33 Questions
Which molecular tool is most useful to differentiate fibrous dysplasia from other fibro-osseous lesions?
GNAS1 mutations
What is the clinical presentation of fibrous dysplasia?
Thickening of skull bones
Which imaging modality is important for investigating fibro-osseous lesions?
CT and CBCT
What is the most common fibrous dysplasia subtype encountered in the maxillofacial region?
Craniofacial FD
What is the genetic cause of fibrous dysplasia?
GNAS1 mutations
What are the radiographic features of early onset fibrous dysplasia lesions?
Ground glass appearance
At what age is fibrous dysplasia commonly diagnosed?
Second decade of life
What is the characteristic feature of benign fibro-osseous lesions?
Hypercellular fibroblastic stroma
Which syndrome is a clinical subtype of fibrous dysplasia?
McCune—Albright syndrome
What is the pattern of involvement for craniofacial fibrous dysplasia?
Maxilla-zygoma-sphenoid-frontal-nasal bones
What is the key requirement for the diagnosis of benign fibro-osseous lesions?
Pathology reports
Where are cemento-ossifying fibromas (COF) primarily found?
Mandible and maxilla
In which age group do COFs show aggressiveness and often cause displacement of teeth?
Younger age groups
What is the peak age for the occurrence of COFs?
3rd and 4th decades of life
What is the preferred initial treatment option for COFs?
Surgical curettage
Where is Juvenile-Ossifying Fibroma (JOF) predominantly found?
Extragnathic bones of the craniofacial skeleton
What is the characteristic feature of COFs on radiographs as the lesion matures?
Becoming mixed with radiopaque foci or purely radiopaque
What is the recurrence rate of COFs?
Variable and unpredictable
What is the more aggressive variant seen in younger age groups?
Juvenile-Ossifying Fibroma (JOF)
What is the preferred treatment option for lesions exhibiting aggressive behavior and recurrence?
Resection
Who proposed a classification for fibro-osseous lesions in 1985?
Charles A. Waldron
Which classification includes entities like ossifying fibroma and central giant cell granuloma?
WHO's 2005 classification
What is replaced by connective tissue and fibroblasts in fibro-osseous lesions?
Normal bone
What is the basis for the final diagnosis of fibro-osseous lesions?
Correlation of clinical, radiological, and histological features
What reflects the spectrum of clinicopathological entities in fibro-osseous lesions?
The classifications
What is the most important factor for the final diagnosis of fibro-osseous lesions?
Correlation of clinical, radiological, and histological features
What is the typical presentation of fibrous dysplasia (FD)?
Slow growth and may stabilize over time
What is the histological appearance of fibrous dysplasia (FD)?
Trabeculae of immature woven bone dispersed in cellular fibroblastic stroma
What factors determine the treatment for fibrous dysplasia?
Patient's age, presence of cosmetic and functional deficits, and growth rate
What are the categories under benign fibro-osseous lesions (BFOL)?
Reactive processes and proliferative periostitis
When is optic canal decompression advised?
For orbital dysplasia and decreasing visual acuity
What is the role of non-surgical treatment in fibrous dysplasia?
Limited role, with some promising options for symptom reduction
What needs to be differentiated from fibrous dysplasia?
Cemento-ossifying fibroma (COF)
Study Notes
Fibro-Osseous Lesions of the Craniofacial Complex: Clinical Features, Treatment, and Prognosis
- Fibrous dysplasia (FD) typically presents with slow growth and may stabilize over time, surgical treatment is indicated for functional deficits or significant cosmetic deformity
- Histologically, FD shows irregularly shaped trabeculae of immature woven bone dispersed in cellular fibroblastic stroma, with bone trabeculae evolving directly from the stroma
- Treatment for FD is based on patient's age, presence of cosmetic and functional deficits, and growth rate, options include observation, conservative surgical recontouring, or radical surgical resection and reconstruction
- Sclerosing osteomyelitis is a differential diagnosis for FD, and its pathology report may show varied results under the benign fibro-osseous lesion (BFOL) spectrum
- Inflammatory reactive processes containing focal sclerosing osteomyelitis and proliferative periostitis are categorized under BFOL
- Differential diagnosis of sclerosing osteomyelitis can be challenging, as seen in a case with initial FD diagnosis but pathology report showing sclerosing osteomyelitis
- Trabecular pattern alterations are evident in cases of sclerosing osteomyelitis, and surgical intervention may be necessary for significant involvement
- Chen's treatment scheme offers guidance for the approach to lesions based on their extent and location within different craniofacial zones
- Optic canal decompression is advised for patients with orbital dysplasia and decreasing visual acuity, and early treatment is recommended for progressive sensory disturbances in craniofacial dysplasia
- Non-surgical treatment has a limited role in fibrous dysplasia, with radiotherapy contraindicated and steroids, calcitonin, and pamidronate showing some promise in reducing symptoms
- Fibrous dysplasia needs to be differentiated from other fibro-osseous lesions like cemento-ossifying fibroma (COF) and diffuse-sclerosing osteomyelitis of the mandible
- COF, previously included under BFOLs, is now accepted as odontogenic in origin and has a new classification, with variants including cemento-ossifying fibroma, juvenile trabecular-ossifying fibroma, and juvenile psammomatoid-ossifying fibroma
Test your knowledge of fibro-osseous lesions of the craniofacial complex with this quiz on clinical features, treatment options, and prognosis. Explore differential diagnoses, histological characteristics, and treatment guidelines for conditions such as fibrous dysplasia, sclerosing osteomyelitis, and cemento-ossifying fibroma.
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