Tumors and Other Lesions of Bone
This section includes the following conditions:
Fibrous dysplasia; no ICD-O code
Ossifying fibroma; no ICD-O code
Osteoma; ICD-O 9180
Osteoblastoma; ICD-O 9200/0
Osteoid osteoma; ICD-O 9191/0
Aneurysmal bone cyst; no ICD-O code
Giant cell lesion; no ICD-O code
Giant cell tumor of bone; ICD-O 9250/1
Cherubism; no ICD-O code
“Brown tumor” of hyperparathyroidism; no ICD-O code
Osseous metaplasia; no ICD-O code
Paget′s disease; no ICD-O code
Osteosarcoma; ICD-O 9200/0
Benign Fibro-Osseous Lesions
Definition
This grouping comprises tumors and proliferative disorders that can affect the jaws in which the common denominator is replacement of normal bone architecture by collagen, fibroblasts, and varying amounts of bone or osteoid. They can range from incidental findings on imaging to massive disfiguring lesions and can affect all regions of the paranasal sinuses.1–3 The commonest fibroosseous lesions of the sinonasal tract are fibrous dysplasia and ossifying fibroma. Other lesions that are sometimes considered in this group include giant cell tumors and osteoblastoma, but it is an area subject to much change in pathological nomenclature to the extent that ossifying fibroma, as such, no longer features in the latest WHO classification (see pp. 42–44). A simpler classification of the conditions is shown in Table 13.1 and the most important features of fibrous dysplasia, ossifying fibroma, and osteoma are shown in Table 13.2.
Given the considerable overlap in histological composition, the diagnosis can certainly be difficult for the histopathologist, who will often require clinical and radiological information in addition to the tissue and recourse to a dedicated bone pathologist may be necessary in some instances.
Fibromatosis | Ossifying fibroma |
Developmental | Fibrous dysplasia |
Reactive/reparative | Giant cell tumor |
“Brown tumor” | |
Paget′s disease | |
Neoplasms | Osteoma |
Osteoblastoma |
In our series of 112 cases of benign fibro-osseous conditions (Table 13.3), patients’ age range was 2 to 80 years, mean 27 years, the ratio of men to women was 2.2:1 and the patients were referred by ophthalmologists (44%), or ENT colleagues (56%), most often with proptosis, diplopia, visual loss, and/or epiphora.
Key Points
Benign fibro-osseous disease:
Exhibits a spectrum of disease.
Is difficult to diagnose on histology alone.
Requires clinical history, histology, and imaging to make diagnosis.
Does not always require treatment!
Requires an appropriate surgical approach to be chosen to encompass the lesion.
Fibrous Dysplasia
Definition
Fibrous dysplasia (FD) was first described by Lichtenstein in 1938 and later by Lichtenstein and Jaffe as a monostotic or polyostotic tumorlike lesion that is composed of fibrous stroma and osseous tissue.1,5–7
Etiology
Pensler et al8 investigated cultures derived from the involved bone in two children with monostotic disease and in one child with McCune-Albright syndrome and showed a 2- to 3-fold increased level of estrogen and progesterone receptors by radioimmunoassay and immunocytochemical assay. From their results they concluded that estrogen may play a major role in the bony metabolism of FD. The actual cause of fibrous dysplasia and related disorders, including McCune-Albright syndrome, has recently been defined as a set of mutations in the GNAS1 gene, located on chromosome 20q13.2, which normally codes for the α subunit of the G-protein.9–11 It should, therefore, be regarded as a genetically based developmental anomaly and probably as a hamartomatous process.
Synonyms
Over 33 different terms have been used for this condition, ranging from focal fibrosis of bone through osteitis fibrosis localis to fibrosis chondroficans.
