Agaimy , Case #6 49 yo male with disability for 5 yrs. Lab.: low - - PowerPoint PPT Presentation

▶
agaimy case 6 49 yo male with disability for 5 yrs lab
SMART_READER_LITE
LIVE PREVIEW

Agaimy , Case #6 49 yo male with disability for 5 yrs. Lab.: low - - PowerPoint PPT Presentation

Mar 30, 2024 33 likes •204 views

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

slide-1
SLIDE 1
  • 49 yo male with disability for 5 yrs.
  • Lab.: low serum phosphate & high urine phosphate.
  • Serum FGF23 significantly elevated.
  • No obvious cause detected.

Agaimy , Case #6

slide-2
SLIDE 2
  • Left hand thenar mass detected on clinical examination and excised.
  • Disability resolved gradually within 6 mo.
  • 3 yrs later no evidence of disease.
slide-3
SLIDE 3

Eosinophilic cells within microcystic stroma

slide-4
SLIDE 4

Microcystic stroma Focal myopericytoma-like vessels

focal myopericytoma-like vessels

slide-5
SLIDE 5

grungy (dirty) matrix (bluish)

slide-6
SLIDE 6
slide-7
SLIDE 7

characteristic weak ERG

slide-8
SLIDE 8

SATB2

slide-9
SLIDE 9
  • 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.

Case #11 in this series

slide-10
SLIDE 10
slide-11
SLIDE 11

A C E D B Agaimy et al, AJSP, 2017

slide-12
SLIDE 12

Agaimy et al, AJSP, 2017

Phosphaturic mesenchymal tumors: Classical mixed connective Tissue type: most common. Soft tissue chondroma-like: uncommon.

slide-13
SLIDE 13

A B C E F D

Agaimy et al, AJSP, 2017

Phosphaturic mesenchymal tumors: angiomyolipoma-like

slide-14
SLIDE 14

A B C F E D G H I

Agaimy et al, AJSP, 2017

Uncommon variants:

  • Hemangiopericytoma-like
  • Chondromyxoid fibroma-

like

  • Osteoblastoma-like
  • Giant cell granuloma-like
  • Sarcomatous
slide-15
SLIDE 15

Somatostatin receptor 2A CD56

Agaimy et al, AJSP, 2017

IHC+: CD56: 100% SATB2: 90% ERG: 90% SSTR2a: 79% Negative: S100, STAT6, betacatenin

slide-16
SLIDE 16
slide-17
SLIDE 17

Any questions?

Clinical examination is of atmost importance given that 50% of soft tissue PMTs are acral and small (usually not included in imaging)