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Bone Pathology II Neoplastic Bone Lesions. Richard Anderson, MD President, Associated Pathology Consultants, S.C. Edward Hospital & Elmhurst Memorial Hospital Clinical Instructor, Department of Pathology, UIC College of Medicine Chairman of the Board, Heartland Blood Centers.

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bone pathology ii neoplastic bone lesions

Bone Pathology IINeoplastic Bone Lesions

Richard Anderson, MD

President, Associated Pathology Consultants, S.C.

Edward Hospital & Elmhurst Memorial Hospital

Clinical Instructor, Department of Pathology, UIC College of Medicine

Chairman of the Board, Heartland Blood Centers

UIC M2 Pathology - 2004

bone forming tumors
Bone-Forming Tumors
  • Benign
      • Osteoid Osteoma
      • Osteoblastoma
  • Malignant
      • Osteogenic sarcoma
osteoid osteoma
Osteoid Osteoma
  • Signs/Symptoms:
      • Pain, characteristically more intense at night, relieved by NSAIA and eliminated by excision
      • Vertebral lesions may cause scoliosis
  • Age:
      • 10-30 years
  • Sex:
      • M > F (2:1)
  • Anatomic Distribution:
      • Nearly every location, most frequent in femur, tibia, humerus, bones of hands and feet, vertebrae and fibula
      • Over 50% of cases in femur or tibia
      • Metaphysis of long bones
slide4

Central radiolucent nidus with or without a radiodense center; surrounded by thickened sclerotic bone

slide7

Nidus contains interlacing network of osteoid and bony trabeculae with variable amount of mineralization, lying in vascular fibrous tissue

osteoid osteoma1
Osteoid Osteoma
  • Ancillary Testing:
      • N/A
  • Prognosis/Treatment:
      • Surgical excision is treatment of choice
      • Recurrence unlikely with complete excision
osteoblastoma giant osteoid osteoma
Osteoblastoma (Giant Osteoid Osteoma)
  • Signs/Symptoms:
      • Pain
      • Gait disturbances
  • Age:
      • 80% of patients < 30 years
  • Sex:
      • M >> F (3:1)
  • Anatomic Distribution:
      • Predilection for vertebral column
      • Metaphysis of long bones
osteoblastoma giant osteoid osteoma1
Osteoblastoma (Giant Osteoid Osteoma)
  • Radiographic Findings:
      • Similar to osteoid osteoma, though much larger (up to 11.0 cm)
  • Gross and Microscopic Findings:
      • Similar to osteoid osteoma, though much larger nidus
  • Ancillary Testing:
      • N/A
  • Prognosis/Treatment:
      • Curettage followed by bone grafting
      • If incompletely removed, tumor may recur
      • Malignant change to osteosarcoma has been rarely reported
osteogenic sarcoma osteosarcoma
Osteogenic Sarcoma (Osteosarcoma)
  • Most frequent primary malignant bone tumor
  • Malignant cells must produce osteoid
  • Most tumors arise de novo, though others arise in the setting of:
          • Paget’s disease
          • Previous RT
          • Previous chemo (especially alkylating agents)
          • Fibrous dysplasia
          • Osteochondromatosis
          • Chondromatosis
          • Chronic osteomyelitis
osteogenic sarcoma osteosarcoma1
Osteogenic Sarcoma (Osteosarcoma)
  • Signs/Symptoms:
      • Pain and swelling
      • Pathologic fracture is uncommon
  • Age:
      • Peak in 2nd decade with gradual decrease thereafter
  • Sex:
      • M > F
  • Anatomic Distribution:
      • 50% arise around the knee
      • Metaphysis of long bones
osteogenic sarcoma osteosarcoma2
Osteogenic Sarcoma (Osteosarcoma)
  • Microscopic Findings:
      • Tumor cells produce osteoid or calcified osteoid
      • Osteoblastic, chondroblastic or fibroblastic types
      • Osteoclast-like giant cells may be present
cartilage forming tumors
Cartilage-Forming Tumors
  • Benign:
      • Chondroma
      • Osteochondroma
      • Chondroblastoma
      • Chondromyxoid Fibroma
  • Malignant:
      • Chondrosarcoma
chondroma
Chondroma
  • Benign tumor of mature hyaline cartilage
  • Most within bone (enchondroma)
  • 2 syndromes characterized by multiple chondromas:
      • Ollier’s disease
          • Multiple enchondromas, usually unilateral
      • Maffucci’s syndrome
          • Multiple enchondromas associated with soft tissue hemangiomas
      • Both disorders have 25% risk of malignant transformation to chondrosarcoma
  • Enchondroma is the most common tumor of the bones of the hand
chondroma1
Chondroma
  • Signs/Symptoms:
      • Usually asymptomatic lesions; pain with pathologic fracture
  • Age:
      • Evenly distributed
  • Sex:
      • F > M
  • Anatomic Distribution:
      • 50% of lesions within small bones of hands and feet (mostly the phalanges)
slide29