Osteoma | 55 |
Ossifying fibroma | 31 |
Fibrous dysplasia | 18 |
Other, e.g., osteoblastoma | 8 |
Total | 112 |
Site
Fibrous dysplasia can be polyostotic (15–30%), involving more than one bone, or monostotic (70–85%), involving only one bone.13 The femur and other long bones are often involved as well as the jaws, skull, and ribs. Twenty-five percent of monostotic cases arise in the facial skeleton.14 The maxilla and mandible are the most common sites in the head and neck, although it has been reported throughout the maxillofacial skeleton, including all the other paranasal sinuses.13,15–17 In our cohort of 18 cases, 8 involved the sphenoid and orbital apex (Figs. 13.1 and 13.2). A third rare disseminated form, McCune-Albright syndrome, has been described that is part of the polyostotic group.12,18
Diagnostic Features
Clinical Features
Fibrous dysplasia is variously described as more common in females than in males,19,20 usually in the polyostotic forms, having a ratio of 1:119 in monostotic forms, or, as in our group of 18 cases, 2:1 (male-to-female ratio) (Table 13.4).
The condition usually presents in the first two decades.1,12,21,22 The condition tends to stabilize after puberty and may burn out in the patient′s thirties.12,19 Our patient group ranged from 20 to 66 years with a mean age of 37 years. McCune-Albright syndrome, a polyostotic form of fibrous dysplasia characterized by precocious puberty and café au lait spots, is the rarest form and preferentially involves young girls.23 Asymptomatic fibrous dysplasia is often an incidental finding on radiographs obtained for other reasons (trauma or evaluation of hearing loss). The sphenoid bone and central skull base are frequently involved in such cases.
Cosmetic changes due to facial swelling and asymmetry are the most common clinical signs.14,17,22,24 These are most often painless but some patients may complain of pain, ocular symptoms, and neurological changes.12,19,25,26 Proptosis is not uncommon and ultimately visual loss may occur but the nerve can withstand an impressive degree and duration of compromise when it occurs slowly (Fig. 13.2).
Imaging
For the diagnosis of FD the “ground glass” bone appearance on CT scans27 with bone window is the most useful radiographic sign.28 However, the lesion changes with time. Early lesions can be radiolucent or cystic, radiopaque in mid-stage lesions, and a mixture of radiolucent and radiopaque in late-stage lesions (Figs. 13.1 and 13.2). The lesion is not encapsulated and infiltrates the bone widely, sometimes spreading to adjacent bones (e.g., maxilla and zygoma).
FD is sometimes associated with expansion of the adjacent sinus or pneumosinus dilatans, a condition that has been described in association with meningioma, with FD, or spontaneously. It was first described by Benjamin in 191829 and more recently by Lloyd in 1985.30
Histological Features and Differential Diagnosis
The World Health Organization defined FD as “a nonencapsulated lesion showing replacement of normal bone by fibrous connective tissue of varying cellularity and containing islands of trabecular or immature nonlamellar metaplastic bone. Osteoblastic rimming is inconspicuous or absent.”31
Histology shows slow replacement of medullary bone by abnormal fibrous tissue with different stages of bone metaplasia.1,12 It must be distinguished from the “brown tumor” of hyperparathyroidism, from osseous metaplasia, and from osteosarcoma.7
Natural History
Growth is variable and usually slows after puberty, but this is not invariable.32 However, in most cases the condition burns out in the patient′s thirties. Fibrous dysplasia has a low rate of malignant transformation into osteosarcoma.13,14,33 Transformation occurs in 0.5% of polyostotic forms and in 4% of lesions in patients with McCune-Albright syndrome.13,34
Treatment and Outcome
As the condition is eventually self-limiting in most cases, it may be best to adopt a policy of “watchful waiting,” particularly as neither medical therapy nor surgery can readily cure the problem. Follow-up imaging every one or two years until there is no sign of change on sequential scans is recommended, but because the process is best shown on CT, there is an issue with regard to radiation, which should be considered and discussed with the patient. Radiotherapy is contraindicated in the treatment of the condition.
