Radiology Case Reports Volume 6, Issue 4, 2011
Osteofibrous dysplasia: A case report and review of the literature Paolo Simoni, MD; Laura Scarciolla, MD; Eugene Mutijima, MD; and Bruno Beomonte Zobel, MD Osteofibrous dysplasia (OFD) is a rare bone tumor affecting young individuals. The differential diagnosis between OFD and adamantinoma may be challenging in some cases on imaging. We present a case of OFD and discuss the key imaging and histological findings. We also discuss the differential diagnosis between OFD and classical adamantinoma on the basis of recent literature. sues was observed. The medullary bone marrow surrounding the lesion was normal (Fig. 3). On the basis of the imaging features, a diagnosis of OFD was made. This diagnosis was confirmed by biopsy, which was performed under CT guidance.
Case report A 27-year-old man presented with a complaint of a mild pain in his right leg, persisting for some months. The pain occurred mainly during the day, with some occasional pain during the night. At clinical examination, the pain was elicited by passive movement and digital compression. Leg mobility was normal. Radiographs of his right knee and leg showed a large focal area of cortical thickening measuring 5.5 x 1.5 cm along the anterior proximal aspect of the tibial shaft (Fig. 1). Multiple roundish, radiolucent lacunae ranging from 5 to 10 mm in size were visible within the lesion, with a “soap bubble” appearance. The lesion borders were well marginated, without a transitional zone. No periosteal reaction was observed on plain films. Computed tomography (CT) confirmed the absence of a transitional zone and periosteal reaction (Fig. 2). On MR imaging, the lesion showed very low signal intensity on both T1-weighted and T2-weighted images. The radiolucent lacunae observed on X-rays and CT were of intermediate signal in all sequences. No invasion of the nearby soft tis-
Citation: Simoni P, Scarciolla L, Mutijima E, Zobel BB. Osteofibrous dysplasia: A case report and review of the literature. Radiology Case Reports. (Online) 2011;6:546. Copyright: © 2011 The Authors. This is an open-access article distributed under the terms of the Creative Commons Attribution-NonCommercial-NoDerivs 2.5 License, which permits reproduction and distribution, provided the original work is properly cited. Commercial use and derivative works are not permitted. Dr. Simoni is in the Department of Diagnostic Imaging and Dr. Mutijima is in the Department of Pathology, both at the University Hospital of Liège, Liège, France. Dr. Scarciolla and Dr. Zobel are both in the Department of Diagnostic Imaging, University Campus Bio-medico, Rome, Italy. Contact Dr. Simoni at [email protected]. Competing Interests: The authors have declared that no competing interests exist.
Figure 1. 27-year-old man with osteofibrous dysplasia. Lateral (A) and anteroposterior (B) radiographs of the right proximal tibia.
DOI: 10.2484/rcr.v6i4.546
RCR Radiology Case Reports | radiology.casereports.net
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2011 | Volume 6 | Issue 4
Osteofibrous dysplasia: A case report and review of the literature
Figure 2. 27-year-old man with osteofibrous dysplasia. Axial (A) and sagittal (B) CT scan. The radiolucent lacunae have a “soap bubble” appearance (white arrows). The cortical is thickened without any periosteal reaction.
Figure 3. 27-year-old man with osteofibrous dysplasia. Axial (A, B) and sagittal (C, D) T1- and T2-weighted MR images. The cortical lesion is of low intensity in both sequences (white arrows). The lacunae within the lesion are of intermediate signal on T1- and T2-weighted images.
