Chondroid Origin

(En)Chondroma

Definition

  • Chondroma: a benign intramedullary neoplasm consisting of mature hyaline cartilage ▸ it is commonly centrally located (and then referred to as an enchondroma)

  • It is the 2 nd commonest benign chondral lesion (after an osteochondroma)

Clinical Presentation

  • An incidental finding ▸ a pathological fracture (60%) ▸ pain in the absence of a fracture or a rapid size increase is potentially malignant

  • Age: 10–80 years (M = F)

Location

The tubular bones of the hands (40–65%): proximal phalanges > metacarpals or middle phalanges ▸ femur, tibia and humerus (25%) ▸ small bones of the feet (7%)

  • A metaphyseal or diaphyseal location (rarely located within the epiphysis) ▸ often eccentric

  • It is not found within the skull (it is only found within bones that are formed by endochondral ossification)

Radiological Features

XR

A well-defined oval or lobulated eccentric lytic lesion ▸ cortical expansion and chondral (popcorn) type calcification ▸ the overlying cortex may be thin and scalloped ▸ a narrow or sclerotic zone of transition ▸ no periosteal reaction (unless fractured) ▸ a solitary lesion in 75%

  • ‘Reverse Madelung’ deformity: a short distal ulna

MRI

Lobular margin ▸ T1WI: intermediate SI ▸ T2WI: high SI (due to the water content of hyaline cartilage) ▸ punctate areas of signal void (matrix mineralization) ▸ there is a hypointense rim with septations ▸ T1WI + Gad: septal enhancement

Scintigraphy

No increased uptake

Pearls

  • Differentiation between a large enchondroma + grade 1 chondrosarcoma can be difficult ▸ lesion size >5–6 cm and deep endosteal scalloping are suggestive of chondrosarcoma

Periosteal chondroma

Rare and occurs in children and young adults ▸ it affects the long bone metaphyses (proximal humerus ▸ femur, tibia and the tubular bones of the hands and feet) ▸ no malignant potential ▸ the differential includes a periosteal chondrosarcoma/periosteal osteosarcoma

  • XR: a 1–3 cm well-defined area of cortical erosion ▸ a mature periosteal reaction ▸ chondral-type calcification (50%) ▸ rarely a thin external shell of bone is present

  • MRI: T2WI: a lobulated high SI mass adjacent to but not infiltrating the underlying cortex

Ollier's disease

Characterized by multiple enchondromas ▸ sporadic (and not inherited) ▸ usually unilateral ▸ bone growth may be impeded (with bowing or angulation)

  • Malignant change (5–30%) ▸ an associated risk of a glioma, pancreatic or ovarian carcinoma

Maffucci's syndrome

A rare condition characterized by multiple enchondromas and soft tissue haemangiomas (phleboliths) ▸ unilateral in 50%

  • Malignant change (20%) ▸ usually > 40 years

Enchondroma. AP radiograph of the index finger showing a lobular, mildly expansile lesion with typical chondral matrix mineralisation (arrow). **

AP XR of a proximal phalangeal enchondroma with associated pathological fracture (arrow). *

Sagittal T2WI MRI of a distal femoral chondroma – calcification is manifest as focal areas of signal void. *

Periosteal chondroma. (A) Lateral XR showing a calcified surface lesion. (B) Axial fat-suppressed T2WI showing a hyperintense lobulated lesion, without medullary infiltration. *

Chondromyxoid Fibroma (CMF)

Definition

A benign tumour composed of immature myxoid mesenchymal tissue with early cartilagenous differentiation

Clinical Presentation

  • Pain and swelling (it can be asymptomatic)

  • 75% of patients present between 10 and 30 years of age (75%) ▸ (M : F, 2 : 1)

Location

60% are found within the long bones (25% are within the upper of the tibia) ▸ 40% are found within the flat bones (10% within the ilium) ▸ 17% are found within the small tubular bones of the hands and feet ▸ metaphyseal and eccentric in the medulla

Radiological Features

XR

An eccentric lobulated metaphyseal lytic lesion arising within the medulla ▸ it can cross into the epiphysis ▸ an associated well-circumscribed sclerotic border with thinning and expansion of the cortex ▸ the lesion long axis parallels the bone long axis ▸ periosteal reaction or soft tissue extension is uncommon ▸ matrix calcification in seen in 12% of cases

MRI

There are no characteristic features

Scintigraphy

Increased uptake

Pearl

Differential: chondrosarcoma ▸ adamantinoma ▸ fibrous dysplasia ▸ ABC

Chondromyxoid fibroma of the distal femoral metaphysis. (A,B) Large osteolytic defect bulging outward. *

Chondromyxoid fibroma. Lateral radiograph of ankle shows a well-defined, eccentric lytic lesion arising eccentrically within the distal tibial metaphysis. ©35