Medical therapy for FD is restricted to symptomatic relief. Bisphosphonates such as disodium pamidronate have been shown to decrease the incidence of fractures and bony pain35–37 but also carry potential complications of their own such as osteonecrosis of the jaw.38 The endocrinological effects of McCune-Albright syndrome can also be treated medically: octreotide can decrease growth hormone secretion; radioactive iodine, methimazole, and propylthiouracil can be applied for hyperthyroidism; spironolactone and ketoconazole can be used as antiandrogens.27
The decision to operate depends on the patient′s symptoms, the extent of the disease, and the patient′s age.12,18,19,25,39 It is not possible to generalize as no large series exist in the literature. Remodeling of the facial contour may be considered but is probably best left until the disease has burnt out. Some authors have reported endonasal endoscopic treatment of FD, especially for the relief of ocular symptoms (optic nerve decompression) or chronic rhinosinusitis.22,25,33,39–43 However, the bone is by definition abnormal and bleeds freely, so the procedure can be technically challenging and should only be contemplated by an experienced surgeon. Nonetheless, it is possible to remove involved bone in the medial half on the orbital apex extending to the canal and thus achieve space while the condition runs its course. This has been undertaken in 10 of our 18 cases with good effect, but the objective of the surgery is not cure. It is not normally necessary to incise the orbital periosteum/perineurium in these circumstances.
Key Points
FD is a genetically based developmental anomaly.
There is benign monostotic (80%) or polyostotic (20%) unencapsulated proliferation of fibrous tissue and woven bone.
FD often starts when the patient is <20 years old and eventually stops growing in the majority but may continue into the late thirties or forties.
Medical treatment is with bisphosphonates; if surgical treatment is needed, it should if possible be deferred until the condition is burnt out.
Ossifying Fibroma
Definition
Ossifying fibroma (OF) is a true benign encapsulated tumor composed of bone, fibrous tissue, calcification, and cementum.1,44–46 The World Health Organization defined OF as “consisting of spindle-shaped fibroblastic cells usually arranged in a whorled pattern and containing small islands and spicules of metaplastic bone and mineralized masses. It may appear encapsulated. The bony spicules may rarely show a lamellar structure peripherally and are frequently rimmed by osteoblasts.”
Etiology
The etiology of OF has so far not been clarified. Trauma has been reported to play a major role in the development of OF, especially of the cemento-ossifying fibroma.47 It seems from studies on a small number of cases that there may also be a genetic basis.48
Synonyms
Ossifying fibroma was first described by Montgomery in 192749 and has its origin mostly in the mandibular bone.45,50 A multiplicity of terms have been applied to this condition, including cemento-ossifying fibroma, cementifying fibroma, and psammomatoid OF, although it has been suggested that these all relate to and should be replaced by juvenile active OF.51
Incidence
Terminological and histopathological confusion has made an accurate assessment of true incidence difficult. Eversole et al,21 albeit 30 years ago, examined 841 cases of fibro-osseous disease, of whom 309 were diagnosed as having fibrous dysplasia and 225 ossifying fibroma. In our own series of 112 patients, 28% had ossifying fibroma (Table 13.4).
Site
OF can be found in any bones ossified in membrane and is most commonly located in the facial skeleton where it most often affects the mandible (~75% of cases) and where it can be clinically silent for a long period. However, OF can occasionally occur in other areas such as the maxilla (10–15%), and rarely the ethmoid bone including the middle turbinate and nasal cavity (Figs. 13.3, 13.4, 13.5).20,45 The maxilla was the commonest area in our series of 31 cases (38%), although the nasal cavity, ethmoid, and frontal were also affected.
Diagnostic Features
Clinical Features
The literature suggests that OF is more frequent in women than men8 with a male-to-female ratio of 1:5.52 This has also been our experience in 11 cases affecting older patients (Table 13.4). Patients usually present between the second and fourth decades of life, somewhat older than those with fibrous dysplasia. The mean age of our patients was 40.5 years.
Depending on its extent, it can lead to swelling, facial pain, nasal obstruction, rhinosinusitis, and ocular symptoms such as proptosis, diplopia, and epiphora.44,47,53,54 There may be changes to dentition if the alveolus is involved and distortion of the mid-face and external nose.
An aggressive variant of OF is termed “juvenile OF,” which starts at an earlier age and was defined by Reed and Hagy55 as “a localized actively growing destructive lesion occurring predominantly in children and teenagers.” The terms “active” or “aggressive” are also included in the name by some authors. We have a group of 20 patients with age range 6 to 20 years, mean 11.3 years (Fig. 13.4). This condition predominantly affects male subjects (in our group 18:2) and is clinically more aggressive with extension of the tumor to the skull base and orbit.56 The condition has been studied in 112 cases by Johnson et al57 and appears to favor the ethmoid and frontal with maxillary lesions constituting 20%.
