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Soft Tissue Tumors Soft Tissue Tumors Soft Tissue Non-Rhabdomyosarcomatous Soft Soft Tissue Tumors Tissue Sarcomas & Germ Cell Tumors of Childhood Other Than Rhabdomyosarcoma Jesse J. Jenkins, III, M.D. Director of Pathology for the International Outreach Program St. Jude Children’s Research Hospital 1 2 Soft Tissue Tumors • Heterogenous with diverse patterns – Extracellular matrix proteins • collagen Soft Tissue Tumors • laminin • chondroitin sulfate – Cytoskeletal elements Can be easy or very difficult to • keratin diagnose! • vimentin – Energy storing & enzymatic proteins • myoglobin • creatine kinase 3 4 Soft Tissue Tumors Soft Tissue Tumors • Histopathologic diagnosis • Cytoskeleton/Matrix Relation – Morphology defined by – Defines tumor cell shape • Cell shapes • Dense collagen - compresses (narrow, elongated) • Spatial organization • Chondroid matrix - surrounds in lacunar fashion – Patterns produced • Myxoid matrix - allows polygonal expansion • Organization of cellular elements • Interaction with surrounding tissues 5 6 1 Soft Tissue Tumors Soft Tissue Tumors • Complex structures • Recognizable distinguishing characteristics – Produced or induced by tumor – Light microscopy (still the primary standard) • Epithelial slits (synovial sarcoma, nerve sheath – Electron microscopy (decreasingly used) tumors) – Immunohistochemistry (now the secondary • Blood vessels (hemangiopericytoma) standard) • Inflammatory cells (recruited by all of them) – Mast cells – Cytogenetics – Lymphocytes – Molecular diagnostics (increasingly important) 7 8 Soft Tissue Tumors Soft Tissue Tumors •Benign •Benign – Scar – Fibrohistiocytic lesions – Nodular fasciitis • Fibrous histiocytoma – Proliferative fasciitis & myositis • Giant cell fibroblastoma • Plexiform fibrohistiocytic tumor – Myositis ossificans • Juvenile xanthogranuloma – Fibrodysplasia ossificans progressiva • Xanthoma – Various Fibromatoses • Tenosynovial giant cell tumor – Fibrous pseudotumor (inflammatory – Vascular lesions pseudotumor) • More than 16 varieties described – Angiofibroma – Smooth muscle lesions 9 10 Soft Tissue Tumors Soft Tissue Tumors • Intermediate Clinical Behavior •Benign –Fibromatoses – Nerve sheath lesions • At least 5 varieties • At least 7 varieties – Vascular lesions –Fatty lesions • Epithelioid hemangioendothelioma • Lipoblastoma/lipoblastomatosis • Spindle cell hemangioendothelioma • Fibrous hamartoma of infancy • Giant cell angioblastoma • Kaposi sarcoma • Fibrosarcoma of infancy • Dermatofibrosarcoma protuberans 11 12 2 Soft Tissue Tumors Soft Tissue Tumors • Malignant – Synovial sarcoma • Malignant – Hemangiopericytoma – Clear cell sarcoma (malignant melanoma of soft parts – Malignant peripheral nerve sheath tumor –Angiosarcoma – Epithelioid sarcoma –Liposarcoma – Malignant fibrous histiocytoma – Leiomyosarcoma –Fibrosarcoma – Extraskeletal mesenchymal chrondrosarcoma – Myofibrosarcoma – Extraskeletal myxoid chondrosarcoma – Alveolar soft part sarcoma 13 14 One Month Old Boy Right Upper Quadrant Mass 15 16 17 18 3 19 20 21 22 23 24 4 Infantile Hemangioendothelioma Final Diagnosis • Cavernous angioma Liver tumor • Angioendothelioma • Capillary hemangioma Infantile Hemangioendothelioma 25 26 Infantile Hemangioendothelioma Infantile Hemangioendothelioma • 90% in first 6 months of life • Jaundice • Males < Females • Thrombocytopenia • Often multinodular • Heart failure (up to 25%) • Involves other organs commonly – Skin (20-40%) –Lung – Lymph nodes –Bone 27 28 One Month Old Girl Soft Tissue Mass in Left Calf Open Biopsy 29 30 5 31 32 Soft Tissue of Left Calf Thirteen Year Old Girl Open Biopsy Congenital (Infantile) Fibrosarcoma Mass in Right Thigh 33 34 Mass in thigh Mass in thigh 35 36 6 Alveolar Soft Part Sarcoma • Adolescence & early adulthood • 40% in deep thigh or buttock • Everywhere else has been reported Final Diagnosis • Indolent growth & no pain • 20% metastatic rate at diagnosis – Lung, Bone, Brain Alveolar Soft Part Sarcoma • Uniform histology – Organoid clusters of bland cells – Thin fibrovascular septa – Central necrosis give alveolar pattern 37 38 Alveolar Soft Part Sarcoma Soft Tissue Sarcomas References • No consistent immunophenotype – Myogenin/MyoD1 but cytoplasmic • Coffin CM, Dehner LP, O’Shea PA. –+/-desmin Pediatric Soft Tissue Tumors. A Clinical, • Rhomboid, PAS+ crystals in 22-80% but Pathological, and Therapeutic Approach. pathognomonic Williams & Wilkins, Baltimore, 1997. • Weiss SW, Goldblum JR. Enzinger and Weiss’s Soft Tissue Tumors. Fourth Edition. Mosby, St. Louis, 2001. 39 40 Germ Cell Tumors Germ Cell Tumors • 2 to 3 per 1,000,000 births in USA • Biphasic age distribution • Primordial germ cell origin – First peak at 2 years of age • Heterogenous group of tumors • Extragonadal and gonadal – Mature teratoma • Site- and age-specific differences in – Immature teratoma (20% have yolk sac carcinoma) biology, prognosis, and therapy – Yolk sac carcinoma • Biphasic age distribution – Second peak at 15-20 years of age – First peak at 2 years of age • Mostly gonadal – Second peak at 15-20 years of age 41 42 7 Germ Cell Tumors One Month Old Girl • Teratoma • Immature teratoma • Embryonal carcinoma • Gastroschisis • Germinoma (dysgerminoma; seminoma) • Sacrococcygeal region mass • Choriocarcinoma • Yolk sac carcinoma (endodermal sinus tumor) • Gonadoblastoma 43 44 45 46 47 48 8 Primitive neural tube formation Final Diagnosis Sacrococcygeal teratoma (immature teratoma, grade I) 49 50 Immature Teratoma Immature Teratoma • Virtually all grade 1 and 2 are benign in • No i(12p) in childhood cases children • Immaturity in one or more of the three • Extraovarian sites extremely rare - ? layers but usually neuroepithelial tissue sufficient numbers to evaluate the grading • Grades (?) system – 0 = no immaturity • Elevated serum α-fetoprotein – 1 = no more than one low power field – Usually means small foci of yolk sac carcinoma – 2 = >1 to <4 low power fields that may not stain with immunoperoxidase – 3 = many consecutive fields – May mean fetal liver (or hepatoid pattern yolk 51 sac carcinoma) - ?? immature tissue or tumor 52 One Month Old Boy Presacral Tumor Mass with Pulmonary Metastases 53 54 9 55 α fetoprotein 56 57 α fetoprotein 58 Yolk Sac Carcinoma FINAL DIAGNOSIS • Commonest malignant GCT in prepuberal children Needle biopsy of presacral tumor: • Pre-existing teratoma in most Yolk sac carcinoma • Usually elevated alpha fetoprotein • Lots of confusing histologic patterns and overlap with embryonal carcinoma 59 60 10 Jesse J. Jenkins, III, M.D. Department of Pathology St. Jude Children’s Research Hospital 332 North Lauderdale Street Memphis, Tennessee 38105-2794 USA [email protected] Phone: (901)495-3516 Fax: (901)495-3100 Web site: www.Cure4Kid.org 61 11.
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