Developmental Bone Diseases Quiz
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Questions and Answers

Which of the following is NOT classified as a developmental disease of bone?

  • Fibrous dysplasia (correct)
  • Osteopetrosis
  • Cherubism
  • Cleidocranial dysplasia

What is the genetic inheritance pattern of cherubism?

  • Mitochondrial
  • X-linked dominant
  • Autosomal dominant (correct)
  • Autosomal recessive

The appearance of individuals with cherubism is mainly due to:

  • Excessive bone formation
  • Decreased osteoclast activity
  • Loss of bone replaced by fibrous tissue (correct)
  • Cartilage overgrowth

What gene mutation is primarily responsible for cherubism?

<p>SH3BP2 (A)</p> Signup and view all the answers

Which cellular activity is increased due to the SH3BP2 gene mutation in cherubism?

<p>Both osteoblast and osteoclast activity (A)</p> Signup and view all the answers

Histologically, what is a characteristic feature of cherubism lesions?

<p>Numerous multinucleated giant cells in fibrous stroma (D)</p> Signup and view all the answers

The multinucleated giant cells in cherubism are positive for which enzyme?

<p>Tartrate-resistant acid phosphatase (D)</p> Signup and view all the answers

Which of the following is NOT a neoplastic bone disease?

<p>Osteomyelitis (C)</p> Signup and view all the answers

Which of the following is NOT considered a known etiological factor in bone diseases characterized by uncontrolled alternating resorption and deposition?

<p>Metabolic disturbances (D)</p> Signup and view all the answers

What is a distinct histological characteristic of bone affected by uncontrolled alternating resorption and deposition, often described as a "jigsaw puzzle" or "mosaic" appearance?

<p>Presence of basophilic reversal lines in bone (C)</p> Signup and view all the answers

Which cell type is characteristically proliferated in Langerhans cell disease?

<p>Langerhans's cells (D)</p> Signup and view all the answers

What is the name of the unique rod/racquet shaped granules found within the cytoplasm of Langerhans's cells?

<p>Birbeck granules (B)</p> Signup and view all the answers

Which protein do Langerhans's cells stain positively for, aiding in their diagnostic distinction from normal histiocytes?

<p>S-100 protein (B)</p> Signup and view all the answers

What key feature is used to classify central giant cell granulomas as a non-neoplastic lesion?

<p>The reparative nature of the cells within the lesion (B)</p> Signup and view all the answers

Which cell type primarily forms the basis of giant cells found in central giant cell granulomas?

<p>Osteoclasts (A)</p> Signup and view all the answers

Aneurysmal bone cysts are most often observed in which of the following locations?

<p>Long bones and vertebrae (C)</p> Signup and view all the answers

Which of the following best describes the primary cause of osteopetrosis?

<p>Defective bone remodeling due to impaired osteoclast function. (B)</p> Signup and view all the answers

Which of these is a synonym of Osteogenesis Imperfecta?

<p>Lobstein's Disease (A)</p> Signup and view all the answers

What is the main underlying issue in Osteogenesis Imperfecta?

<p>Impaired collagen maturation and synthesis, specifically of type I collagen. (A)</p> Signup and view all the answers

What is the key feature observed in the bone histology of osteopetrosis?

<p>Tortuous lamellar trabeculae replacing cancellous bone and globular amorphous bone deposition in marrow spaces. (B)</p> Signup and view all the answers

Which of the following best describes the osteoclasts in Osteopetrosis?

<p>Increased, decreased or normal in number, but nonfunctional. (A)</p> Signup and view all the answers

Which of the following is a characteristic finding in the bone of a patient with Osteogenesis Imperfecta?

<p>Abnormal collagen synthesis by abnormal osteoblasts and reduced bone mass. (A)</p> Signup and view all the answers

Which of these is the primary site affected by Paget's disease?

<p>Areas of both resorption and deposition. (B)</p> Signup and view all the answers

A patient's bone biopsy shows thin cortical bone and delicate cancellous bone with microfractures. Which disorder best fits this description?

<p>Osteogenesis Imperfecta (B)</p> Signup and view all the answers

What is a key characteristic of the trabeculae found in the lesions described in the provided content?

<p>They possess a unique curvilinear shape, resembling Chinese script, and are not interconnected. (C)</p> Signup and view all the answers

What is the primary tissue type characteristically seen in the early stages of cemento-osseous dysplasia?

