Primary Bone Tumors: Overview

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Questions and Answers

What genetic mutation is most closely associated with hereditary multiple exostoses, often linked to osteochondroma formation?

  • TP53
  • RB1
  • APC
  • EXT1 or EXT2 (correct)

A patient presents with bone pain that is worse at night and is relieved by NSAIDs. Imaging reveals a small bony mass with a radiolucent osteoid core. These findings are most consistent with which diagnosis?

  • Osteoid osteoma (correct)
  • Osteoblastoma
  • Chondrosarcoma
  • Osteosarcoma

A radiograph reveals a bone tumor in the epiphysis of the distal femur of a 16-year-old male that has not yet reached skeletal maturity. Which of the following is the most likely diagnosis?

  • Chondroblastoma (correct)
  • Osteosarcoma
  • Giant cell tumor
  • Osteochondroma

Which bone tumor is characterized by neoplastic mononuclear cells expressing RANKL and reactive multinucleated giant cells, often described as osteoclastoma?

<p>Giant cell tumor (D)</p> Signup and view all the answers

A patient is diagnosed with Gardner syndrome. What primary bone tumor are they most predisposed to developing?

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

Which of the following bone tumors is most likely to be found in the metaphysis of long bones, particularly around the knee, and appear as a lateral bony projection of the growth plate?

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

Compared to osteoid osteomas, osteoblastomas exhibit which distinct characteristic?

<p>Larger size and pain unresponsive to NSAIDs (D)</p> Signup and view all the answers

Which of the following radiographic findings is most suggestive of a giant cell tumor?

<p>&quot;Soap bubble&quot; appearance (D)</p> Signup and view all the answers

A 30-year-old female presents with a bone tumor in the epiphysis of her tibia. Radiographic imaging reveals a "soap bubble" appearance. Which of the following is the most likely diagnosis?

<p>Giant cell tumor (B)</p> Signup and view all the answers

What is a key histological characteristic that differentiates a giant cell tumor from other bone tumors?

<p>Numerous multinucleated giant cells (B)</p> Signup and view all the answers

Flashcards

Osteochondroma (exostosis)

Most common benign bone tumor, males < 25 years old. Lateral bony projection of growth plate, points away from joint. EXT1 or EXT2 gene mutation. Rarely transforms to chondrosarcoma

Osteoma

Middle age. Surface of facial bones. Associated with Gardner syndrome.

Osteoid osteoma

Adults < 25 years old, males > females. Cortex of long bones. Classically presents as bone pain (worse at night) caused by prostaglandins; thus relieved by NSAIDs. Bony mass (< 1.5 cm) with radiolucent osteoid core.

Osteoblastoma

Males > females. Vertebrae. Similar histology to osteoid osteoma. Larger size (>2 cm); pain unresponsive to NSAIDs. X-ray similar to aneurysmal bone cyst.

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Giant cell tumor

20-40 years old, females > males. Epiphysis of long bones after skeletal maturation (often in knee region), radiographic epicenter is metaphysis. Locally aggressive benign tumor, neoplastic mononuclear cells that express RANKL. "Soap bubble" appearance on x-ray.

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Chondroblastoma

Adolescents, males > females. Epiphysis of long bones before skeletal maturation (often in knee region). May complain of joint pain. Cross physis on x-ray.

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Study Notes

Primary Bone Tumors Overview

  • Metastatic disease is more common than primary bone tumors
  • Benign bone tumors end with "-oma."
  • Tumors starting with "c" and "o" are more common in boys.
  • Malignant tumors usually have the ending "-sarcoma."

Osteochondroma (Exostosis)

  • Most common benign bone tumor
  • Typically occurs in males under 25 years old.
  • Located in the metaphysis of long bones, often around the knee-distal femur
  • Characterized by a lateral bony projection of the growth plate that is continuous with the marrow space
  • Covered by a cartilaginous cap
  • Points away from the joint
  • May involve EXT1 or EXT2 gene mutation, leading to hereditary multiple exostoses
  • Rarely transforms to chondrosarcoma

Osteoma

  • Occurs in middle-aged individuals
  • Found on the surface of facial bones
  • Associated with Gardner syndrome

Osteoid Osteoma

  • Typically affects adults under 25 years old, with males more commonly affected than females.
  • Located in the cortex of long bones
  • Classically presents as bone pain, which is worse at night, is caused by prostaglandins, and relieved by NSAIDs
  • Appears as a bony mass smaller than 1.5 cm with a radiolucent osteoid core

Osteoblastoma

  • Males are more frequently affected than females
  • Occurs in the vertebrae
  • Similar histology to osteoid osteoma
  • Larger in size (greater than 2 cm)
  • Pain is unresponsive to NSAIDs
  • X-ray appearance similar to aneurysmal bone cyst

Giant Cell Tumor

  • Typically affects individuals in their 20s to 40s, with females more commonly affected than males.
  • Located in the epiphysis of long bones after skeletal maturation, often in the knee region; radiographic epicenter is the metaphysis
  • Locally aggressive benign tumor composed of neoplastic mononuclear cells that express RANKL and reactive multinucleated giant (osteoclast-like) cells, also known as "osteoclastoma"
  • Exhibits a "soap bubble" appearance on X-ray

Chondroblastoma

  • Occurs in adolescents, with males more commonly affected than females
  • Located in the epiphysis of long bones before skeletal maturation, often in the knee region
  • Patients may complain of joint pain
  • Crosses the physis on X-ray

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