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Referat

GIANT CELL TUMOR

Arranged by:

Elvina Indra Diva

(G0007062)

Guided by:

Dr. Tangkas Sibarani., Sp.OT, FICS.

THE CLINICAL DEPARTMENT OF SURGERY

MEDICAL FACULTY OF SEBELAS MARET UNIVERSITY

THE ORTHOPAEDIC HOSPITAL OF PROF. DR. SOEHARSO

SURAKARTA

2011

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LEGALITY SHEET

Orthopaedic surgery paper with the title Giant Cell Tumor is arranged to

fulfill the requirement of Clinical Department of Orthopaedic Surgery Sebelas Maret

University/ Dr. Moewardi Hospital during stage RSOP by:

Elvina Indra Diva

(G0007062)

Has been approved by the case presentation tutorial of the orthopaedic hospital

of Prof. Dr. Soeharso Hospital, Surakarta on August, 2011.

Consultant,

Dr. Tangkas Sibarani., Sp.OT, FICS.

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CHAPTER I

PREFACE

Bone cancer occurs when bone cells grow uncontrollably. Unlike normal cells,

cancer cells do not stop reproducing after they have doubled 50-60 times. These

abnormal cells form clumps of tissue, called tumors, inside bones. The first symptom

of bone cancer is typically pain in the affected bone(s). Sometimes, a bump either on

the bone or in the tissues surrounding the bone may be felt.

Primary bone cancer, or cancer that starts in the bone cells, is rare. Fewer than

2,500 Americans are diagnosed with this type of cancer each year. Children are more

likely to develop primary bone cancer than adults.

Most cases of bone cancer occur when cancer from another part of the body,

such as breast, prostate, or lung, has spread to bone cells. This is sometimes called

metastatic bone cancer or secondary bone cancer.

There are several types of bone cancer, such as osteosarcoma, chondrosarcoma,

Ewing's sarcoma and Giant cell Tumor. These cancers can be primary or secondary

cancer.

Surgery is often the main treatment for bone cancer. In addition to having bone

tumors surgically removed, patients may also undergo chemotherapy, and/or radiation

therapy. In some cases, patients may need to undergo a surgical amputation, but this

is performed less often today. Specific treatment options depend on the type of bone

cancer, as well as its location, size, and stage.

In general, the prognosis for patients with bone cancer is based on many factors,

including the type of cancer, at what stage the cancer was discovered, and where the

tumor is located. For instance, if the tumor is small and limited to a localized area, the

patient's prognosis is generally better than if the cancer has spread to other parts of

the body.

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CHAPTER II

Discussion

A. BackgroundGCTs of bone have been described as the most challenging benign bone tumors.

Although benign, GCTs show a tendency for significant bone destruction, local

recurrence, and occasionally metastasis. The natural history of GCTs varies widely

and can range from local bony destruction to local metastasis, metastasis to the lung,

metastasis to lymph nodes (rare), or malignant transformation (rare).

Gross specimen of a giant cell tumor that fills the entire distal radius. Despite

cortical disruption, the periosteum remains intact (arrow). Once again, note the blood-

filled cystic areas and areas of orange discoloration.

Approximately 3% of GCTs metastasize to the lung. The metastases appear as

clusters of GCTs located within the lung.

GCTs metastasis generally appear an average of 3-5 years after the initial

diagnosis of the primary lesion. However, GCTs metastasis may not be detected for

10 years or longer.

The natural history of these lung metastases is unpredictable. Pulmonary

metastases that spontaneously regress, remain stable, continuously grow slowly, or

rapidly progress have been reported.

B. DefinisionA giant cell tumor is one that is made up of a large number of benign (non-

cancerous) cells that form an aggressive tumor - usually near the end of the bone near

a joint. The location of a giant cell tumor is often in the knee, but can also involve the

bones of the arms and the legs, or the flat bones such as the sternum (breastbone) or

pelvis.

