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Bone Tumors in the Lower Extremity How to identify benign and malignant osseous tumors in the foot and ankle
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Bone Tumors

May 07, 2015

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Page 1: Bone Tumors

Bone Tumors in the Lower Extremity

How to identify benign and malignant osseous tumors in the

foot and ankle

Page 2: Bone Tumors

Bone Tumors in the Lower Extremity

Benign tumors usually present with–

Well-defined sclerotic borders

Geographic type of bone destruction

Uninterrupted solid periosteal reaction

No soft tissue mass

Malignant tumors usually present with–

Poorly defined border with a wide zone of transition

Moth eaten or permeative bone destruction

Interrupted periosteal reaction (sunburst or onion skin)

Adjacent soft tissue mass

Page 3: Bone Tumors

Bone Tumors in the Lower Extremity

• Bone tumors can be primary or secondary (metastatic) • Staging of these tumors is done once the type of tumor has

been determined • This staging is done by plain films, bone scans, CT, MRI,

arteriography and biopsy• Appearance of the lesion can guide the diagnosis

– Appearance includes the pattern of destruction: geographic, moth- eaten or permeative in nature

– The size, shape and the margin of the tumor must also be considered.

Page 4: Bone Tumors

Bone Tumors in the Lower Extremity

• The matrix of the tumor can be identified as calcified (indicating cartilaginous origin), or ossification (of bony origin, seen in the osteoid osteoma and the malignant osteogenic sarcoma)

• Cortical erosive patterns , periosteal response, area of the bone involved (metaphysis, epiphysis or diaphysis) are also involved in the identification of the tumor.

• Treatment usually involves either surgical excision or in the case

of metastatic spread, irradiation and/or chemotherapy.

Page 5: Bone Tumors

Bone Tumors in the Lower Extremity

• Surgical excision of benign tumors involves a biopsy to confirm the diagnosis prior to any surgical procedure, done as a two- stage procedure (biopsy done first and the surgical excision done at a later date)

• The biopsy is done under general anaesthesia when suspicious of a malignancy, as use of a local can seed sarcomas

• Make as small a longitudinal incision as possible, so as to allow

adequate skin covering.

Page 6: Bone Tumors

Bone Tumors

• Some researchers have suggested that bone tumors affect the

feet in a disproportionately small number of cases

• Keep in mind that most major studies on the subject have been

assembled at major centers for the treatment of cancer

• Therefore, lesions that have clearly benign clinical or radiologic

features are largely omitted

Page 7: Bone Tumors

Bone Tumors

• In order to identify bone tumors of the feet accurately, one must understand the relative frequency with which various

tumors of bone occur in that location

• Occasional tumors of bone (such as Dupuytren’s exostosis)

occur in the feet in the vast majority of cases

• Osteochondroma, the most common benign bone tumor, is rare in the small bones of the feet

Page 8: Bone Tumors

Bone Tumors

• A relatively large number of reactive or reparative lesions of bone are seen within bone study series.

• Usually identified conditions such as Florid Reactive Periostitis, Bizarre Parosteal Osteochondromatous Proliferation (Nora’s lesion) and Giant Cell Reparative Granuloma were seen at a much higher rate than has been

described outside of the hands and feet

• There is an overwhelming predominance of benign lesions, as

compared to malignant lesions, in these locations

Page 9: Bone Tumors

Bone Tumor Classification

Divide bone lesions of the small bones of the feet into three categories:

1. reactive/reparative lesions 2. benign neoplasms 3. malignant neoplasms

Page 10: Bone Tumors

Bone Tumor Classification

• Reactive/reparative lesions of bone are non-neoplastic proliferations or hyperplasias that are incited by trauma or stress in most cases

• Neoplasms are autologous new growths that are clonal in nature in that they emanate from a single genetically identical “mother cell”

• In most cases, true neoplasms do not form in response to trauma or involute upon removal of a stimulus

Page 11: Bone Tumors

Reactive/Reparative Lesions

• Giant Cell Reparative Granuloma, Florid Reactive Periostitis, Dupuytren’s Exostosis and Bizarre Parosteal Osteochondromatous Proliferation accounted for 50 of the 151 benign lesions in the series published by Ostrowski and Spjut (excluding those lesions that were classified as a reaction

to injury, fracture or osteomyelitis)

