Sarcopedia

BenignBone

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