Bone tumor

Chondroblastoma
Essential features
• Rare (< 1% of primary bone neoplasms)
• Peaks in second decade of life, with slight male predilection
• Common location includes epiphysis of long bones > metaphysis; may involve skull
in older patients

• Sheets of chondroblasts admixed with osteoclast-like giant cells and eosinophilic

chondroid matrix; pericellular chicken wire type calcification may be present

• K36M mutations commonly identified

 CASE: 14 y/o male who presented with h/o pain in left
knee joint

A sharply defined oval lucent lesion is

centered on the epiphysis of the distal
femur, and appears to transgress the
growth plate (which remains open).

It has a narrow zone of transition and no

convincing matrix calcification. A joint
effusion is present. No periosteal reaction is
present.

This case demonstrates typical

appearances of a chondroblastoma, in a
typical location.

The lesion was curetted and the diagnosis

was confirmed on histolopathologic
plain film examination
 CT confirms the plain film appearance of the previous image, revealing a sharply demarcated
epiphyseal lucent lesion but with faintly sclerotic margins.

It transgresses the growth plate into the anterior part of the metaphysis.

There is no periosteal reaction.

The presence of a lucent lesion involving both sides of an open epiphyseal plate (as seen here) is
diagnostic of chondroblastoma
CT confirms the plain film appearance of the previous image, revealing a sharply demarcated
epiphyseal lucent lesion but with faintly sclerotic margins.

It transgresses the growth plate into the anterior part of the metaphysis.

There is no periosteal reaction, however there does appear to be a cortical breach

anterosuperiorly into the knee joint.
No matrix calcification or extra-osseous soft tissue component can be demonstrated
 sheets of
histiocyte-like
tumor cells in the
center

pink areas of
chondroid
differentiation
The tumor cells have scant amount of clear or pink cytoplasm with well-defined
cytoplasmic borders.
Some tumors are composed of epithelioid cells with abundant pink cytoplasm
 The nuclei of tumor cells in chondroblastoma may be oval, indented or lobulated and may
have longitudinal grooves giving rise to coffee-bean appearance.
Some may resemble the nuclei of Langerhans� cells.
Mitotic figures may be occasionally seen in chondroblastomas; however, atypical mitoses are never found.
This image shows 2-3 recognizable mitotic figures
 Sheets of chondroblasts

Sheets of polygonal cells with defined cell borders and eosinophilic cytoplasm.
Chondroblastomas usually contain many multinucleated giant cells.
This should not lead to the mistaken diagnosis of a giant cell tumor
 About one-third of chondroblastomas show some calcification.
The calcification is delicate, lace-like and is deposited between degenerating tumor cells
(chicken-wire calcification
• Besides mononuclear cells, chondroblastoma may also contain a spindle
cell component.
• Foci of delicate calcification are also present in this image.
 Chondroblastoma
• Chondroblastoma is a benign tumor of cartilaginous derivation seen most often in
the 2nd decade of life with a male predominance.

• The lesion is usually centered on the epiphyseal region at the end of long tubular
bones.

• Proximal humerus, proximal tibia, and distal femur are the most commonly
involved locations.

• Local pain is frequently the presenting symptom

• The treatment of chondroblastomas is curettage with or without bone graft.

• Local recurrences can usually be treated by repeat curettage.

• Prognosis is good.

• Rare cases of benign chondroblastoma metastasizing to the lung have

been reported. Most of these cases occurred after surgical treatment of
the primary tumor.
Chondroblastoma of the acromion process of the scapula in a 28 y/o
male

The bone is infiltrated by a whitish tumor resembling cartilage.

The tumor penetrates the cortical bone and invades the soft tissue.
This is a recurrent lesion 2 years after curettage.
• Local recurrence in long bone lesions is approximately 10% and is higher
for chondroblastomas arising in flat bones, especially those lesions arising
in the vicinity of the triradiate cartilage
 This is a case of recurrence in soft tissues of the toe

Chondroblastomas that recur in soft tissues tend to be well-circumscribed.

 Most local recurrences can be treated by repeated curettage

Well-circumscribed tumor
nodules can be seen in the
subcutaneous tissues.
 Higher magnification of the previous image

image shows bluish foci of delicate lace-like calcification, benign giant cells, spindle cell
proliferation, and scattered mononuclear cells
Chondroblastomas that recur in soft tissues often have a shell of ossification as shown
in this image
Higher magnification of the mononuclear cells in a recurrent chondroblastoma

•The cytoplasm stains pink and is

well-defined.

• Many of the nuclei show grooves

or have a cleaved appearance.

•Mitotic figures are easily found

in chondroblastomas but are not
abundant.

• Atypical mitotic figures are never

seen
Positive stains
• H3.3B (H3F3B) p.Lys36Met (K36M): diffuse nuclear staining may be
helpful to distinguish from aneurysmal bone cyst.

Molecular / cytogenetics description

• K36M mutations commonly identified within H3F3B and rarely in H3F3A
An 18 year old man presents with a 3 cm, well defined lucent lesion within the epiphysis
of the distal femur. Microscopic features of the surgical curettage specimen are
represented by the H&E images. The best diagnosis is

A. Chondroblastoma
B. Chondroblastoma-like osteosarcoma
C. Chondromyxoid fibroma
D. Giant cell tumor of bone
Which of the following is a feature of chondroblastoma?

