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

  • Levy Hermes Rau
  • Angélica ReinheimerEmail author
  • Maria Inês Meurer
  • Aline Luiza Marodin
  • Catherine Schmitz Espezim
  • Leandro Eduardo Klüppel
  • Paula Cristina dos Santos Vaz Fernandes
  • Elena Riet Correa Rivero
Case Report

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.

Keywords

Fibrous dysplasia Fibro-osseous lesions Aneurysmal bone cyst Craniofacial surgery 

Notes

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 statement

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).

Ethical approval

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

Informed consent was obtained from the patient included in the study.

References

  1. 1.
    Verma A, Jindal N, Singh V, Sethi P (2013) Unusual presentation of monostatic fibrous dysplasia in zygoma. J Craniofac Surg 24:592–594.  https://doi.org/10.1097/SCS.0b013e31829ad561 Google Scholar
  2. 2.
    El-Mofty SK (2014) Fibro-osseous lesions of the craniofacial skeleton: an update. Head Neck Pathol 8:432–444.  https://doi.org/10.1007/s12105-014-0590-0 Google Scholar
  3. 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. 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
  5. 5.
    Cheng J, Wang Y, Yu H, Wang D, Ye J, Jiang H, Wu Y, Shen G (2012) An epidemiological and clinical analysis of craniomaxillofacial fibrous dysplasia in a Chinese population. Orphanet J Rare Dis 7:80.  https://doi.org/10.1186/1750-1172-7-80 Google Scholar
  6. 6.
    Rahman AM, Madge SN, Billing K, Anderson PJ, Leibovitch I, Selva D, David D (2009) Craniofacial fibrous dysplasia: clinical characteristics and long-term outcomes. Eye (Lond) 23:2175–2218.  https://doi.org/10.1038/eye.2009.6 Google Scholar
  7. 7.
    Gupta D, Garg P, Mittal A (2017) Computed tomography in craniofacial fibrous dysplasia: a case series with review of literature and classification update. Open Dent J 11:384–403.  https://doi.org/10.2174/1874210601711010384 Google Scholar
  8. 8.
    Sasaki H, Nagano S, Shimada H, Yokouchi M, Setoguchi T, Ishidou Y, Kunigou O, Maehara K, Komiya S (2017) Diagnosing and discriminating between primary and secondary aneurysmal bone cysts. Oncol Lett 13:2290–2296.  https://doi.org/10.3892/ol.2017.5682 Google Scholar
  9. 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
  10. 10.
    Kransdorf MJ, Sweet DE (1995) Aneurysmal bone cyst: concept, controversy, clinical presentation, and imaging. AJR Am J Roentgenol 164:573–580.  https://doi.org/10.2214/ajr.164.3.7863874 Google Scholar
  11. 11.
    Lee JW, Kim JH, Han SH, Kang HI (2010) Fibrous dysplasia with aneurysmal bone cyst presenting as painful solitary skull lesion. J Korean Neurosurg Soc 48:551–554.  https://doi.org/10.3340/jkns.2010.48.6.551 Google Scholar
  12. 12.
    Arden RL, Bahu SJ, Lucas DR (1997) Mandibular aneurysmal bone cyst associated with fibrous dysplasia. Otolaryngol Head Neck Surg 117:153–156.  https://doi.org/10.1016/s0194-59989770087-1 Google Scholar
  13. 13.
    Birk H, Winkler EA, Bonney PA, Berger MS, McDermott MW (2017) Calvarial aneurysmal bone cyst associated with fibrous dysplasia: case report and literature review. Interdiscip Neurosurg 7:50–52.  https://doi.org/10.1016/j.inat.2016.11.011 Google Scholar
  14. 14.
    Branch CL Jr, Challa VR, Kelly DL Jr (1986) Aneurysmal bone cyst with fibrous dysplasia of the parietal bone. Report of two cases. J Neurosurg 64:331–335.  https://doi.org/10.3171/jns.1986.64.2.0331 Google Scholar
  15. 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. 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. 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. 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. 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. 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. 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
  22. 22.
    Lee JY, Ko YI, Kwon H, Jung SN (2014) Aneurysmal bone cyst of the zygomatic bone. J Craniofac Surg 25:148–149.  https://doi.org/10.1097/scs.0000000000000463 Google Scholar
  23. 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. 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. 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. 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
  27. 27.
    Mattei TA, Mattei JA, Ramina R, Aguiar PH (2005) Fibrous dysplasia in combination with aneurysmal bone cyst presenting as a subarachnoid haemorrhage. Neurol Sci 26:178–181.  https://doi.org/10.1007/s10072-005-0458-x Google Scholar
  28. 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
  29. 29.
    Ngo CS, Propst EJ, Ngan BY, Smith DR, Reginald YA (2015) Aneurysmal bone cyst with fibrous dysplasia resulting in partial third nerve palsy. Can J Ophthalmol 50:27–29.  https://doi.org/10.1016/j.jcjo.2014.12.002 Google Scholar