Chondromyxoid fibroma. Lateral XR showing expansion of the anterior cortex. *

Osteochondroma

Definition

  • A cartilage-capped exostosis (representing a bony outgrowth) ▸ usually a developmental anomaly ▸ it possesses its own growth plate and will stop growing with skeletal maturity

  • The most common benign bone lesion

Clinical presentation

  • It presents between 2 and 60 years (M : F, 2 : 1)

  • It can present with mechanical problems: an enlarging mass ▸ pressure on any adjoining structures (e.g. nerves or blood vessels) ▸ bursa formation (due to irritation of local soft tissues)

    • Rarely the stem can fracture

Location

The long bones – especially around the knee (35%) ▸ the proximal humerus or femur ▸ the flat bones (e.g. the ilium or scapula)

  • Lesions are initially metaphyseal but migration to the diaphysis occurs over time (classically pointing away from the joint)

Radiological features

XR

A continuous bony outgrowth from a normal cortex ▸ it can have a long slim neck (pedunculated) or be broad based (sessile) ▸ the marrow cavity extends into the exostosis (essential feature for diagnosis) ▸ the cartilage cap develops punctuate calcification with age

US/CT/MRI

These allow assessment of the cartilage cap – it should be < 5 mm (T2WI: high SI)

Scintigraphy

There will be variable activity

Pearls

  • Differential: periosteal chondroma ▸ parosteal osteosarcoma

  • Chondrosarcomatous change within the cartilage cap (occurring in <1% of cases) should be suspected if:

    • There is an increase in pain or size (particularly after skeletal maturation)

    • If the cartilage cap measures >1 cm (CT) or >2 cm (MRI)

    • The development of ill-defined margins

Diaphyseal aclasia (multiple hereditary exostosis)

A rare autosomal disorder with multiple osteochondromas occurring particularly at the ends of the long bones, ribs, scapulae and iliac bones (they may be larger than in the solitary form) ▸ this may lead to shortened or deformed limbs (e.g. a reverse Madelung deformity with a short distal ulna) ▸ the metaphyses can be widened and dysplastic

  • Vertebral involvement is rare ▸ the cranial vault is spared ▸ malignant degeneration in 3–5%

Bizarre parosteal osteochondromatous proliferation (BPOP)

  • A rare, tumour-like disorder

  • Calcified masses arise adjacent to the cortex of the small bones of the hands and feet – there is no continuity between the lesion and the underlying bone ▸ there is no cartilaginous cap but it can resemble an osteochondroma

  • Differential: soft tissue chondroma ▸ florid reactive periostitis

Dysplasia epiphysealis hemimelica (Trevor's disease)

Irregular overgrowth of part of an epiphysis which lies on one side of a single limb (resembling an osteochondroma) ▸ the leg is commonly affected

Osteochondroma. MRI features. (A) Axial PDW FSE MRI through the proximal tibia showing medullary continuity (arrow) between the osteochondroma and host bone. The cartilage cap is mildly hyperintense and surrounded by a thin, hypointense perichondrium (arrowheads). **

Diaphyseal aclasia demonstrating the typical femoral neck modelling deformity (asterisks), a small osteochondroma arising from the left superior pubic ramus (arrow) and malignant transformation of a left iliac wing osteochondroma (arrowhead). A coin was left in the right pocket.

Osteochondroma. AP XR showing a typical pedunculated osteochondroma. *

BPOP. AP XR showing a bizarre parosteal osteochondromatous proliferation adjacent to the middle phalanx. *

Chondroblastoma

Definition

A benign cartilage tumour with proliferation of immature cartilage cells (1% of all bone tumours)

Clinical presentation

  • 80–90% occur between 5 and 25 years (M : F, 3 : 1)

  • It presents with joint pain (± a synovial reaction) ▸ commonly presents as a monoarthropathy (typically located in the epiphyses and provoking a synovial reaction)

Location

It is usually seen in skeletally immature patients, affecting the long bone epiphyses (40% are seen around the knee, and 33% within the proximal femur)

  • Also found within apophyses and sesamoid bones (epiphyseal equivalents) such as the femoral greater trochanter and patella (the commonest patella tumour)

  • It usually affects the calcaneus or talus in the foot

  • The flat bones are mostly affected if >30 years old

Radiological features

XR

An eccentric spherical or lobular lytic lesion with a fine sclerotic margin centred within the epiphysis (40%) ▸ it extends into the metaphysis (55%) with partial closure of the growth plate

  • Matrix mineralization (30%) ▸ linear periosteal reaction (30–50%)

MRI

T1WI: intermediate SI ▸ T2WI: variable SI including fluid levels due to secondary aneurysmal bone cyst change (15%) ▸ there is associated marrow and soft tissue oedema ▸ there may be a reactive joint effusion

Pearl

An ‘aggressive’ (atypical) chondroblastoma

A rare variant associated with cortical destruction and soft tissue involvement ▸ rarely associated with lung metastases