Localized central lytic lesion surrounded by sharp rim of sclerosis; cortex usually not involved, though may be thin

slide33

Composed of mature lobules of hyaline cartilage with foci of myxoid degeneration, calcification and endochondral ossification; may be quite cellular

chondroma2
Chondroma
  • Ancillary Testing:
      • N/A
  • Prognosis/Treatment:
      • Solitary chondromas of long or flat bones need no treatment
      • If fracture occurs, treat with curettage and bone grafting
      • Recurrence unusual
osteochondroma
Osteochondroma
  • Most frequent benign bone tumor
  • Probably not a true neoplasm, but rather a tumor produced by growth of aberrant foci of cartilage on the surface of bone
  • Autosomal dominant disorder of osteochrondromatosis with risk of malignant transformation to chondrosarcoma
osteochondroma1
Osteochondroma
  • Signs/Symptoms:
      • Palpable mass of long duration
      • Pain from compression of regional structures
  • Age:
      • 60% of patients < 20 years
      • Average age 10 years
  • Sex:
      • M > F
  • Anatomic Predilection:
      • May occur in any bone; usually metaphysis of long bones (lower end of femur, upper end of humerus and upper end of tibia are most frequent)
slide37

Projection with cortex continuous with underlying bone; may be pedunculated; cartilaginous cap with frequent calcification

osteochondroma2
Osteochondroma
  • Ancillary Testing:
      • N/A
  • Prognosis/Treatment:
      • Surgical removal only for pain or cosmetic reasons
      • Lesion cured by complete removal; if recurs, suggests the lesion was probably a low grade chondrosarcoma
chondroblastoma
Chondroblastoma
  • Rare benign tumor
  • Most common primary epiphyseal tumor in children
  • Signs/Symptoms:
      • Local pain and swelling; tumors 1.0 to 7.0 cm
  • Age:
      • 2nd decade of life
  • Sex:
      • M > F
  • Anatomic Distribution:
      • Epiphysis of long bones
      • 40% in distal femur or proximal tibia
chondroblastoma1
Chondroblastoma

Lytic lesion of epiphysis with thin sclerotic rim; thinning without destruction of cortex

slide44

Cellular lesion with polyhedral chondroblasts; giant cells and areas of calcification may be seen; mitoses common

chondroblastoma2
Chondroblastoma
  • Ancillary Testing:
      • N/A
  • Prognosis/Treatment:
      • Curettage with bone grafting
      • May recur locally if not completely removed
chondromyxoid fibroma
Chondromyxoid Fibroma
  • Rare benign tumor
  • Signs/Symptoms:
      • Pain and swelling
  • Age:
      • 2nd and 3rd decades
  • Sex:
      • M > F
  • Anatomic Distribution:
      • Metaphysis of long bones, though may abut the epiphysis
      • 30% of tumors in tibia
chondromyxoid fibroma1
Chondromyxoid Fibroma
  • Ancillary Testing:
      • N/A
  • Prognosis/Treatment:
      • En bloc resection
      • Curettage is associated with 25% rate of recurrence
chondrosarcoma
Chondrosarcoma
  • Signs/Symptoms:
      • Local swelling and pain
  • Age:
      • Adulthood (60% between 30-60 years)
      • Rare in childhood
  • Sex:
      • M > F
  • Anatomic Distribution:
      • Trunk, shoulder girdle, upper ends of femur and humerus
slide57

