Giant Cell Tumours & Aneurysmal Bone Cysts - PowerPoint Presentation

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Giant Cell Tumours & Aneurysmal Bone Cysts

Miss S Rinkoff

Slide2

Aims and Objectives

For both GCTs and ABCs to cover:

Background

Signs and Symptoms

Imaging

Histology

Treatment

Slide3

Giant Cell Tumours

Benign but aggressive

Mostly in epiphysis of long bones (can extend to metaphysis)

50% located around the knee

Occasionally occur in the vertebrae (2% to 5%) and the sacrum (10%)

Can be locally destructive, demonstrate local recurrence and can metastasise

20 -30 years old

W>M

In the US and Europe:

5% of all primary bone tumours

21% of all benign bone tumours

In China:

20% of all primary bone tumours

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Signs and Symptoms

Pain

Swelling in the area of the tumour

Decreased motion in the adjacent joint

An effusion or pathologic fracture

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Metastasis

Metastasise to the lung

Rarely malignant de novo

The metastases appear as clusters of GCTs located within the lung

M

etastases appear 3-5 years later

Overall prognosis is good

Pulmonary metastasis cause of death in 16-25%

Local recurrence determined by the adequacy of surgical removal

Malignancy after local recurrence

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Bertoni et al (1998)

Malignant GCT cases

17 patients:

5 primary malignant GCTs

12 secondary malignant GCTs

½ secondary GCTs occurred after radiation

Mean age:

1

◦

: 67 years

2

◦

: 40 years

Typical benign GCT: 20 and 30 years.

They concluded that malignancy associated with GCTs is always high grade with a poor prognosis

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Radiographic Appearance

GCTs appear lucent

Eccentrically located

Aggressive with extensive local bony destruction, cortical breakthrough, and soft-tissue expansion

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Grading

Campanacci

grading system is based on radiographic appearance:

Grade 1 lesion (latent): well-defined margin and an intact cortex

Grade 2 lesion (active): relatively well-defined margin but no radiopaque rim, and the cortex is thinned and moderately expanded

G

rade 3 lesion (aggressive): indistinct borders and cortical destruction

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Grading

Jaffe:

Grade I (completely benign)

Grade II (borderline)

Grade III (frankly

sarcomatous

).

In general, grades I and II do not correlate well with biologic behaviour.

Poor correlation between the histologic pattern and the tendency for recurrence or malignant transformation.

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MRI

MRI can be

used to

delineate the extent of the neoplasm.

Homogeneous signal

W

ell circumscribed lesion

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Macroscopic Appearance

Chocolate brown, soft, spongy, and friable

Cystic cavities are common

Variable degree of cortical expansion and disruption with intact periosteum

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Histological appearance

Spindle-shaped and ovoid cells

Multinucleated giant cells.

Osteoid product

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Treatment

Surgical resection

Resection is curative in 90%

Curettage = recurrence rate: 40% to 75%

Johnson and

Dahlin

(1,2)

reported a recurrence rate of 29% within 1 year of curettage and of 54.1% within 5 years.

O’Donnell et al.

(3)

reported an overall recurrence rate of approximately 40%

Johnson EJ,

Dahlin

DC. Treatment of giant cell

tumor

of bone: an evaluation of 24 cases treated at the Johns Hopkins Hospital between 1925-1955.

Clin

Orthop

1969;62:187. 100.

Johnson EJ,

Dahlin

CD. Treatment of giant cell

tumor

of bone.

J Bone Joint

Surg

Am 1959;41:895.

O’Donnell RJ, Springfield DS,

Motwani

HK, et al. Recurrence of giant-cell

tumors

of the long bones after curettage and packing with cement.

Bone Joint

Surg

Am 1994;76(12): 1827.

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Treatment

Primary resection of a joint has a significant morbidity

Amputation is reserved for massive recurrence, malignant transformation, or infection

Curettage is usually curative

Decreased the rate of local recurrence

Bone graft and PMMA (polymethacrylate) for reconstruction

A Canadian

multicenter

study

(1)

of 186 cases shows no difference in function, health status, or recurrence rate whether cement or bone graft was used after curettage

The heat given off by the hardening PMMA is thought to lead to thermal necrosis

Turcotte

RE,

Wunder

JS, Isler MH, et al. Giant cell

tumor

of long bone: a Canadian Sarcoma Group study.

Clin

Orthop

2002;397:248

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Treatment

O’Donnell et al

(1)

:

Massachusetts General Hospital

60 patients with GCTs treated by curettage and packing with PMMA

Overall rate of local recurrence was 25% (15 of 60 patients), occurring at an average of 4 years.