  • Differential of a lytic epiphyseal lesion:

    • Children: Brodie's abscess

    • Adults: subchondral cyst ▸ clear cell chondrosarcoma

Chondroblastoma in the immature and mature skeleton. (A) AP XR showing a lobulated, lytic lesion (arrow) adjacent to the open growth plate and limited to the epiphysis. (B) AP XR showing extension of the lesion across the fused growth plate. *

Chondroblastoma of the left femoral head. Coronal fat-suppressed T2WI showing the hypointense lesion with surrounding marrow oedema and reactive joint effusion. *

Osteoid Origin

Osteoblastoma

Definition

  • A benign bone tumour producing osteoid and woven bone ▸ it is histologically similar to an osteoid osteoma, but is differentiated by its size (> 1.5–2 cm) ▸ tends to show a more aggressive growth pattern ▸ does not resolve spontaneously

Clinical Presentation

  • Any pain is usually more chronic and less severe than with an osteoid osteoma (and is rarely relieved by aspirin) ▸ there can be a painful scoliosis (with tumour at the concavity apex)

    • 80% of cases present at < 30 years of age (M : F, 2–3 : 1)

Location

40–50% are within the spine and sacrum – 90% are eccentrically located within the neural arch with a painful scoliosis (± expansion or absence of the pedicle) ▸ potential extraosseous extension

  • Other major sites: the diaphyses or metaphyses of the long bones (commonly the humerus)

Radiological Features

XR

A predominantly lytic lesion (>2 cm) ▸ larger lesions have greater matrix mineralization and may expand the bone with surrounding reactive sclerosis

CT

This often reveals occult calcification (punctate/nodular/generalized)

MRI

Reactive marrow and soft tissue changes (which may extend across several vertebrae) dominate ▸ there may also be secondary ABC changes ▸ T1WI: low or intermediate SI ▸ T2WI: intermediate or high SI ▸ T1WI + Gad: enhancement

Scintigraphy

This is always positive

Pearls

  • An osteoblastoma may produce an extracortical mass causing spinal cord compression with a soft tissue component

  • ‘Aggressive osteoblastoma’: a locally aggressive lesion which is commonly within the sacrum and capable of producing metastases

  • Differential (long bones): Brodie's abscess ▸ chondromyxoid fibroma ▸ Langerhans' cell histiocytosis

Osteoblastoma. (A) AP radiograph of the left proximal humerus showing a large mixed lytic-sclerotic lesion (arrows) in the medullary cavity with associated periosteal thickening. (B) Coronal CT MPR shows the oval, mineralised lesion (arrows). (C) Coronal STIR MRI demonstrates a hypointense tumour (arrows) with extensive reactive oedema-like marrow changes (arrowheads). **

Bone Island (Enostosis)

Definition

  • A congenital developmental focus of medullary cortical bone

Clinical Presentation

  • An asymptomatic incidental finding

Location

It is commonly seen within the pelvis, femur and other long bones

Radiological Features

XR

A dense sclerotic focus with a spiculated margin blending with the trabeculae of adjacent bone

MRI

It is hypointense on all sequences (equivalent to cortical bone)

  • Unlike a sclerotic metastasis, it will tend to have straight edges and the very dense bone can cause adjacent susceptibility artefacts

Scintigraphy

There is usually no uptake (25% of giant bone islands show increased uptake)

Osteopoikilosis and osteopathia striata

An autosomal dominant condition with multiple bone islands located in a periarticular distribution ▸ they can appear either round (osteopoikilosis) or elongated (osteopathia striata) in shape

Giant bone island. AP XR showing a uniformly sclerotic bone island (arrow) in the supra-acetabular region of the ilium. *

Osteopoikilosis. †

Osteoid Origin/Tumours of Neural Tissue

Osteoma

Definition

  • A slow-growing tumour consisting of predominantly cortical or, less often, cancellous bone ▸ it represents a dysplastic developmental anomaly

Clinical Presentation

  • It is usually asymptomatic, although it can affect sinus drainage (leading to mucocoele formation) ▸ it can also cause CSF rhinorrhoea, pneumocephalus or meningitis

Location

Cortical (ivory) osteomas commonly affect the paranasal sinuses (frontal and ethmoid > sphenoid sinuses) ▸ it is less often found within the mandible, long bones or spine (causing backache)

Radiological Features

XR/CT

A homogeneous smooth dense lesion with a well-defined spherical margin ▸ it is attached to underlying bone ▸ it rarely exceeds 2–3 cm diameter

Pearl

  • Multiple osteomas occur in Gardner's syndrome

Osteoma. (A) Plain XR. (B) Coronal STIR images underestimate the lesion since bone and air are both black. (C) Axial CT shows extension into the orbit. *

Osteoid Osteoma

Definition

  • A benign hamartoma affecting the cortex (80%), medullary cavity or subperiosteum ▸ it produces osteoid and woven bone

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