GCT case

GCT PROXIMAL FIBULA Dr Anton priyantha warnakulasuriya MBBS (sl) MS ( orth) 0resident nams :

GCT PROXIMAL FIBULA Dr Anton priyantha warnakulasuriya MBBS ( sl ) MS ( orth ) 0resident nams

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Clinical History Mr R . K.Upreti ,18 years old gentleman from Kavre DOA -24-12-2067 INPATIENT NO - 63440 DOD -26-01-2068 Presenting complain Pain over right upper leg-6 months Swelling over right upper leg -3months

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History of present complain Pain –Gradual onset , Localised over right upper leg , moderate, dull aching , progressive , , worse at night and increase with activity - for last 6 months Swelling - First notice 3 month back ,over lateral aspect of right upper leg , associated with pain , initially small in size and gradually increased over time , doesn’t disappear with knee movement, no other similar lump in the body No - fever ,night sweat or weight loss

Previous history :

Previous history No similar problem in past, no history of tuberculosis TREATMENT HISTORY Not on any long term medication FAMILY HISTORY not known any familial disease , good family support

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SOCIAL AND OCCUPATIONAL HISTORY Un married , Law student PERSONAL HISTORY Non alcoholic ,non smoker

Clinical examination :

Clinical examination GENERAL EXAMINATION Average build , Afebrile , NO- anemia ,jaundice ,cyanosis, clubbing or pedal edema No –multiple bone or joint deformity Cardiovascular , Respiratory , Abdominal and neurological examination revealed normal

LOCAL EXAMINATION :

LOCAL EXAMINATION Inspection Swelling over anterior aspect of right upper leg Globular in shape No dilated blood vessels , overlying skin is stretched Skin colour not changed over lump

LOCAL EXAMINATION cont…:

LOCAL EXAMINATION cont… PALPATION 7x5 cm size Globular shape lump local temperature not raised Mild tenderness present smooth surface Margin ill defined Boney hard lump Fixed to underlying bone skin over lump move freely in all direction Non Flactulant ,irreducible ,non pulsatile Right inguinal lymph nodes enlarged ,3 in number Right knee , ankle range of movement full Distal neurovascular status intact

IMAGING:

IMAGING X –RAY R/PROXIMAL LEG expansile lesion of the head of the fibula Lesion is osteolytic , radiolusent , lacking sclerotic margins No periosteal reaction present There is surrounding “egg shell” rim indicating the periosteum is intact. There are internal trabeculations within the lesion The entire head of the fibula is destroyed by the lesion

IMAGING cont.. :

IMAGING cont.. MRI OF RIGHT LEG Well defined heterogenous signal lesion with cystic and solid component involving epiphysis and meta physis of right fibula . There were no “fluid-fluid” levels that would indicate cystic changes. most likely of GCT proximal fibula

Imaging cont..:

Imaging cont .. CHEST-X-RAY – Normal findings CT CHEST – No abnormality detected ULTRASOUND SCAN ABDOMEN & PELVIS – Normal findings

Laboratory tests :

Laboratory tests TLC-6600 DLC-N-65%,L32%,E3% Hb 14.6% ESR-40 mm in first hour Glucose-87mg% Urea-22mg% Creatinine-1 Sodium-146 meq % Potassium 5.3 meq % Ca -9mg% Alkaline phosphatase -75u Total protein -6gm%, Albumin 4gm% urine RE - normal

Differential diagnosis :

Differential diagnosis The radiographic differential diagnosis Giant Cell Tumor Aneurysmal Bone Cyst Chondroblastoma Enchondroma Osteoblastoma Atypical Infection Desmoplastic Fibroma Non-ossifying Fibroma

Differential diagnosis cont..:

Differential diagnosis cont.. The radiographic studies support the diagnosis of a benign aggressive neoplasm. The lesion expands the bone and the periosteum appears to be intact and to contain the lesion. There is a sharp zone of transition between the tumor and normal bone Given the age, benign aggressive appearance, origin in the metaphysis and involvement of the epiphysis and lack of mineralization, the most likely radiological diagnosis is a Giant Cell Tumor of Bone .

Differential diagnosis cont..:

Differential diagnosis cont.. The lack of mineralization argues against a chondroblastoma , enchondroma and osteoblastoma although these lesions do not always demonstrate mineralization. The epiphyseal involvement suggests a chondroblastoma however this would be a very rare site for a chondroblastoma and chondroblastomas usually do not show internal trabeculations .

Differential diagnosis cont..:

Differential diagnosis cont.. The differential diagnosis of internal trabeculations includes desmoplastic fibroma , chondromyxofibroma , hemangioma , aneurysmal bone cyst, nonossifying fibroma and giant cell tumor. Desmoplastic fibroma is extremely rare and this would be an unusual age and location for a desmoplastic fibroma . This would also be an extrmely rare site for a chondromyxofibroma . Chondromyxofibromas also usually arise eccentrically from the bone and have a border that is very expansile and another border with an indolent appearance.

Differential diagnosis cont..:

Differential diagnosis cont.. Nonossifying fibromas are usually sharply circumscribed, arise eccentrically from the bone and do not expand and destroy the bone. This is also an unusual site for a nonossifying fibroma . Aneurysmal Bone Cyst : ABCs arise in this age group. This would be an unusual site and there were no “fluid-fluid” levels detected on the MRI which would be consistent with a primary or secondary ABC.

Differential diagnosis cont..:

Differential diagnosis cont.. Infections can be considered within the differential. TB and Fungal infections can present in an unusual manner such as this. However, the patient gave no history of exposure to tuberculosis and he had no fevers, night sweats and all blood tests were normal.(Except ESR) The key to an accurate diagnosis lies in the biopsy of the lesion.

INCISIONAL Biopsy:

INCISIONAL Biopsy 10-12-2067 The pathology demonstrated many giant cells dispersed amongst a sea of uniform mononuclear cells The nuclei of the mononuclear cells resembled the nuclei in the giant cells There was no evidence of ossification or calcification There was no matrix production There were no granulomas Cultures was negative Conclusive of Giant Cell Tumor

staging:

staging Enneking - stage I: - benign latent giant cell tumor Radiological grading ( campanacci ) Grade2 : -well defined margin cortex and rim of reactive bone are thin Expanded lesion ,but not perforated

Operation –wide local excision 07-01-2068 :

Operation –wide local excision 07-01-2068 14x9x2.5 cm tumor in proximal fibula involving perineous longus and soleus and externser digitorium Deep peroneal nerve was also involved. sacrificed during surgery

Problem encountered:

Problem encountered Abharent anatomy of vessel- branching of politeal artery was much more distal than expected ; it was close to tumor mass and it was very difficult to dissect the tumor mass. Artery injured , it was Repaired with 10 – 0 prolene

Specimen send for histopathological assessment :

Specimen send for histopathological assessment Specimen –mass of lateral aspect of right proximal leg Gross – single ,brownish tissue with muscle and bone measuring alltogether 14x9x2.5 cm Microscopy - Featurs are consistant with giant cell tumour

Post op period :

Post op period Distal pulsation present Patient developed Foot drop . Patient was able to walk with foot drop splint

Salient features :

Salient features 18 year old boy co- pain swelling of right upper leg for 3-6 months duration OE-7x5 cm size Globular shape hard lump arising from right proximal fibula . Xray , Incisional biopsy ,MRI suggestive of GCT CXR ,CT Chest –No secondary lesion in lung Wide local excision , Deep branch of common peroneal nerve sacrificed Excision biopsy- GCT Post operative foot drop Plan Regular folow up Tendon transfer for foot drop .

Thank you:

Thank you