Classification of Bone Tumors

Rapid Review

Dpt. Aamir Memon

8/12/2013

Classification of primary benign bone tumors Cartilage tumors

Osteochondroma

Peak age (years) Most common sites

1030 M 2:1 (<20) Distal femur, proximal tibia, proximal humerus, rarely from flat bones Hands, feet, long tubular bones Proximal humerus, distal femur, hip region, and pelvis Distal femur, proximal tibia and humerus, calcaneus Proximal tibia, distal femur, pelvis, feet (metatarsal) Proximal femur, any long bones

Comments
> 2 cm cartilage cap may indicate malignant transformation. Characteristic Radiographic Feature: Radio-opaque with Bony stalk capped with cartilage, oriented away from involved joint Characteristic Radiographic Feature: Radio-lucent expansion with well-defined margins. Sharply demarcated from cortex Typically epiphyseal

Enchondroma Periosteal chondroma Chondroblastoma Chondromyxoid fibroma Osteogenic tumors Osteoid osteoma

1040 1040 1030 1030 525 {M>F (<15)}

Distinguished from osteoblastoma by size and imaging Characteristic Radiographic Feature: Radio-opaque with Lucent nidus surrounded by sclerotic halo (circle within a circle).

Osteoblastoma Fibrogenic tumors Desmoplastic fibroma Fibrohistiocytic tumors Benign fibrous histiocytoma Giant cell tumor

1040 1030

Spine, long tubular bones, jaws Mandible, femur, pelvis Very rare; distinction from FD, low-grade osteosarcoma, and fibrosarcoma may be difficult Diaphyseal or metaphyseal; rarely used concept, distinguished from nonossifying fibroma only by clinical setting Characteristic Radiographic Feature: Soap bubble appearance in metaphysis, with expansive some subarticular expansion.al; pulmonary metastases occur in 2%; very rarely transformation to high-grade sarcoma Hemangiomas are often multicentric

20-60 20-45 {F 3:2}

Pelvis, femur Distal femur, proximal tibia, distal radius, sacrum

Vascular tumor Hemangioma (cavernous, capillary, epithelioid, etc.) Angiomatosis, lymphangioma(tosis) Glomus tumor Hemangiopericytoma Epithelioid hemangioendothelioma Soft tissue type tumors Lipoma Schwannoma Leiomyoma FD fibrous dysplasia

Classic hemangiomas, usually adults Often children Usually adults Usually adults Adults

Craniofacial bones, vertebrae

Highly variable Hands, distal phalanx Pelvis Long tubular bones, spine

Adults

Femur, calcaneus Sacrum, mandible Mandible, tibia

All very rare

Classification of primary malignant bone tumors

Histologic type
Chondrosarcoma Primary Secondary Differentiated Clear cell Mesenchymal Osteosarcoma Conventional Telangiectatic osteosarcoma Low-grade central osteosarcoma Parosteal osteosarcoma Periosteal osteosarcoma High-grade surface Secondary osteosarcoma Pagets associated Post-radiation Other conditions Ewings sarcoma, PNET Fibrosarcoma, MFH, spindle cell sarcoma Malignant giant cell tumor Chordoma

Peak age (years) Most common sites

M 2:1 (40-60)

Comments
Usually large, intraosseous; very rarely periosteal In Olliers/Maffuccis at any site affected Usually small component of low-grade chondrosarcoma juxtaposed with high-grade osteo-, spindle cell-,MFH-, or other sarcoma Typically epiphyseal location 2030% occur in soft tissues Typically metaphyseal Typically metaphyseal; ABC-like, purely lytic May dedifferentiate to high grade May invade the bone, may dedifferentiate to high grade Diaphyseal, surface lesion, predominantly chondroblastic, intermediate grade Diaphyseal or metaphyseal High-grade osteosarcoma High-grade osteosarcoma

Characteristic Radiographic Feature: Radio-lucent, with Lucent expansive lesion with well-defined border. Associated soft tissue reaction.

5080 2060 5070 2560 1040

M 2:1 (15-25)

Pelvis, proximal/distal femur, proximal humerus, ribs Ex osteochondroma(tosis): pelvis, hip and shoulder Pelvis, femur, humerus Proximal femur, humerus Jaws, ribs, pelvis, spine Distal femur, proximal tibia, hip and shoulder Femur, tibia, humerus Distal femur, proximal tibia Posterior distal femur, proximal humerus Femur, tibia Distal femur, shoulder Pelvis, hip and shoulder, Craniofacial Pelvis, craniofacial, hip and shoulder, chest wall Bones affected by FD, bone infracts, chronic osteomyelitis, etc. Pelvis, long bones of lower and upper extremities Knee, hip and shoulder regions, pelvis Knee region, pelvis, shoulder region Sacrococcygeal, skull base, vertebrae

Characteristic Radiographic Feature: Radio-opaque (diffuse sclerosis) with poorly defined border with dense ivory (diffuse sclerosis) regions, soft tissue mass visible on x-ray.