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Discussion Osteofibrous dysplasia (OFD), also known as ossifying fibroma of the long bones, is a rare benign tumor. OFD occurs during the first decade of the life, with a slight prevalence in males (sex ratio= 3:2) (1). OFD is typically located at the tibial diaphysis. Rarely, OFD can be observed in the fibula (2), radius, and ulna (3). OFD is generally asymptomatic. Therefore symptomatic OFD should raise the question of an associated pathological fracture (1). When sufficiently advanced, OFD can lead to enlargement or a bowing of the tibia (1). On imaging, the typical problem with OFD is distinguishing it from adamantinoma, a malignant bone tumor of young individuals, aged between 15 and 40 years, and typically arising from the tibia (1). On radiographs and CT, OFD appears as a cortical thickening interspersed with multiple roundish lytic areas with a "soap bubble" appearance. Usually, neither periosteal reaction nor a transitional zone are observed around OFD (1, 2, 3). Unlike OFD, classical adamantinoma presents moth-eaten borders and extensive involvement of the cancellous bone. OFD is generally smaller than classical adamantinomas; the mean reported size is 13.2 cm and 6.1 respectively (4). OFD is typically solitary, while fibular skip lesions may be associated with adamantinoma (1). At histology, OFD is characterized by the presence of osteoid tissue, fibrous tissue, and a small amount of epidermoid cells (1, 3, 5) (Fig. 4). An immunohistochemical essay is mandatory in some cases to detect epidermoid cells, which are not visible on standard hematoxyline and eosine (H & E) because of their small number. References 1. 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. [PubMed]
2011 | Volume 6 | Issue 4
Osteofibrous dysplasia: A case report and review of the literature 2. Campanacci M, Laus M. Osteofibrous dysplasia of the tibia and fibula. J Bone Jt Surg [Am] 1981; 69-A: 367– 375. [PubMed] 3. Wang J, Shih C, Chen W. Osteofibrous dysplasia (ossifying fibroma of long bones): a report of four cases and review of the literature. Clin Orthop Relat Res 1992; 69A: 367–375. [PubMed] 4. Bohndorf K, Nidecker A, Mathias K, Zidkova H, Kauffmann H, Jundt G. The radiologic findings in adamantinoma of the long tubular bones. Rofo Fortschr Geb Rontgenstrahl Neuen Bildgeb Verfahr 1992; 157: 239– 244. [PubMed] 5. Szendrői M, Antal I, Arató G. Adamantinoma of long bones: A long-term follow-up study of 11 cases. Pathol. Oncol. Res. 2009 15:209–216. [PubMed] 6. Park YK, Unni KK, McLeod RA, Pritchard DJ. Osteofibrous dysplasia: clinicopathological study of 80 cases. Hum Pathol 1993; 24: 1339–1347. [PubMed]
RCR Radiology Case Reports | radiology.casereports.net
Figure 4. 27-year-old man with osteofibrous dysplasia. Immunohistochemical specimen:(cytokeratin antibodies; magnification x40). Note the paucity of cytokeratin-positive cells, confirming the diagnosis of OFD (see text). OM: osteoid matrix; FT: fibrous tissue.
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2011 | Volume 6 | Issue 4
Osteofibrous dysplasia: A case report and review of the literature Paolo Simoni, MD; Laura Scarciolla, MD; Eugene Mutijima, MD; and Bruno Beomonte Zobel, MD Osteofibrous dysplasia (OFD) is a rare bone tumor affecting young individuals. The differential diagnosis between OFD and adamantinoma may be challenging in some cases on imaging. We present a case of OFD and discuss the key imaging and histological findings. We also discuss the differential diagnosis between OFD and classical adamantinoma on the basis of recent literature. sues was observed. The medullary bone marrow surrounding the lesion was normal (Fig. 3). On the basis of the imaging features, a diagnosis of OFD was made. This diagnosis was confirmed by biopsy, which was performed under CT guidance.
Case report A 27-year-old man presented with a complaint of a mild pain in his right leg, persisting for some months. The pain occurred mainly during the day, with some occasional pain during the night. At clinical examination, the pain was elicited by passive movement and digital compression. Leg mobility was normal. Radiographs of his right knee and leg showed a large focal area of cortical thickening measuring 5.5 x 1.5 cm along the anterior proximal aspect of the tibial shaft (Fig. 1). Multiple roundish, radiolucent lacunae ranging from 5 to 10 mm in size were visible within the lesion, with a “soap bubble” appearance. The lesion borders were well marginated, without a transitional zone. No periosteal reaction was observed on plain films. Computed tomography (CT) confirmed the absence of a transitional zone and periosteal reaction (Fig. 2). On MR imaging, the lesion showed very low signal intensity on both T1-weighted and T2-weighted images. The radiolucent lacunae observed on X-rays and CT were of intermediate signal in all sequences. No invasion of the nearby soft tis-
Citation: Simoni P, Scarciolla L, Mutijima E, Zobel BB. Osteofibrous dysplasia: A case report and review of the literature. Radiology Case Reports. (Online) 2011;6:546. Copyright: © 2011 The Authors. This is an open-access article distributed under the terms of the Creative Commons Attribution-NonCommercial-NoDerivs 2.5 License, which permits reproduction and distribution, provided the original work is properly cited. Commercial use and derivative works are not permitted. Dr. Simoni is in the Department of Diagnostic Imaging and Dr. Mutijima is in the Department of Pathology, both at the University Hospital of Liège, Liège, France. Dr. Scarciolla and Dr. Zobel are both in the Department of Diagnostic Imaging, University Campus Bio-medico, Rome, Italy. Contact Dr. Simoni at [email protected]. Competing Interests: The authors have declared that no competing interests exist.
