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.
- 3.Albright F, Butler AM, Hampton AO, Smith P (1937) Syndrome characterized by osteitis fibrosa disseminata, areas of pigmentation and endocrine dysfunction, with precocious puberty in females - report of five cases. N Engl J Med 216:727–746Google Scholar
- 4.Lichtenstein L, Jaffe HL (1942) Fibrous dysplasia of bone. A condition affecting one, several or many bones, the graver cases of which may present abnormal pigmentation of skin, premature sexual development, hyperthyroidism or still other extra skeletal abnormalities. Arch Pathol 33:777–816Google Scholar
- 9.Jordan RC, Koutlas I, Raubenheimer E (2017) Aneurysmal bone cyst. In: El-Naggar AK, Chan JK, Grandis JR, Takata T, Slootweg P (eds) WHO classification of head and neck tumours: giant cell lesions and bone cysts, 4th edn. IARC Press, Lyon, pp 258–259Google Scholar
- 15.Buraczewski J, Dabska M (1971) Pathogenesis of aneurysmal bone cyst. Relationship between the aneurysmal bone cyst and fibrous dysplasia of bone. Cancer 28:597–604Google Scholar
- 16.Dani R, Gandhi V, Thakkar G, Prajapati H, Rathod G (2006) An aneurysmal bone cyst arising from fibrous dysplasia in frontal bone. Ind J Radiol Imag 16:49–52Google Scholar
- 17.El Deeb M, Sedano HO, Waite DE (1980) Aneurysmal bone cyst of the jaws. Report of a case associated with fibrous dysplasia and review of the literature. Int J Oral Surg 9:301–311Google Scholar
- 18.Elleuch W, Briki S, Karray F, Dhouib M, Abdelmoula M (2016) Aneurysmal bone cyst in cranial fibrous dysplasia: a case report. J Neurol Disord 4:4Google Scholar
- 19.Haddad GF, Hambali F, Mufarrij A, Nassar A, Haddad FS (1998) Concomitant fibrous dysplasia and aneurysmal bone cyst of the skull base. Pediatr Neurosurg 28:147–153Google Scholar
- 20.Iseri PK, Efendi H, Demirci A, Komsuoglu S (2005) Fibrous dysplasia of the cranial bones: a case report and review of the literature. Yale J Biol Med 78:141–145Google Scholar
- 21.Itshayek E, Spector S, Gomori M, Segal R (2002) Fibrous dysplasia in combination with aneurysmal bone cyst of the occipital bone and the clivus: case report and review of the literature. Neurosurgery 51:815–817Google Scholar
- 23.Lin WC, Wu HT, Wei CJ, Chang CY (2004) Aneurysmal bone cyst arising from fibrous dysplasia of the frontal bone. Eur Radiol 14:930–932Google Scholar
- 24.Lucarelli MJ, Bilyk JR, Shore JW, Rubin PA, Yaremchuk MJ (1995) Aneurysmal bone cyst of the orbit associated with fibrous dysplasia. Plast Reconstr Surg 96:440–445Google Scholar
- 25.Malik R, Pandya V, Awasthi P (2006) Secondary aneurysmal bone cyst developing in fibrous dysplasia. Ind J Radiol Imag 16:27–28Google Scholar
- 26.Manjila S, Zender CA, Weaver J, Rodgers M, Cohen AR (2013) Aneurysmal bone cyst within fibrous dysplasia of the anterior skull base: continued intracranial extension after endoscopic resections requiring craniofacial approach with free tissue transfer reconstruction. Childs Nerv Syst 29:1183–1192. https://doi.org/10.1007/s00381-013-2034-7 Google Scholar
- 28.Moorthy N, Shruthi S, Chander M, Rao GP (2015) Secondary aneurysmal bone cyst with fibrous dysplasia - a case report. Int J Curr Med Appl Sci 5:140–143Google Scholar
- 30.Oliver LP (1973) Aneurysmal bone cyst. Report of a case. Oral Surg Oral Med Oral Pathol 35:67–76Google Scholar
