Volume 2 | Issue 2 | May-Aug 2016 | Page 23-25 | Pankaj Panda1, Ashish Gulia1
Authors: Pankaj Panda, Ashish Gulia
Orthopedic Oncology Services, Department of Surgical Oncology, Tata Memorial Hospital, Mumbai
Address of Correspondence
Dr Ashish Gulia, MS (Ortho), Mch (Surgical Oncology)
Associate Professor, Orthopedic Oncology, Dept. of Surgical Oncology, Tata Memorial Hospital, Mumbai – 400012, India.
Introduction: Osteofibrous dysplasia (OFD) is a rare, benign, self-limiting, fibro-osseous lesion occurring in long bones especially of lower limbs. Patients typically presents with painless swelling with or without anterior bowing of tibia .The diagnosis can be confirmed by peculiar radiological feature of well defined intracortical lytic lesion with variable degree of osteolysis and osteosclerosis. Admantinoma is close differential of this lesion . Most cases regress spontaneously by puberty , surgical intervention is required only for progressive lesions or in case of pathological fracture .
Keywords: Osteofibrous Dysplasia , Management
Osteofibrous dysplasia (OFD) is a rare, benign, self-limiting, fibro-osseous lesion occurring in long bones especially of lower limbs. It is also called as Kempson-Campanacci lesion or cortical fibrous dysplasia. The prominence of the osteoblasts led Kempson in 1966 to describe the entity as ossifying fibroma of the long bones . In 1976, Campanacci, gave the term “osteofibrous dysplasia of the tibia and fibula” in reference to its histological features, developmental origin and anatomic location .
Etiopathogenesis – Exact etiology is not known. A few of the cases are known to have occurred in families. It has also been reported that OFD may act as a precursor of adamantinoma which is supported by occurrence of OFD like adamantinomas. The evidence for this is limited and most of the cases are considered to be arising spontaneously.
Incidence – OFD is a rare benign self-limiting tumor, which accounts for about 0.2% of all primary bone tumors . These lesions are mainly seen in the first two decades of life. It is very uncommon after skeletal maturity with any gender predilection .
Site – It is invariably a disorder of the tibia and fibula. The lesion usually has its epicenter in anterior cortex of tibia. Tibial mid-diaphysis and proximal metaphysis are affected the most. Ipsilateral or contra lateral fibula may be involved. Even though most of the lesions are confined to a limited portion of the bone a few may grow rapidly and involve almost entire bone. Isolated fibular involvement is rare. Forearm bones (radius and ulna) are other uncommon sites of affection .
Clinical features – The typical presentation is painless swelling with or without anterior bowing of the tibia. Pain is only present in about one third of the cases and is usually due to pathological fracture. About a third of the cases are detected incidentally .
Radiograph – The lesion appears as a well defined intracortical lytic lesion, with variable degree of osteolysis and osteosclerosis located in the anterior cortex of the tibia. These lesions may present as a single focus or multiple elongated foci interspersed with reactive bone. The overlying cortical shell presents itself in a wavy pattern giving it a “saw tooth appearance”. Most of the lesions are associated with anterior tibial bowing and buttress type of benign periosteal reaction. Aggressive lesions may involve entire diaphysis and metaphysic and may have associated pathological fractures [7, 8].
Computed Tomography – It is helpful in assessing exact extent of the lesion, cortical involvement, periosteal reaction and pathological fractures and acts as an adjuvant to MRI in the overall assessment of the lesion.
Magnetic resonance imaging – MRI helps in delineating the cortical based lesion and to assess its medullary or soft tissue extension. The lesion demonstrates mixed signals on T1 and high intensity lesions on T2 weighted images. MRI is helpful in surgical planning and differentiating OFD from adamantinoma .
On gross examination, a typical specimen appears as a whitish or yellowish solid lesion with surrounding gritty bony architecture. The cortex may be expanded and thinned out deficient at places with intact periosteum. Lesions may show medullary extension, which is usually demarcated by a sclerotic rim .
Microscopically, OFD demonstrates a zonal architecture with loose fibrous tissue containing spicules of woven bone in the centre which is lined by a layer of lamellar bone lined by prominent osteoblasts at the periphery. This shows a progressive maturation of the bone trabeculae from a central zone of delicate trabecular bone in a vascular fibrous stroma, to an outer zone of lamellar bone. The fibrous component in most cases contains cells which react positively for pan-cytokeratin. Desmosomes, tonofilaments, and microfilaments are seen on electron microscopy [2, 11].
