Fibrous Dysplasia

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Questions and Answers

What is fibrous dysplasia?

A benign developmental bone disorder (correct)

A malignant bone tumor

A type of bone infection

A genetic disorder affecting bone growth

Which form of fibrous dysplasia is more common?

Both forms are equally common

Polyostotic form

Monostotic form (correct)

Fibrous dysplasia is a rare condition

What is the main method of diagnosing fibrous dysplasia?

Genetic testing

Biopsy

Blood tests

Clinical and radiographic features (correct)

What is the most common symptom of fibrous dysplasia?

Bony expansion and remodeling Signup and view all the answers

What is the management goal for fibrous dysplasia?

Establishing the extent of the disease and maintenance of bone quality Signup and view all the answers

What is a potential complication of monostotic fibrous dysplasia?

Bony expansion and remodeling Signup and view all the answers

What is sarcomatous dedifferentiation?

A malignant transformation of fibrous dysplasia Signup and view all the answers

Who first described fibrous dysplasia?

Louis Lichtenstein Signup and view all the answers

What is fibrous dysplasia?

A developmental benign medullary fibro-osseous process characterized by the failure to form mature lamellar bone and arrest as woven bone that can be multifocal. Signup and view all the answers

What is the most common form of fibrous dysplasia?

Monostotic form Signup and view all the answers

At what age does the monostotic form of fibrous dysplasia usually become symptomatic?

2nd to 3rd decade Signup and view all the answers

What is the main method for diagnosing fibrous dysplasia?

Clinical and typical radiographic features Signup and view all the answers

When is histological confirmation necessary for the diagnosis of fibrous dysplasia?

In cases with atypical imaging appearance or in isolated monostotic lesions with clinical symptoms or other concerning features. Signup and view all the answers

What is the usual outcome for patients with fibrous dysplasia?

Excellent prognosis with no other treatment required Signup and view all the answers

What is the most common sarcomatous dedifferentiation seen in fibrous dysplasia?

Osteosarcoma Signup and view all the answers

Who first described fibrous dysplasia?

Louis Lichtenstein Signup and view all the answers

Study Notes

Fibrous Dysplasia: Characteristics, Diagnosis, and Management

Fibrous dysplasia is a developmental benign medullary fibro-osseous process characterized by the failure to form mature lamellar bone and arrest as woven bone that can be multifocal.
It can affect any bone and occur in a monostotic form involving only one bone or a polyostotic form involving multiple bones.
Fibrous dysplasia accounts for one of the 'F's in the popular mnemonic for lucent bone lesions FEGNOMASHIC.
The monostotic form is far more common accounting for 70-80% of cases and is usually asymptomatic until the 2nd to 3rd decade but can be seen throughout adulthood, while the polyostotic form accounts for 20-30% and presents earlier, typically in childhood with about 60% showing symptoms before the age of 10 years.
The diagnosis of fibrous dysplasia is mainly based on clinical and typical radiographic features, and if the imaging features are characteristic the lesion does not require histology.
Histological confirmation is indicated in cases with atypical imaging appearance or in isolated monostotic lesions with clinical symptoms or other concerning features.
The condition is often an incidental finding and is usually painless. Alternatively, it may present with bony expansion and remodeling or with pain.
Management aims to establish the extent of the disease and the maintenance of bone quality via dietary measures and exercise. The prognosis is excellent and usually, no other treatment is required.
However, monostotic fibrous dysplasia can lead to deformities leg-length differences and impingement or nerve compression syndromes. If a mass effect is severe, then surgery excision may be considered.
Bone affected by fibrous dysplasia is weaker than normal and thus susceptible to pathological fractures.
Sarcomatous dedifferentiation (most commonly osteosarcoma, fibrosarcoma, malignant fibrous histiocytoma, or rarely chondrosarcoma) is occasionally seen (< 1%) and is more common in the polyostotic form.
Fibrous dysplasia was first described by the American bone pathologist Louis Lichtenstein in 1938 and the clinical, radiological and histological spectrum of findings has been later characterized by him and his colleague Henry Louis Jaffe in 1942.

Fibrous Dysplasia: Characteristics, Diagnosis, and Management

Fibrous dysplasia is a developmental benign medullary fibro-osseous process characterized by the failure to form mature lamellar bone and arrest as woven bone that can be multifocal.
It can affect any bone and occur in a monostotic form involving only one bone or a polyostotic form involving multiple bones.
Fibrous dysplasia accounts for one of the 'F's in the popular mnemonic for lucent bone lesions FEGNOMASHIC.
The monostotic form is far more common accounting for 70-80% of cases and is usually asymptomatic until the 2nd to 3rd decade but can be seen throughout adulthood, while the polyostotic form accounts for 20-30% and presents earlier, typically in childhood with about 60% showing symptoms before the age of 10 years.
The diagnosis of fibrous dysplasia is mainly based on clinical and typical radiographic features, and if the imaging features are characteristic the lesion does not require histology.
Histological confirmation is indicated in cases with atypical imaging appearance or in isolated monostotic lesions with clinical symptoms or other concerning features.
The condition is often an incidental finding and is usually painless. Alternatively, it may present with bony expansion and remodeling or with pain.
Management aims to establish the extent of the disease and the maintenance of bone quality via dietary measures and exercise. The prognosis is excellent and usually, no other treatment is required.
However, monostotic fibrous dysplasia can lead to deformities leg-length differences and impingement or nerve compression syndromes. If a mass effect is severe, then surgery excision may be considered.
Bone affected by fibrous dysplasia is weaker than normal and thus susceptible to pathological fractures.
Sarcomatous dedifferentiation (most commonly osteosarcoma, fibrosarcoma, malignant fibrous histiocytoma, or rarely chondrosarcoma) is occasionally seen (< 1%) and is more common in the polyostotic form.
Fibrous dysplasia was first described by the American bone pathologist Louis Lichtenstein in 1938 and the clinical, radiological and histological spectrum of findings has been later characterized by him and his colleague Henry Louis Jaffe in 1942.

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Description

Test your knowledge on Fibrous Dysplasia with this informative quiz! Learn about the characteristics, diagnosis, and management of this benign medullary fibro-osseous process that can affect any bone in the body. Discover the differences between monostotic and polyostotic forms, the typical imaging features, and when histological confirmation is necessary. Explore the various management options, potential complications, and prognosis of this condition. Challenge yourself and enhance your understanding of Fibrous Dysplasia with this engaging