CASE 28
By: Dr. Khalid Abdullah AL-Ismail MD
Describe your findings?
Name the most likely deferential diagnosis?
Label the arrowed structure?
How can you suggest the best diagnosis taking in considerations the pt. info?
FINDINGS:
Radiograph:
Lateral X-ray of the tibia.
Skeletally immature pt.
Eccentric, intracortical, expansive osteolytic lesion, mainly involving and deforming with bowing of the anterior cortex diaphysis and encroaching the proximal and distal metaphysis.
No pathologic fracture or soft tissue extension.
No periosteal reaction.
Thick surrounding rim of sclerosis.
MRI:
Trans axial T1: iso-signal to muscles intracortical lesion with low signal septae. minimal intramedullary canal narrowing however No edema.
Trans axial T2 FS: Moderate bright T2 signal of the above described lesion.
Sag. T1 FS post Gd enhancement showing the heterogeneous pattern of the soft tissue component. No adjacent soft tissue involvement.
DIFFERENTIAL DIAGNOSIS:
Mono-stotic fibrous dysplasia.
Non-ossifying fibroma.
Adamantinoma.
Brown tumor of hyperparathyroidism.
DIAGNOSIS:
Osteofibrous dysplasia.
Anatomy:
Nutrient artery a branch of posterior tibial artery.
Enters the tibia at the origin of the soleus muscle along the oblique line of the tibia.
The nutrient artery of the tibia has three ascending branches and one descending branch. The distal aspect of the tibia is supplied by periosteal anastomoses that enter the bone adjacent to the ankle joint.
Supporting features:
Age of the patient usually less than 10 years compared to Adamantinoma group usually older age.
Less aggressive histopathologic features compared to Adamantinoma with no sarcomatous transformation.
No frequent association with soft tissue mass, periosteal reaction or early pathologic fractures compared to Adamantinoma.
Cortical involvement compared to FD that mainly affect the medullary region.
Expansion towards outer cortex compared to NOF that expand towards the medullary canal.
Normal bone density compared to brown tumor that show multiplicity of the disease and sub-periosteal resorption.
PEARLS AND DISCUSSION:
Osteofibrous dysplasia is a rare, non-neoplastic, non-inherited developmental tumor-like fibro-osseous condition of unknown etiology that have near-exclusive predilection for the tibial diaphysis. It frequently is asymptomatic. Often called as Campanacci syndrome.
Younger age group of children aged 1-5 years with no sex preponderance.
Originally described by Frangenheim in 1921and is named by many terms such as congenital fibrous dysplasia and ossifying fibroma of the long bones.
Biopsy is not usually indicated unless features of adamantinoma or aggressive appearance are seen.
Once aggressive features are confirmed surgery is highly recommended.
A diagnosis of classical adamantinoma is suggested by an extensive lesion with moth-eaten margins and complete involvement of the medullary cavity on axial MR imaging. Misdiagnosis on needle biopsy may occur in up to one fifth of cases, and radiological features can assist in making the correct diagnosis.
FURTHER READING:
R. S. Lee, S. Weitzel, D. M. Eastwood, F. Monsell, J. Pringle, S. R. Cannon, T. W. R. Briggs. Osteofibrous dysplasia of the tibia is there a need for a radical surgical approach? DOI: 10.1302/0301-620X.88B5.17358 Published 27 April 2006
Soo Bong Hahn, Sung Hun Kim, Nam Hoon Cho,Chul Jun Choi, Bom Soo Kim, and Ho Jung Kang.Treatment of Osteofibrous Dysplasia and Associated Lesions
Khanna M, Delaney D, Tirabosco R, Saifuddin A. Osteofibrous dysplasia, osteofibrous dysplasia-like adamantinoma and adamantinoma: correlation of radiological imaging features with surgical histology and assessment of the use of radiology in contributing to needle biopsy diagnosis. Skeletal Radiol. 2008 Dec;37(12):1077-84. doi: 10.1007/s00256-008-0553-1. Epub 2008 Aug 9.
