Benign Lesions of Bone

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Benign Lesions of Bone
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Osteoma

• Benign osseous tumor, usually affecting
  membranous bone
• Painless, slowly enlarging
• Paranasal sinus involvement is common
• Jaw lesions are usually associated with the
  condylar area

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Gardner Syndrome

• Autosomal dominant trait uncommon
• One of the multiple intestinal polyposis
  syndromes
• Characterized by osteomas of the facial bones
• Epidermoid cysts and desmoid tumors

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Gardner Syndrome

• Radiographically, the osteomas of Gardner
  syndrome could resemble florid cemento-osseous
  dysplasia or osteitis deformans
• May see impacted supernumerary teeth or, less
  commonly, odontomas

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Gardner Syndrome

• The most significant aspect of the syndrome is
  the development of precancerous polyps of the
  colon
• 50 of patients develop adenocarcinoma of the
  colon by 30 years of age

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Gardner Syndrome

• Management includes
• Prophylactic colectomy
• Removal of cosmetically problematic cysts and
  osteomas
• Genetic counseling

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Central Giant Cell Granuloma

• Benign, somewhat controversial, lesion of the
  jaws
• Some pathologists feel this is the same lesion as
  the giant cell tumor of bone, which typically
  arises in long bones, particularly in the knee
  area

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Central Giant Cell Granuloma

• 2nd to 4th decade of life
• 21 female predilection
• Usually affects the mandible, often crossing the
  midline
• Unilocular when small larger lesions often
  multilocular and expansile

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Central Giant Cell Granuloma

• Histopathologically, granulation tissue with
  numerous benign multinucleated giant cells

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Central Giant Cell Granuloma

• Treatment generally consists of aggressive
  curettage
• Recurrence rate of 15-20
• Prognosis is generally considered to be good

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Langerhans Cell Histiocytosis

• Considered to be a group of related disorders
  caused by proliferation of Langerhans cells
• Langerhans cells are related to monocytes and
  serve as antigen-presenting cells
• Evidence that these are monoclonal
  proliferations, consistent with neoplasia

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Langerhans Cell Histiocytosis

• At least three clinical presentations
• Acute disseminated histiocytosis
• Chronic disseminated histiocytosis
• Eosinophilic granuloma, which may be monostotic
  or polyostotic

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Langerhans Cell Histiocytosis

• Acute disseminated histiocytosis (also known as
  Letterer-Siwe disease) is seen in infants
• Skin rash, splenic and hepatic involvement
• Usually a very aggressive, malignant course

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Langerhans Cell Histiocytosis

• Chronic disseminated histiocytosis (also known as
  Hand-Schuller-Christian disease) is seen in older
  children
• Classic triad of exophthalmos, diabetes
  insipidus, and bone lesions (well-defined
  radiolucencies) is actually rather uncommon

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Langerhans Cell Histiocytosis

• Eosinophilic granuloma of bone affects teenagers
  and adults
• May be polyostotic (teenagers) or monostotic
  (older adults)
• Well-defined, but non-corticated radiolucency

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Histopathologic Features

• Sheets of large, histiocytic-appearing cells
  (neoplastic Langerhans cells)
• Variable numbers of eosinophils (eosinophilic
  granuloma)

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Management and Prognosis

• Acute chemotherapy poor prognosis
• Chronic radiation and/or chemotherapy guarded
  prognosis
• Eosinophilic granuloma curettage or radiation
  good prognosis

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Central Ossifying Fibroma

• Uncommon benign neoplasm that probably arises
  from periodontal ligament fibroblasts
• Probably confused with focal cemento-osseous
  dysplasia in the past
• Mandibular premolar/molar region adult female,
  3rd to 4th decade

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Central Ossifying Fibroma

• Swelling may be present if the lesion is large
• Well-circumscribed radiolucency with variable
  amount of central opacity
• Range from almost purely radiolucency to very
  radiodense with a defined lucent border

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Central Ossifying Fibroma

• Cellular fibrous connective tissue containing
  mineralized trabeculae and spherules of material
  that may resemble cellular cementum or woven bone
• Microscopically similar to fibrous dysplasia, so
  radiographs are often necessary

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Central Ossifying Fibroma

• Treatment consists of enucleation lesion tends
  to shell out as one mass
• Prognosis is considered to be excellent, and
  recurrence is very uncommon

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Malignancies Affecting Bone
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Chondrosarcoma

• Rare malignancy of cartilaginous differentiation
• Most are seen in adult males (4th to 6th decade)
  and affect femur, pelvis or ribs
• 10 occur in the head and neck region
• May present with pain, swelling or loose teeth