<p>Fibroblastic proliferation, possibly with initial osteoid formation. (D)</p> Signup and view all the answers

Which of the following best describes the relationship between the lesion and the surrounding normal bone when the lesion is present?

<p>The lesion and normal bone fuse directly with no capsule or line of separation. (A)</p> Signup and view all the answers

What is the typical composition of the calcified material found in ossifying fibromas?

<p>A mix of irregular osteoid trabeculae, and basophilic, globular calcifications resembling cementum. (B)</p> Signup and view all the answers

What is the suggested origin of cemento-osseous dysplasias based on their proximity to a specific dental structure?

<p>Developing from the periodontal ligament due to their close proximity and similarity. (D)</p> Signup and view all the answers

What is characteristic about the jaw and skull lesions when compared with similar skeletal lesions in the context mentioned?

<p>They are more ossified, compared to lesions in the rest of the skeleton. (D)</p> Signup and view all the answers

In fibro-osseous lesions, which of the following features will most likely NOT be seen?

<p>A capsule or demarcation from surrounding tissues. (A)</p> Signup and view all the answers

Which of the following best describes the final stage of cemento-osseous dysplasia?

<p>A lesion composed of dense, mineralized tissue. (B)</p> Signup and view all the answers

What is the primary distinction between juvenile ossifying fibroma (JOF) and ossifying fibroma (OF) based on?

<p>Age incidence, site predilection, and clinical behavior (C)</p> Signup and view all the answers

Which of the following best describes the mineralized component of trabecular Juvenile Ossifying Fibroma (JOF)?

<p>Irregular strands of osteoid lined by plump osteoblasts (A)</p> Signup and view all the answers

What distinguishes the mineralized material in psammomatoid Juvenile Ossifying Fibroma (JOF)?

<p>Concentric, lamellated, and spherical ossicles with basophilic centers and eosinophilic osteoid rims (B)</p> Signup and view all the answers

Which of the following is NOT typically considered a neoplastic disease of bone?

<p>Tori (D)</p> Signup and view all the answers

What distinguishes compact osteoma from cancellous osteoma?

<p>Amount of marrow tissue and bone structure (C)</p> Signup and view all the answers

Where are osteomas commonly located?

<p>Craniofacial skeleton (D)</p> Signup and view all the answers

Which type of cell is primarily responsible for the development of osteosarcoma?

<p>Mesenchymal cells capable of producing osteoid (C)</p> Signup and view all the answers

Besides osteoid, what other materials might be seen in osteosarcoma?

<p>Chondroid and fibrous material (D)</p> Signup and view all the answers

Osteosarcomas are classified based upon the relative amounts of which three components produced by the tumor?

<p>Chondroid, osteoid, and fibers (B)</p> Signup and view all the answers

Which type of osteosarcoma is characterized by the presence of neoplastic cartilage merging with tumor bone?

<p>Chondroblastic osteosarcoma (A)</p> Signup and view all the answers

A benign tumor composed of mature hyaline cartilage, commonly found in short bones of hands and feet, is known as:

<p>Chondroma (B)</p> Signup and view all the answers

Which feature is NOT typically associated with the histopathology of a chondroma?

<p>Abundant eosinophilic matrix (D)</p> Signup and view all the answers

Which of the following best describes a chondrosarcoma?

<p>A malignant tumor characterized by cartilage formation, but not bone, by the tumor cells (D)</p> Signup and view all the answers

Which of the following is a histologic feature of chondrosarcoma?

<p>Proliferation of atypical chondrocytes that permeate medullary spaces (B)</p> Signup and view all the answers

The histogenesis of Ewing's sarcoma is:

<p>Of neuroectodermal origin (C)</p> Signup and view all the answers

Which of the following best describes the cellular arrangement in Ewing's sarcoma?

<p>Composed of small round cells proliferating in sheets without pattern (C)</p> Signup and view all the answers

Flashcards

Cherubism

A rare developmental jaw condition characterized by bilateral swelling of the jaws, giving the affected person a 'cherubic' appearance.

Osteopetrosis

A genetic disorder where bones become abnormally dense due to impaired bone resorption. This can lead to fractures, joint stiffness, and neurological complications.

Osteogenesis Imperfecta

A group of genetic disorders characterized by brittle bones that fracture easily. This can cause skeletal deformities and complications.

