Osteoma

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For more information about osteoid osteoma that is not associated with sino-orbital osteoma, see osteoid osteoma

Osteoma Microchapters

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Patient Information

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

X Ray

CT

MRI

Ultrasound

Other Imaging Findings

Other Diagnostic Studies

Treatment

Medical Therapy

Surgery

Primary Prevention

Secondary Prevention

Cost-Effectiveness of Therapy

Future or Investigational Therapies

Case Studies

Case #1

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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

Historical Perspective

  • 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

Osteoma can be classified based on imaging findings.

Enneking (MSTS) Staging System

  • 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

  • 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

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

Causes

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

Differentiating ((Page name)) from Other Diseases

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 tumours, fibrous displasia, 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

  • 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

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

Screening

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

  • If left untreated, osteoma progression occurs slowly and is then followed by facial distortion.[14]
  • Common complications of osteoma include:[15]
  • Prognosis is generally excellent,the lesion does not recur after surgical excision and it is not associated with malignant change.[15]

Diagnosis

Diagnostic Study of Choice

The diagnosis of [disease name] is made when at least [number] of the following [number] diagnostic criteria are met: [criterion 1], [criterion 2], [criterion 3], and [criterion 4].

OR

The diagnosis of [disease name] is based on the [criteria name] criteria, which include [criterion 1], [criterion 2], and [criterion 3].

OR

The diagnosis of [disease name] is based on the [definition name] definition, which includes [criterion 1], [criterion 2], and [criterion 3].

OR

There are no established criteria for the diagnosis of [disease name].

History and Symptoms

  • Small osteomas are asymptomatic and usually incidental findings.
  • Common symotoms of osteoma include:[16][17]

Physical Examination

  • 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

There are no diagnostic laboratory findings associated with osteoma.

Electrocardiogram

There are no ECG findings associated with osteoma.

X-ray

There are no x-ray findings associated with [disease name].

OR

An x-ray may be helpful in the diagnosis of [disease name]. Findings on an x-ray suggestive of/diagnostic of [disease name] include [finding 1], [finding 2], and [finding 3].

OR

There are no x-ray findings associated with [disease name]. However, an x-ray may be helpful in the diagnosis of complications of [disease name], which include [complication 1], [complication 2], and [complication 3].

Echocardiography or Ultrasound

There are no echocardiography/ultrasound findings associated with osteoma.

CT scan

There are no CT scan findings associated with [disease name].

OR

[Location] CT scan may be helpful in the diagnosis of [disease name]. Findings on CT scan suggestive of/diagnostic of [disease name] include [finding 1], [finding 2], and [finding 3].

OR

There are no CT scan findings associated with [disease name]. However, a CT scan may be helpful in the diagnosis of complications of [disease name], which include [complication 1], [complication 2], and [complication 3].

MRI

There are no MRI findings associated with [disease name].

OR

[Location] MRI may be helpful in the diagnosis of [disease name]. Findings on MRI suggestive of/diagnostic of [disease name] include [finding 1], [finding 2], and [finding 3].

OR

There are no MRI findings associated with [disease name]. However, a MRI may be helpful in the diagnosis of complications of [disease name], which include [complication 1], [complication 2], and [complication 3].

Other Imaging Findings

There are no other imaging findings associated with osteoma.

Other Diagnostic Studies

There are no other diagnostic studies associated with [disease name].

OR

[Diagnostic study] may be helpful in the diagnosis of [disease name]. Findings suggestive of/diagnostic of [disease name] include [finding 1], [finding 2], and [finding 3].

OR

Other diagnostic studies for [disease name] include [diagnostic study 1], which demonstrates [finding 1], [finding 2], and [finding 3], and [diagnostic study 2], which demonstrates [finding 1], [finding 2], and [finding 3].

Treatment

Medical Therapy

There is no treatment for [disease name]; the mainstay of therapy is supportive care.

OR

Supportive therapy for [disease name] includes [therapy 1], [therapy 2], and [therapy 3].

OR

The majority of cases of [disease name] are self-limited and require only supportive care.

OR

[Disease name] is a medical emergency and requires prompt treatment.

OR

The mainstay of treatment for [disease name] is [therapy].

OR   The optimal therapy for [malignancy name] depends on the stage at diagnosis.

OR

[Therapy] is recommended among all patients who develop [disease name].

OR

Pharmacologic medical therapy is recommended among patients with [disease subclass 1], [disease subclass 2], and [disease subclass 3].

OR

Pharmacologic medical therapies for [disease name] include (either) [therapy 1], [therapy 2], and/or [therapy 3].

OR

Empiric therapy for [disease name] depends on [disease factor 1] and [disease factor 2].

OR

Patients with [disease subclass 1] are treated with [therapy 1], whereas patients with [disease subclass 2] are treated with [therapy 2].

Surgery

Surgical intervention is not recommended for the management of [disease name].

OR

Surgery is not the first-line treatment option for patients with [disease name]. Surgery is usually reserved for patients with either [indication 1], [indication 2], and [indication 3]

OR

The mainstay of treatment for [disease name] is medical therapy. Surgery is usually reserved for patients with either [indication 1], [indication 2], and/or [indication 3].

OR

The feasibility of surgery depends on the stage of [malignancy] at diagnosis.

OR

Surgery is the mainstay of treatment for [disease or malignancy].

Primary Prevention

There are no established measures for the primary prevention of [disease name].

OR

There are no available vaccines against [disease name].

OR

Effective measures for the primary prevention of [disease name] include [measure1], [measure2], and [measure3].

OR

[Vaccine name] vaccine is recommended for [patient population] to prevent [disease name]. Other primary prevention strategies include [strategy 1], [strategy 2], and [strategy 3].

Secondary Prevention

There are no established measures for the secondary prevention of [disease name].

OR

Effective measures for the secondary prevention of [disease name] include [strategy 1], [strategy 2], and [strategy 3].

References

  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.
  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.
  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.
  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.
  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.
  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.


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