Giant cell tumors most often occur when skeletal bone growth is complete. Most

occur in the long bones of the legs and arms. They are most prevalent after age 20

and are rare after age 55. It is rare, but these tumors can occur in children. At any age,

they are more commonly seen in females than males.
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Approximately 50% of giant cell tumors are located around the knee. The most

common locations are the distal femur, the proximal tibia, and the proximal humerus

and distal radius.

Pain is the most common presenting symptom. Swelling and deformity are

associated with larger lesions. Soft-tissue extension is common. The incidence of

pathologic fracture at presentation is 11-37%.

The presence of tumor is the indication for surgery. See Treatment, below, for

detailed information.

E. The Symptoms of a Giant Cell TumorThe following are the most common symptoms of a giant cell tumor. However,

each individual may experience symptoms differently. Symptoms may include:

1.Pain at the adjacent joint2.A visible mass3.Swelling4.Bone fracture5.Limited movement in the adjacent joint6.Fluid accumulation in the joint adjacent to the affected boneThe symptoms of a giant cell tumor may resemble other medical conditions or

problems. Always consult your physician for a diagnosis.

F. DiagnosisIn addition to a complete medical history and physical examination, diagnostic

procedures for giant cell tumors may include the following:

1. Radiographically, these lesions are lucent and eccentrically located withinthe bone. GCTs can appear aggressive and are often characterized by

extensive local bony destruction, cortical breakthrough, and soft-tissue

expansion. When located in the epiphysis, GCTs generally extend to the

articular surface. Although radiographs of GCTs demonstrate a narrow zone

of transition, GCTs generally lack the dense peripheral sclerosis seen in

nonossifying fibromas. Mineralization of the primary lesion is rare.

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However, when GCTs occur in the soft tissue (metastasis or local

recurrence), peripheral calcifications are common.

Giant cell tumor. Anteroposterior radiograph of the distal femur reveals an

expansile lytic metaphyseal-epiphyseal lesion.

Giant cell tumor. Lateral radiograph of the same distal femur as in Image 2 in

Multimedia reveals an expansile lytic metaphyseal-epiphyseal lesion.
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2. A grade 2 lesion (active) has a relatively well-defined margin but noradiopaque rim, and the cortex is thinned and moderately expanded.

3. A grade 3 lesion (aggressive) has indistinct borders and corticaldestruction).

4. No correlation exists between the grading systems and the incidence oflocal recurrence or metastases.

MRI often is performed to delineate the extent of the neoplasm.

5. In the typical GCT, the signal intensity is homogeneous, and the lesionis well circumscribed.

6. The lesions have low signal intensity on T1-weighted images andintermediate signal intensity on T2-weighted images (see Image 6).

CT scans of the lesion reveal an absence of bone and intralesional

mineralization.

2. Radionuclide bone scans - a nuclear imaging method to evaluate anydegenerative and/or arthritic changes in the joints; to detect bone diseases and

tumors; to determine the cause of bone pain or inflammation. This test is to

rule out any infection or fractures.

3. Biopsy - a procedure in which tissue samples are removed (with a needle orduring surgery) from the body for examination under a microscope; to

determine if cancer or other abnormal cells are present.

G. HistopatologyOn gross inspection, these lesions are characteristically chocolate brown, soft,

spongy, and friable. Yellowish-to-orange discoloration due to hemosiderin may be

present. Cystic cavities within the tumor are common. Often, these cavities are blood

filled. Examination of resected specimen reveals a variable degree of cortical

expansion and disruption. Despite the cortical disruption, the periosteum remains

intact.

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Giant cell tumor. Anteroposterior radiograph of the distal tibia demonstrates

extension of the lesion to the articular surface.

Giant cell tumor. Lateral radiograph of the same distal tibia as in Image 7 inMultimedia demonstrates extension of the lesion to the articular surface.

Histologically, the lesions tend to be cellular. Although the multinucleated giant

cell is the characteristic cell type, these lesions have a background network of stromal

mononuclear cells. The mononuclear cells are plump and round, oval, or spindle

shaped. They may have prominent mitotic activity, but cellular atypia is rare. The

degree of mitotic activity has no prognostic significance.