• Being aware of these lesions, which share atypical histopathologic and clinical features, can help prevent erroneous clinical diagnoses and facilitate referrals for outside pathology

consultations when indicated

Page 12: Bone Tumors

Giant Cell Reparative Granuloma• Intraosseous tumors comprised of a mixture of giant cells and

fibrous stroma • They are believed to arise in response to hemorrhage • Some investigators have viewed giant cell reparative granuloma

(GCRG) as a solid variant of aneurysmal bone cyst • Most cases will present as solitary painful and swollen lesions

among teenagers or people who are in their twenties • These lesions more commonly affect the hands than the feet

with rare history of trauma

Page 13: Bone Tumors

Giant Cell Reparative Granuloma

• Radiologically, GCRG appear as radiolucent lesions measuring from 2 to 2.5 cm in greatest diameter

• You will see bone expansion and cortical thinning but you usually won’t see evidence of destruction

• There is no surrounding sclerosis nor intralesional calcifications

and the epiphyseal ends of long bones are spared in most cases

Page 14: Bone Tumors

Florid Reactive Periostitis• A reactive process leading to metaplastic bone and cartilage

formation• Has radiologic and histopathologic similarities with parosteal

osteogenic sarcoma and osteochondroma• FRP most commonly causes pain and swelling• Slight female predominance and a history of trauma is noted in

roughly 40 percent of the cases• Radiologically a fusiform soft tissue mass enveloping the

proximal phalanx, but can affect any of the tubular bones of the hands and feet

• A laminated periosteal reaction is evident in about a third of cases

Page 15: Bone Tumors

Dupuytren’s exostosis (DE)• Unlike osteochondromas, which are neoplastic proliferations of

epiphyseal cartilage, DE are metaplastic osteochondral proliferations that result from persistent microtrauma

• First described by Dupuytren in 1847, this tumor most commonly arises within the soft tissue deep to the medial nail bed of the hallux and thus was originally designated as a “subungual exostosis”

• Unlike an osteochondroma, a neoplastic tumor of bone, DE does not show a tendency toward malignant transformation and is not associated with familial osteochondromatosis

Page 16: Bone Tumors

Nora’s Lesion• Bizarre parosteal osteochondromatous proliferation (Nora’s

lesion) was first characterized in 1983 by Nora and colleagues of the Mayo Clinic

• Typically arises around the proximal phalanges, metacarpals or metatarsals, and presents as a painless and rapidly growing mass

• When patients do report symptoms, they are most often related

to tumefaction

Page 17: Bone Tumors

Nora’s Lesion

• Awareness of bizarre parosteal osteochondromatous proliferation (BPOP) is particularly important due to the lesion’s tendency for local recurrence and its atypical histopathologic features

• If this diagnosis is a clinical possibility, be aware that it can be mistaken for sarcoma

• Radiologically, one will see BPOPs as well-delineated partially mineralized masses emanating from the cortical surface of the involved bone. Larger lesions are lobulated and exhibit a

haphazard pattern of mineralization

Page 18: Bone Tumors

Benign Tumors Osteochondromas

• Osteochondromas of cartilaginous origin• The most common benign tumor of bone

– account for between 40 to 50 percent of all benign neoplasms of bone

– represent only 3 to 8 percent of such neoplasms in the feet

• Radiographic signs are usually diagnostic. Appears sessile or pedunculated

• If >2.5 cm in size, the lesion has a predilection to progress to a chondrosarcoma

• Appears in the lower metaphysis of the femur and the upper

metaphysis of the tibia

Page 19: Bone Tumors

Benign Tumors Enchondroma

• Exhibit hyaline cartilage differentiation• Lesions in the bones of the hands and feet are more commonly

symptomatic than those arising elsewhere in the skeletal system• Are solitary lesions although multiple bones may be involved,

especially in the setting of Maffucci syndrome (enchondroma seen with hemangiomatosis) or Ollier disease (enchondromatosis)

• Typically see bone expansion and cortical thinning• Characteristically radiolucent and well-delineated• Cortical expansion and endosteal erosion in large long bones

warrant a suspicion of malignancy

Page 20: Bone Tumors

Benign Tumors Chondroblastoma

• A benign but locally aggressive neoplasm• 23% of all chondroblastomas arise in the bones of the

hands and feet• 90% of the chondroblastomas that arose in the feet

were located in the rearfoot tarsal bones• Male predominance (83%)• The most common presenting complaint is pain

without a corresponding history of trauma, occasional edema

• Moderately well circumscribed lesions; consistently radiolucent with geographic zones of bone destruction with little or no matrix production