Cleidocranial Dysplasia

A genetic disorder affecting the development of the collarbones (clavicles) and skull, leading to abnormalities in these bones.

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Ossifying Fibroma

A benign bone tumor characterized by excessive bone formation, commonly found in the jaw. It typically remits spontaneously as the child reaches puberty.

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Fibrous Dysplasia

A developmental disorder affecting bone development. It involves excessive production of fibrous tissue, replacing normal bone, leading to enlargement of the jaws. It's often associated with facial asymmetry and dental issues.

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Cemento-osseous Dysplasia

A condition affecting the jawbone, involving abnormal bone formation due to a change in bone structure.

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Paget's Disease

A rare bone disorder that leads to excessive bone turnover, potentially causing bone deformities, pain, and fractures.

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Failure of osteoclast resorption

A histological finding seen in Osteopetrosis where osteoclasts fail to effectively resorb skeletal tissue leading to increased bone density.

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Tortuous lamellar trabeculae

A histological finding in Osteopetrosis where the normal cancellous bone is replaced by abnormally shaped and dense bone trabeculae (bone beams).

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Globular amorphous bone deposition

A histological finding in Osteopetrosis where abnormal bone material fills up the marrow cavities, hindering blood cell production.

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Abnormal collagen synthesis

A histological finding in Osteogenesis Imperfecta where osteoblasts produce abnormal collagen, resulting in weakened and fragile bone matrix.

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Reduced bone mass with thin cortical bone

A histological finding in Osteogenesis Imperfecta where bone mass is reduced, and the cortical and cancellous bone are thin and fragile.

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Langerhans cells

Specialized cells normally found in the epidermis, mucosa, lymph nodes, and bone marrow. They present antigens to immune cells.

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Central giant cell granuloma

A benign, proliferative lesion characterized by the presence of multinucleated giant cells in a background of mesenchymal cells.

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Langerhans cell disease

A non-neoplastic lesion characterized by the proliferation of histiocyte-like cells (Langerhans cells) with varying numbers of eosinophils, lymphocytes, and multinucleated giant cells.

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Aneurysmal bone cyst

A bone cyst that is characterized by blood-filled spaces and a lining of fibrous tissue.

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Osteoclasts

Specialized cells responsible for breaking down bone tissue during bone remodeling.

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Osteogenesis

The process of bone formation, where new bone tissue is created by specialized cells called osteoblasts.

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Birbeck granules

Characteristic rod- or racquet-shaped intracellular granules found in Langerhans cells, which help identify the cell.

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Histological Features of Ossifying Fibroma

Irregular, woven bone trabeculae in a cellular, vascular stroma, often resembling Chinese characters.

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Periapical Cemento-Osseous Dysplasia

A type of Cemento-Osseous Dysplasia affecting the tooth-bearing region of the jaw, typically near the periodontal ligament.

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Florid Cemento-Osseous Dysplasia

A type of Cemento-Osseous Dysplasia occurring in multiple areas of the jaw, usually affecting both sides.

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Focal Cemento-Osseous Dysplasia

A type of Cemento-Osseous Dysplasia affecting a specific, localized area of the jaw.

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Histopathology of Cemento-Osseous Dysplasia

In early stages: Fibroblastic proliferation with minimal osteoid formation. Later stages: Increased deposition of bone or cementum-like material. Final stages: Dense mineralized tissue.

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Calcified Material in Ossifying Fibroma

The fibrous tissue in an Ossifying Fibroma can contain bone, cementum-like spherules or a mixture of both.

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Juvenile ossifying fibroma

A more aggressive form of ossifying fibroma, often found in younger individuals and with a greater tendency to grow.

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Trabecular juvenile ossifying fibroma

A pattern seen in juvenile ossifying fibromas where the bone formation appears as irregular strands resembling woven threads.

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Psammomatoid juvenile ossifying fibroma

A pattern observed in juvenile ossifying fibromas, characterized by spherical bone formations with a layered structure.

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Osteoma

A benign bone tumor that is composed entirely of mature bone, typically found in the skull.

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Compact osteoma

A type of osteoma that is characterized by compact, dense bone.

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Cancellous osteoma

A type of osteoma that contains trabeculae (tiny beams or struts) of bone, along with fibrous tissue and fatty marrow.

