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Table 3 Differential diagnostic features

From: Chondromyxoid fibroma-like osteosarcoma: a case series and literature review

Characteristic

CMF-OS

CMF

LGCO

CHS

COS

Age (years)

All ages

All ages, preferably < 30

30–40

>  40

10–14 and > 40

Site

Craniofacial part; femur

Proximal tibia, distal femur; ilium

Distal femur, proximal tibia

Bones of the pelvis; proximal femur, proximal humerus, distal femur; rib

Distal femur, proximal tibia, proximal humerus

Location in the long bone

Variable

Metaphysis; eccentric in long bones

Metaphysis, diametaphysis

Metaphysis or Diaphysis; Centric in long bones

Metaphysis

CR

poorly defined osteolytic, osteogenic or expansive lesion

Well-defined osteolytic lesion

Large, lytic coarsely trabeculated lesion with a focal aggressive feature

Osteolytic lesion with endosteal scalloping; cortical thickening

Large, poorly defined, mixed lytic and blastic mass

CT

Cortex destruction

Sclerotic margin

Cortex destruction; periosteal reaction

Mineralization; cortex erosion or destruction

Mineralization, ossification; cortex destruction; periosteal reaction

MRI

Soft tissue mass; Soft tissue and bone marrow oedema

Peripheral nodular enhancement; no bone marrow or soft tissue oedema

Soft tissue extension; difficult to distinguish from COS

Ring-and-arc enhancement; soft tissue oedema

Soft tissue mass; soft tissue and bone marrow oedema

Histology

Loose aggregates of cells supported in a highly myxoid stroma; Neoplastic bone

Lobular pattern with stellate or spindle-shaped cells in a myxoid background

Hypocellular to moderately cellular fibroblastic proliferation with variable amounts of osteoid production; neoplastic bone

Abundant blue-grey cartilage matrix production; atypical chondrocyte varying in size and shape with enlarged, hyperchromatic nuclei

Neoplastic cells with severe anaplasia and pleomorphism; neoplastic bone

Immunohistochemistry

Vimentin (+); S100 (−)

S100 (+); collagen II, SOX9 (+) in the centre of lobules

MDM2, CDK4 (+)

20% IDH1(+) with IDH R132H antibody

osteocalcin, osteonectin, S100, action, SMA, NSE, CD99 (+); Factor VIII, CD31, CD45 (−)

Genetic

None

Chromosome 6 aberration

Gain or amplification of MDM2 (12q13–15)

IDH1 mutation

Multiple numerical and structural chromosomal aberrations

  1. CMF-OS Chondromyxoid fibroma-like osteosarcoma, CMF Chondromyxoid fibroma, LGCO Low-grade central osteosarcoma, CHS Chondrosarcoma, COS Conventional osteosarcoma