Osteoma

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Overview

Historical Perspective

Classification

Pathophysiology

Causes

Differentiating Osteoma from other Diseases

Epidemiology and Demographics

Risk Factors

Screening

Natural History, Complications and Prognosis

Diagnosis

Staging

History and Symptoms

Physical Examination

Laboratory Findings

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MRI

Ultrasound

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief:  Rohan A. Bhimani, M.B.B.S., D.N.B., M.Ch.[2]

Synonyms and keywords: Osteoma; Osteomata; Osteoncus; Ivory osteoma; Mature osteoma; Mixed osteoma; Homoplastic osteoma; Heteroplastic osteoma; Osteomas; Ivory exostosis; Sino-orbital osteoma; Sino-nasal osteoma; Paranasal sinus osteoma; Skull vault osteoma; Mandibular osteoma

## Overview

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Osteoma (also known as Osteomata) is a slow growing benign tumor of bone, occurring most commonly in the craniofacial skeletal structures, primarily in the nasal and paranasal (75-90%) sinuses. Osteoma arises from bone overgrowth, which is normally composed of connective tissue. Osteomas are slow growing tumors composed of compact or mature trabecular bone limited to craniofacial bones. Osteoma may be incidentally identified as a mass in the skull, mandible, or as the underlying cause of sinusitis or mucocele formation within the paranasal sinuses. When they are multiple, Gardner syndrome should be considered. Osteoma represents the most common benign neoplasm of the nose and paranasal sinuses. The causes remain uncertain, but commonly accepted theories propose embryologic, traumatic, or infectious causes. Osteomas are usually asymptomatic. Excision may be performed if osteoma is responsible for symptoms.

## Historical Perspective

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* In 1898, the description of craniofacial osteoma was first reported by Paul Schulze.[1]
* In 1951, Eldon J. Gardner (1909–1989) a geneticist first described the occurrence of multiple osteomas in hereditary familial adenomatous polyposis (FAP).
* In 2014, The Lancet published an article named "Did René Descartes have a giant ethmoidal sinus osteoma?" the authenticity has been confirmed by anthropological and historical investigations to be true.[2]

## Classification

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Osteoma can be classified based on imaging findings.

### Enneking (MSTS) Staging System

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* The Enneking surgical staging system (also known as the MSTS system) for benign musculoskeletal tumors based on radiographic characteristics of the tumor host margin.[3]
* It is widely accepted and routinely used classification.

Stages | Description
1  | Latent: Well demarcated borders
2  | Active: Indistinct borders
3  | Aggressive: Indistinct borders

## Pathophysiology

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* The exact etiology of osteoma is unknown.[4]
* The possibility of a reactive mechanism, triggered by trauma or infection, has been suggested.[5]
* Osteoma arises from bone overgrowth, which is normally composed of connective tissue.[6]
* Osteomas are slow growing tumors composed of compact or mature trabecular bone limited to craniofacial bones.
* Very rarely osteomas of the facial bones may be associated with Gardner's syndrome.
* Osteomas have a particular frequency distribution within the paranasal sinuses: frontal sinuses 80%, ethmoid air cells 15%, maxillary sinuses 5% and sphenoid sinus rare.

### Genetics

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* The hallmark of multiple osteomas is a mutation in the APC gene, that results in the Gardner syndrome.[7]

## Causes

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* The cause of osteoma has not been identified.[8]

## Differentiating ((Page name)) from Other Diseases

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Osteoma must be differentiated from other diseases that cause sinus or facial pain, headache, and changes to or loss of sense of smell, such as other osteogenic tumors, fibrous dysplasia, and chronic sinusitis.[9][10]

Differential Diagnosis | Similar Features | Differentiating Features
Fibrous dysplasia |

* Benign, often an incidental finding, affects the same group of patients, and symptoms include facial pain and headache

* In fibrous dysplasia, differentiating features include: More common presentation is on ribs: 28%, no gender predilection, and complete resection is usually not possible

Osteoblastoma |

* Benign, incidental, and male predilection

* In osteoblastoma, differentiating features include: normally affect the axial skeleton, lesions are typically larger than 2 cm, and surgical excision is often the treatment of choice

