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. 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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1. DIAGNOSIS OF SPINDLE-CELL SOFT TISSUE TUMORSMark R. Wick, MDUniversity 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 Zimmerman’s 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-PositiveTumors
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 Ewing’s 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