  30. 30.
    Oliver LP (1973) Aneurysmal bone cyst. Report of a case. Oral Surg Oral Med Oral Pathol 35:67–76Google Scholar
  31. 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
  32. 32.
    Rappaport ZH (1989) Aneurysmal bone cyst associated with fibrous dysplasia of the skull. Neurochirurgia 32:192–194.  https://doi.org/10.1055/s-2008-1054037 Google Scholar
  33. 33.
    Salmasi V, Blitz AM, Ishii M, Gallia GL (2011) Expanded endonasal endoscopic approach for resection of a large skull base aneurysmal bone cyst in a pediatric patient with extensive cranial fibrous dysplasia. Childs Nerv Syst 27:649–656.  https://doi.org/10.1007/s00381-010-1341-5 Google Scholar
  34. 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. 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. 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. 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. 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. 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. 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. 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
  42. 42.
    Cottalorda J, Bourelle S (2007) Modern concepts of primary aneurysmal bone cyst. Arch Orthop Trauma Surg 127:105–114.  https://doi.org/10.1007/s00402-006-0223-5 Google Scholar
  43. 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
  44. 44.
    Park HY, Yang SK, Sheppard WL, Hegde V, Zoller SD, Nelson SD, Federman N, Bernthal NM (2016) Current management of aneurysmal bone cysts. Curr Rev Musculoskelet Med 9:435–444.  https://doi.org/10.1007/s12178-016-9371-6 Google Scholar
  45. 45.
    Diah E, Morris DE, Lo LJ, Chen YR (2007) Cyst degeneration in craniofacial fibrous dysplasia: clinical presentation and management. J Neurosurg 107:504–508.  https://doi.org/10.3171/jns-07/09/0504 Google Scholar
  46. 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
  47. 47.
    Lee JS, FitzGibbon EJ, Chen YR, Kim HJ, Lustig LR, Akintoye SO, Collins MT, Kaban LB (2012) Clinical guidelines for the management of craniofacial fibrous dysplasia. Orphanet J Rare Dis 7:S2.  https://doi.org/10.1186/1750-1172-7-s1-s2 Google Scholar
  48. 48.
    Alves N, de Oliveira RJ, Takehana D, Deana NF (2016) Recurrent monostotic fibrous dysplasia in the mandible. Case Rep Dent 2016:1–6.  https://doi.org/10.1155/2016/3920850 Google Scholar
  49. 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. 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. 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
  52. 52.
    Park B, Abode-Iyamah K, Lee SL, Kirby P, El-Khoury G, Wilson S (2015) Fibro-osseous lesion of the cranium in an adolescent patient. Surg Neurol Int 6:12.  https://doi.org/10.4103/2152-7806.149848 Google Scholar
  53. 53.
    Valentini V, Cassoni A, Marianetti TM, Terenzi V, Fadda MT, Iannetti G (2009) Craniomaxillofacial fibrous dysplasia: conservative treatment or radical surgery? A retrospective study on 68 patients. Plast Reconstr Surg 123:653–660.  https://doi.org/10.1097/PRS.0b013e318196bbbe Google Scholar
  54. 54.
    Chen YR, Chang CN, Tan YC (2006) Craniofacial fibrous dysplasia: an update. Chang Gung Med J 29:543–549Google Scholar
  55. 55.
    Fowell C, Edmondson S, Martin T, Praveen P (2015) Rapid prototyping and patient-specific pre-contoured reconstruction plate for comminuted fractures of the mandible. Br J Oral Maxillofac Surg 53:1035–1037.  https://doi.org/10.1016/j.bjoms.2015.06.018 Google Scholar
  56. 56.
    Murray DJ, Edwards G, Mainprize JG, Antonyshyn O (2008) Advanced technology in the management of fibrous dysplasia. J Plast Reconstr Aesthet Surg 61:906–916.  https://doi.org/10.1016/j.bjps.2007.08.029 Google Scholar

Copyright information

© Springer-Verlag GmbH Germany, part of Springer Nature 2019

Authors and Affiliations

  • Levy Hermes Rau
    • 1
    • 2
    • 3
  • Angélica Reinheimer
    • 4
    Email author
  • Maria Inês Meurer
    • 5
  • Aline Luiza Marodin
    • 1
  • Catherine Schmitz Espezim
    • 1
  • Leandro Eduardo Klüppel
    • 2
  • Paula Cristina dos Santos Vaz Fernandes
    • 3
  • Elena Riet Correa Rivero
    • 6
  1. 1.Department of Maxillofacial SurgeryJoana de Gusmão Children’s HospitalFlorianópolisBrazil
  2. 2.Department of Implant DentistryLatin American Institute of Dental Research and EducationCuritibaBrazil
  3. 3.Faculty of Dental MedicineUniversity of PortoPortoPortugal
  4. 4.Postgraduate Program in DentistryFederal University of Santa CatarinaFlorianópolisBrazil
  5. 5.Department of PathologyFederal University of Santa CatarinaFlorianópolisBrazil
  6. 6.Department of Pathology, Center of Health SciencesFederal University of Santa CatarinaFlorianópolisBrazil

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