Bilateral Symmetric Sporadic Osteofibrous Dysplasia: an Unusual Case
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CASE REPORT
Bilateral Symmetric Sporadic Osteofibrous Dysplasia: an Unusual Case Asit Ranjan Mridha 1 & Anubhav Narwal 1 & Adarsh Barwad 1 & Venkatesan Sampat Kumar 1 & Shivanand Gamanagatti 2 & Prashant Ramteke 3 Received: 5 May 2020 / Accepted: 17 September 2020 # Indian Association of Surgical Oncology 2020
Case History A 7-year-old male presented to our hospital with complaints of pain and bowing of both legs for the last 1 year. There was no history of trauma, fever, anorexia, and weight loss. Family history was unremarkable. Physical examination revealed mild shortening and anterolateral bowing of bilateral legs associated with mild tenderness. No lymph nodes or any abdominal organs were palpable. Radiographs of legs revealed symmetric involvement of upper and middle third of diaphysis of the bilateral tibia by well-demarcated, expansile, longitudinally oriented, confluent, and separate oval-shaped bubbly multiloculated lytic lesions with thinning of the anterior cortex. The radiolucent areas were surrounded by sclerotic rim towards the medullary bone. Periosteal reaction or associated soft tissue involvement was not seen (Fig. 1 a and b). Magnetic resonance imaging (MRI) showed well-defined hyperintense multicystic osseous lesions in bilateral tibia on T2W images (Fig. 2). Biopsy from the lesion showed irregularly arranged woven bone with intervening cellular stroma with a mild myxoid appearance at places. The bone trabeculae were rimmed by prominent osteoblasts, and the stroma contained bland spindle cells and collagen (Figs. 3a–c). Epithelial cells were absent, which was also confirmed by immunohistochemical studies with pan-cytokeratin and epithelial membrane antigen (EMA) (Fig. 3d). Surgical
* Prashant Ramteke [email protected] 1
Department of Orthopedics, All India Institute of Medical Sciences, New Delhi, India
2
Department of Radiodiagnosis, All India Institute of Medical Sciences, New Delhi, India
3
Department of Pathology, All India Institute of Medical Sciences, Ansari Nagar, New Delhi 110029, India
intervention with curettage and bone grafting was done in the left tibia; however, conservative management is being provided for the right tibia.
Discussion Osteofibrous dysplasia is a self-limited, benign fibro-osseous lesion, which shares many features like anatomic location, cellular origin, and histologic similarity with fibrous dysplasia [1]. It is primarily seen during the first two decades of life with male predominance and uncommon after skeletal maturation [2]. It was first described by Campanacci in 1976 and is considered to be a developmental skeletal disorder [1]. OFDs were presumed to be the precursor of adamantinoma, which was supported by the presence of an intermediate OFD-LA lesion [3]. However, long-term prospective studies failed to demonstrate the progression of OFD to adamantinoma [4, 5]. According to some authors, OFD-LA represents a spontaneously regressing adamantinoma [6]. But no such case has been reported in the English literature. A recent study found a ME
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