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Bone Tumors in the Lower
       Extremity

   How to identify benign and
 malignant osseous tumors in the
         foot and ankle
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
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.
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.
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.
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
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
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
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
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
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
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
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
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
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
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
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
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
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
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
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
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
Benign Tumors
                    Unicameral Bone Cyst
• Fibrous in nature
• Radiographic signal is the quot;fallen fragment signquot;
• 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
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
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
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
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
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
Benign Tumors
                   Eosinophillic Granuloma
• Presentation: bone pain, local swelling, irritability
• Bones 50 - 75% solitary/monostotic with skull/mandible (50%),
  with quot;punched-outquot; lucencies, quot;hole within a holequot;, quot;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
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
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
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
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
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
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                       intramedullary
• One may sub-classify these exceedingly aggressive
  neoplasms even further, according to their
  histologic appearance, as either osteoblastic,
  chondroblastic, fibroblastic or giant cell rich
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
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 quot;starburstquot; 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
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
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
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
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       Low-grade
  opposed to enchondromas, which are uniformly
  painless and more common in young adults
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
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
                                                           2º to Paget’s
• 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
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
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
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
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
Malignant Tumors of Bone
     Angiosarcomas and Epithelioid Hemangioendotheliomas

• The trend is to use the term quot;hemangioendotheleomaquot; to
  denote those lesions that are low-grade (well differentiated) and
  quot;angiosarcomaquot; 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
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.
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
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
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
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
Tumors by Type
 Tumors that form bone
 Osteoma
 Osteoid osteoma
 Osteoblastoma
 Osteosarcoma
 Conventional Intramedullary Osteosarcoma
 Extraosseous osteosarcoma
 Multifocal Osteosarcoma
 Telangectatic Osteosarcoma
 Small Cell Osteosarcoma
 Intraosseous Well-differentiated Osteosarcoma
 Intracortical Osteosarcoma
 Periosteal Osteosarcoma
 Parosteal Osteosarcoma
 High-Grade Surface Osteosarcoma
Tumors by Type
Tumors that form Cartilage
 Osteochondroma
 Osteochondromatosis (HMOCE)
 Periosteal Chondroma
 Juxtacortical Chondroma
 Enchondroma
 Enchondromatosis (Ollier's Disease)
 Mafucci Syndrome
 Chondroblastoma
 Chondromyxoid Fibroma
 Chondrosarcoma
 Dedifferentiated chondrosarcoma
 Clear Cell Chondrosarcoma
 Mesenchymal Chondrosarcoma
 Juxtacortical Chondrosarcoma
Tumors by Type
 That form Fibrous Tissue
 Nonossifying fibroma (fibrous cortical defect)
 Fibrous Dysplasia
 Osteofibrous Dysplasia
 Desmoplastic Fibroma
 Extragnathic Fibromyxoma
 Fibrosarcoma
 Benign Fibrous Histiocytoma

 Malignant fibrous hystiocytoma
Tumors by Type
Lesions of Marrow Elements
Eosinophilic Granuloma
Multiple myeloma
Solitary myeloma (plasmacytoma)
Leukemia
Non Hodgkin lymphoma
Hodgkin lymphoma of bone
Granulocytic Sarcoma in bone
Systemic mastocytosis
Sinus histiocytosis with Massive
   Lymphadenopathy
Tumors by Type
 Forming Vascular Tissue
 Skeletal lymphangiomatosis
 Hemangioma
 Skeletal Angiomatosis
 Massive Osteolysis
 Epithelioid hemangioendothelioma
 Epithelioid hemangioma
 Angiosarcoma - low grade
 Angiosarcoma - high grade
 Hemangiopericytoma
 Glomus tumor
Tumors by Type
      Miscellaneous
Chordoma
Conventional Chordoma
Chondroid chordoma
Dedifferentiated chordoma
Schwannoma of bone
Neurofibroma of bone
Liposarcoma of bone
Post - Paget's Sarcoma
Malignant Mesenchymoma of bone
Tumors by Type
    Metabolic Disease
Brown Tumor of Hyperparathyroidism
Bone lesions of Gaucher's Disease
Paget's disease
Disappearing bone disease
Tumors by Type
    Metastatic to Bone
Metastatic   Breast Cancer
Metastatic   Lung Cancer
Metastatic   Kidney Cancer
Metastatic   Prostate Cancer

Metastatic Thyroid Cancer
LOCATION/SITE      TYPICAL PATHOLOGY

                   Chondroblastoma, chondrosarcoma, giant
Epiphyseal
                   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
                   Mets, myeloma, eosinophilic granuloma, chondroma, Paget’s, haemangioma
body)