Imaging
CT images show a characteristic well-demarcated benign expansile mass covered by a thick shell of bone, sometimes with a multiloculated internal appearance and a content of varying density,45,58 and as a consequence is sometimes mistaken for a mucocele. There may be areas of low density or scattered calcification. The mass expands, thins, and destroys adjacent bone, displacing structures such as teeth, nasal septum, the orbital contents, and the skull base (Figs. 13.4, 13.5a).
The MRI appearances will vary depending on the cystic change and calcification. However, the bony walls present signal voids or low density on T2W images and are isointense with gray matter on T1W scans. The contents are isointense with muscle on T1W and T2W scans and gadolinium contrast usually produces only mild homogeneous enhancement on T1W images (Fig. 13.5c).59
Histological Features and Differential Diagnosis
Macroscopically OF presents a circumscribed lesion with defined margins consisting of homogeneous pinkish gray granular/gritty material with a firm consistency. Microscopically it is composed of fibrous tissue with varying amounts of mineralized or calcified psammomatoid bodies.60 This has led to the term “cementifying ossifying fibroma.”
The juvenile OF is characteristically composed of “ossicles,” a stroma, and “chondricles” in myxomatous areas. Some believe that this tumor derives from myxoid tissue, the precursor of cartilage and bone found in the septations of the paranasal sinuses where it forms the mucoperiosteum. In some individuals a completely myxomatous tumor can result (See Myxoma, p. 169).
As previously stated, distinguishing this lesion from fibrous dysplasia (and other fibro-osseous lesions) can be difficult on histology alone and clinical and radiological information must be provided to the histopathologist (Table 13.2).
Natural History
OF continues to grow throughout life and is therefore regarded as having a more aggressive behavior than fibrous dysplasia due to local destruction.
Treatment and Outcome
The treatment depends on the location but in general requires complete surgical resection. The lesion will regrow if not completely removed, so total extirpation should be undertaken whenever possible.52,61 Due to its rarity, there are few large series in the literature that include a wide range of surgical approaches including craniofacial resection, midfacial degloving, and lateral rhinotomy as well as some case reports of entirely endoscopic resection.45,47,62 Draf et al63 reported on endonasal microendoscopic resection of four OFs without any complications. In two cases the surgery was done for optic nerve decompression. In OF with significant intracranial extension, most authors recommend a combined approach including craniofacial resection.20,61,64 All 31 cases in our series underwent surgery (Table 13.4) which comprised midfacial degloving in 42%, craniofacial or osteoplastic flap approaches in 32%, and endoscopic resection in 13%.
Recurrence after surgery is directly related to the thoroughness of excision, but up to one-third of patients with the juvenile OF have recurrence despite repeated operations. In our series 10% (3 cases) recurred and were dealt with either by another midfacial degloving (2 cases) or endoscopic resection (1 case). Recurrence manifested itself 3, 6, and 10 years later and in one patient recurred again 18 years after the previous surgery, suggesting lifelong surveillance may be needed in selected cases.
Radiotherapy is not effective and could induce sarcomatous transformation.
Key Points
OF is an encapsulated true benign neoplasm at one end of the benign fibro-osseous disease spectrum.
There is proliferation of fibrous tissue and woven bone, sometimes with scattered cementum-like or psammomatoid spherules leading to the description of cementifying ossifying fibroma or the “juvenile” form which is more aggressive.
It manifests as an often painless swelling that continues to grow slowly and is locally destructive.
Treatment is by complete surgical excision by whatever approach appropriate.