Mesenchymal Chondrosarcoma

Myxoid Chondrosarcoma

chondrosarcoma1
Chondrosarcoma
  • Ancillary Testing:
      • IHC
          • S100 – positive
  • Prognosis/Treatment:
      • Must completely excise; biopsy leads to soft tissue implantation
      • RADIORESISTANT; surgery is Tx of choice
      • Recurrence may occur 5-10 years after primary
      • 5-year survival 80%
      • Hematogenous metastasis to lung in high grade lesions
miscellaneous tumors of bone
Miscellaneous Tumors of Bone
  • Giant Cell Tumor
  • Ewing’s Sarcoma / Primitive Neuroectodermal Tumor (PNET)
  • Chordoma
giant cell tumor
Giant Cell Tumor
  • Signs/Symptoms:
      • Pain, loss of mobility, fracture
  • Age:
      • 80% of patients > 20 years
  • Sex:
      • F > M
  • Anatomic Distribution:
      • Epiphysis of long bones
      • 50% around knee with most in distal femur
      • Most common primary epiphyseal tumor of adults
giant cell tumor1
Giant Cell Tumor
  • Ancillary Testing:
      • N/A
  • Prognosis/Treatment:
      • Curettage is TX of choice
      • Chemical or thermal cautery of walls advocated; defect filled with bone chips
      • Recurrence rate 20-30%
      • Malignant transformation to fibrosarcoma or osteosarcoma may rarely occur
ewing s sarcoma pnet
Ewing’s Sarcoma / PNET
  • Signs/Symptoms:
      • May simulate osteomyelitis as patients often present with pain, fever and leukocytosis
  • Age:
      • 5-20 years
  • Sex:
      • M > F
  • Anatomic Distribution:
      • Long bones of extremities
  • Gross Findings:
      • Solid masses of degenerating gray-white tumor
ewing s sarcoma pnet1
Ewing’s Sarcoma / PNET
  • Ancillary Testing:
      • Cytogenetics
          • t(11;22)(q24;q12)
  • Prognosis/Treatment:
      • Widely metastatic tumors with spread to lung, pleura and bones
      • Previous 5-year survival dismal, now with surgery, chemo and RT, 5-year survival up to 75%
chordoma
Chordoma
  • Signs/Symptoms:
      • Pain
      • Spheno-occipital lesions may lead to cranial nerve signs
  • Age:
      • 5th and 6th decades
  • Sex:
      • M > F
  • Anatomic Distribution:
      • Sacrococcygeal or spheno-occipital region
chordoma1
Chordoma
  • Ancillary Testing:
      • IHC
          • S100 and cytokeratin – positive
  • Prognosis/Treatment:
      • Classic history is incomplete resection followed by local recurrence
      • Some tumors radiosensitive
      • Distant metastasis to skin may occur
tumor like lesions of bone
Tumor-Like Lesions of Bone
  • Metaphyseal Fibrous Defect (Nonossifying Fibroma)
nonossifying fibroma
Nonossifying Fibroma
  • Signs/Symptoms:
      • Usually found incidentally; may cause pain
  • Age:
      • Children and adolescents
  • Sex:
      • M > F
  • Anatomic Distribution:
      • Metaphysis of long bones, usually distal femur and tibia
nonossifying fibroma1
Nonossifying Fibroma
  • Ancillary Testing:
      • N/A
  • Prognosis/Treatment:
      • Most lesions undergo spontaneous regression