Adamantinomas |

* Benign, slow growing, and similar clinical onset

* In adamantinomas, differentiating features include: locally aggressive tumor, common in the 3rd to 5th decades of life, and location is usually confined to the jaw

Chronic sinusitis |

* Affects same group of population (young to middle aged adults) and the clinical presentation is similar

* In chronic sinusitis, differentiating features include: fever, previous history of acute sinusitis, lack of facial deformation or imaging findings compatible with osteoma

## Epidemiology and Demographics

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* The prevalence of osteoma is approximately 3000 per 100,000 individuals worldwide.[11]
* The incidence of osteoma remains unknown.
* Patients of all age groups may develop osteoma.
* The average patient age varies from 25 to 35 years.
* The mean age of the patients with osteoma is is 37 years.[9]
* Men are more commonly affected than women, with a 3:1 ratio.[12]
* There is no racial predilection to osteoma.

## Risk Factors

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There are no established risk factors for osteoma.[6]

## Screening

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Screening for multiple osteomas is recommended among patients with family history or/and a confirmed diagnosis of Gardner syndrome. Thyroid exam and annual ultrasound, should be performed starting at age 10 to 12 years.[13]

## Natural History, Complications, and Prognosis

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* If left untreated, osteoma progression occurs slowly and is then followed by facial distortion.[14]
* Common complications of osteoma include:[15]
* Proptosis
* Facial deformity
* Airway obstruction
* Sensory loss
* Anosmia
* Visual loss
* Prognosis is generally excellent,the lesion does not recur after surgical excision and it is not associated with malignant change.[15]

## Diagnosis

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### Diagnostic Study of Choice

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Biopsy is the diagnostic study of choice for the diagnosis of osteoma.

* Gross appearance of osteoma include:
* Osteomas have a spongy to densely appearance, conformed of in a polypoid and lobullated shape.[1]
* The median size tumor size is 3.0 cm (range 0.5-8 cm).
* Osteomas have a smooth surface and composed of dense compact bone (ivory osteoma), trabecular bone (mature osteoma, or both patterns).

Histology of osteoma.Source: Case courtesy of Nephron [CC BY-SA 4.0 (https://creativecommons.org/licenses/by-sa/4.0) or GFDL (http://www.gnu.org/copyleft/fdl.html), from Wikimedia Commons]

* Histological appearance includes:
* Presence of dense compact mature bone in paucicellular fibrous stroma.
* Large trabeculae of mature lamellar bone can be also be seen.

### History and Symptoms

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* Small osteomas are asymptomatic and usually incidental findings.
* Common symotoms of osteoma include:[16][17]
* Headache
* Nasal congestion
* Facial pain
* Facial tenderness
* Loss of the sense of smell

### Physical Examination

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X-ray showing osteoma of the frontal sinus.Source: Case courtesy of Dr Ahmed Abdrabou, Radiopaedia.org, rID: 42250

* Patients with osteoid osteoma usually appears well.
* Common physical examination findings of osteoid osteoma include:[18]
* Facial tenderness
* Nasal obstruction and discharge
* Tapping over a sinus area produces dull sound
* Physical deformity over mastoid or facial area

### Laboratory Findings

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There are no diagnostic laboratory findings associated with osteoma.

### Electrocardiogram

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There are no ECG findings associated with osteoma.

### X-ray

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* Three views of affected bone or joint are recommended.[19]
* Radiological findings for osteoma include:
* Well circumscribed mass
* Varying amounts of central lucency
* Lobulated mass occupying frontal or maxillary sinus

### Echocardiography or Ultrasound

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CT showing osteoma of the mandible.Source: Case courtesy of Dr Ahmed Abdrabou, Radiopaedia.org, rID: 42250

There are no echocardiography/ultrasound findings associated with osteoma.

### CT scan

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* CT findings of osteoma include:[20]
* Well-circumscribed mass of variable density
* Ground-glass appearance
* Exophytical mass growing out of a sinus

### MRI

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* MRI findings of osteoma include:[21]
* Low signal on all sequences.
* Mature osteomas may demonstrate some marrow signal, but are also predominantly low on all sequences

### Other Imaging Findings

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There are no other imaging findings associated with osteoma.