References
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Am J Clin Pathol 2002;118(Suppl):S50–S70 8. Pensler JM, Langman CB, Radosevich JA, et al. Sex steroid hormone receptors in normal and dysplastic bone disorders in children. J Bone Miner Res 1990;5(5):493–498 9. Shenker A, Weinstein LS, Moran A, et al. Severe endocrine and nonendocrine manifestations of the McCune-Albright syndrome associated with activating mutations of stimulatory G protein GS. J Pediatr 1993;123(4):509–518 10. Ringel MD, Schwindinger WF, Levine MA. Clinical implications of genetic defects in G proteins. The molecular basis of McCune-Albright syndrome and Albright hereditary osteodystrophy. Medicine (Baltimore) 1996; 75(4):171–184 11. Levine MA. Clinical implications of genetic defects in G proteins: oncogenic mutations in G alpha s as the molecular basis for the McCune-Albright syndrome. Arch Med Res 1999;30(6):522–531 12. Ferguson BJ. Fibrous dysplasia of the paranasal sinuses. Am J Otolaryngol 1994;15(3):227–230 13. MacDonald-Jankowski DS. Fibro-osseous lesions of the face and jaws. Clin Radiol 2004;59(1):11–25 14. Tsai TL, Ho CY, Guo YC, Chen W, Lin CZ. Fibrous dysplasia of the ethmoid sinus. J Chin Med Assoc 2003;66(2):131–133 15. Waldron CA. Fibro-osseous lesions of the jaws. J Oral Maxillofac Surg 1993;51(8):828–835 16. Lustig LR, Holliday MJ, McCarthy EF, Nager GT. Fibrous dysplasia involving the skull base and temporal bone. Arch Otolaryngol Head Neck Surg 2001;127(10):1239–1247 17. Chan EK. Ethmoid fibrous dysplasia with anterior skull base and intraorbital extension. Ear Nose Throat J 2005;84(10):627–628 18. Ramsey HE, Strong EW, Frazell EL. Fibrous dysplasia of the craniofacial bones. Am J Surg 1968;116(4):542–547 19. Rojas R, Palacios E, Kaplan J, Wong LK. Fibrous dysplasia of the frontal sinus. Ear Nose Throat J 2004;83(1):14–15 20. Mehta D, Clifton N, McClelland L, Jones NS. Paediatric fibro-osseous lesions of the nose and paranasal sinuses. Int J Pediatr Otorhinolaryngol 2006;70(2):193–199 21. Eversole LR, Sabes WR, Rovin S. Fibrous dysplasia: a nosologic problem in the diagnosis of fibro-osseous lesions of the jaws. J Oral Pathol 1972;1(5):189–220 22. Mladina R, Manojlovic S, Markov-Glavas D, Heinrich Z. Isolated unilateral fibrous dysplasia of the sphenoid sinus. Ann Otol Rhinol Laryngol 1999;108(12):1181–1184 23. Pacini F, Perri G, Bagnolesi P, Cilotti A, Pinchera A. McCune-Albright syndrome with gigantism and hyperprolactinemia. J Endocrinol Invest 1987;10(4):417–420 24. Ozcan KM, Akdogan O, Gedikli Y, Ozcan I, Dere H, Unal T. Fibrous dysplasia of inferior turbinate, middle turbinate, and frontal sinus. B-ENT 2007;3(1):35–38 25. Ikeda K, Suzuki H, Oshima T, Shimomura A, Nakabayashi S, Takasaka T. Endonasal endoscopic management in fibrous dysplasia of the paranasal sinuses. Am J Otolaryngol 1997;18(6):415–418 26. Kim SW, Kim DW, Kong IG, et al. Isolated sphenoid sinus diseases: report of 76 cases. Acta Otolaryngol 2008;128(4):455–459 27. Hullar TE, Lustig LR. Paget′s disease and fibrous dysplasia. Otolaryngol Clin North Am 2003;36(4):707–732 28. Tehranzadeh J, Fung Y, Donohue M, Anavim A, Pribram HW. Computed tomography of Paget disease of the skull versus fibrous dysplasia. Skeletal Radiol 1998;27(12):664–672 29. Benjamin C. Pneumosinus frontalis dilatans. Acta Otolaryngol 1918;1:412–422 30. Lloyd GA. Orbital pneumosinus dilatans. Clin Radiol 1985;36(4):381–386 31. Slootweg PJ. Maxillofacial fibro-osseous lesions: classification and differential diagnosis. Semin Diagn Pathol 1996;13(2):104–112 32. Chen YR, Fairholm D. Fronto-orbito-sphenoidal fibrous dysplasia. Ann Plast Surg 1985;15(3):190–203 33. London