Page 21: Bone Tumors

Benign Tumors Osteoblastoma

• Relatively uncommon neoplasms of bone, they have a disproportionately high frequency in the bones of the feet

• Usually presents in the second or third decade of life with marked male predominance

• Nearly all patients (97%) complain of low grade aching pain and about one in four experience concurrent swelling

• Round or oval, well circumscribed, and range from 2 to 10 cm in size. Most form radiolucent expansile masses that lack the sclerotic margin seen in association with osteoid osteoma

• Talus is the most commonly affected bone

Page 22: Bone Tumors

Benign Tumors Osteoid Osteoma

• Typically present with pain ranging from dull to intense and usually shows nocturnal exacerbation, with 80% of patients experience relief with NSAIDs

• Demonstrates unmyelinated nerve fibers and excessive prostaglandin E2 production in the nidus

• Children and adolescents are most commonly affected by osteoid osteoma and may be initially misdiagnosed as “growing pains”

• Dense, often fusiform, ring of sclerosis surrounding a radiolucent nidus with ossification of the nidus

leading to a targetoid radiologic appearance

Page 23: Bone Tumors

Benign Tumors Unicameral Bone Cyst

• Fibrous in nature• Radiographic signal is the "fallen fragment sign"• Look for attenuated cortical wall lined by a fibrous membrane

with serosanguinous fluid• Diagnosis needs 4 criteria:

– typical radiograph– yellow fluid aspirate– arterial pressure 15-18mm

– cyst filling with renografin

Page 24: Bone Tumors

Benign Tumors Fibrous Dysplasia

• A skeletal developmental anomaly of the bone- forming mesenchyme that manifests as a defect in osteoblastic differentiation and maturation

• Usually monostotic• When polystotic, you also see melanotic

pigmentation at the skin (café-au-lait) and endocrine dyscrasias. The combination of fibrous dysplasia, hormonal disturbances, and skin pigmentation is called McCune-Albright syndrome

• Patients who have fibrous dysplasia in only one bone usually do not develop McCune-Albright syndrome. The genetic abnormality that has been found in bone is also found in other tissues that have abnormal function

• Has a diagnostic ground glass appearance

Page 25: Bone Tumors

Benign Tumors Giant Cell Tumor

• A benign but locally aggressive tumor• Characterized by a dense infiltrate of reactive osteoclast-like

giant cells scattered among a neoplastic population of small epithelioid or slightly spindled mononuclear cells

• Giant cell tumors account for approximately 20% of all benign tumors with the incidence within the small bones of the feet estimated to be about 1.8%

• One of the few tumors of bone where there is a significant female predominance

• Commonly present with either localized pain and swelling, or with pain in the absence of swelling

• Most often affects people in their third decade of life• Like chondroblastomas, benign GCTs may also exhibit

pulmonary spread

Page 26: Bone Tumors

Benign Tumors Giant Cell Tumor

• In the bones of the feet, GCTs average almost 3cm• When within long bones, they invariably involve the

physis• Largely lytic, but may disclose trabeculation that can

vary from fine to moderately coarse leading to the characteristic “soap-bubble” appearance

• Rarely see periosteal reactions typical of osteosarcoma (Codman’s triangle, sunburst) and Ewing’s sarcoma (onion skinning)

• Many GCTs in the small bones of the feet produce cortical erosion and expansion with the lesion covered by a thin layer of periosteal new bone that is not apparent radiologically

Page 27: Bone Tumors

Benign Tumors Non-ossifying Fibroma

• Seen at the metaphysis of the long bones• Heal spontaneously• One of the more common benign tumors found in children and

adolescents• The cause of a non-ossifying fibroma is unknown. It is believed

to be a developmental defect associated with a vascular

disturbance or related to a hemorrhage inside the bone

Page 28: Bone Tumors

Benign Tumors Eosinophillic Granuloma

• Seen in the 1st decade of life (age 5 - 10 yrs)• Moth-eaten cortical pattern• 60-80% of histiocytosis X (Hand-Schuller-Christian Syndrome)

– Disease of the immune system, where histiocytes and eosinophils grow uncontrollably, usually in the lungs and bone. These cells constitute malignant or benign macrophages in the tissues, or Langerhans' cells in the bone marrow.

