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Multiple skeletal metastases from a giant cell tumour of the distal fibula with fatal oucome
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,
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:
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
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 :
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
Traditional Intralesional curettage & bone grafting Local recurrence rates 40-60%
Liquid N2
Phenol
Cryotherapy
Irrigate cartilage Liquid nitrogen is a
Enbloc Resection
bones : prox fibula / distal ulna high recurrence with other Tx stage III lesions
Reconstruction
CASE REPORT
in 1997
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
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
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.
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
DISSCUSSION
In this case, the mechanism of tumour spread is unclear,
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.
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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