1 Non-Rhabdomyosarcomatous Soft Tissue Sarcomas & Germ Cell Tumors of Childhood Jesse J. Jenkins, III, M.D. Director of Pathology for the International Outreach Program St. Jude Children’s Research Hospital 2 Soft Tissue Tumors Other Than Rhabdomyosarcoma 3 Soft Tissue Tumors Can be easy or very difficult to diagnose! 4 Soft Tissue Tumors • Heterogenous with diverse patterns – Extracellular matrix proteins • collagen • laminin • chondroitin sulfate – Cytoskeletal elements • keratin • vimentin – Energy storing & enzymatic proteins • myoglobin • creatine kinase 5 Soft Tissue Tumors • Histopathologic diagnosis – Morphology defined by • Cell shapes • Spatial organization – Patterns produced • Organization of cellular elements • Interaction with surrounding tissues 6 Soft Tissue Tumors • Cytoskeleton/Matrix Relation – Defines tumor cell shape • Dense collagen - compresses (narrow, elongated) • Chondroid matrix - surrounds in lacunar fashion • Myxoid matrix - allows polygonal expansion
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• 90% in first 6 months of life• Males < Females• Often multinodular• Involves other organs commonly
– Skin (20-40%)– Lung– Lymph nodes– Bone
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Infantile Hemangioendothelioma
• Jaundice• Thrombocytopenia• Heart failure (up to 25%)
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One Month Old GirlSoft Tissue Mass in Left Calf
Open Biopsy
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Soft Tissue of Left CalfOpen Biopsy
Congenital (Infantile) Fibrosarcoma
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Thirteen Year Old Girl
Mass in Right Thigh
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Mass in thigh
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Mass in thigh
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Final Diagnosis
Alveolar Soft Part Sarcoma
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Alveolar Soft Part Sarcoma• Adolescence & early adulthood• 40% in deep thigh or buttock• Everywhere else has been reported• Indolent growth & no pain• 20% metastatic rate at diagnosis
– Lung, Bone, Brain• Uniform histology
– Organoid clusters of bland cells– Thin fibrovascular septa– Central necrosis give alveolar pattern
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Alveolar Soft Part Sarcoma
• No consistent immunophenotype– Myogenin/MyoD1 but cytoplasmic– +/- desmin
• Rhomboid, PAS+ crystals in 22-80% but pathognomonic
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Soft Tissue SarcomasReferences
• Coffin CM, Dehner LP, O’Shea PA. Pediatric Soft Tissue Tumors. A Clinical, Pathological, and Therapeutic Approach. Williams & Wilkins, Baltimore, 1997.
• Weiss SW, Goldblum JR. Enzinger and Weiss’s Soft Tissue Tumors. Fourth Edition. Mosby, St. Louis, 2001.
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Germ Cell Tumors• 2 to 3 per 1,000,000 births in USA• Primordial germ cell origin• Heterogenous group of tumors• Site- and age-specific differences in
biology, prognosis, and therapy• Biphasic age distribution
– First peak at 2 years of age– Second peak at 15-20 years of age
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Germ Cell Tumors
• Biphasic age distribution– First peak at 2 years of age
• Extragonadal and gonadal– Mature teratoma– Immature teratoma (20% have yolk sac carcinoma) – Yolk sac carcinoma
– Second peak at 15-20 years of age• Mostly gonadal