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Osteosarcoma

A malignant bone tumor characterized by the production of immature bone by cancerous cells.

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Osteoblastic osteosarcoma

A type of osteosarcoma characterized by the production of a large amount of osteoid by tumor cells.

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Chondroblastic osteosarcoma

A type of osteosarcoma characterized by the production of a large amount of chondroid by tumor cells.

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Fibroblastic osteosarcoma

A type of osteosarcoma characterized by the production of a large amount of fibrous tissue by tumor cells.

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Chondroma

A benign tumor of cartilage, commonly found in the hands and feet.

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Chondrosarcoma

A malignant tumor of cartilage. It is characterized by the formation of cartilage, but not bone, by the tumor cells.

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Ewing's Sarcoma

A primary malignant tumor of bone. Its origin is thought to be neuroectodermal.

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Conventional Chondrosarcoma

A type of chondrosarcoma characterized by well-differentiated hyaline cartilage.

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Dedifferentiated Chondrosarcoma

A type of chondrosarcoma characterized by a rapid growth rate and poorly differentiated cells.

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Study Notes

Diseases of Bones & Joints

  • Diseases of bones and joints are categorized into developmental, endocrine, idiopathic, reactive, fibro-osseous, inflammatory, and neoplastic diseases.

Classification of Diseases of Bone

  • Developmental Diseases:

    • Cherubism
    • Osteopetrosis
    • Osteogenesis imperfecta
    • Cleidocranial dysplasia

  • Endocrine Diseases:

    • Hyperparathyroidism

  • Idiopathic Diseases:

    • Idiopathic osteosclerosis
    • Massive osteolysis
    • Langerhan's cell disease (Histiocytosis-X)
    • Paget's disease

  • Reactive Diseases:

    • Giant cell lesion of bone
    • Aneurysmal bone cyst
    • Simple/Traumatic bone cyst

  • Fibro-osseous Lesions:

    • (i) Non-neoplastic lesions:
      • Fibrous dysplasia
      • Cemento-osseous dysplasia
    • (ii) Neoplasms:
      • Ossifying fibroma

  • Inflammatory Diseases:

    • (i) Specific:
      • Tuberculosis
      • Actinomycosis
    • (ii) Non-specific:
      • Osteomyelitis
      • Dry socket
      • Periapical cyst/abscess/granuloma
      • Osteoradionecrosis

  • Neoplastic Diseases:

    • (i) Benign:
      • Osteoma
      • Osteoid osteoma & osteoblastoma
      • Chondroma
      • Chondromyxoid fibroma
    • (ii) Malignant:
      • Osteosarcoma
      • Ewing's sarcoma
      • Chondrosarcoma

Developmental Disorders

  • Cherubism:
    • Rare developmental jaw condition
    • Autosomal dominant trait
    • Bilateral occurrence
    • Child appears plump, cheeked, "cherubic"
    • Jaw lesions remit spontaneously during puberty
    • Associated with facial deformity and malocclusion
  • Osteopetrosis:
    • Rare disease
    • Excessive density of all bones
    • Obliteration of marrow cavities
    • Defective bone remodeling due to failure of osteoclasts
    • Poor mechanical properties of bone.
  • Osteogenesis Imperfecta:
    • Developmental, inherited bone disorder
    • Autosomal dominant or recessive pattern
    • Connective tissue disorder with bone fragility
    • Genetic defect in collagen maturation/synthesis of type I collagen
  • Cleidocranial Dysplasia:
    • Developmental bone disorder

Pathogenesis of Cherubism

  • Due to mutation in SH3BP2 gene mapped to chromosome 4p16.
  • This mutation increases osteoclast and osteoblast activity during tooth eruption.

Histological Features of Cherubism

  • Normal bone is partially replaced by pathological tissue.
  • Numerous multinucleated giant cells and vacuoles are observed within fibrous connective tissue stroma.
  • Freshly harvested osteoclasts are also present.

Pathogenesis of Osteopetrosis

  • Mutation in SH3BP2 gene on chromosome 4p16.

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OAOL Diseases of Bones and Joints PDF

Description

Test your knowledge on developmental diseases of bone, focusing on conditions like cherubism and their genetic underpinnings. This quiz covers key characteristics, histology, and differentiating factors between various bone diseases. Dive into the complexities of bone pathology and expand your understanding.

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