- 31.Pasquini E, Ceroni Compadretti G, Sciarretta V, Ippolito A (2002) Transnasal endoscopic surgery for the treatment of fibrous dysplasia of maxillary sinus associated to aneurysmal bone cyst in a 5-year-old child. Int J Pediatr Otorhinolaryngol 62:59–62Google Scholar
- 34.Skladzierin J, Oles K, Zagolski O, Moskala M, Sztuka M, Strek P, Wierzchowski W, Tomik J (2008) A giant cranial aneurysmal bone cyst associated with fibrous dysplasia. B-ENT 4:29–33Google Scholar
- 35.Suzuki F, Fukuda S, Yagi K, Chida E, Inuyama Y (2011) A rare aneurysmal bone cyst of the maxillary sinus: a case report. Auris Nasus Larynx 28:S131–S137Google Scholar
- 36.Terkawi AS, Al-Qahtani KH, Baksh E, Soualmi L, Mohamed Ael B, Sabbagh AJ (2011) Fibrous dysplasia and aneurysmal bone cyst of the skull base presenting with blindness: a report of a rare locally aggressive example. Head Neck Oncol 3(15). https://doi.org/10.1186/1758-3284-3-15
- 37.Urgun K, Yilmaz B, Toktas ZO, Akakin A, Konya D, Demir MK, Kilic T (2016) Craniospinal polyostotic fibrous dysplasia, aneurysmal bone cysts, and Chiari type 1 malformation coexistence in a patient with McCune-Albright syndrome. Pediatr Neurosurg 51:253–256. https://doi.org/10.1159/000444937 Google Scholar
- 38.Wojno KJ, McCarthy EF (1994) Fibro-osseous lesions of the face and skull with aneurysmal bone cyst formation. Skelet Radiol 23:15–18Google Scholar
- 39.Yokoyama T, Ihara K, Kato S, Orita T (1988) Fibrous dysplasia associated with aneurysmal bone cyst. Case report. Neurol Med Chir 28:833–837Google Scholar
- 40.Yollu U, Aslan M, Yilmaz M, Inan HC, Akil F, Comunoglu N, Yener HM (2017) Aneurysmal bone cyst of the maxillary sinus secondary to fibrous dysplasia: report of a rare case and review of the literature. Biomed Res Rev 1:1–3. https://doi.org/10.15761/BRR.1000104
- 41.Yuen VH, Jordan DR, Jabi M, Agbi C (2002) Aneurysmal bone cyst associated with fibrous dysplasia. Ophthal Plast Reconstr Surg 18:471–474. https://doi.org/10.1097/01.iop.0000031375.82320.f2 Google Scholar
- 43.Dabezies EJ, D'Ambrosia RD, Chuinard RG, Ferguson AB (1982) Aneurysmal bone cyst after fracture. A report of three cases. J Bone Joint Surg Am 64:617–621Google Scholar
- 46.Martinez V, Sissons HA (1988) Aneurysmal bone cyst. A review of 123 cases including primary lesions and those secondary to other bone pathology. Cancer 61(11):2291–2304Google Scholar
- 49.Leithner A, Windhager R, Lang S, Haas OA, Kainberger F, Kotz R (1999) Aneurysmal bone cyst. A population based epidemiologic study and literature review. Clin Orthop Relat Res 363:176–179Google Scholar
- 50.Fries JW (1957) The roentgen features of fibrous dysplasia of the skull and facial bones; a critical analysis of thirty-nine pathologically proved cases. Am J Roentgenol Radium Therapy, Nucl Med 77:71–88Google Scholar
- 51.Ciniglio Appiani M, Verillaud B, Bresson D, Sauvaget E, Blancal JP, Guichard JP, Saint Maurice JP, Wassef M, Karligkiotis A, Kania R, Herman P (2015) Ossifying fibromas of the paranasal sinuses: diagnosis and management. Acta Otorhinolaryngol Ital 35:355–361. https://doi.org/10.14639/0392-100x-533 Google Scholar
- 54.Chen YR, Chang CN, Tan YC (2006) Craniofacial fibrous dysplasia: an update. Chang Gung Med J 29:543–549Google Scholar