1030 1030 2040 2050 1030 1040 5090 5080 4070 530{M 2:1 (1025)} 4070 2060 M 3:1 3080

Characteristic Radiographic Feature: Periosteal reaction on bone surface gives classic onion skin appearance

High-grade sarcoma arising in GCT; classic GCT may rarely metastasize May rarely dedifferentiate

ABC aneurysmal bone cyst, FD fibrous dysplasia, GCT giant cell tumor, MFH malignant fibrous histiocytoma, PNET primitive neuroectodermal tumor

Age group
0 - 10

Most common benign lesions

simple bone cyst eosinophilic granuloma

Most common malignant tumors

Ewing's sarcoma leukemic involvement metastatic neuroblastoma osteosarcoma, Ewing's sarcoma, adamantinoma

10-20

20-40 40 and above

non-ossifying fibroma fibrous dysplasia simple bone cyst aneurysmal bone cyst osteochondroma (exostosis) osteoid osteoma osteoblastoma chondroblastoma chondromyxoid fibroma enchondroma giant cell tumor osteoma

chondrosarcoma metastatic tumors myeloma leukemic involvement chondrosarcoma osteosarcoma (Paget'sassociated) MFH chordoma

SITE OF LONG BONE INVOLVEMENT (Most primary bone tumors have favored sites within long bones; this may provide a clue to diagnosis). 1. Epiphyseal lesions: o Chondroblastoma (Ch) and Giant Cell Tumor (GCT) are almost invariably centered in the epiphysis. o Chondroblastoma is a rare tumor seen in children and adolescents with open growth plates. GCT is the most common tumor of epiphyses in skeletally mature individuals with closed growth plates. GCT often shows metaphyseal extension. 2. Metaphyseal intramedullary lesions: o Osteosarcoma is usually centered in the metaphysis. o Chondrosarcoma and fibrosarcoma often present as metaphyseal lesions. Osteoblastoma, enchondroma, fibrous dysplasia, simple bone cyst, and aneurysmal bone cyst are common in this location. 3. Metaphyseal lesions centered in the cortex: o Classic location for a non-ossifying fibroma (NOF). o Also, a common site for osteoid osteoma. 4. Metaphyseal exostosis: o Osteochondroma 5. Diaphyseal intramedullary lesions: o Favored location for Ewing's sarcoma, lymphoma, myeloma. o Common for fibrous dysplasia and enchondroma. 6. Diaphyseal lesions centered in the cortex: o Adamantinoma, osteoid osteom

PATTERNS OF GROWTH and BONE DESTRUCTION o Benign and non-growing (or extremely slowly growing) lesions are well circumscribed and show geographic pattern of bone destruction with a sclerotic rim. Geographic pattern refers to a well defined area of lysis. The sclerotic rim is more commonly seen in the weight-bearing bones and represents bone reaction to the lesion. Its presence means that the bone has been given sufficient time to react. Some authors say that the sclerotic rim signifies benignancy to about 95%. o If the lesion is growing more rapidly, it may still show a well-demarcated zone of bone destruction (geographic pattern), but it will lack a sclerotic rim. With continued growth, such lesions may show cortical expansion. Expansile growth pattern is defined as visible widening of the affected portion of bone. In many cases, an interrupted periosteal rim will surround the expanded portion of bone. This pattern may be seen in locally aggressive tumors and in low-grade malignancies. o Rapidly growing lesions are poorly defined and may show aggressive, infiltrative patterns of bone destruction (permeative or "motheaten"). "Moth-eaten" pattern is defined as an ill-defined zone of multiple small radiolucencies that may coalesce. o Permeative pattern is characterized by numerous tiny radiolucencies in between the residual bone trabeculae. Due to the minute size of radiolucencies the lesion may be difficult to see and to delineate on the plain film. Generally, the more rapidly growing a lesion, the more difficult it is to see on plain film. "Moth-eaten" and permeative patterns are indicative of destruction involving both medullary and cortical bone. They are seen in high-grade malignant neoplasms and in osteomyelitis. TYPES OF PERIOSTEAL REACTION The periosteum responds to traumatic stimuli or pressure from an underlying growing tumor by depositing new bone. The radiographic appearances of this response reflect the degree of aggressiveness of the tumor. o Slow-growing tumors provoke focal cortical thickening (solid periosteal reaction, or "buttress") o Rapidly growing lesions penetrate through the cortex causing separation of the periosteum and formation of lamellated new bone. If the periosteum elevates to a significant degree, it can break forming an acute angle (Codman's triangle). This is seen in malignant bone tumors and in some other rapidly growing lesions such as aneurysmal bone cyst, or in reactive processes (osteomyelitis, and subperiosteal hematoma). Codman's triangle is usually free of tumor unless infiltrated through its open end or by transcortical growth. o Other types of periosteal reaction in response to a rapidly growing lesion include "onion-skinning" and spiculated "hair-on-end" types. PATTERNS OF MATRIX MINERALIZATION Mineralization patterns (calcification or ossification) are helpful in identification of bone producing and cartilage producing tumors. o Osteoid: Malignant osteoid can be recognized radiologically as cloudlike or ill-defined amorphous densities with haphazard mineralization. This pattern is seen in osteosarcoma. Mature osteoid, or organized bone, shows more orderly, trabecular pattern of ossification. This is characteristic of the benign bone-forming lesions such as osteoblastoma. o Chondroid: Radiologically, it is usually easier to recognize cartilage as opposed to osteoid by the presence of focal stippled or flocculent densities, or in lobulated areas as rings or arcs of calcifications. They are best demonstrated by CT. Whatever the pattern, it only suggests the histologic nature of the tissue (cartilage) but does not reliably differentiate between benign and malignant processes.