Adamantinoma & Osteofibrous Dysplasia

 

AdamantinomaAdamantinoma 2

Osteofibrous dysplasia or Adamantinoma? Require adequate biopsy

 

Osteofibrous dysplasia

 

Definition

 

Rare, benign, self-limited fibro-osseous lesion

Anterior cortex of tibial diaphysis +/- fibula

Young child - first two decades of life

 

Clinical

 

Swelling

Pain

Anterior bow

Pathological fracture

Incidental finding

 

X-ray

 

Multiple small radiolucent lesions surrounded by sclerosis

Thickened cortex

 

Doesn't progress on xray

 

Adamantinoma tibiaOFD tibia

 

DDx

 

#Adamantinoma#

 

FOGMACHINES / Lucent lesion

- Fibrous dyplasia

- Osteofibrous dysplasia / Ossifying fibroma / Osteosarcoma

- Giant cell tumour

- ABC

- Chondrosarcoma

- Infection / osteomyelitis

- Simple bone cyst

 

Diaphyseal lesion / HALFEE

- Histiocytoma

- Adamantinoma

- Lymphoma

- Fibrous Dysplasia / OsteoFibrous Dyplasia

- Eosinophilic granuloma

- Ewings

 

MRI

 

OFDys xray 1OFdys xray 2OFD MRI

 

OFD tibiaOFD MRI 2OFD MRI 1

 

Biopsy

 

Exclude adamantinoma

 

Sampling errors

- need adequate tissue to differentiate between osteo-fibrous dypslasia and adamantinoma

- typically open and extensive biopsy

 

Natural history

 

Progression of the lesion halts with skeletal maturity

 

Management

 

Observation

 

Sweet et al Am J Surg Pathol 1992

- 30 cases of osteofibrous dysplasia

- none progressed to adamantinoma

 

Dala-Ali et al Bone Joint J 2022

- 101 cases of tibial osteofibrous dysplasia in 99 patients

- presented with pain / swelling / fracture

- 40% had radiological deformity (>10 degrees bowing)

- no malignant transformation

 

Surgery

 

Indications

- progressive deformity

- fracture

 

OFD xray 1OFD MRI 1OFD MRI 2

 

Options

- currettage

- wide excision

 

Results

 

Lu et al Orthop Traumatol Surg Res 2023

- osteofibrous dysplasia of the tibia

- 4/6 treated with currettage and allograft had local recurrence

- 1/9 treated with currettage / allograft / elastic nails had local recurrence

- 0/5 treated with wide resection and bone transport had local recurrence

- longer healing rates and increased complications with bone transport

 

Adamantinoma

 

Adamantinoma 1Adamantinoma 2

Biopsy proven adamantinoma

 

Definition

 

Rare low-grade malignant tumour

 

Clusters of epithelial cells surrounded by bland spindle cell osteofibrous component

 

Epidemiology

 

Usually second or third decade of life

 

Site

 

90% diaphysis of tibia 

 

Mandible

 

Symptoms

 

Swelling

Pain

Pathological fracture

 

X-ray

 

Most common anterior cortex of tibia

 

Soap-bubble appearance

 

Eccentrically located

- well circumscribed

- slightly expansile

- cortical thickening

- little or no periosteal reaction

 

MRI

 

Khanna et al Skeletal Radiol 2008

- 24 cases of OFD / adamantinoma

- adamantinomas tend to be larger / cortical destruction / complete marrow involvement

 

DDx

 

#Osteofibrous Dysplasia#

 

FOGMACHINES / Lucent lesion

- Fibrous dyplasia

- Osteofibrous dysplasia / Ossifying fibroma / Osteosarcoma

- Giant cell tumour

- ABC

- Chondrosarcoma

- Infection / osteomyelitis

- Simple bone cyst

 

Diaphyseal lesion / HALFEE

- Histiocytoma

- Adamantinoma

- Lymphoma

- Fibrous Dysplasia / OsteoFibrous Dyplasia

- Eosinophilic granuloma

- Ewings

 

Pathology

 

Two distinctly different components / biphasic

 

1.  Epithelial origin 

- composed of squamous cells and epithelial pearls 

 

2.  Mesenchymal origin 

- composed of immature mesenchymal cells and spicules of dysplastic bone

 

Natural History

 

Schutgens et al JBJS Am 2020

- 190 cases of adamantinoma

- 16% pathological fracture

- local recurrence after wide resection 24%

- metastatic disease in 18%

 

Management

 

Wide excision

 

Chemo and radiotherapy ineffective

 

Wide resection

- address bone loss

- vascularised fibular graft / allograft / bone transport / prosthesis

 

Prognosis

 

Qureshi et al JBJS Am 2000

- multicentred retrospective review of 70 cases

- average age 31, more common in males

- wide operative margins associated with lower recurrence rates

- 87% 10 year survival

 

Aytekin et al J Oncol 2020

- 92 cases of adamantinoma

- 5 year survival 99%

- 10 year survival 92%