Fibrous dysplasia with secondary aneurysmal bone cyst—a rare case report and literature review
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.
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.
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.
KeywordsFibrous dysplasia Fibro-osseous lesions Aneurysmal bone cyst Craniofacial surgery
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.
This study was funded by the authors themselves.
Compliance with ethical standards
Conflict of interest
The authors declare that they have no conflict of interest of any nature (financial or non-financial).
All procedures performed in this study involving human participants were in accordance with the ethical standards of the Institutional and/or National Research Committee and with the 1964 Declaration of Helsinki and its later amendments or comparable ethical standards.
Informed consent was obtained from the patient included in the study.
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