Paediatric Bone Tumours

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Paediatric Bone Tumours

• William Aston
• Consultant Sarcoma and Joint Reconstruction
  Surgeon
• Royal National Orthopaedic Hospital
• Stanmore
• Paedriatric Revision Course 2009

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Paediatric Bone Tumours

• Diagnosis
• Staging
• Benign
• Malignant
• Treatment

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Paediatric Bone Tumours

• Diagnosis
• - History
• - Examination
• - Imaging
• At any stage Tertiary referral to MDT

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Paediatric Bone Tumours

• Staging - Characterise the lesion
• - Examination
• - X ray
• - U/S /- Angiogram
• - CT
• - MRI /- Whole Body
• - Bone Scan
• - PET

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Paediatric Bone Tumours

• Staging - Characterise the lesion
• - If MDT not sure of diagnosis then Biopsy
• Benign
• Malignant - Image whole compartment
• - Distant Staging
• CT Chest, Bone Scan, PET, Whole body MRI

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Classification of Tumours

• Enneking W F. A system of staging
  musculoskeletal neoplasms. Clin Orthop. 1986
  204 9-24.
• 1 Low grade
• 2 High grade
• 3 Any grade with metastases
• A Intracompartmental
• B Extracompartmental

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Considerations before biopsy

• Mankin H J, Lange T A, Spanier S S. The hazards
  of biopsy in patients with malignant primary bone
  and soft-tissue tumours. J. Bone and Joint Surg.
  1982 64-A 1121-27.
• Biopsy complication rate 3-5 times higher when
  performed by an inexperienced surgeon than by a
  member of the MSTS
• If the surgeon or institution is not equipped
  to investigate the patient appropriately, perform
  definitive surgery and administer adjuvant
  therapy then the patient should be referred to a
  treatment centre before biopsy

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Considerations before biopsy

• Open vs Needle Biopsy
• Always needle if possible
• In line of surgical incision
• Experienced Person
• Direct route
• Tract marking
• Dont contaminate other compartments

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The ideal biopsy

• - Adequate volume of representative tissue
• - Carefully planned site
• - Minimal contamination
• - Appropriately prepared specimen
• - Examined by expert pathologist

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Paediatric Bone Tumours

• Benign
• - Bone forming
• - Cartilage forming
• - Fibrous and Cystic tumours
• - Others

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Classification by anatomy
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Classification by cell type
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Behaviour

• Benign/latent
• Slow growth during normal growth of individual,
  then stop. Never become malignant. (Non-ossifying
  fibroma)
• Benign/active
• Progressive growth (Aneurysmal bone cyst)
• Benign/aggressive
• Locally aggressive but do not metastasize. There
  is a pseudocapsule with tumour extension into the
  reactive zone. Local control can only be achieved
  by complete removal of the lesion. (Giant-cell
  tumour)

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Paediatric Bone Tumours

• Benign Bone Forming
• - Osteoid Osteoma
• - Osteoblastoma

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Osteoid Osteoma

• MgtF
• Any bone- cortical
• Classic hx pain
• -Night pain
• -Relieved by salicylate
• Xray, bone scan, CT
• lt 1cm
• Rx by
• -Radiofrequency ablation
• -(Excision)

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Radiofrequency ablation
76 good response after 1 treatment 92 good
response after 2 treatments Safe NICE approval
March 2004
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Osteoblastoma

• 1 bone tumours
• MF 31
• Spine sacrum
• gt1cm
• Rx curettage
• -Saqlik et al 2007
• -20 pts
• -13 local recurrence
• Must exclude osteoblastic osteosarcoma

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Paediatric Bone Tumours

• Benign Cartilage Forming
• - Enchondroma
• - Osteochondroma
• - Chondroblastoma
• - Chondromyxoid fibroma

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Enchondroma

• Intramedullary lesion
• Hands feet
• Rx Curettage /- grafting

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Enchondroma - Olliers

• Multiple hereditary enchondromas
• 25-30 at chance of developing chondrosarcoma _at_
  40yrs
• Regular surveillance

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Enchondroma Maffuccis Syndrome

• Multiple enchondromata haemangiomas
• Risk of malignancy 25-100

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Osteochondroma

• Developmental malformation
• 90 solitary
• Distal femur, prox tibia, prox humerus
• Malignant transformation in 1
• MRI if suspicious- cartilage cap gt1cm thick
  malignant
• Excision if symptomatic or suspicious

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Multiple hereditary ostechondromas (diaphyseal
aclasis)

• Autosomal dominant
• Malignant transformation 0.5-3
• Regular surveillance
• N.B. pelvic lesions

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Chondroblastoma

• Rare
• 10-25yrs, MgtF
• 75 in long bones
• Epiphyseal
• MRI biopsy
• Rx curettage

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Chondromyxoid fibroma

• lt2 bone tumours
• Any site
• Eccentric, expansile
• Scalloped, eroded cortex
• Sclerotic margin 80
• Calcified 2
• Rx curettage
• 15 recurrence

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Paediatric Bone Tumours

• Benign Fibrous and Cystic
• - Fibrous Dysplasia
• - Non Ossifying Fibroma/ Fibrous cortical defect
• - Unicamral (Simple) Bone Cyst
• - Aneurysmal Bone Cyst

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Fibrous dysplasia

• Location
• 35 head
• 35 femur/tibia
• 30 ribs
• Monostotic or polyostotic (NB McCune-Albright Sy)
• Rx
• observation
• bisphosphonatess for pain
• osteotomy/internal fixation

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Osteofibrous dysplasia

• Tibia
• 10-15 yr olds
• Variant of FD
• Must distinguish from
• Adamantinoma
• Surgery if symptomatic

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McCune Albright Syndrome

• Polyostotic fibrous dysplasia
• Café au lait spots (coast of Maine)
• Endocrinopathy