### Other Diagnostic Studies

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### Nasal Endoscopy

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Nasal endoscopy findings include:[22]

* Direct visualization of the nasal passages structures, and sinuses.
* Tumor location, size, and adjacent structure evaluation.

## Treatment

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### Medical Therapy

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There is no medical treatment for osteoma; the mainstay of therapy is surgery.[23]

### Surgery

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Surgery is the mainstay of treatment for osteoma.[24][25]

Indication

* For symptomatic lesions, local excision is performed.

Types of Surgery

* Medial maxillectomy with a lateral rhinotomy
* Craniofacial resection
* Endoscopic resection

Recurrence Rare recurrence may occur after several years.

### Primary Prevention

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There are no established measures for the primary prevention of osteoma.

### Secondary Prevention

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There are no established measures for the secondary prevention of osteoma.

## References

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1. ↑ Peabody, Terrance (2014). Orthopaedic oncology : primary and metastatic tumors of the skeletal system. Cham: Springer. ISBN 9783319073224.
2. ↑ Charlier P, Froesch P, Benmoussa N, Froment A, Shorto R, Huynh-Charlier I (2014). "Did René Descartes have a giant ethmoidal sinus osteoma?". Lancet. 384 (9951): 1348. doi:10.1016/S0140-6736(14)61816-X. PMID 25307842.
3. ↑ Jawad MU, Scully SP (2010). "In brief: classifications in brief: enneking classification: benign and malignant tumors of the musculoskeletal system". Clin Orthop Relat Res. 468 (7): 2000–2. doi:10.1007/s11999-010-1315-7. PMC 2882012. PMID 20333492.
4. ↑ Athwal P, Stock H (2014). "Osteoid osteoma: a pictorial review". Conn Med. 78 (4): 233–5. PMID 24830123.
5. ↑ Bilkay U, Erdem O, Ozek C, Helvaci E, Kilic K, Ertan Y; et al. (2004). "Benign osteoma with Gardner syndrome: review of the literature and report of a case". J Craniofac Surg. 15 (3): 506–9. PMID 15111819.CS1 maint: Explicit use of et al. (link) CS1 maint: Multiple names: authors list (link)
6. ↑ 6.0 6.1 Abdel Tawab HM, Kumar V R, Tabook SM (2015). "Osteoma presenting as a painless solitary mastoid swelling". Case Rep Otolaryngol. 2015: 590783. doi:10.1155/2015/590783. PMC 4341844. PMID 25767729. Vancouver style error: name (help)
7. ↑ Bisgaard ML, Bülow S (2006). "Familial adenomatous polyposis (FAP): genotype correlation to FAP phenotype with osteomas and sebaceous cysts". Am J Med Genet A. 140 (3): 200–4. doi:10.1002/ajmg.a.31010. PMID 16411234.
8. ↑ Kaplan I, Calderon S, Buchner A (1994). "Peripheral osteoma of the mandible: a study of 10 new cases and analysis of the literature". J Oral Maxillofac Surg. 52 (5): 467–70. PMID 8169708.CS1 maint: Multiple names: authors list (link)
9. ↑ 9.0 9.1 Erdogan N, Demir U, Songu M, Ozenler NK, Uluç E, Dirim B (2009). "A prospective study of paranasal sinus osteomas in 1,889 cases: changing patterns of localization". Laryngoscope. 119 (12): 2355–9. doi:10.1002/lary.20646. PMID 19780030.
