Craniofacial fibrous dysplasia

Camilleri, A. E.

doi:10.1017/s0022215100116974

Cited by 55 publications

(31 citation statements)

References 14 publications

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“…In the craniofacial complex, fibrous dysplasia is most commonly seen in the maxilla and calvarium, whereas in the remainder of the skeleton, it is seen in most frequently seen in the rib, femur, and tibia (45). The process usually stabilizes during puberty, persisting in a nearly quiescent state indefinitely.…”

Section: Fibrous Dysplasiamentioning

confidence: 99%

Odontogenic Cysts, Odontogenic Tumors, Fibroosseous, and Giant Cell Lesions of the Jaws

2002

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Odontogenic cysts that can be problematic because of recurrence and/or aggressive growth include odontogenic keratocyst (OKC), calcifying odontogenic cyst, and the recently described glandular odontogenic cyst. The OKC has significant growth capacity and recurrence potential and is occasionally indicative of the nevoid basal cell carcinoma syndrome. There is also an orthokeratinized variant, the orthokeratinized odontogenic cyst, which is less aggressive and is not syndrome associated. Ghost cell keratinization, which typifies the calcifying odontogenic cyst, can be seen in solid lesions that have now been designated odontogenic ghost cell tumor. The glandular odontogenic cyst contains mucous cells and ductlike structures that may mimic central mucoepidermoid carcinoma. Several odontogenic tumors may provide diagnostic challenges, particularly the cystic ameloblastoma. Identification of this frequently underdiagnosed cystic tumor often comes after one or more recurrences and a destructive course. Other difficult lesions include malignant ameloblastomas, calcifying epithelial odontogenic tumor, squamous odontogenic tumor, and clear-cell odontogenic tumor. Histologic identification of myxofibrous lesions of the jaws (odontogenic myxoma, odontogenic fibroma, desmoplastic fibroma) is necessary to avoid the diagnostic pitfall of overdiagnosis of similar-appearing follicular sacs and dental pulps. Fibroosseous lesions of the jaws show considerable microscopic overlap and include fibrous dysplasia, ossifying fibroma, periapical cementoosseous dysplasia, and low-grade chronic osteomyelitis. The term fibrous dysplasia is probably overused in general practice and should be reserved for the rare lesion that presents as a large, expansile, diffuse opacity of children and young adults. The need to use clinicopathologic correlation in assessing these lesions is of particular importance. Central giant cell granuloma is a relatively common jaw lesion of young adults that has an unpredictable behavior. Microscopic diagnosis is relatively straightforward; however, this lesion continues to be somewhat controversial because of its disputed classification (reactive versus neoplastic) and because of its management (surgical versus. medical). Its relationship to giant cell tumor of long bone remains undetermined.

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Section: Fibrous Dysplasiamentioning

confidence: 99%

Odontogenic Cysts, Odontogenic Tumors, Fibroosseous, and Giant Cell Lesions of the Jaws

2002

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“…The aggressive and destructive nature of the lesion and its recurrences may require surgical intervention with extensive resection and reconstruction, as occurred in our patients. Because this form of fibrous dysplasia can be very vascular with arteriovenous malformations, patients may have extensive bleeding at the time of surgery similar to angiofibroma [3,9,30]. Although aggressive fibrous dysplasia was relatively vascular in our patients, no bleeding difficulties were encountered at surgery.…”

Section: Discussionmentioning

confidence: 70%

Aggressive fibrous dysplasia of the maxillary sinus

et al. 1993

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Fibrous dysplasia is usually a slowly progressive, benign disease that develops over several years and presents with deformity or mild symptomatology. Five of 34 patients (ages 4-21 years), who were subsequently diagnosed histologically as having fibrous dysplasia of the maxillary sinus, rapidly developed soft tissue masses of the malar region over a period of less than 4 months with accompanying pain (2 patients) and nasal obstruction and exophthalmos (2 patients). Each was clinically suspected of having a sarcoma; two had been thought to have an "osteofibrosarcoma" on initial biopsy at outside hospitals. After resection, all lesions developed regrowth. At histopathologic examination, both initial and recurrent masses proved to be typical fibrous dysplasia with spicules of woven bone in cellular, sometimes vascular, fibrous tissue. No malignant degeneration was found. On conventional radiography, aggressive fibrous dysplasia produced opacification and expansion of the maxillary sinus and apparent disruption of its wall with an associated soft tissue mass. Computed tomography (CT) demonstrated voluminous heterogeneous masses with "ground glass appearance", calcifications, areas of enhancement, low attenuation, cystic areas, and a thinned, sometimes interrupted, maxillary wall. Despite the aggressive clinical course for both initial and recurrent lesions, the CT findings of a "ground glass" mass with calcifications surrounded by a maxillary sinus wall, even if incomplete, can suggest the diagnosis of aggressive fibrous dysplasia.

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“…Most common are facial deformities, atypical facial pain and headaches 7 8. Additionally, cranial nerve dysfunction has also been reported including visual impairment and labyrinth involvement with sensorineural hearing loss and dizziness 9–11.…”

Section: Discussionmentioning

confidence: 99%

Olfactory dysfunction as first presenting symptom of cranial fibrous dysplasia

et al. 2013

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Fibrous dysplasia (FD) is a benign bone disorder presenting with a variety of clinical manifestations. This is the first reported case of anosmia as presenting symptom of FD. We present the case of a 72-year-old female patient with a progressive olfactory dysfunction. Clinical examination revealed evidence of chronic rhinosinusitis; therefore the patient was treated with a course of oral corticosteroids. The patient had no improvement in her olfactory ability and imaging studies were ordered. Bony lesions characteristic of craniofacial FD were found, causing obstruction of the central olfactory pathway. This case emphasises the need to conduct further investigations in patients with rhinosinusitis and olfactory dysfunction especially when they present no response to oral steroid treatment.

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Craniofacial fibrous dysplasia

Cited by 55 publications

References 14 publications

Odontogenic Cysts, Odontogenic Tumors, Fibroosseous, and Giant Cell Lesions of the Jaws

Odontogenic Cysts, Odontogenic Tumors, Fibroosseous, and Giant Cell Lesions of the Jaws

Aggressive fibrous dysplasia of the maxillary sinus

Olfactory dysfunction as first presenting symptom of cranial fibrous dysplasia

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