– This syndrome is characterized by three, simultaneous conditions: • Diabetes Insipidus• Exopthalmus• Destructive Bone Lesions

Page 29: Bone Tumors

Benign Tumors Eosinophillic Granuloma

• Presentation: bone pain, local swelling, irritability• Bones 50 - 75% solitary/monostotic with skull/mandible (50%),

with "punched-out" lucencies, "hole within a hole", "button sequestrum

• lungs involved in <10%, signals worse prognosis– Langerhans cell histiocytosis (LCH) describes a group of syndromes

that share the common pathologic feature of infiltration of involved tissues by Langerhans cells. Typically, the skeletal system is involved, with a characteristic lytic bone lesion form that occurs in young children or a more acute disseminated form that occurs in infants

Page 30: Bone Tumors

Benign Tumors Chondromyxoid Fibroma

• Least common of the tumors – less than 1% of primary osseous neoplasms

(approximately 2% of benign bone tumors)

• Consists of chondroid, myxoid, and fibrous tissue in variable amounts

• Most often found in the long tubular bones, especially the tibia and femur near the knee joint

• Osteoclast-like giant cells also may be present, as may small cysts and hemorrhagic zones and focal calcification is found microscopically occasionally,

although any gross evidence of calcification is rare

Page 31: Bone Tumors

Benign Tumors Aneurysmal Bone Cyst

• Traditionally been classified as either primary or secondary• Primary ABCs emerge in the absence of an alternate neoplasm

while secondary ABCs are engrafted upon another tumor with approximately 50 to 71 percent of ABC cases are primary in nature

• Associated neoplasms include chondroblastoma, giant cell tumor, non-ossifying fibroma, osteoblastoma and osteosarcoma

Page 32: Bone Tumors

Benign Tumors Aneurysmal Bone Cyst

• Most often present with localized pain and swelling. In a small number of cases, one may see pathologic fractures

• Unlike most other tumors of bone, ABC will cross open growth plates in 23% of cases

• Expansion of the involved bone with cortical thinning is a characteristic feature of ABC and is found in up to 93% of cases

• Roughly half of the cases, the radiographic margin is distinct but not sclerotic. However, keep in mind that sclerosis is present in about a third of cases

• These lesions cannot be radiologically distinguished from malignancies in 42% of cases

Page 33: Bone Tumors

Malignant Tumors of Bone Osteogenic Sarcoma

• The most common of all non-hematopoietic primary malignant neoplasms of bone found in the skeletal system

• Osteogenic Sarcoma or Osteosarcoma encompass a number of distinct tumor subtypes with a spectrum of clinical aggressiveness

• single unifying feature is the production of osteoid by a population of malignant cells

• classified into various subtypes based on the site of origin, pattern of growth, cytologic features, the type of stroma

produced and the tumor grade

Page 34: Bone Tumors

Malignant Tumors of Bone Osteogenic Sarcoma

• One may see tumors arise on the external surface of the involved bone, within the cortex or within the medullary canal

• Alternatively, neoplastic cells may form large telangiectatic spaces filled with blood (telangiectatic OS) or, from a cytologic perspective, might be comprised of small hyperchromatic cells reminiscent of Ewing's sarcoma (small cell OS)

• The tumor grade is related to the degree of cytologic atypia,

mitotic activity and necrosis

Page 35: Bone Tumors

Malignant Tumors of Bone Osteogenic Sarcoma (high grade)

• The high-grade portion of the OS spectrum consists of conventional intramedullary OS, high-grade surface OS, telangiectatic OS and small cell OS

• Conventional intramedullary is by far the most common variety of osteogenic sarcoma, accounting for more than 90% of all cases

• One may sub-classify these exceedingly aggressive neoplasms even further, according to their histologic appearance, as either osteoblastic,

chondroblastic, fibroblastic or giant cell rich

intramedullary

Page 36: Bone Tumors

Malignant Tumors of Bone Osteogenic Sarcoma (high grade)

• Male predominance that becomes less pronounced with progressive age

• Over 60% of OS cases occur in people under the age of 25, but there is a second peak beyond 60, largely related to secondary factors such as Paget's disease and radiation exposure

• most common presenting complaint is pain but concurrent swelling is a routine finding

• the calcaneus is the most commonly affected bone, followed by

the talus and the metatarsals

Page 37: Bone Tumors

Malignant Tumors of Bone Osteogenic Sarcoma (high grade)

• Most pedal cases disclose transcortical penetration with soft tissue extension at diagnosis.