SD, Schlosser RJ, Gross CW. Endoscopic management of benign sinonasal tumors: a decade of experience. Am J Rhinol 2002;16(4):221–227 34. Schwartz DT, Alpert M. The malignant transformation of fibrous dysplasia. Am J Med Sci 1964;247:1–20 35. Liens D, Delmas PD, Meunier PJ. Long-term effects of intravenous pamidronate in fibrous dysplasia of bone. Lancet 1994;343(8903):953–954 36. Lala R, Matarazzo P, Bertelloni S, Buzi F, Rigon F, de Sanctis C. Pamidronate treatment of bone fibrous dysplasia in nine children with McCune-Albright syndrome. Acta Paediatr 2000;89(2):188–193 37. Zacharin M, O′Sullivan M. Intravenous pamidronate treatment of polyostotic fibrous dysplasia associated with the McCune Albright syndrome. J Pediatr 2000; 137(3):403–409 38. Crépin S, Laroche M-L, Sarry B, Merle L. Osteonecrosis of the jaw induced by clodronate, an alkylbiphosphonate: case report and literature review. Eur J Clin Pharmacol 2010;66(6):547–554 39. Kessler A, Berenholz LP, Segal S. Use of intranasal endoscopic surgery to relieve ostiomeatal complex obstruction in fibrous dysplasia of the paranasal sinuses. Eur Arch Otorhinolaryngol 1998;255(9):454–456 40. Brodish BN, Morgan CE, Sillers MJ. Endoscopic resection of fibro-osseous lesions of the paranasal sinuses. Am J Rhinol 1999;13(1):11–16 41. Kingdom TT, Delgaudio JM. Endoscopic approach to lesions of the sphenoid sinus, orbital apex, and clivus. Am J Otolaryngol 2003;24(5):317–322 42. Eviatar E, Vaiman M, Shlamkovitch N, Segal S, Kessler A, Katzenell U. Removal of sinonasal tumors by the endonasal endoscopic approach. Isr Med Assoc J 2004;6(6):346–349 43. Socher JA, Cassano M, Filheiro CA, Cassano P, Felippu A. Diagnosis and treatment of isolated sphenoid sinus disease: a review of 109 cases. Acta Otolaryngol 2008;128(9):1004–1010 44. Chong VF, Tan LH. Maxillary sinus ossifying fibroma. Am J Otolaryngol 1997;18(6):419–424 45. Choi YC, Jeon EJ, Park YS. Ossifying fibroma arising in the right ethmoid sinus and nasal cavity. Int J Pediatr Otorhinolaryngol 2000;54(2-3):159–162 46. Granados R, Carrillo R, Nájera L, García-Villanueva M, Patrón M. Psammomatoid ossifying fibromas: immunohistochemical analysis and differential diagnosis with psammomatous meningiomas of craniofacial bones. Oral Surg Oral Med Oral Pathol Oral Radiol Endod 2006;101(5):614–619 47. Yilmaz I, Bal N, Ozluoglu LN. Isolated cementoossifying fibroma of the ethmoid bulla: a case report. Ear Nose Throat J 2006;85(5):322–324 48. Sawyer JR, Tryka AF, Bell JM, Boop FA. Nonrandom chromosome breakpoints at Xq26 and 2q33 characterize cemento-ossifying fibromas of the orbit. Cancer 1995;76(10):1853–1859 49. Montgomery AH. Ossifying fibromas of the jaw. Arch Surg 1927;15:30–44 50. Kendi AT, Kara S, Altinok D, Keskil S. Sinonasal ossifying fibroma with fluid-fluid levels on MR images. AJNR Am J Neuroradiol 2003;24(8):1639–1641 51. Robinson R. Head and Neck Pathology: Atlas for Histologic and Cytologic Diagnosis. Philadelphia: Wolters Kluwer, Lippincott Williams and Wilkins; 2010:102 52. Commins DJ, Tolley NS, Milford CA. Fibrous dysplasia and ossifying fibroma of the paranasal sinuses. J Laryngol Otol 1998;112(10):964–968 53. Hauser MS, Freije S, Payne RW, Timen S. Bilateral ossifying fibroma of the maxillary sinus. Oral Surg Oral Med Oral Pathol 1989;68(6):759–763 54. Cheng C, Takahashi H, Yao K, et al. Cemento-ossifying fibroma of maxillary and sphenoid sinuses: case report and literature review. Acta Otolaryngol Suppl 2002; 547(547, Suppl)118–122 55. Reed RJ, Hagy DM. Benign nonodontogenic fibro-osseous lesions of the skull; Report of two cases. Oral Surg Oral Med Oral Pathol 1965;19:214–227 56. Khoury NJ, Naffaa