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CASE REPORT

Multiple skeletal metastases from a giant cell tumour of the distal fibula with fatal oucome

Giant Cell Tumor of Bone

Definition
rare aggressive non-cancerous (benign) tumor potential for : recurrence metastasis Statistically :

80% benign course, with a local rate of recurrence of 20% to 50%. 10% undergo malignant transformation at recurrence 1% to 4% give pulmonary metastases even in cases of

Epidemiology
5- 10% primary bone tumors
20 % benign bone tumors F : M 1,5 : 1

70- 80 % age 20 40
higher incidence rates in Asia,

20% of all primary bone tumors in China

Location
usually prefers the

epiphyses of long bones. half of the cases knee joint distal femur, proximal tibia, distal radius & proximal humerus. other sites: fibula, sacrum, & distal tibia

Diagnose
History age progessivity of tumor pain swelling

Clinical symptom:

hard and painfull mass


swelling joint effusion tenderness, muscle atrofi patologic fractur (11-37%)

neurogical deficit (sacrum)

Radiography :

o lytic lession
o radiolucent area at the end

of the bone with no firm boundaries. o transition zone between normal and pathological bone <1 cm. o ekstentrik cortex becomes thinner.

Scan : o improves detection of cortical thinning fracture, pathological, and periosteal reaction. o to accurately determine the location and soft tissue mass.
CT

MRI

o help determine extent of

tumor destruction o indicated when the tumor has eroded thorugh the cortex and allows determination of whether neurovascular structures are involved o help evaluate subchondral penetration

Angiography

Histology
macroscopic :

brown mass and soft haemorrhage and necrosis

microscopic : multinucleated giant cells mononuclear stroma (round / ovoid / spindle), large nuclei and indistinct nucleoli mitoses

Classification
Enneking (radiological, histological and clinical

classification) : stage I benign latent GCT (15%) stage II benign active GCT (70%) stage III locally aggressive tumors (15%) malignant sarcomatous lesion contiguous with benign GCT ( very rare)

Treatment
Principal : Excise the lesion Sterilize the cavity

Reconstruct the defect

Traditional Intralesional curettage & bone grafting Local recurrence rates 40-60%

Modern Adjuvant Treatment (Polymethylmethacrylate) PMMA

Liquid N2
Phenol

Cryotherapy
Irrigate cartilage Liquid nitrogen is a

chemical reagent used in cryotherapy

Enbloc Resection

reserved for expendable

bones : prox fibula / distal ulna high recurrence with other Tx stage III lesions

Reconstruction

CASE REPORT
in 1997

A 25-year-old woman with no significant past medical history

underwent curettage and grafting with spongiosa for a lesion at the lateral malleolus. Histological presence of a giant cell tumour. Malignancy was not suspected and recovery was uneventful.
in April 2002 the tumour recurred locally

treated again with curettage and grafting.


pathology laboratory : recurrent giant cell tumour

In Feb 2003

recurrent pain in the lateral aspect of her right ankle radiographs progressive destruction of the distal fibula recurrence of the giant cell tumour.

MRI a lesion in the distal fibula, 4 cm long and 3 cm wide.Extensive destruction of bone was localised to the area of the lateral malleolus.

Tx : EN BLOCK RESECTION histology : giant cell tumor with mononucleal cell (spindle) with mild

to moderat nuclear atypia and some mitosis

3 month normal ankle and chest

in Nov 2003

severe pain around the right iliac crest radiographs of the pelvic region, a whole body scan and a whole body CT scan, revealed multiple bony metastases.

no abnormality was detected in the viscera or the lungs

Histology : recurrence of the giant cell tumour. Tx : chemotherapy with radiotherapy she developed signs and symptoms of raised

intracranial pressure. Her condition deteriorated and she died in a state of epileptic shock.
In the absence of a post-mortem examination it is

not possible to be certain that there was no lung involvement.

DISSCUSSION
In this case, the mechanism of tumour spread is unclear,

particularly considering the lack of pulmonary metastases.


One possible explanation might be retrograde embolisation via

superficial veins.
Vessel destruction within the tumour does not necessarily indicate

a higher risk of metastases. About 40% of all GCT exhibit vessel destruction but very few of them develop metastases.

In this case, metastasis via the blood stream as

seen in other malignant tumours has to be assumed. In this patient, passage through the lung without pulmonary cell colonisation may have occurred. giant cell tumours show a tendency to local recurrence Recurrences after the treatment mostly caused by curettage and can reach up to 85%. To suppress recurrence rate, curettage thermal cautery using 5% phenol, bone cement or with liquid nitrogen are recommended. In this way, the number recurrence after curettage action can be suppressed up to 20%. The most frequent recurrence occurred within a period of 2-3 years post surgery.

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