Several tumor and tumor like lesions can mimic Osteofibrous dysplasisas on radiographs . The differential diagnoses are that of a cortical, lytic, expansile lesion. Adamantinoma is the most closest differential diagnosis as both lesions are very similar clinico- radiologically and even on histopathology. Adamantinomas are more aggressive lesions and may lead to local and distant recurrences. These commonly involve the medullary cavity, but there is usually cortical infiltration, break and soft tissue component. Other differentials include Fibrous dysplasia, Nonossifying fibroma, Aneurysmal bone cyst, Chondromyxoid fibroma, Langerhans cell histiocytosis, Osteomyelitis and Hemangioendothelioma . A thorough clinico-pathological correlation substantiated with characteristic radiological findings is very essential for a definitive diagnosis of OFD.
According to the case series on OFDs from the Rizzoli Institute in Milan and the Mayo Clinic, these lesions, owing to their benign nature, seldom progress during childhood and undergo spontaneous regression at puberty, thus can be carefully observed with serial plain radiographs and clinical evaluation at regular intervals. If associated with significant or progressive bowing then conservative treatment in the form of bracing may be helpful to minimize deformity and prevent pathological fracture [5, 14].
Surgical intervention is mainly required in extensive cases with progressive deformity or for pathologic fracture. Extraperiosteal “shark-bite” excision is the most widely considered surgical option for OFDs. The resultant defects may be reconstructed with auto or allo-strut grafts. Other surgical interventions may include curettage bone grafting and internal fixation after correction of deformity. .
Prognosis – OFD has a very good prognosis. Most of the lesion even though they grow in first decade of life get stablised during the second decade and heal by spontaneous resolution. Deformities may persist for a longer time and may remodel slowly. Aggressive lesions may have severe deformity or pathological fracture, which usually heal well with surgical intervention. Excisions are mostly curative. A few lesions may progress to OFD like adamantinoma or adamantinoma and require aggressive treatment accordingly [3, 6].
1. Campanacci M, Olmi R. Ossifying fibroma of the long bones. A light and electron microscopic study. Arch Pathol. 1966 Sep;82(3):218-33
2. Kempson RL.Ossifying fibroma of the long bones. A light and electron microscopic study. Arch Pathol. 1966 Sep;82(3):218-33.
3. Most MJ, Sim FH, Inwards CY. Osteofibrous dysplasia and adamantinoma. J Am Acad Orthop Surg 2010;18:358-66.
4. Hahn SB, Kim SH, Cho NH, Choi CJ, Kim BS, Kang HJ. Treatment of osteofibrous dysplasia and associated lesions. Yonsei Med J. 2007 Jun 30;48(3):502-10.
5. Park YK, Unni KK, McLeod RA, Pritchard DJ. Osteofibrous dysplasia: clinicopathologic study of 80 cases. Hum Pathol. 1993 Dec;24(12):1339-47.
6. Gleason BC, Liegl-Atzwanger B, Kozakewich HP, Connolly S, Gebhardt MC, Fletcher JA, Perez-Atayde AR. Osteofibrous dysplasia and adamantinoma in children and adolescents: a clinicopathologic reappraisal. Am J Surg Pathol. 2008 Mar;32(3):363-76..
7. Levine SM, Lambiase RE, Petchprapa CN. Cortical lesions of the tibia: characteristic appearances at conventional radiography. RadioGraphics 2003;23:157-77.
8. Greenspan A. Malignant bone tumors II. In: Orthopedic imaging: a practical approach. 5th ed. Philadelphia, USA: Lippincott Williams & Wilkins; 2011. p. 754.
9. 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;37:1077-1084
10. Fitzpatrick KA, Taljanovic MS, Speer DP, Graham AR, Jacobson JA, Barnes GR, HunterTB.Imaging findings of fibrous dysplasia with histopathologic and intraoperative correlation. AJR Am J Roentgenol. 2004 Jun;182(6):1389-98.
11. Kahn L. Adamantinoma, osteofibrous dysplasia and differentiated adamantinoma. Skeletal Radiol 2003;32:245-58.
12. Levine SM, Lambiase RE, Petchprapa CN. Cortical lesions of the tibia:characteristic appearances at conventional radiography. RadioGraphics 2003;23:157-77.
13. Izquierdo FM, Ramos LR, Sánchez-Herráez S, Hernández T, de Alava E, Hazelbag HM. Dedifferentiated classic adamantinoma of the tibia: a report of a case with eventual complete revertant mesenchymal phenotype. Am J Surg Pathol. 2010 Sep;34(9):1388-92.
14. Campanacci M, Laus M. Osteofibrous dysplasia of the tibia and fibula. J Bone Jt Surg Am 1981;69(A):367-75.
|How to Cite this article:1. Panda P, Gulia A. Osteofibrous Dysplasia – an update. Journal of Bone and Soft Tissue Tumors May- Aug 2016;2(2):23-25 .|