• There is an admixture of lysis, sclerosis and rarefaction in most cases, although a single pattern often predominates.

• One may see "starburst" phenomenon and Codman's triangle in the bones of the feet but these are not typical findings

• In one study OS of the foot was misdiagnosed in 50% of cases• May resemble giant cell tumor or aneurysmal bone cyst in plain

film

Page 38: Bone Tumors

Malignant Tumors of Bone Osteogenic Sarcoma (intermediate grade)

• The sole subtype that falls into the category of intermediate grade is periosteal (juxtacortical chondroblastic) OS

• Accounts for roughly 2% of all cases• Unlike conventional OS, periosteal OS commonly

affects the diaphysis of long bones• Associated with a significantly better prognosis than

conventional OS• Arises on the external surface of long bones, the

tumor is not typically confused with conventional intramedullary OS, but must be differentiated from other surface tumors

Page 39: Bone Tumors

Malignant Tumors of Bone Osteogenic Sarcoma (low grade)

• Comprised of parosteal OS, intracortical OS and well-differentiated (low-grade) intramedullary OS.

• Parosteal OS accounts for roughly 80% of all cases of low-grade OS

• Parosteal OS arises on the external surface of long bones with a predilection for the distal posterior femur (70%), tibia and humerus

• Chief complaint with regard to parosteal OS is pain- free swelling

• Tendency toward local recurrence although metastases at presentation are extremely uncommon

• Radiologically, parosteal OS appears in plain film radiographs as a dense, heavily mineralized mass attached to the external surface of tubular bones by a broad base

Page 40: Bone Tumors

Malignant Tumors of Bone Chondrosarcoma

• Roughly 98% of all CHS is seen within bones external to the foot

• Primary bone CHS may arise within the medullary canal or upon the periosteal surface.

• There are several histopathologic subtypes and a spectrum of tumor grades, each exhibiting different clinical behavior

• Conventional CHS accounts for over 90% of all lesions with the remaining 10% being comprised of an admixture of clear cell CHS, myxoid CHS, dedifferentiated CHS and mesenchymal CHS

• Largely a disease of middle-aged adults and the elderly

Page 41: Bone Tumors

Malignant Tumors of Bone Chondrosarcoma

• Slight male predominance• Calcaneus most common, but may affect any

bone, including the sesamoids• Commonly presents with pain, swelling or both. In

the foot, the presence of pain and an older patient age may be important clues in diagnosing CHS as opposed to enchondromas, which are uniformly painless and more common in young adults

Low-grade

Page 42: Bone Tumors

Malignant Tumors of Bone Chondrosarcoma

• Radiological appearance of CHS is exceedingly important for the correct diagnosis of low and intermediate grade lesions.

• In many instances, the histopathologic diagnosis of low-grade CHS cannot be done without radiographic analysis

• In the feet, the most common single feature is endosteal erosion (94%) with or without frank cortical destruction (92%)

• Bony expansion, soft tissue extension and a permeative pattern of growth are additional findings that suggest the diagnosis of

CHS over enchondroma

Page 43: Bone Tumors

Malignant Tumors of Bone Malignant Fibrous Histiocytoma

• High-grade without osteoid production • Appears as an osteolytic lesion at the metaphysis

with permeative destructive pattern • No periosteal reaction, no matrix produced • Associated with Paget's, fibrous dysplasia and

osteomyelitis• May occur following radiation treatments• Among the most common types of soft tissue

tumors found in adults, it is rarely found in children

• Originates in primitive mesenchymal cells

2º to Paget’s

Page 44: Bone Tumors

Malignant Tumors of Bone Fibrosarcoma

• Can occur as a soft tissue mass or as a primary or secondary bone tumor

• Primary fibrosarcoma is a fibroblastic malignancy that produces variable amounts of collagen. It is either central, arising within the medullary canal, or peripheral, arising from the periosteum

