case 1 srdp, sibiu, october 20121. authors: sabina zurac 1, razvan andrei 1, tiberiu tebeica 1,...

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  • Case 1SRDP, Sibiu, october 2012*

  • Authors:Sabina Zurac 1, Razvan Andrei1, Tiberiu Tebeica1, Florica Staniceanu1,

    Adrian Rebosapca, Bogdan Andreescu2

    1Pathology2Plastic Surgery Colentina University Hospital, Bucharest, RomaniaSRDP, Sibiu, october 2012*

  • SRDP, Sibiu, october 2012*

  • SRDP, Sibiu, october 2012*

  • SRDP, Sibiu, october 2012*

  • SRDP, Sibiu, october 2012*

  • SRDP, Sibiu, october 2012*

  • Male82 yrs oldUlcerated tumor, left temporal area, max diameter 1.5 cmSRDP, Sibiu, october 2012*

  • SRDP, Sibiu, october 2012*

  • IHC negative markersAE1-AE3MNF116CK7CK14CK8/18CK5/634E12BER-EP 4P63HMB45Melan AT311S100

    SMADesminHHF35CLACD20CD3CD30EMACD34CD31CD105F VIII

    SRDP, Sibiu, october 2012*

  • Atypical fibroxantomaSRDP, Sibiu, october 2012*

  • Atypical fibroxantomahighly pleomorphic tumor histologyBenign clinical behaviourlittle, if any, potential for metastasisbenign tumor when strict diagnostic criteria are appliedBarnhill Dermatopathology, McGraw Hill, 2010

    diagnosis of exclusion

    SRDP, Sibiu, october 2012*

  • Atypical fibroxantomaClinical appearance not characteristicold adults sun-damaged skin Associated pathologyxeroderma pigmentosum ImmunosuppressionRapid growthRather small (< 1.5 cm)

    !!! Rarely identified solely on macroscopic aspect (MM, BCC, SCC)!!!Young pts, no actinic damage more likely atypical dermatofibromaSRDP, Sibiu, october 2012*

  • Atypical fibroxantomaUltraviolet-induced p53 mutationsAFX vs undifferentiated high grade sarcomaCGH analysisCommon alterations: deletions on chromosomes 9p and 13q. Differences: deletions on 1q, 3p, 5q, 11p, 11q, gains on 7q, 12q; high level gains on 5p and 11q.Mihic-Probst et al Anticancer Res. 2004 Jan-Feb;24(1):19-26.SRDP, Sibiu, october 2012*

  • Atypical fibroxantomaMicroscopysymmetric, well-circumscribedexophytic Dermal, very superficial adipose extensiongrenz zoneulceration.NO pagetoid spreadepidermal collaretterelatively storiform or fascicular pattern

    SRDP, Sibiu, october 2012*

  • Atypical fibroxantomaMicroscopyTumor cells highly pleomorphic (marked irregular and hyperchromatic nuclei, bizarre multinucleated tumor giant cells)uniform spindle cellsuniform cytologic atypia in tumor mass.Mitoses: frequent, numerous, atypical. multinucleated giant histiocytes, some Touton-typeNO extensive extension in adipose tissueNO necrosis and vascular or perineural invasion

    SRDP, Sibiu, october 2012*

  • Atypical fibroxantomaImmunophenotype highly nonspecific+ vimentin, 1-AT, 1-ACT, CD68, SMA, +CD10, +CD117Large panels to exclude dif malignancies.Aware to reactive/entrapped cells: epithelial, Langherhans, vessels Longacre TA et al. Am J Surg Pathol. 1993;17:1199-1209.Singh M et al. J Oral Maxillofac Surg. 2012 Apr 9.SRDP, Sibiu, october 2012*

  • Atypical fibroxantomaIf strict criteria are followed, atypical fibroxanthoma may be regarded as benign neoplasms cured by simple excisionBarnhill Dermatopathology, McGraw Hill, 2010Local recurrences, no metastases series of 140 atypical fibroxanthomasFretzin, Helwig. Cancer.1973;31:1541-1552.series of 171 atypical fibroxanthomasBeer TW et al Am J Dermatopathol. 2010 ;32:533-40

    IHC tests to exclude malignant fibrous histiocytoma, angiosarcoma, malignant melanoma, and squamous cell carcinoma Morphologic alterationsNO extensive extension in adipose tissueNO necrosis and vascular or perineural invasion

    SRDP, Sibiu, october 2012*