1. Case 1 SRDP, Sibiu, october 20121

2. Authors: Sabina Zurac 1, Razvan Andrei 1, Tiberiu Tebeica 1, Florica Staniceanu 1, Adrian Rebosapca, Bogdan Andreescu 2 1 Pathology 2 Plastic Surgery Colentina University Hospital, Bucharest, Romania SRDP, Sibiu, october 20122

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8. Male 82 yrs old Ulcerated tumor, left temporal area, max diameter 1.5 cm SRDP, Sibiu, october 20128

9. vimentin SRDP, Sibiu, october 20129

10. IHC – negative markers AE1-AE3MNF116CK7CK14CK8/18CK5/6 34βE12 BER-EP 4 P63HMB45 Melan A T311S100SMADesminHHF35CLACD20CD3CD30EMACD34CD31CD105 F VIII SRDP, Sibiu, october 201210

11. Atypical fibroxantoma SRDP, Sibiu, october 201211

12. Atypical fibroxantoma highly pleomorphic tumor histology Benign clinical behaviour –“little, if any, potential for metastasis” –“benign tumor when strict diagnostic criteria are applied” Barnhill Dermatopathology, McGraw Hill, 2010 diagnosis of exclusion SRDP, Sibiu, october 201212

13. Atypical fibroxantoma Clinical appearance – not characteristic old adults sun-damaged skin Associated pathology –xeroderma pigmentosum –Immunosuppression Rapid growth Rather small (< 1.5 cm) !!! Rarely identified solely on macroscopic aspect (MM, BCC, SCC) !!!Young pts, no actinic damage – more likely atypical dermatofibroma SRDP, Sibiu, october 201213

14. Atypical fibroxantoma Ultraviolet-induced p53 mutations AFX vs undifferentiated high grade sarcoma –CGH analysis Common 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 201214

15. Atypical fibroxantoma Microscopy symmetric, well-circumscribed exophytic Dermal, very superficial adipose extension grenz zone ulceration. NO pagetoid spread epidermal collarette relatively storiform or fascicular pattern SRDP, Sibiu, october 201215

16. Atypical fibroxantoma Microscopy Tumor cells –highly pleomorphic (marked irregular and hyperchromatic nuclei, bizarre multinucleated tumor giant cells) –uniform spindle cells uniform cytologic atypia in tumor mass. Mitoses: frequent, numerous, atypical. multinucleated giant histiocytes, some Touton-type multinucleated giant histiocytes, some Touton-type NO extensive extension in adipose tissue NO necrosis and vascular or perineural invasion SRDP, Sibiu, october 201216

17. Atypical fibroxantoma Immunophenotype highly nonspecific –+ vimentin, α1-AT, α1-ACT, CD68, SMA, –+CD10, +CD117 –Large 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 201217

18. Atypical fibroxantoma “If strict criteria are followed, atypical fibroxanthoma may be regarded as benign neoplasms cured by simple excision” Barnhill Dermatopathology, McGraw Hill, 2010 Local recurrences, no metastases –series of 140 atypical fibroxanthomas Fretzin, Helwig. Cancer.1973;31:1541-1552. –series of 171 atypical fibroxanthomas Beer 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 alterations –NO extensive extension in adipose tissue –NO necrosis and vascular or perineural invasion SRDP, Sibiu, october 201218