Bone Pathology II Neoplastic Bone Lesions


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1 : 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
2 : Bone-Forming Tumors Benign Osteoid Osteoma Osteoblastoma Malignant Osteogenic sarcoma
3 : 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
4 : Central radiolucent nidus with or without a radiodense center; surrounded by thickened sclerotic bone
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6 : Central hemorrhagic nidus surrounded by dense rim of sclerotic bone
7 : Nidus contains interlacing network of osteoid and bony trabeculae with variable amount of mineralization, lying in vascular fibrous tissue
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9 : Osteoid Osteoma Ancillary Testing: N/A Prognosis/Treatment: Surgical excision is treatment of choice Recurrence unlikely with complete excision
10 : 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
11 : 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
12 : 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
13 : 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
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21 : Osteogenic Sarcoma (Osteosarcoma) Microscopic Findings: Tumor cells produce osteoid or calcified osteoid Osteoblastic, chondroblastic or fibroblastic types Osteoclast-like giant cells may be present
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26 : Cartilage-Forming Tumors Benign: Chondroma Osteochondroma Chondroblastoma Chondromyxoid Fibroma Malignant: Chondrosarcoma
27 : 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
28 : 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)
29 : Localized central lytic lesion surrounded by sharp rim of sclerosis; cortex usually not involved, though may be thin
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32 : Ollier’s disease
33 : Composed of mature lobules of hyaline cartilage with foci of myxoid degeneration, calcification and endochondral ossification; may be quite cellular
34 : 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
35 : 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
36 : 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)
37 : Projection with cortex continuous with underlying bone; may be pedunculated; cartilaginous cap with frequent calcification
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41 : 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
42 : 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
43 : Lytic lesion of epiphysis with thin sclerotic rim; thinning without destruction of cortex Chondroblastoma
44 : Cellular lesion with polyhedral chondroblasts; giant cells and areas of calcification may be seen; mitoses common
45 : Chondroblastoma Ancillary Testing: N/A Prognosis/Treatment: Curettage with bone grafting May recur locally if not completely removed
46 : 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
47 : Eccentric, sharply defined radiolucency in metaphysis of long bones; may destroy cortex
48 : Zones of cartilage, fibrous and myxoid tissue; few benign giant cells
49 : Chondromyxoid Fibroma Ancillary Testing: N/A Prognosis/Treatment: En bloc resection Curettage is associated with 25% rate of recurrence
50 : 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
51 : Ill-defined margins; fusiform thickening of shaft; perforation of cortex
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57 : Myxoid Chondrosarcoma Mesenchymal Chondrosarcoma
58 : 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
59 : Miscellaneous Tumors of Bone Giant Cell Tumor Ewing’s Sarcoma / Primitive Neuroectodermal Tumor (PNET) Chordoma
60 : 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
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65 : 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
66 : 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
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69 : 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%
70 : 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
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72 : 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
73 : Tumor-Like Lesions of Bone Metaphyseal Fibrous Defect (Nonossifying Fibroma)
74 : 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
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77 : Nonossifying Fibroma Ancillary Testing: N/A Prognosis/Treatment: Most lesions undergo spontaneous regression

 

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