Agaimy , Case #6 • 49 yo male with disability for 5 yrs. • Lab.: low serum phosphate & high urine phosphate. • Serum FGF23 significantly elevated. • No obvious cause detected.
• Left hand thenar mass detected on clinical examination and excised. • Disability resolved gradually within 6 mo. • 3 yrs later no evidence of disease.
Eosinophilic cells within microcystic stroma
focal myopericytoma-like vessels Microcystic stroma Focal myopericytoma-like vessels
grungy (dirty) matrix (bluish)
characteristic weak ERG
SATB2
Case #11 in this series • Uncommon but often very late recognized (disability for yrs). • Adults. • Half bone & half soft tissue (half acral); head&neck. • Indolent behavior. • IHC: SATB2+/CD56+/SSTR2A+/ERG wk+. • FN/FGFR1 translocation in 50% of cases.
A B C E D Agaimy et al, AJSP, 2017
Phosphaturic mesenchymal tumors: Classical mixed connective Tissue type: most common. Soft tissue chondroma-like: uncommon. Agaimy et al, AJSP, 2017
A B C Phosphaturic mesenchymal tumors: angiomyolipoma-like D E F Agaimy et al, AJSP, 2017
D G A Uncommon variants: o Hemangiopericytoma-like o Chondromyxoid fibroma- like o Osteoblastoma-like o Giant cell granuloma-like H B E o Sarcomatous I F C Agaimy et al, AJSP, 2017
IHC+: Somatostatin receptor 2A CD56 CD56: 100% SATB2: 90% ERG: 90% SSTR2a: 79% Negative: S100, STAT6, betacatenin Agaimy et al, AJSP, 2017
Clinical examination is of atmost importance given that 50% of soft tissue PMTs are acral and small (usually not included in imaging) Any questions?
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