Risk factors for local recurrence were pathologic fracture, stage III disease, anatomic site, and the use of adjuvant treatment.

The distal radius and the proximal tibia had the highest rate of local recurrence

O’Donnell RJ, Springfield DS,

Motwani

HK, et al. Recurrence of giant-cell

tumors

of the long bones after curettage and packing with cement.

Bone Joint

Surg

Am 1994;76(12): 1827.

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Other treatment options

Radiation

GCT is not

radioresistant

Should only be considered an adjuvant to surgery or alternative therapy in cases of GCT that are unresectable or in which excision would result in substantial functional deficits

Denosumab

June 2013, the FDA approved denosumab for the treatment of unresectable GCT

Approval was based on positive results from two open-label trials involving 305 patients

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Other treatment options

Cryosurgery

Effective in eradicating the

tumor

while preserving joint motion and avoiding resection or amputation.

Bisphosphonates

Surgical adjuvant

May reduce the rate of giant cell

tumor

recurrence by inducing apoptosis

Target

osteoclastlike

giant cells, and the autocrine loop of

tumor

osteoclastogenesis

RANK-L

Blocking the RANK-L pathway

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Case discussion

7376036

7368576

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Aneurysmal Bone Cysts

Benign tumorlike lesion

"an expanding osteolytic lesion consisting of blood-filled spaces of variable size separated by connective tissue septa containing trabeculae or osteoid tissue and osteoclast giant cells."

 

Most common in 2

nd

decade

Locally aggressive

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Signs and symptoms

Pain

Swelling

Deformity

Disruption of growth plates or joint surfaces

Neurologic symptoms

Pathological fractures

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Background

Jaffe and Lichtenstein first described ABC as its own entity in 1942

“Soap-bubble" appearance

Jaffe and Lichtenstein suggested that ABCs may have been mistaken for other benign and malignant bone

tumors

in the past

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Anatomy

Long tubular bones is most common site

Plus spine and the flat bones =80%

Eccentrically located in the metaphysis

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Pathophysiology

Theories about vascular malformations

Vascular lesions then cause increased pressure, expansion, erosion, and resorption of the surrounding bone

Local haemorrhage causes reactive osteolytic tissue

t(16;17)(q22;p13) fusion of the 

TRE17/CDH11-USP6

 oncogene

Increased cellular cadherin-11 activity that seems to arrest osteoblastic maturation in a more primitive state

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Prognosis

Generally excellent

Recurrence is the biggest problem

Overall cure rate is 90-95%

 

 

Increased risk of recurrence:

Younger age

Open growth plates

Metaphyseal location

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Imaging

Eccentric

Less commonly, central or subperiosteal lesion

Cystic or lytic

Blown-out, ballooned, or soap-bubble appearance

Eggshell-appearing bony rim

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Imaging

Capanna

et al:

Type I – Central metaphyseal presentation

Type II – Entire segment of bone

Type III – Eccentric metaphyseal location; no or minimal expansion of the cortex

Type IV – Subperiosteal extension

Type V –

Metadiaphyseal

location; inflation of periosteum toward the soft tissues; penetration of the cortex; extension into cancellous bone

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Imaging

MRI can distinguish ABCs from telangiectatic osteosarcoma (TOS

TOS: thick, nodular enhancement of tissue surrounding the cystic spaces

TOS may also demonstrate necrosis

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Histology

Blood-soaked sponge

A thin subperiosteal shell of new bone surrounds the structure and contains cystic blood-filled cavities

Brownish intertwining septa

Proliferative fibroblasts

Spindle cells

Areas of osteoid formation

Multinucleated giant cells: “pigs at the trough” formation

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Treatment

Intralesional curettage

Intralesional excision

En

-bloc resection or wide excision

Curettage with locally applied adjuvants such as liquid nitrogen, PMMA, argon beam photocoagulation, or phenol

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Medical Treatment

Angiography

Reach difficult locations

Save joint function

Complications associated with invasive surgery (

eg

, bleeding) less likely to occur

Intralesional injection

Calcitonin

 

and methylprednisolone injections

ETHIBLOC (Ethicon, Norderstedt, Germany) injection is also performed under CT guidance and

anesthesia

 

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References

https://emedicine.medscape.com

DeVita

, Hellman, and Rosenberg's Cancer:

Principles and Practice of Oncology

(Cancer: Principles & Practice (

DeVita

)(2 Vol.)) Hardcover – 1 May 2008

Imaging of Bone

Tumors

and

Tumor

-Like Lesions: Techniques and Applications

Editors: Davies, A. Mark, Sundaram, Murali, James, Steven J. (Eds.)

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Any Questions?