A. Aggressive lesion with an infiltrative growth pattern

B. Commonly arises in the epiphysis and metaphysis of long bones
C. Generally shows a high degree of cytologic atypia
D. Lacks a pericellular chicken wire calcification

answer #2
B. Commonly arises in the epiphysis and metaphysis of long bones.

Chondroblastomas show pericellular chicken wire calcification and generally display

well demarcated borders and a lesser degree cytologic atypia when compared with
chondroblastoma-like osteosarcoma
Differential diagnosis:

• Chondromyxoid fibroma:
– Metaphyseal, myxoid with pseudolobular pattern with pleomorphic stellate cells
– Negative for K36M

• Giant cell tumor:

– Metaphyseal or epiphyseal in patients with closed epiphysis (third decade of life), clustered
giant cells that are larger and more numerous and uniformly distributed than
chondroblastoma, no chondroid matrix or chicken wire matrix
– Negative for K36M

• Aneurysmal bone cyst:

– Primary: multiloculated, blood filled, destructive, cystic lesion; USP6 gene rearrangements
– Secondary: can be associated with chondroblastoma, giant cell
tumor, osteoblastoma and osteosarcoma

• Chondroblastoma-like osteosarcoma:
– Histologically, chondroblastoma-like osteosarcoma is characterized by monotonous,
minimally to moderately atypical rounded cells with ovoid nuclei resembling
chondroblastoma and abnormal osteoid deposition with destruction of the bone
– Negative for H3K36M
 Giant cell tumor
Essential features
• Bone tumor with compatible imaging
• Osteolytic circumscribed tumor involving the epiphysis and metaphysis,
generally in skeletally mature individual

• Mononuclear cell neoplastic component without atypia together with

numerous osteoclasts

• Detection of H3.3 pGly34 mutated cells / H3.3 pGly34Trp (G34W)

immunoreactivity.
Giant cell tumor

Plain radiographs will show an expanding lytic

lesion in an epiphyseal location extending to
the articular cartilage.

Usually there is no surrounding sclerosis or

periosteal reaction.

Destruction of the cortex and extension into

soft tissue may be seen
 Giant cell tumor of the bone involving radius - a rare presentation.
a 50-year-old man, had no lesions in the other bones

It is normally seen at
the ends of the long
bones and it is purely
lytic (radiolucent) in
most cases.

The above example is accompanied by secondary aneurysmal bone cyst and prominent
periosteal new bone formation making a radiological diagnosis almost impossible.
• The most commonly involved locations include ends (epiphyses) of long
bones - distal femur, proximal tibia and distal radius.

• Roughly half of all cases occur around the knee joint.

• Sarcum is another common location.

• The peak incidence of giant cell tumor is seen in the 3rd decade of life with
a female predominance.
The image shows a typical case with numerous multinucleated giant cells scattered in a
background of uniform mononuclear cells.
The giant cells are believed to arise from the fusion of these mononuclear cells.
 Another typical case of a giant cell tumor of the bone.
The nuclei are round to oval and surrounded by dense eosinophilic cytoplasm. Due to the
similarity of their nuclei, it may be difficult to discern where mononuclear cells end and giant
cells begin
Mitotic figures are quite common in giant cell tumors of the bone and have no prognostic
significance.
However, there should be no abnormal mitotic figures.
It is not uncommon to see 50 to 70 nuclei within giant cells
Giant cell tumors usually do not produce matrix, except when they extend into soft tissues or
metastasize to lungs.

Rarely, there may be abundant reactive bone formation with the potential of misdiagnosis as
osteosarcoma
 Rare cases of giant cell tumors have abundant spindle cells and may be mistaken for
fibrosarcoma.

However, the giant cell tumors lack nuclear atypia and are more cellular than the low-grade
sarcomas
 A focus of spindle growth pattern in a giant cell tumor.
Scattered giant cells as well as numerous hemosiderin laden macrophages are seen.
Giant cell tumor is perhaps the most common bone tumor associated with secondary
aneurysmal bone cyst. In rare instances, such areas predominate.
• Treatment of giant cell tumors is usually curettage followed by filling the
defect with bone chips.

• For destructive lesions, total resection or even amputation may be

required.

• Recurrences occur in about 20% cases, sometimes after many decades.

• Malignant transformation (to osteosarcoma or fibrosarcoma)

and metastases to lungs are other rare complications.
This image from a giant cell tumor shows aggregates of foam cells and a spindle cell
proliferation.

The typical areas of giant cell tumor may sometimes be inconspicuous. Such cases
may be misdiagnosed as fibrous histiocytomas.

The location of the tumor and its radiographic appearance will be helpful clues to the
correct diagnosis.
 Infarct-like necrosis is quite commonly seen in giant cell tumors.
Some tumors may be completely necrotic. Usually, there is no inflammatory response.
Ghost outlines of multinucleated giant cells will still be visible and helpful in the diagnosis
Giant cell tumor arising in the sphenoid bone. This is an exceedingly rare location.
Note the respiratory epithelium on the top.