13

14 Mnphasic synvial sarcma Prly differentiated synvial sarcma Keratin Keratin Take-hme messages EMA SS18 break apart FISH 1. D nt diagnse diseases f jints (especially synvial chndrma(tsis) withut reviewing imaging. It culd be a bne tumr. 2. Just because it s villus and synvial it des nt mean it is Pigmented Villndular Synvitis 3. D nt ver-diagnse a desmin psitive PVNS as rhabdmysarcma 4. Synvial sarcma is a sarcma but has nthing t d with nrmal synvial cells 14