Bizarre Parosteal Osteochondromatous Proliferation
Synonyms: BPOP, Nora lesion
Cytological atypia mimics malignancy but behaviour is Benign
Quick Facts
Behaviour
Benign
Category
Bone
Grade
Not set
Synonyms
- BPOP
- Nora lesion
Category
Bone
Behaviour
Benign
Gender
Both equally
Tissue of Origin
Bone
Epidemiology
- Rare reactive/neoplastic lesion of Bone surface
- Wide age range, peak 3rd–4th decade
- No sex predilection
- Often associated with prior trauma
Clinical Features
- Painful swelling on Bone surface
- Rapid growth raising concern for malignancy
- No systemic symptoms
- May recur locally after excision
Location
- Small Bones of hands and feet most common
- Distal phalanges of fingers
- Rarely long Bones or skull
Imaging
- Pedunculated or sessile surface lesion
- Mineralised matrix (calcified Cartilage and Bone)
- No cortical destruction
- CT shows dense mineralisation arising from cortical surface
Pathology
- Bizarre Cartilage with hypercellularity and nuclear atypia
- Mineralising Fibrous tissue
- 'Blue' Cartilage (basophilic myxoid matrix)
- No continuity with medullary canal (unlike osteochondroma)
Genetics
- t(1;17)(q32-42;q21-23) translocation reported in some cases
- COL6A3-USP6 fusion described
- Uncertain whether neoplastic or reactive
Treatment
- Local excision - marginal or intralesional
- High recurrence rate (50–55%) - multiple excisions may be required
- No Malignant transformation reported
Prognosis
- Benign but locally aggressive with High recurrence rate
- No metastatic potential
- Repeated excisions may be needed
Key Points
- Cytological atypia mimics malignancy but behaviour is Benign
- Lack of medullary continuity distinguishes from osteochondroma
- USP6 rearrangement links it to nodular fasciitis (reactive vs neoplastic debate)
- Recurrence in >50% after simple excision
Workup - Blood Tests
FBC, U&E, LFTs - pre-operative baseline
Workup - Local Imaging
- Plain radiograph - mineralised surface lesion; no medullary continuity
- CT primary site - characterises mineralisation, confirms no cortical destruction
- MRI - if soft tissue extension or malignancy suspected
Workup - Biopsy
- Excision biopsy preferred - complete removal diagnostic and therapeutic
- Histology: 'blue Bone' (basophilic Cartilage), hypercellular with nuclear atypia, no necrosis
- USP6 FISH - rearrangement supports diagnosis in atypical cases
- Exclude parosteal osteosarcoma: MDM2/CDK4 FISH negative in BPOP
Workup - Staging
No staging required
Workup - Other
MDT discussion if imaging ambiguous - exclude parosteal osteosarcoma before excision
Follow-up Summary
- Post-op visit at 6 weeks; plain X-ray + CT of primary site as new baseline
- Year 1–2: 6-monthly clinical review + plain X-ray of affected site
- If recurrence detected (>50% rate): re-excision; continue annual surveillance
- Discharge at 3–5 years if no recurrence
- No systemic metastatic risk - no chest imaging required
- Patient education: advise that multiple local excisions may be necessary
- Red flags: rapid regrowth at excision site, new mass within months of surgery
- High recurrence rate after excision; active surveillance required