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

                   Myosotis, osteosarcoma, chondrosarcoma, chondroma
Periosteal

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

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Bone Tumors

  • 1. Bone Tumors in the Lower Extremity How to identify benign and malignant osseous tumors in the foot and ankle
  • 2. 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
  • 3. 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.
  • 4. 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.
  • 5. 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.
  • 6. 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
  • 7. 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
  • 8. 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
  • 9. 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
  • 10. 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
  • 11. 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
  • 12. 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
  • 13. 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
  • 14. 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
  • 15. 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
  • 16. 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
  • 17. 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
  • 18. 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
  • 19. 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
  • 20. 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
  • 21. 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
  • 22. 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
  • 23. Benign Tumors Unicameral Bone Cyst • Fibrous in nature • Radiographic signal is the quot;fallen fragment signquot; • 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
  • 24. 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
  • 25. 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
  • 26. 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
  • 27. 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
  • 28. 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
  • 29. Benign Tumors Eosinophillic Granuloma • Presentation: bone pain, local swelling, irritability • Bones 50 - 75% solitary/monostotic with skull/mandible (50%), with quot;punched-outquot; lucencies, quot;hole within a holequot;, quot;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
  • 30. 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
  • 31. 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
  • 32. 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
  • 33. 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
  • 34. 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
  • 35. 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 intramedullary • One may sub-classify these exceedingly aggressive neoplasms even further, according to their histologic appearance, as either osteoblastic, chondroblastic, fibroblastic or giant cell rich
  • 36. 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
  • 37. 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 quot;starburstquot; 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
  • 38. 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
  • 39. 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
  • 40. 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
  • 41. 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 Low-grade opposed to enchondromas, which are uniformly painless and more common in young adults
  • 42. 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
  • 43. 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 2º to Paget’s • 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
  • 44. 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
  • 45. 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
  • 46. 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
  • 47. 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
  • 48. Malignant Tumors of Bone Angiosarcomas and Epithelioid Hemangioendotheliomas • The trend is to use the term quot;hemangioendotheleomaquot; to denote those lesions that are low-grade (well differentiated) and quot;angiosarcomaquot; 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
  • 49. 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.
  • 50. 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
  • 51. 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
  • 52. 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
  • 53. 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
  • 54. Tumors by Type Tumors that form bone Osteoma Osteoid osteoma Osteoblastoma Osteosarcoma Conventional Intramedullary Osteosarcoma Extraosseous osteosarcoma Multifocal Osteosarcoma Telangectatic Osteosarcoma Small Cell Osteosarcoma Intraosseous Well-differentiated Osteosarcoma Intracortical Osteosarcoma Periosteal Osteosarcoma Parosteal Osteosarcoma High-Grade Surface Osteosarcoma
  • 55. Tumors by Type Tumors that form Cartilage Osteochondroma Osteochondromatosis (HMOCE) Periosteal Chondroma Juxtacortical Chondroma Enchondroma Enchondromatosis (Ollier's Disease) Mafucci Syndrome Chondroblastoma Chondromyxoid Fibroma Chondrosarcoma Dedifferentiated chondrosarcoma Clear Cell Chondrosarcoma Mesenchymal Chondrosarcoma Juxtacortical Chondrosarcoma
  • 56. Tumors by Type That form Fibrous Tissue Nonossifying fibroma (fibrous cortical defect) Fibrous Dysplasia Osteofibrous Dysplasia Desmoplastic Fibroma Extragnathic Fibromyxoma Fibrosarcoma Benign Fibrous Histiocytoma Malignant fibrous hystiocytoma
  • 57. Tumors by Type Lesions of Marrow Elements Eosinophilic Granuloma Multiple myeloma Solitary myeloma (plasmacytoma) Leukemia Non Hodgkin lymphoma Hodgkin lymphoma of bone Granulocytic Sarcoma in bone Systemic mastocytosis Sinus histiocytosis with Massive Lymphadenopathy
  • 58. Tumors by Type Forming Vascular Tissue Skeletal lymphangiomatosis Hemangioma Skeletal Angiomatosis Massive Osteolysis Epithelioid hemangioendothelioma Epithelioid hemangioma Angiosarcoma - low grade Angiosarcoma - high grade Hemangiopericytoma Glomus tumor
  • 59. Tumors by Type Miscellaneous Chordoma Conventional Chordoma Chondroid chordoma Dedifferentiated chordoma Schwannoma of bone Neurofibroma of bone Liposarcoma of bone Post - Paget's Sarcoma Malignant Mesenchymoma of bone
  • 60. Tumors by Type Metabolic Disease Brown Tumor of Hyperparathyroidism Bone lesions of Gaucher's Disease Paget's disease Disappearing bone disease
  • 61. Tumors by Type Metastatic to Bone Metastatic Breast Cancer Metastatic Lung Cancer Metastatic Kidney Cancer Metastatic Prostate Cancer Metastatic Thyroid Cancer
  • 62. LOCATION/SITE TYPICAL PATHOLOGY Chondroblastoma, chondrosarcoma, giant Epiphyseal 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 Mets, myeloma, eosinophilic granuloma, chondroma, Paget’s, haemangioma body) Spine (Posterior Aneurysmal bone cyst, osteoid osteoma, osteoblastoma elements) Myosotis, osteosarcoma, chondrosarcoma, chondroma Periosteal Mets, myeloma, haemangioma, fibrous dysplasia, osteochondromas, Multiple lesions enchondromas, histiocytosis X