• Secondary fibrosarcoma of bone arises from a preexisting lesion or after radiotherapy to an area of bone or soft tissue. This is a more aggressive tumor with poorer prognosis

• Must be distinguished histologically from similar lesions, such as desmoid tumors, malignant fibrous histiocytoma, malignant schwannoma, and high-grade osteosarcoma

Page 45: Bone Tumors

Malignant Tumors of Bone Ewing’s sarcoma

• Ewing's sarcoma/primitive neuroectodermal tumor (PNET) has been documented as the most common non-hematopoietic primary malignancy of the bones of the feet

• Are considered variations of the same disease, Ewing's sarcoma is commonly taken to connote tumors that lack neuroectodermal differentiation

• Ewing's sarcoma and PNET have a chromosomal translocation t(11; 22) (q24; q12) in common in 85% to 90% of cases. The remaining 10% to 15% of these malignant neoplasms are believed to have alternate mutations involving the Ewing's

sarcoma gene on chromosome 22

Page 46: Bone Tumors

Malignant Tumors of Bone Ewing’s sarcoma

• May affect any bone of the foot with the calcaneus the most common, with calcaneal lesions imparting a worse prognosis

• Usually affects people in their second decade of life with a male predominance

• In the long bones of the appendicular skeleton, a diaphyseal-based tumor with admixed lytic and sclerotic features, and characteristic “onion skinning” is seen

• In the feet, onion skinning is an uncommon finding

Page 47: Bone Tumors

Malignant Tumors of Bone Angiosarcomas and Epithelioid Hemangioendotheliomas

• Exhibit endothelial (vascular) differentiation. In some cases, such differentiation is not apparent by light microscopy and must be demonstrated with immunohistochemical studies or electron microscopy

• Angiosarcoma may exhibit a spectrum of differentiation, ranging from extremely well- differentiated tumors that resemble benign hemangiomas to poorly differentiated neoplasms with few if any vascular features by light microscopy

• The prognosis is worse for higher-grade tumors that are less differentiated

Page 48: Bone Tumors

Malignant Tumors of Bone Angiosarcomas and Epithelioid Hemangioendotheliomas

• The trend is to use the term "hemangioendotheleoma" to denote those lesions that are low-grade (well differentiated) and "angiosarcoma" to refer to those tumors that are of a higher tumor grade (poorly differentiated)

• Together, EHE and AS ranked fourth in frequency amongst non- hematopoietic primary malignancies of the pedal bones

• They account for roughly 1% of all malignant bone tumors• Multicentric in 20% to 50% of cases• Clustering of distinct lesions in a single region is highly

suggestive of EHE or traditional AS

Page 49: Bone Tumors

Malignant Tumors of Bone Adamantinoma

• A rare, slow growing tumor most often found in the tibia or mandible, but are occasionally found in the forearm, hands or feet. Primarily in males between the ages of 10-30, usually after skeletal maturity is reached.

• In about 20% of cases, will metastasize to other parts of the body, usually to the lungs or nearby lymph nodes.

• Of unknown etiology, commonly have a history of trauma, but it is unclear whether trauma is involved with formation. Symptoms may include, but are not limited to: pain (sharp or dull), swelling and/or erythema at the site of the tumor.

Page 50: Bone Tumors

Malignant Tumors of Bone Leiomyosarcoma

• Primary leiomyosarcoma of bone is extremely rare with approximately 50 cases reported in the literature

• A metastatic lesion must be ruled out • Differential Diagnosis is usually not

extensive and includes fibrosarcoma, MFH, and metastatic spindle-cell carcinoma

Page 51: Bone Tumors

Malignant Tumors of Bone Multiple Myeloma

• Multiple myeloma is the most common primary tumor of bone

• The average patient age is over fifty years old and men are affected twice as often as women

• The presenting symptom of multiple myeloma is usually pain

• Bence Jones proteins are present in urine

• Hypercalcemia may cause confusion, weakness and lethargy

Page 52: Bone Tumors

Metastatic Carcinoma

• The most common primary sites to metastasize to the bones of the foot are the lower intestinal tract, followed closely by the genitourinary system and lower respiratory tract

• This contrasts with metastases to the bones of the hand in which pulmonary sources overwhelmingly predominate

