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      Osteofibrous dysplasia: A rare case in 3-day-old female


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          Osteofibrous dysplasia (OFD) is a nonneoplastic tumor-like lesion, made up of fibrous matrix with immature bone tissue surrounded by osteoblasts, occurring usually in the cortex of tibial diaphysis. OFD is usually seen in the first decade of life and, according to literature, it is rarely seen in the newborn period. Diagnosis of congenital OFD in the newborn is challenging because it is uncommon in this age group and can be confused with other bone benign or malignant lesions. Imaging plays an important role in diagnosis, although histological confirmation is often required. Our report presents a rare case of pathologically confirmed congenital OFD in 3-day-old female which presented with a swelling of her right leg. We will focus on imaging findings of OFD and main differential diagnosis of this lesion in neonatal age.

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          Most cited references33

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          Giant cell tumor of bone.

          Giant cell tumor (GCT) of bone is one type of giant cell-rich lesion of bone. This benign mesenchymal tumor has characteristic multinuclear giant cells. Mononuclear stromal cells are the physiologically active and diagnostic cell type. Most GCTs are located in the epiphyseal regions of long bones. The axial skeleton-primarily the sacrum-is a secondary site of involvement. Most patients present with pain, swelling, joint effusion, and disability in the third and fourth decades of life. Imaging studies are important for tumor staging and radiographic grading. Typically, these clinically active but slow-growing tumors are confined to bone, with relatively well-defined radiographic borders. Monostotic disease is most common. Metastatic spread to the lungs is rare. Extended intralesional curettage with or without adjuvant therapy is the primary treatment choice. Local recurrence is seen in ≤ 20% of cases, and a second local intralesional procedure is typically sufficient in cases that are detected early. Medical therapies include diphosphonates and denosumab. Denosumab has been approved for use in osteoporosis as well as breast and prostate cancer metastatic to bone. Medical therapy and radiotherapy can alter the management of GCT of bone, especially in multifocal disease, local recurrences, and bulky central/axial disease.
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            Langerhans cell histiocytosis.

            Langerhans cell histiocytosis (LCH) is a complex disease entity comprised of three distinct clinical syndromes that demonstrate indistinguishable histology. These syndromes are: eosinophilic granuloma, which is predominantly osseous or pulmonary; Hand-Schûller-Christian's disease, which involves multiple organ systems and, most typically, the skull base; and Letterer-Siwe's disease, the most severe disease manifestation, which typically involves the abdominal viscera. This article reviews our current understanding of Langerhans cell histiocytosis by discussing the history, histology, etiology, and treatment of the disease. It focuses on the radiographic findings and imaging modalities that are the most useful in disease diagnosis and management.
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              Osteofibrous dysplasia: clinicopathologic study of 80 cases.

              A thorough review of the clinical, radiologic, and histologic features of 80 cases of long bone osteofibrous dysplasia is presented. Of the six cases of osteofibrous dysplasia immunostained with a cytokeratin antibody, two were cytokeratin positive; however, the five cases of fibrous dysplasia that were stained were all negative. Follow-up (mean, 5.4 years; range, 1 month to 31 years) data were available for 41 cases (51%). From the consultation series nine of the 18 patients studied had recurrences, regardless of the different treatment regimens. The average recurrence interval in these patients was 2.7 years. Two patients had incomplete excision and six had biopsy only. All eight of these patients had residual tumors. Among the 16 Mayo Clinic patients, one had recurrence 5 years after the initial treatment. In two cases, 7-year-old and 6-year-old boys, histologic maturation to fibrous dysplasia was observed 4 and 10 years later, respectively. Adamantinoma has not developed in any of the 41 cases of osteofibrous dysplasia for which we have follow-up information. It seems clear that osteofibrous dysplasia does not progress to adamantinoma. Our study suggests that osteofibrous dysplasia is probably a variant of fibrous dysplasia, as demonstrated by the maturation of two lesions of osteofibrous dysplasia to fibrous dysplasia. Surgical treatment may be appropriate in cases with an extensive lesion, pseudoarthrosis, and accentuated tibial bowing. The overall prognosis is good, even with recurrence.

                Author and article information

                Radiol Case Rep
                Radiol Case Rep
                Radiology Case Reports
                31 December 2021
                March 2022
                31 December 2021
                : 17
                : 3
                : 825-831
                [a ]Advanced Biomedical Sciences Department, University Federico II of Naples, 80131, Naples, Italy
                [b ]Department of Radiology, AORN Santobono Pausilipon, Pediatric Hospital, Naples, Italy
                [c ]Department of Pathology, AORN Santobono Pausilipon, Pediatric Hospital, Naples, Italy
                Author notes
                [* ]Corresponding author at: Via Roma 85, Afragola Napoli Italia annacastaldo90@ 123456libero.it
                © 2021 Published by Elsevier Inc. on behalf of University of Washington.

                This is an open access article under the CC BY-NC-ND license (http://creativecommons.org/licenses/by-nc-nd/4.0/).

                Case Report

                osteofibrous dysplasia- congenital – tibia,x-ray,magnetic resonance- computed tomography


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