Levy Hermes Rau1,2,3, Angélica Reinheimer4, Maria Inês Meurer5, Aline Luiza Marodin1, Catherine Schmitz Espezim1, Leandro Eduardo Klüppel2, Paula Cristina Dos Santos Vaz Fernandes3, Elena Riet Correa Rivero6. 1. Department of Maxillofacial Surgery, Joana de Gusmão Children's Hospital, Florianópolis, SC, Brazil. 2. Department of Implant Dentistry, Latin American Institute of Dental Research and Education, Curitiba, PR, Brazil. 3. Faculty of Dental Medicine, University of Porto, Porto, Portugal. 4. Postgraduate Program in Dentistry, Federal University of Santa Catarina, Florianópolis, SC, Brazil. angelicareinheimer@hotmail.com. 5. Department of Pathology, Federal University of Santa Catarina, University Campus, Trindade, Florianópolis, Santa Catarina, 88040-370, Brazil. 6. Department of Pathology, Center of Health Sciences, Federal University of Santa Catarina, University Campus, Trindade, Florianópolis, 88.040-370, Santa Catarina, Brazil.
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.
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.
Entities:
Keywords:
Aneurysmal bone cyst; Craniofacial surgery; Fibro-osseous lesions; Fibrous dysplasia
Authors: M Ciniglio Appiani; B Verillaud; D Bresson; E Sauvaget; J-P Blancal; J-P Guichard; J-P Saint Maurice; M Wassef; A Karligkiotis; R Kania; P Herman Journal: Acta Otorhinolaryngol Ital Date: 2015-10 Impact factor: 2.124
Authors: J S Lee; E J FitzGibbon; Y R Chen; H J Kim; L R Lustig; S O Akintoye; M T Collins; L B Kaban Journal: Orphanet J Rare Dis Date: 2012-05-24 Impact factor: 4.123