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Chondrosarcoma

• Poorly defined radiolucency with variable amounts
  of radiopacity
• Larger lesions may appear multilocular
• May see widened PDL of the teeth in the area of
  the tumor, similar to osteosarcoma

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Chondrosarcoma

• Histopathologically, this tumor is characterized
  by invasive lobules of atypical cells showing
  cartilaginous differentiation

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Chondrosarcoma

• Treatment is radical surgery
• Prognosis depends on the location and
  histopathologic grade of the tumor, but generally
  is considered poor
• Death is usually due to direct extension of
  tumor, involving vital structures

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Osteosarcoma

• Malignancy showing production of osteoid by tumor
  cells
• Most common primary bone malignancy twice as
  common as chondrosarcoma
• Only 900 new cases in the US annually only 7 of
  these affect the jaws

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Osteosarcoma

• Osteosarcoma of the jaws has a different
  presentation epidemiologically compared to
  osteosarcoma of long bones
• Osteosarcoma of long bones develops around the
  knee area mean age 18 years
• Patients with osteosarcoma of the jaws have a
  mean age of 28 years

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Osteosarcoma

• Initial complaint is often pain, followed by
  swelling, loose teeth or paresthesia
• Mixed radiopaque/radiolucent lesion with
  ill-defined borders
• Symmetrically widened PDL of teeth in the area
  may be seen
• Sun-burst pattern is uncommon in jaws

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Osteosarcoma

• Infiltrating sheets of malignant spindle-shaped
  or angular lesional cells
• Direct production of osteoid or bone
• Mitotic activity can vary from field to field
• Osteoblastic, chondroblastic and fibroblastic
  differentiation may be seen

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Osteosarcoma

• Treatment is radical surgery
• Historically, 5-year survival has been in the
  30-50 range
• One study, using radical surgery, had an 80
  5-year survival
• Death is usually due to uncontrolled local disease

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Plasmacytoma/Multiple Myeloma

• Neoplastic proliferation of plasma cells
• Solitary plasmacytoma
• Multiple myeloma
• Extramedullary plasmacytoma

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Plasmacytoma/Multiple Myeloma

• Comprises 47 of malignancies involving bone,
  excluding metastases
• Over 14,000 cases annually in the US
• Mean age of 70 years rare lt 40 years
• 21 male predilection

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Plasmacytoma/Multiple Myeloma

• Initially present with bone pain
• Myelophthisic anemia may cause fatigue,
  susceptibility to infection, fever, petechial
  hemorrhage
• Renal failure due to circulating monoclonal
  immunoglobulin

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Plasmacytoma/Multiple Myeloma

• Circulating monoclonal immunoglobulin may spill
  over into the urine (Bence Jones protein)
• May be deposited in tissues as eosinophilic
  acellular material amyloid

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Plasmacytoma/Multiple Myeloma

• Monotonous sheets of atypical plasma cells
• Varying stages of differentiation
• Immunohistochemical studies show a monoclonal
  light chain restriction (kappa or lambda) of the
  lesional cell population
• Similar finding of monoclonal gammopathy on serum
  protein immunoelectrophoresis

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Plasmacytoma/Multiple Myeloma

• Treatment chemotherapy (prednisone plus an
  alkylating agent) thalidomide bone marrow
  transplant
• Prognosis poor
• Median survival of 30-36 months after onset of
  symptoms

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Metastatic Disease

• Most common malignant process to involve bone
• Jaws are occasionally affected
• Metastatic deposits from malignancies below the
  neck may affect the jaws through Batsons
  paravertebral plexus of veins no valves

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Metastatic Disease

• Over half of affected patients are over 50 years
  of age
• Mandible 61 of cases
• Maxilla 24 of cases
• Soft tissue 15 of cases

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Metastatic Disease

• Paresthesia, tooth mobility, swelling,
  hemorrhage, pathologic fracture, trismus
• With lack of healing of a tooth socket,
  clinically consider granulation tissue,
  lymphoma, metastatic disease
• Poorly defined radiolucency less commonly,
  radiopacity

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Metastatic Disease

• Histopathologically, these lesions have the
  pattern of the primary malignancy from which they
  arose
• Scattered clusters of lesional cells give a
  seeded effect
• Breast, lung, colon, thyroid, prostate, kidney,
  melanoma are among the more common primary sites

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Metastatic Disease

• Treatment palliation, usually with radiation
  therapy
• Prognosis very poor most patients die within
  one year of the diagnosis
• 22 of jaw metastases represent the initial
  manifestation of the malignant process