• Metastases to bone are indicative of end-stage disease• Patient survival following such events averages less than 12

months

Page 53: Bone Tumors

Metastatic Carcinoma

• In the rare instances when children are affected, the most common offending neoplasms are neuroblastoma, rhabdomyosarcoma and clear cell sarcoma of the kidney

• Metastases may produce lytic lesions, sclerotic (osteoblastic) lesions or a combination of the two

Page 54: Bone Tumors

Tumors by Type

Tumors that form boneOsteomaOsteoid osteomaOsteoblastomaOsteosarcoma Conventional Intramedullary OsteosarcomaExtraosseous osteosarcomaMultifocal OsteosarcomaTelangectatic OsteosarcomaSmall Cell OsteosarcomaIntraosseous Well-differentiated OsteosarcomaIntracortical OsteosarcomaPeriosteal OsteosarcomaParosteal Osteosarcoma

High-Grade Surface Osteosarcoma

Page 55: Bone Tumors

Tumors by Type Tumors that form Cartilage

OsteochondromaOsteochondromatosis (HMOCE)Periosteal ChondromaJuxtacortical ChondromaEnchondromaEnchondromatosis (Ollier's Disease)Mafucci Syndrome ChondroblastomaChondromyxoid FibromaChondrosarcomaDedifferentiated chondrosarcomaClear Cell ChondrosarcomaMesenchymal Chondrosarcoma

Juxtacortical Chondrosarcoma

Page 56: Bone Tumors

Tumors by Type That form Fibrous Tissue

Nonossifying fibroma (fibrous cortical defect)Fibrous DysplasiaOsteofibrous DysplasiaDesmoplastic FibromaExtragnathic FibromyxomaFibrosarcomaBenign Fibrous Histiocytoma

Malignant fibrous hystiocytoma

Page 57: Bone Tumors

Tumors by Type Lesions of Marrow Elements

Eosinophilic GranulomaMultiple myelomaSolitary myeloma (plasmacytoma)Leukemia Non Hodgkin lymphomaHodgkin lymphoma of boneGranulocytic Sarcoma in boneSystemic mastocytosisSinus histiocytosis with Massive

Lymphadenopathy

Page 58: Bone Tumors

Tumors by Type Forming Vascular Tissue

Skeletal lymphangiomatosisHemangiomaSkeletal AngiomatosisMassive OsteolysisEpithelioid hemangioendotheliomaEpithelioid hemangiomaAngiosarcoma - low gradeAngiosarcoma - high gradeHemangiopericytomaGlomus tumor

Page 59: Bone Tumors

Tumors by Type Miscellaneous

ChordomaConventional ChordomaChondroid chordomaDedifferentiated chordomaSchwannoma of boneNeurofibroma of boneLiposarcoma of bonePost - Paget's Sarcoma

Malignant Mesenchymoma of bone

Page 60: Bone Tumors

Tumors by Type Metabolic Disease

Brown Tumor of HyperparathyroidismBone lesions of Gaucher's DiseasePaget's diseaseDisappearing bone disease

Page 61: Bone Tumors

Tumors by Type Metastatic to Bone

Metastatic Breast CancerMetastatic Lung CancerMetastatic Kidney CancerMetastatic Prostate Cancer

Metastatic Thyroid Cancer

Page 62: Bone Tumors

LOCATION/SITE TYPICAL PATHOLOGY

Epiphyseal Chondroblastoma, chondrosarcoma, giant cell tumor, infection

Metaphyseal Any lesion

Diaphyseal Osteoblastoma, Ewing’s eosinophilic granuloma, lymphoma, adamantinoma, fibrous dysplasia

Pelvis Mets, myeloma, Ewing’s, chondrosarcoma, Paget’s Disease.

Proximal humerus Chondroid lesions.

Knee Osteosarcoma, adamantinoma, chondromyxoid fibroma

Ribs Mets, myeloma, Ewing’s chondrosarcoma, fibrous dysplasia

Spine (Vertebral body) Mets, myeloma, eosinophilic granuloma, chondroma, Paget’s, haemangioma

Spine (Posterior elements) Aneurysmal bone cyst, osteoid osteoma, osteoblastoma

Periosteal Myosotis, osteosarcoma, chondrosarcoma, chondroma

Multiple lesions Mets, myeloma, haemangioma, fibrous dysplasia, osteochondromas, enchondromas, histiocytosis X

Page 63: Bone Tumors

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