Bone Tumors Flashcards by Andrew Cibulas

What areas of the body with Paget’s disease most likely afflict?

Skull, Pelvis, Vertebrae, Tibia (Long bone)

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What areas of the body with fibrous displasia most likely afflict?

Shoulder (Joints)

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What areas of the body will enchondroma most likely afflict?

Wrist and Ankle (hands and feet)

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What areas of the body will Osteochondroma and Chondroblastoma likely afflict?

Elbow and Knee (bones on either side of the joint)

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What areas of the body with Chordoma likely afflict?

Nose and Ribs

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What areas of the body with osteoblastoma likely afflict?

Neck (Vertebrae)

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What ares of the body will Osteosarcoma likely afflict?

Long bones (proximal humerus, shaft of the femur, distal femur, proximal tibia shown)

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What areas of the body will Chondrosarcoma likely affect?

Hip

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What areas of the body will Giant cell tumors affect?

Shown are distal radius, all parts of the femur, and proximal tibia

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What benign tumors occur in the epiphysis?

Chondroblastoma and Giant Cell tumors

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What benign tumors occur in the metaphysis?

Osteoblastoma, osteochondroma, non-ossifying fibroma, osteoid osteoma, chondromyxoid fibroma, giant cell tumor

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What benign tumors occur in the diaphysis?

Enchondroma, Fibrous dysplasia

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What malignant tumors occur in the diaphysis?

Ewing’s sarcoma, chondrosarcoma

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What malignant tumors occur in the metaphysis?

Osteosarcoma, juxacortical osteosarcome

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Describe fibrous dysplasia.

Benign, localized developmental arrest in which all components of the bone are present but do not mature

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What are the 3 distinctive patterns of fibrous dysplasia?

Monostotic (single bone), polyostotic (multiple bones), and McCune Albright syndrome which is polyostotic, cafe-au-lait skin pigmentation, and endocrinopathies

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What may polyostotic disease progress to?

Osteosarcoma

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On x-ray describe fibrous dysplasia

Lucent ground glass appearance with well demarcated borders

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What are the histologic buzzwords of fibrous dysplasia?

Loose whorled pattern of fibroblastic tissue; irregular spicules

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What is fibrous corticol defect?

An eccentric, sharply delineated, metaphyseal lesion in long bones of adolescents (A.K.A. benign fibrous histiocytoma if mass forming and involving medullary cavity)

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What are the clinical features of fibrous corticol defect?

30-50% of all children > 2 (COMMON!), few to no symptoms except pain, and fractures can occur in thinned cortex

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Where does fibrous corticol defect occur and how is it found?

Proximal tibia or distal femur and detected incidentally on radiography (Scooped out lesions! and about half are bilateral)

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How is fibrous corticol defect treated?

It isn’t, usually resolves spontaneously

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Describe gross pathology of fibrous corticol defect.

Granular dark brown/red, eccentric, sharply delimited

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What is the buzzword for Nonossifying fibromas for x-ray?

Scerotic margin with a woven mat/storiform pattern

What are solitary bone cysts?

They are benign fluid filled cysts and tend to have very thin margins of bone that appear lytic

Where do solitary bone cysts occur?

Generally in the humerus or femur (2/3rds)

What are the clinical features of solitary bone cysts?

Usually asymptomatic but if large enough they can contribute to a fracture

What are aneurysmal bone cysts?

They are bloody, cystic lesions that are also not true tumors

Where do aneurysmal bone cysts form?

Anywhere but usually found in the long bones and vertebral column

What is the complication with aneurysmal bone cysts?

May contribute to fractures, but if not expected can lead to operative challenges due to bleeding; generally grow slowly but can come to attention from rapid expansion

When are benign primary bone neoplasms most frequent?

First 3 decades of life

How are benign primary bone neoplasms found and treated?

They are usually found only incidentally on x-ray and only removed if they cause pain

Do primary bone neoplasms undergo malignant transformation?

Rarely, except chondromas

What lesions are included in the group of benign primary bone lesions?

Osteoma, osteoid osteoma, osteochrondroma/chondroma, fibroma, giant cell tumors

What is an osteoma?

It is a bone forming tumor; they are round tumors that project from sub or endosteal surfaces

Are osteomas multiple or single?

Usually single and multiple in Gardner's syndrome

Are osteomas clincally significant?

