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Fibrous dysplasia with secondary aneurysmal bone cyst—a rare case report and literature review

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Abstract

Introduction

Fibrous dysplasia (FD) and aneurysmal bone cyst (ABC) are uncommon non-neoplastic intraosseous lesions, and the occurrence of concomitant FD and ABC is extremely rare.

Case report

An 11-year-old boy presented with gradual progressive enlargement of his right zygomatic bone over 4 years prior to presentation. Computed tomography revealed a lesion with a central bony area showing a ground-glass appearance surrounded by a well-defined expansile lesion with internal septations. An incisional biopsy was performed and suggested a fibro-osseous lesion. Resection was performed, followed by immediate reconstruction using autogenous bone graft from the iliac crest. Histopathological examination revealed irregularly shaped trabeculae comprising immature woven bone in a fibroblastic cell-rich stroma. Blood-filled sinusoidal spaces lined by fibrous septa containing scattered multinucleated giant cells were observed peripherally. These findings were compatible with concomitant FD and ABC. The patient was disease-free at the time of his 10-month follow-up.

Conclusions

Based on our literature search, this report is the first to describe concomitant monostotic FD and ABC in the zygomatic bone. Accurate diagnosis requires careful investigation and examination of clinical, radiographical, and histopathological features. The treatment of choice should provide an esthetic and functional improvement in the patient.

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Acknowledgements

We are grateful to Dr. André Andujar, pediatric orthopedic surgeon at Joana de Gusmão Children’s Hospital, and to Dr. Bruno Gribel for donating the 3D printed biomodel.

Funding

This study was funded by the authors themselves.

Author information

Authors and Affiliations

  1. Department of Maxillofacial Surgery, Joana de Gusmão Children’s Hospital, Florianópolis, SC, Brazil

    Levy Hermes Rau, Aline Luiza Marodin & Catherine Schmitz Espezim

  2. Department of Implant Dentistry, Latin American Institute of Dental Research and Education, Curitiba, PR, Brazil

    Levy Hermes Rau & Leandro Eduardo Klüppel

  3. Faculty of Dental Medicine, University of Porto, Porto, Portugal

    Levy Hermes Rau & Paula Cristina dos Santos Vaz Fernandes

  4. Postgraduate Program in Dentistry, Federal University of Santa Catarina, Florianópolis, SC, Brazil

    Angélica Reinheimer

  5. Department of Pathology, Federal University of Santa Catarina, University Campus, Trindade, Florianópolis, Santa Catarina, 88040-370, Brazil

    Maria Inês Meurer

  6. Department of Pathology, Center of Health Sciences, Federal University of Santa Catarina, University Campus, Trindade, Florianópolis, 88.040-370, Santa Catarina, Brazil

    Elena Riet Correa Rivero

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  1. Levy Hermes Rau
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Correspondence to Angélica Reinheimer.

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Rau, L.H., Reinheimer, A., Meurer, M.I. et al. Fibrous dysplasia with secondary aneurysmal bone cyst—a rare case report and literature review. Oral Maxillofac Surg 23, 101–107 (2019). https://doi.org/10.1007/s10006-019-00741-w

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