10. ↑ Larrea-Oyarbide N, Valmaseda-Castellón E, Berini-Aytés L, Gay-Escoda C (2008). "Osteomas of the craniofacial region. Review of 106 cases". J Oral Pathol Med. 37 (1): 38–42. doi:10.1111/j.1600-0714.2007.00590.x. PMID 18154576.CS1 maint: Multiple names: authors list (link)
11. ↑ Earwaker J (1993). "Paranasal sinus osteomas: a review of 46 cases". Skeletal Radiol. 22 (6): 417–23. PMID 8248815.
12. ↑ Boysen M (1978). "Osteomas of the paranasal sinuses". J Otolaryngol. 7 (4): 366–70. PMID 691104.
13. ↑ Septer S, Slowik V, Morgan R, Dai H, Attard T (2013). "Thyroid cancer complicating familial adenomatous polyposis: mutation spectrum of at-risk individuals". Hered Cancer Clin Pract. 11 (1): 13. doi:10.1186/1897-4287-11-13. PMC 3854022. PMID 24093640.
14. ↑ Sayan NB, Uçok C, Karasu HA, Günhan O (2002). "Peripheral osteoma of the oral and maxillofacial region: a study of 35 new cases". J Oral Maxillofac Surg. 60 (11): 1299–301. PMID 12420263.CS1 maint: Multiple names: authors list (link)
15. ↑ 15.0 15.1 Wijn MA, Keller JJ, Giardiello FM, Brand HS (2007). "Oral and maxillofacial manifestations of familial adenomatous polyposis". Oral Dis. 13 (4): 360–5. doi:10.1111/j.1601-0825.2006.01293.x. PMID 17577321.CS1 maint: Multiple names: authors list (link)
16. ↑ GARDNER EJ, PLENK HP (1952). "Hereditary pattern for multiple osteomas in a family group". Am. J. Hum. Genet. 4 (1): 31–6. PMC 1716387. PMID 14933371.
17. ↑ Smith ME, Calcaterra TC (1989). "Frontal sinus osteoma". Ann Otol Rhinol Laryngol. 98 (11): 896–900. doi:10.1177/000348948909801111. PMID 2817682.
18. ↑ Fu YS, Perzin KH (1974). "Non-epithelial tumors of the nasal cavity, paranasal sinuses, and nasopharynx. A clinicopathologic study. II. Osseous and fibro-osseous lesions, including osteoma, fibrous dysplasia, ossifying fibroma, osteoblastoma, giant cell tumor, and osteosarcoma". Cancer. 33 (5): 1289–305. PMID 4207295.
19. ↑ Schajowicz, Fritz (1994). Tumors and Tumorlike Lesions of Bone : Pathology, Radiology, and Treatment. Berlin, Heidelberg: Springer Berlin Heidelberg. ISBN 9783642499562.
20. ↑ Schajowicz, Fritz (1994). Tumors and Tumorlike Lesions of Bone : Pathology, Radiology, and Treatment. Berlin, Heidelberg: Springer Berlin Heidelberg. ISBN 9783642499562.
21. ↑ Schajowicz, Fritz (1994). Tumors and Tumorlike Lesions of Bone : Pathology, Radiology, and Treatment. Berlin, Heidelberg: Springer Berlin Heidelberg. ISBN 9783642499562.
22. ↑ Li Y, Zhang L, Zhou B, Han D (2009). "[Resection of frontal ethmoid sinus osteomas with nasal endoscopy]". Lin Chung Er Bi Yan Hou Tou Jing Wai Ke Za Zhi (in Chinese). 23 (14): 628–30. PMID 19894552.CS1 maint: Unrecognized language (link)
23. ↑ Gorini E, Mullace M, Migliorini L, Mevio E (2014). "Osseous choristoma of the tongue: a review of etiopathogenesis". Case Rep Otolaryngol. 2014: 373104. doi:10.1155/2014/373104. PMC 4279709. PMID 25580337.CS1 maint: Multiple names: authors list (link)
24. ↑ Kim WH, Kim DW, Kim CG, Kim MH (2013). "Additional Detection of Multiple Osteomas in a Patient with Gardner's Syndrome by Bone SPECT/CT". Nucl Med Mol Imaging. 47 (4): 297–8. doi:10.1007/s13139-013-0225-5. PMC 4035179. PMID 24900131.CS1 maint: Multiple names: authors list (link)
25. ↑ Alexander AA, Patel AA, Odland R (2007). "Paranasal sinus osteomas and Gardner's syndrome". Ann Otol Rhinol Laryngol. 116 (9): 658–62. doi:10.1177/000348940711600906. PMID 17926587.CS1 maint: Multiple names: authors list (link)

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