Updated: 4/1/2020

Malignant Bone Tumors

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Topic
Overview
Ewing Sarcoma
  • Introduction
    • anaplastic small blue cell tumor
      • neuroectoderm origin
      • involves an 11;22 translocation  
    • boys < 15 has highest incidence
    • most often seen in diaphysis of long bones, pelvis, scapula, and ribs
  • Evaluation
    • layered "onion-skin" deposition of bone 
  • Prognosis, Prevention, and Complications
    • early metastasis
    • responsive to chemotherapy
Chondrosarcoma
  • Introduction
    • malignant cartilaginous tumor
    • seen in men 30-60 years
    • most commonly involves the central skeleton
    • may occur de novo or from progression of osteochondroma
  • Evaluation
    • expansile glistening mass 
    • located in the medullary cavity
Osteosarcoma (osteogenic sarcoma)
  • Introduction
    • tumor of osteoblasts
    • most common primary malignant tumor of bone
    • commonly found in the metaphysis of long bones
      • e.g. distal femur or proximal tibia
    • risk factors
      • Paget disease of bone 
        • results in onset in elderly
      • bone infarcts
      • radiation
      • familial retinoblastoma
        • Rb mutation
        • results in onset before third decade
  • Evaluation
    • histology
      • large pleomorphic cells producing increased osteoid 
    • Radiograph 
      • Codman's triangle or sunburst pattern 
        • from elevation of periosteum from the cortical bone
  • Prognosis, Prevention, and Complications
    • poor prognosis
Bone Metastasis
  • Introduction
    • typically result in osteolytic lesions
    • exception is prostatic carcinoma
      • produces osteoblastic lesion 

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(M1.ON.17.4723) A 12-year-old Caucasian male presents with his mother to the pediatrician’s office complaining of right thigh pain. He reports that he has noticed slowly progressive pain and swelling over the distal aspect of his right thigh over the past two months. He denies any recent trauma to the area and his temperature is 100.9°F (38.3°C). On exam, there is swelling and tenderness overlying the distal right femoral diaphysis. Laboratory evaluation is notable for an elevated white blood cell (WBC) count and elevated erythrocyte sedimentation rate (ESR). A radiograph of the patient’s right leg is shown. Biopsy of the lesion demonstrates sheets of monotonous small round blue cells with minimal cytoplasm. Which of the following genetic mutations is most likely associated with this patient’s condition? Tested Concept

QID: 108547
FIGURES:
1

t(8;14)

14%

(32/232)

2

t(11;22)

49%

(113/232)

3

APC inactivation

8%

(18/232)

4

TP53 inactivation

8%

(19/232)

5

RB1 inactivation

17%

(40/232)

M 1 C

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