LN, Shabb NS, Haddad MC. Juvenile ossifying fibroma: CT and MR findings. Eur Radiol 2002;12(Suppl 3):S109–S113 57. Johnson LC, Yousefi M, Vinh TN, Heffner DK, Hyams VJ, Hartman KS. Juvenile active ossifying fibroma. Its nature, dynamics and origin. Acta Otolaryngol Suppl 1991;488(Suppl):1–40 58. Levine PA, Wiggins R, Archibald RW, Britt R. Ossifying fibroma of the head and neck: involvement of the temporal bone- and unusual and challenging site. Laryngoscope 1981;91(5):720–725 59. Bendet E, Bakon M, Talmi YP, Tadmor R, Kronenberg J. Juvenile cemento-ossifying fibroma of the maxilla. Ann Otol Rhinol Laryngol 1997;106(1):75–78 60. Wenig BM, Vinh TN, Smirniotopoulos JG, Fowler CB, Houston GD, Heffner DK. Aggressive psammomatoid ossifying fibromas of the sinonasal region: a clinicopathologic study of a distinct group of fibro-osseous lesions. Cancer 1995;76(7):1155–1165 61. Lund VJ, Howard DJ, Wei WI, Cheesman AD. Craniofacial resection for tumors of the nasal cavity and paranasal sinuses—a 17-year experience. Head Neck 1998;20(2):97–105 62. Post G, Kountakis SE. Endoscopic resection of large sinonasal ossifying fibroma. Am J Otolaryngol 2005;26(1):54–56 63. Draf W, Schick B, Weber R, Keerl R, Saha A. Endonasal micro-endoscopic surgery of nasal and paranasal-sinuses tumors. In: Stamm AC, Draf W, eds. Micro-Endoscopic Surgery of the Paranasal Sinuses and the Skull Base. Berlin: Springer; 2000:481–8 64. Howard DJ, Lund VJ, Wei WI. Craniofacial resection for tumors of the nasal cavity and paranasal sinuses: a 25-year experience. Head Neck 2006;28(10):867–873Osteoma
Definition
(ICD-O code 9180)
A slow-growing benign bony tumor.
Etiology
There is some debate whether osteomas are tumors or merely reactive bone hyperplasia due to some irritant stimulus. There are three main theories: developmental, traumatic, and infectious. In the developmental theory, previously silent embryonic stem cells become activated later in life and lead to uncontrolled bone formation. In the traumatic and infectious theories, an inflammatory process is regarded as the inciting factor for the osteoma formation.1–4
The predilection for osteomas to occur in the sinuses near the junction between the skull vault (formed in membrane) and skull base (formed in cartilage) suggests an area of instability and supports the developmental theory.
Gardner′s syndrome is a rare autosomal dominant condition of multiple osteomas in the skull associated with colonic polyps, which may become malignant, and other soft tissue tumors.5
History and Synonyms
Viega is credited as being the first physician who reported a sinus osteoma case, which was successfully removed by him in 1506.6,7 Vallisnieri described the true bony origin of sinonasal osteoma in 1733, and in 1857 Bickersteth reported an antral osteoma that he had to saw in half to remove.8–10
Incidence
Osteoma is the most common bone tumor of the facial region.11 The incidence of osteoma ranges from 0.43%12 (skull radiographic study) to 3% in an analysis of CT scans.13 This figure of 3% was confirmed in a more recent prospective study of 1,889 CT scans done for chronic sinus disease.14 It has been suggested that there were some geographic/ethnic variations with large series being reported from Egypt,15 but this may have simply reflected local patterns of referral.
Site
Osteoma is a benign, slowly growing neoplasm and arises mostly from the frontal sinus (57%) followed by the ethmoid, maxillary sinus, and sphenoid sinus.8,13,16–20 A number cross the midline, especially in the frontal region, and can become markedly bilateral (Figs. 13.6, 13.7, 13.8).
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