Multinucleated giant cells, as the name suggests, have numerous centrally

located nuclei as opposed to the peripherally located nuclei ofLangerhans -type giant

cells seen in atypical infections. The nuclei tend to be compact and oval and contain

prominent nucleoli. These are similar in appearance to those of the surrounding

stromal cells, and the giant cell often appears to be a syncytium of these stromal cells.
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Giant cell tumor. Sagittal MRI of the same distal tibia as in Images 7-8 in

Multimedia demonstrates extension of the lesion to the articular surface.

Anteroposterior radiograph of a wrist arthrodesis performed for a giant cell

tumor. Soft tissue recurrence is present. Note the peripheral mineralization about thesoft-tissue recurrence (arrow).

Giant cells generally are distributed throughout the lesion. The concentration of

multinucleated giant cells varies considerably from tumor to tumor. Some tumors

have many multinucleated giant cells, whereas others have a few giant cells nestled in

swirls of spindle-shaped stromal cells. The concentration of multinucleated giant cells

is not related to the incidence of local recurrence or metastases. In some lesions, giant

cells invade the small perforating vessels. This intravascular invasion can be found in

approximately 5% of cases. This invasion, although appearing aggressive, is not

correlated with the prognosis.

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Sagittal T1-weighted MRI shows a giant cell tumor with low signal intensity.

Giant cell tumor. CT scan of the distal femur reveals an absence of matrix within

the lesion.

At histologic analysis, the differential diagnosis includes brown tumors of

hyperparathyroidism; aneurysmal bone cysts; and, rarely, chondroblastoma,

osteoblastoma, or osteosarcoma.

In an attempt to relate the histologic features with the clinical course, several

histologic grading systems have been developed. The earliest was devised by Jaffe et

al in 1940. In grade I at the benign end of the spectrum, giant cells are numerous,

mononuclear cells are rare, and mitotic activity is absent. In grade II, mononuclear

stromal cells are numerous, and moderate atypia and mitotic activity is seen. In grade

III, giant cells are few and small, atypia and pleomorphism are common, and mitotic

activity is frequent.

However, this grading system has no prognostic significance. In an attempt to

improve the prognostic relevance of the histologic grading system, several authors
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have modified the staging system of Jaffe et al. Generally, these staging systems

include sarcomatous lesions as grade III lesions. Unfortunately, these modified

systems, like that of Jaffe et al, are of little value in predicting patient outcomes.

H. TreatmentSpecific treatment for giant cell tumors will be determined by your physician

based on:

1.your age, overall health, and medical history2.extent of the disease3.your tolerance for specific medications, procedures, or therapies4.expectations for the course of the disease5.your opinion or preferenceThe goal for treatment of a giant cell tumor is to remove the tumor and prevent

damage to the affected bone. Treatment may include:

1. Medical TherapyPulmonary metastases have been cited as the cause of death in 16-25% of

reported cases. The need for early detection and treatment of these metastases has

been emphasized. Pulmonary metastases have been treated with wide resection,

chemotherapy, radiation therapy, and interferon alpha. When possible, wide surgical

resection is the treatment of choice.

When the pulmonary metastases cannot be completely surgically excised,

adjuvant treatment, such as chemotherapy or radiation therapy, has been advocated.

In addition, in situations when the metastases are unresectable, both chemotherapy

and radiation have been used as solitary agents. At University of Texas MD Anderson

Cancer Center, interferon has been used with promising results.

Spontaneous malignant transformation of GCT is not uncommon. Malignant

transformation has been defined as a sarcoma associated with a benign typical GCT at

presentation or as a sarcoma arising at the site of a preexisting GCT. Malignant

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transformations have resulted in osteosarcoma, fibrosarcoma, or malignant

histiocytoma. Periods of 4-40 years for malignant transformation have been reported.

2. Surgical TherapyIn the past, GCTs were treated with amputation or with wide resection and

reconstruction. However, with the knowledge that GCT is a locally aggressive yet

benign disease, the surgical treatment of GCTs is intralesional for most locations.