Generally no, slow growing, and only a problem if they cause obstruction

What is an osteoid osteoma?

A more common representation of osteomas and generally <2 cm in size, occurring in teens/twenties and more in males

Where are osteoid osteomas usually found?

50% in tibia and fibula, usually cortical

What is the primary characteristic of osteoid osteomas?

They are painful, and produce PGE2; more painful at night; relieved by aspirin; alcohol increases pain

What is an osteochondroma?

Synonym: exostosis; they are benign cartilage capped tumors that attach to underlying skeleton by a stalk

What are osteochondromas related to?

Displacement of growth plate in endochondral bone

What is multiple hereditary exostosis?

It is AD and rarely gives rise to chondrosarcomas

If you see a little outpouch of cartilage near the epiphyseal plate, waht is it?

Osteochondromas

What is a chondroma?

A benign tumor of hyaline cartilage

What are endhondromas?

chondromas that arise in medullary cavity, typically in the hands and feet

What are juxtacortical chondromas?

They arise on the surface of bones

Are enchondromas usually multiple or solitary?

Solitary

What diseases are multiple enchondromas/enchondromatosis seen in?

Ollier dz (nonhereditary syndrome) and Maffucci syndrome (multiple enchondromas and hemangiomas)

What are giant cell tumors of bone?

Multinucleated osteoclast type giant cells that are benign but uncommon, locally aggressive, occur from 20's-40's, undergo cystic degeneration, and arise around the knee (2ndary area is wrist)

What are malignant bone tumors?

They are rare primary bone neoplasms, half of which are derived from blood forming cells (commonest is multiple myeloma)

What are the most common bone tumors?

(Secondary) metastatic bone tumors

What are the sites from which metastases occur to the bone?

Breast, prostate, lung, kidney, and thyroid

What is special about the occurrence of malignant primary bone tumors?

They occur in specific bones at certain ages

Where do osteosarcomas occur and how common are they (with respect to primary lesions)?

Most common primary malignant bone tumor in the knee

Where do chondrosarcomas occur and when?

Adults and in cartilage, trunk and limb girdles

What is ewing's sarcoma?

Peripheral primitive neuroectodermal tumor, occurs in children and young adults at the diaphysis of bones

75% of osteosarcomas occur in what patient group?

Patients younger than 20; but also in the elderly, in patients with Paget's, or who have been irradiated.

What are osteosarcomas associated with?

Retinoblastomas; Rb; MDM2

Are osteosarcomas painful?

Yes, also enlarging, and fracture

When osteosarcomas result in periosteal elevation what is this called?

The codman triangle on radiography; it is NOT necessarily diagnostic

Where do chondrosarcomas occur?

In the axial skeleton: trunk, pelvis, vertebrae, ribs; rare in the distal extremeties; In the diaphysis or metaphysis

What age group are chondrosarcomas prevalent in?

Ages 35-60

How common are chondrosarcomas?

2nd most common primary malignant bone tumor

How do you treat chondrosarcomas?

Surgery, insensitive to chemotherapy

What is an Ewing sarcoma?

Small round blue cell tumor

When do ewing sarcomas occur?

Ages 10-15; in whites

What translocation is Ewing sarcoma associated with?

t(11,22)+

Where do ewing sarcomas arise?

Medulla; primarily in diaphysis of long bones

In cross section, what is unique about ewing sarcoma?

Their onion skin appearance; layers of reactive periosteum

What is the treatment for ewing sarcoma?

75% 5-year survival with surgery and chemo

What cells are characteristic of ewing sarcoma?

Cmall round blue cells; they are glycogen rich cells that tend to form small structures called Homer-Wright rosettes which are tumor cells arranged in a circle around a central fibrillary space

How frequent are metastatic bone tumors?

They are the MOST COMMON TUMORS IN BONE! and >75% of skeletal metastasis originate from prostate, breast, lung, and kidney

What are metastatic bone tumors associated with in children?

Neuroblastoma, wilm's tumor, osteosarcoma, ewing sarcoma, and rhabdomyosarcoma

Are metastatic bone tumors multifocal?

Yes: vertebrae, pelvis, ribs, skull, and sternum (marrow rich areas)

What will you see with an XR with metastatic bone tumors?

Lytic, blastic, or mixed