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Fibrous cortical defect Non ossifying fibroma

• FCD lt 1 cm, NOF gt 1 cm
• Characteristic x-ray appearance
• Asymptomatic
• No treatment required

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Simple/unicameral bone cyst

• ? Caused by Inc. PGE2
• MF 21
• Prox femur prox humerus
• _at_ 25-50 will sustain pathological

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Treatment of SBC

• Observation until resolution

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Treatment of SBC

• Observation until resolution
• Fracture may stimulate resolution
• Aspiration steroid injection
• Aspiration bone marrow injection
• Curettage /- grafting
• Nancy nails

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Aneurysmal bone cyst

• Metaphyseal
• Max diameter of tumour gt max diameter of physis
• MRI Bx indicated
• - Must exclude telangiectatic osteosarcoma
• Embolisation curettage
• Occasionally requires excision

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Paediatric Bone Tumours

• Benign Others
• - Giant Cell Tumour
• - Langerhans Cell Histiocytosis

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Giant Cell Tumour

• Young adults FM 1.51
• Most commonly distal femur, prox tibia
• Osteoclast rich
• MRI biopsy indicated

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Eosinophilic Granuloma / histiocytosis X /
Langerhans cell histiocytosis

• Any bone
• Usually diaphyseal
• Bx to exclude Ewings sarcoma
• Rx chemotherapy

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Paediatric Bone Tumours

• Malignant
• - Osteosarcoma
• - Ewings Sarcoma (MRCT)
• Other tumours that present with bone lesions in
  Children

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Behaviour

• Malignant/Low-grade
• Low potential to metastasize. There is a
  pseudocapsule and tumour nodules exist within the
  reactive zone but rarely beyond. Local control by
  excision of normal cuff of tissue. (Parosteal
  osteosarcoma)
• Malignant/High-grade
• Rapid growth and early metastasis. Require local
  control and systemic therapy to prevent
  metastasis. (Osteosarcoma)

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Analysis of radiograph
AGGRESSIVE
wide transition, ill-defined margin

• margin
• pattern
• cortical response
• soft-tissue mass
• periosteal reaction
• matrix

moth-eaten, permeative
destroyed
often
spiculated, onion-skin, interrupted
/ - bone (OS) cartilage (CS)
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Osteosarcoma

• Conventional central OS
• Age 10-25 years
• Site
• -Distal femur
• -Proximal tibia
• -Proximal humerus
• -Pelvis
• -Spine
• -Jaw

• Histology
• Osteoblastic
• Chondroblastic
• Fibroblastic
• Mixed
• Osteoclast rich
• Telangiectatic

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CENTRAL OSTEOSARCOMA
CLASSICAL FEATURES Aggressive bone forming tumour
Mixed lytic/sclerotic Metaphyseal
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CENTRAL OSTEOSARCOMA
VARIANTS Densely sclerotic
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CENTRAL OSTEOSARCOMA
VARIANTS Lytic OS 10-13 cases
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Paediatric Bone Tumours

• Prognostic factors - Osteosarcoma
• Mets at diagnosis p gt 0.0001
• Axial Location p .005
• Necrosis lt90 p .001
• Raised Alk Phos p .002
• Proximal vs Distal p .002
• Amputation vs LSS p .04

William Aston
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Malignant Round Cell Tumours

• Age
• - Neuroblastoma - 1-5 years
• - Ewing/PNET - 5-15 years
• - 1o Bone Lymphoma - gt30 years
• Site
• - Femur, humerus, tibia, flat bones
• - Pelvis
• - Red Marrow distribution dependant
• Radiologically similar
• - Permeative bone destruction
• - Large ST mass

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EWINGS SARCOMA

• Described 1921 by James Ewing
• t1122 translocation
• Historical good response to R/T
• But systemic relapse
• Chemotherapy dramatically improved survival
• Prognostic factors
• Mets at diagnosis (especially bone)
• Systemically unwell (fever, raised ESR)
• Large tumours
• Axial tumours
• Poor response to chemotherapy

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MRCT

EWING SARCOMA Cortical saucerization
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MRCT

EWING SARCOMA Metadiaphyseal Periosteal reaction
-hair-on-end - onion skinning
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Ewings sarcoma cortex apparently intact
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MRCT

PELVIC EWING SARCOMA Affects older age group
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STAGING Incidence of metastases

• Osteo Ewings
• Bone Scan lt5 10
• CT Chest 10 25
• MRI - skip 5 2
• Incidence size ie. 5cm 5, 10cm 10

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Paediatric Bone Tumours

• Treatment
• Neo-adjuvant
• Surgical excision, ablation/salvage
• Adjuvant
• Os Adriamycin, Cisplatin, HDMTX, Ifosfamide
• Ewings Adriamycin, Ifos, Vincristine,
  etoposide, Actinomycin D
• /- Radiotherapy - margins
• Survival

William Aston
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Classification of Surgical Procedures by Margin

• Enneking
• Clin Orth 1980

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Paediatric Bone Tumours

• Salvage vs Ablation.
• - Neurovascular status.
• - Joint involvement.
• - Is it reconstructable after a wide margin?
• Autograft, Eg. Fibula, ECI
• Allograft
• Endoprosthesis
• - Function? If not ablative surgery

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Paediatric Bone Tumours

• Malignant tumours that present with bone lesions
• - Neuroblastoma
• - Leukimia
• - Lymphoma of bone
• - Clear Cell sarcoma of the Kidney (Wilms)
• - Retinoblastoma
• - Alveolar Rhabdomyosarcoma
• - Hepatoblastoma

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Paediatric Bone Tumours

• Questions

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Paediatric Bone Tumours

• Thank You
• Osteomyelitis
• DD Infection, Metabolic, Tumour