Various treatment options have been advocated, including the following:

a.Curettageb.Curettage and bone graftingc.Curettage and insertion of polymethylmethacrylate (PMMA)d.Primary resectione.Radiation therapyf.Embolization of the feeding vessels

3. ResectionAlthough intralesional procedures remain the treatment of choice for most GCTs,

wide en bloc resection offers the lowest recurrence rate and can be performed in

expandable bone. In the proximal fibula, wide resection without reconstruction is

often performed. Similarly, GCTs of the distal radius often are resected and

reconstructed with autograft or allograft.

Intraoperative photograph of giant cell tumor in the distal femur.

However, in the long bones, resection necessitates prosthetic or allograft

reconstruction and is generally reserved for grade III lesions.
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Photomicrograph of a giant cell tumor reveals the typical appearance.

Multinucleated giant cells are dispersed throughout on a background of mononuclear

cells.

The liquid nitrogen is left in the cavity until it all evaporates. The surrounding

tissues are irrigated with warm sodium chloride solution in an attempt to prevent or

minimize thermal injury to the surrounding tissues. The process is repeated 2-3 times,

resulting in cellular death at a depth of approximately 1-2 cm. The cavitary defect is

then reconstructed with PMMA or bone grafts.

Recurrence rates with cryosurgery have been reported to be 2-12%. The

disadvantages of cryosurgery include the need for wide exposure, the need to protect

the soft tissues, skin necrosis, osteonecrosis, and fracture. Fracture is the mostcommonly reported and gravest complication.

Malawer et al noted that internal fixation with Steinmann pins and reconstruction

of the cavitary defect with PMMA significantly reduced the incidence of fracture and

suggested that all patients who undergo cryosurgery receive internal stabilization as

well.
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Photomicrograph of a giant cell tumor reveals the typical appearance.

Multinucleated giant cells are dispersed throughout on a background of mononuclear

cells.

Some authors, as an alternative to cryosurgery and phenol therapy, have

advocated argon-beam coagulation. It lacks the application hazards identified with

both phenol and liquid nitrogen. Thermal coagulation applied through a concentrated

argon gas is used to paint the tumor cavity.

Photomicrograph of a giant cell tumor reveals prominent mitotic activity and rare

cellular atypia.

The penetration is approximately 2-3 mm. Recurrence rates for this procedure

when paired with PMMA have been reported at 7%. No acute complications were

noted. Long-term follow-up is warranted to assess the effect of argon beam

coagulation on joint and/or subchondral physiology and on the incidence of

pathologic fracture.

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CHAPTERIII

Conslusion

Giant cell tumor accounts for 5 to 9 percent of all primary bony tumors. It

possibly is the most common bone tumor in the young adults aged 25 to 40. Giant

cell tumor is are found more commonly in women than men, and occur most often

during the third decade.

Most patients present with slowly progressive pain, with or without a mass.

Symptoms arise when the lesion begins to destroy the cortex and irritate the

periosteum or when the weakening of the bone caused by the tumor causes pain due

to imminent pathologic fracture. Some giant cell tumors present with a pathologic

fracture.

Radiologic findings demonstrate the lesion is most often eccentrically placed to

the long axis of the bone. The center is most radiolucent with increasing density

towards the periphery.

Treatment of giant cell tumors is by medical therapy, surgery, resection,

Intralesional procedures, adjuvant therapies.

.

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REFERENCES:

Balke M, Schremper L, Gebert C, et al. (March 2008). "Giant cell tumor of bone:

treatment and outcome of 214 cases".J. Cancer Res. Clin. Oncol.

Dickson, B. C., Li, S.-Q., Wunder, J. S., Ferguson, P. C., Eslami, B., Werier, J. A., et

al. (2008). Giant-cell tumor of bone express p63. Modern Pathology

Thomas, D. M., & Skubitz, T. (2009). Giant-cell tumour of bone. Current Opinion in

Oncology

Valerae O Lewis, MD; Chief Editor: Harris Gellman, MD.2011. Giant Cell

Tumor.www.emedicine.medscape.com

Lesley-Ann Goh, MBBS, FRCR; Chief Editor: Felix S Chew, MD, MBA,

EdM.2011.Giant Cell Tumor Imaging. www.emedicine.medscape.com

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