Figure 1. 27-year-old man with osteofibrous dysplasia. Lateral (A) and anteroposterior (B) radiographs of the right proximal tibia.
DOI: 10.2484/rcr.v6i4.546
RCR Radiology Case Reports | radiology.casereports.net
6
2011 | Volume 6 | Issue 4
Osteofibrous dysplasia: A case report and review of the literature
Figure 2. 27-year-old man with osteofibrous dysplasia. Axial (A) and sagittal (B) CT scan. The radiolucent lacunae have a “soap bubble” appearance (white arrows). The cortical is thickened without any periosteal reaction.
Figure 3. 27-year-old man with osteofibrous dysplasia. Axial (A, B) and sagittal (C, D) T1- and T2-weighted MR images. The cortical lesion is of low intensity in both sequences (white arrows). The lacunae within the lesion are of intermediate signal on T1- and T2-weighted images.
RCR Radiology Case Reports | radiology.casereports.net
2
Discussion Osteofibrous dysplasia (OFD), also known as ossifying fibroma of the long bones, is a rare benign tumor. OFD occurs during the first decade of the life, with a slight prevalence in males (sex ratio= 3:2) (1). OFD is typically located at the tibial diaphysis. Rarely, OFD can be observed in the fibula (2), radius, and ulna (3). OFD is generally asymptomatic. Therefore symptomatic OFD should raise the question of an associated pathological fracture (1). When sufficiently advanced, OFD can lead to enlargement or a bowing of the tibia (1). On imaging, the typical problem with OFD is distinguishing it from adamantinoma, a malignant bone tumor of young individuals, aged between 15 and 40 years, and typically arising from the tibia (1). On radiographs and CT, OFD appears as a cortical thickening interspersed with multiple roundish lytic areas with a "soap bubble" appearance. Usually, neither periosteal reaction nor a transitional zone are observed around OFD (1, 2, 3). Unlike OFD, classical adamantinoma presents moth-eaten borders and extensive involvement of the cancellous bone. OFD is generally smaller than classical adamantinomas; the mean reported size is 13.2 cm and 6.1 respectively (4). OFD is typically solitary, while fibular skip lesions may be associated with adamantinoma (1). At histology, OFD is characterized by the presence of osteoid tissue, fibrous tissue, and a small amount of epidermoid cells (1, 3, 5) (Fig. 4). An immunohistochemical essay is mandatory in some cases to detect epidermoid cells, which are not visible on standard hematoxyline and eosine (H & E) because of their small number. References 1. 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. [PubMed]
2011 | Volume 6 | Issue 4
Osteofibrous dysplasia: A case report and review of the literature 2. Campanacci M, Laus M. Osteofibrous dysplasia of the tibia and fibula. J Bone Jt Surg [Am] 1981; 69-A: 367– 375. [PubMed] 3. Wang J, Shih C, Chen W. Osteofibrous dysplasia (ossifying fibroma of long bones): a report of four cases and review of the literature. Clin Orthop Relat Res 1992; 69A: 367–375. [PubMed] 4. Bohndorf K, Nidecker A, Mathias K, Zidkova H, Kauffmann H, Jundt G. The radiologic findings in adamantinoma of the long tubular bones. Rofo Fortschr Geb Rontgenstrahl Neuen Bildgeb Verfahr 1992; 157: 239– 244. [PubMed] 5. Szendrői M, Antal I, Arató G. Adamantinoma of long bones: A long-term follow-up study of 11 cases. Pathol. Oncol. Res. 2009 15:209–216. [PubMed] 6. Park YK, Unni KK, McLeod RA, Pritchard DJ. Osteofibrous dysplasia: clinicopathological study of 80 cases. Hum Pathol 1993; 24: 1339–1347. [PubMed]
RCR Radiology Case Reports | radiology.casereports.net
Figure 4. 27-year-old man with osteofibrous dysplasia. Immunohistochemical specimen:(cytokeratin antibodies; magnification x40). Note the paucity of cytokeratin-positive cells, confirming the diagnosis of OFD (see text). OM: osteoid matrix; FT: fibrous tissue.
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2011 | Volume 6 | Issue 4
