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DIAGNOSIS OF SPINDLE-CELL SOFT TISSUE TUMORS Mark R. Wick, MD University of Virginia Medical Center

. CLINICAL INFORMATION . MORPHOLOGY. SPECIAL STUDIES. CLINICAL INFORMATION. AgeSexPrevious historyPresentationEvolutionLocalization. Selected Pediatric Tumors of Soft Tissue. Juvenile Aponeurotic Fibroma Fibrous Hamartoma of InfancyMyofibroma/MyofibromatosisInfantile

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DIAGNOSIS OF SPINDLE-CELL SOFT TISSUE TUMORS Mark R. Wick, MD University of Virginia Medical Center

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    1. DIAGNOSIS OF SPINDLE-CELL SOFT TISSUE TUMORS Mark R. Wick, MD University of Virginia Medical Center

    3. CLINICAL INFORMATION Age Sex Previous history Presentation Evolution Localization

    4. Selected Pediatric Tumors of Soft Tissue Juvenile Aponeurotic Fibroma Fibrous Hamartoma of Infancy Myofibroma/Myofibromatosis Infantile Hemangiopericytoma Congenital Fibrosarcoma

    5. Impact of Laboratory Data Phosphaturia & osteomalacia History of radiation History of neurofibromatosis

    6. Localization Topography (distal vs. proximal extremities; trunk; serosal cavities; head & neck) Depth (superficial vs. deep soft tissues)

    7. Depth of Tumor Superficial: Superficial to fascial planes on the extremities & trunk Deep: Involving or penetrating fascia in: Retroperitoneum Mediastinum & chest wall Head & neck Pelvis Spermatic cord Axilla

    9. MORPHOLOGIC FEATURES Circumscription, encapsulation, infiltration Cellularity; mitotic activity Stromal features Growth pattern Cytologic features

    10. Circumscription

    11. Cellularity/Mitotic Activity

    12. Stroma Myxoid Fibrotic/hyalinized Calcific Formation of metaplastic tissue Multinucleated giant cells

    13. Cytologic Features

    15. Degree of Pleomorphism Leiomyosarcoma MFH MPNST Synovial sarcoma Neurofibroma Fibromatosis Myofibromatosis

    16. Growth Patterns Fascicular Herringbone Hemangiopericytoid Palisading Storiform Fibrotic/hyalinizing Myxoid

    17. Fascicular Growth (Myo-)Fibrosarcoma Leiomyosarcoma Nodular Fasciitis Fibromatosis Solitary Fibrous Tumor Peripheral nerve sheath tumors

    19. Herringbone Growth

    20. Other Herringbone Tumors Synovial sarcoma Neurofibrosarcoma Solitary Fibrous Tumor

    21. Storiform Growth

    23. Storiform Growth Malignant fibrous histiocytoma Dermatofibrosarcoma protuberans (DFSP) (best representative of this group) Other fibrohistiocytic tumors Occasional leiomyosarcomas Some examples of malignant peripheral nerve sheath tumor Occasional synovial sarcomas Solitary fibrous tumor

    26. Hemangiopericytoid Growth Pattern Stout & Murray: Hemangiopericytoma: a vascular tumor featuring Zimmermans pericytes Annals of Surgery, 1942.

    28. Potentially-Hemangiopericytoid Tumors Solitary fibrous tumor Synovial sarcoma Mesenchymal chondrosarcoma Adult myofibroma Some leiomyosarcomas Some examples of MFH Endometrial stromal sarcoma Some examples of MPNST

    33. Palisading Growth

    35. Potentially Fibrotic & Hyalinizing Tumors Solitary fibrous tumor Fibrosarcoma Fibromatosis MFH Sclerosing epithelioid fibrosarcoma Fibrosing & hyalinizing tumor with giant rosettes Myofibroblastomas

    43. Potentially Myxoid Tumors Myxoid MFH Low-grade myxofibrosarcoma of Angervall Fibromyxosarcoma of Evans Aggressive angiomyxoma Myxoinflammatory fibrosarcoma of the distal extremities Occasional examples of leiomyosarcoma, synovial sarcoma, & MPNST

    49. Immunohistochemistry

    50. Recommended Basic IHC Panel for Spindle Cell Tumors Vimentin MSA/SMA Desmin Calponin Caldesmon CD34 CD99 Bcl-2 S-100 protein MART-1 Keratin (cocktail)

    51. Fine Tuning Actin-Positive Tumors

    52. Fine Tuning Keratin-Positive Tumors

    53. Fine Tuning S100 Protein-Positive Tumors

    54. What Should One Do When the Immunoprofile is Indeterminate? (E.g., CD34+, + bcl-2)

    56. Role of Electron Microscopy Schwannian & perineurial tumors Synovial sarcoma Myofibroblastic tumors Melanocytic tumors Metastatic carcinomas

    60. Cytogenetics Ewings tumor/PNET t(11;22) Myxoid liposarcoma t(12;16) Clear-cell sarcoma t(12;22) Alv. rhabdomyosarcoma t(2;13) or t(1;13) Congenital fibrosarcoma t(12;15) DFSP t(17;22) Synovial sarcoma t(X;18)

    63. Summary Multidisciplinary Approach: Clinical data Macroscopic features Histology Inmunohistochemistry Ultrastructure Cytogenetics

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