Bone Pathology

1
Bone Pathology

• Advanced Oral Pathology

2
Osteogenesis Imperfecta

• A heterogeneous group of heritable disorders
  characterized by impairment of collagen
  maturation.
• Mutation of COL1A1 (chromosome 17) or COL1A2 (7).
• Most common type of heritable bone disease
  (1/8000).

3
Osteogenesis Imperfecta

• Main features are osteopenia, bone fragility,
  (fractures), blue sclera, altered teeth,
  hypoacusis (hearing loss) long bone and spine
  deformity and joint hyperextensibility.
• Oral findings include dentinogenesis imperfecta,
  maxillary hypoplasia with class III malocclusion
  and radiographic lesions similar to florid
  cemento-osseous dysplasia.

4
Osteogenesis Imperfecta

• Type I AD Most common and mildest form, 10
  congenital fracture 10 never fracture blue
  sclera hearing loss before 30
  hyperextensibility and tooth defects variable.
• Type II AR/AD Most severe form 90 stillborn
  or die before 4 weeks blue sclera opalescent
  teeth may be present.

5
Osteogenesis Imperfecta

• Type III AD/AR Moderate to severe bone
  fragility blue sclera fades over time
  hyperextensibility and hearing loss common 2/3
  die in childhood from cardiopulmonary failure
  secondary to kyphoscoliosis some have opalescent
  dentin.
• Type IV AD Mild to moderate bone fragility-50
  congenital fractures with frequency decreasing
  with time blue sclera fades over time
  opalescent dentin variable.

6
Osteogenesis Imperfecta
7
Osteogenesis Imperfecta
8
Osteopetrosis (Albers-Schönberg Disease)

• A group of rare hereditary skeletal defects of
  increased bone density resulting from defective
  remodeling due to failure in osteoclast function.
• Endochondral, endosteal and periosteal growth
  without concomitant resorption results in
  thickening of bone.
• Incidence estimated between 1 in 100,000-500,000.
  

9
Osteopetrosis

• Infantile (malignant form)
• AR marrow failure, fracture and cranial nerve
  compression (blindness, deafness, facial
  paralysis.
• Hepatosplenomegaly, infections and osteomyelitis.
• Delayed tooth eruption extraction osteomyelitis.

10
Osteopetrosis

• Adult (benign form)
• AD limited mostly to axial skeleton bone pain
  is common but nearly 40 are asymptomatic marrow
  failure rare.
• Variants with cranial nerve compression-without
  fracture or fracture without cranial nerve
  compression .
• Treatment of infantile with BMT for cure
  interferon, calcitriol, steroids, PTH, MCSF,
  erythropoietin.
• Supportive measures transfusion, antibiotics,
  surgery hyperbaric oxygen.

11
Bone marrow transplantation can correct
osteopetrosis

• Uncorrected Corrected

Geneva Foundation for Medical Education and
Research
12
Osteopetrosis
13
Osteopetrosis
14

• Our son Anton was born on July 15, 2004. He was
  born full-term, weighed 3,990 gm and was 52 cm
  long. During the first month of his life, Anton
  developed paleness and inflammation of lymph
  nodes, and was admitted to the Children's
  hospital No. 1 in St. Petersburg for examination.
  In two months of his stay at the hospital, Anton
  was on the operating table under the anesthesia
  three times. He had biopsies of liver, spleen and
  bone marrow. All of them were necessary for
  proper diagnostics. As a result of the tests, my
  child was issued a terrifying diagnosis,
  osteopetrosis, or "marble bone" disease. This is
  a very rare disease, and unfortunately, the only
  cure is bone marrow transplant. Since we have no
  matching donor in our family, we have to look for
  an unrelated donor at the international registry.
  The cost of the search and collection of bone
  marrow from the donor is 15,000 euros, and the
  delivery of it to St. Petersburg costs another
  2,500 euros. This is an enormous amount of money
  for us, and we will never be able to pay it on
  our own. At this time, there is still hope that
  after the transplant Anton will be able to live a
  normal life, because he can still see and hear.
  Unfortunately, this disease makes loss of vision
  and hearing unavoidable. Anton is now 18 months
  old. He is considered disabled and is under
  constant monitoring of a specialist in oncology
  and hematology. Our problems at this time are
  persistent anemia, high leukocytes and presence
  of blasts in Anton's blood, low immunity
  (repetitive colds) and enlarged liver and spleen.
  At his age, Anton only has 6 teeth and even they
  are starting to crumble. The main thing for us
  though is that he can still see and hear!,
  However, nobody knows how soon he will lose his
  sight and hearing. Please help us while it is
  still possible! Please give my child a chance to
  live happy life. I cannot imagine how terrible it
  must be to live without seeing or hearing
  anything. Close your eyes and cover your ears,
  imagine that it will last a lifetime, and you
  will be able to feel our despair.

15
Cleidocranial Dysplasia

• Defect in CBFA1 gene (6p21) AD with 40 sporadic
  cases defect in osteoblastic differentiation and
  bone formation.
• Clavicles show hypoplasia or malformation about
  10 one/both clavicles are absent.
• Short stature, large head with frontal parietal
  bossing, hypertelorism, broad nose, depressed
  nasal bridge.
• Persistent open fontanels and cranial sutures.

16
Cleidocranial Dysplasia

• Dental gnathic manifestations include narrow,
  high-arched palate w/o cleft, prolonged retention
  of deciduous teeth, delayed eruption of permanent
  teeth, numerous unerupted permanent and
  supernumerary teeth (lack secondary cementum).
  Narrow ramus, pointed coronoid processes, thin
  zygomatic arch, small/absent maxillary sinuses,
  acute gonial angle, prognathism.

17
Cleidocranial Dysplasia
18
Cleidocranial Dysplasia
19
Cleidocranial Dysplasia
20
Focal Osteoporotic Marrow Defect

• Non-pathologic area of hematopoietic marrow
  sufficient in size to appear radiographically as
  a radiolucency.
• Theories as to cause include aberrant bone
  regeneration following tooth extraction,
  persistent fetal marrow or marrow hypoplasia due
  to increased demand for erythrocytes.
• Asymptomatic ill-defined circumscribed lucency
  from a few to several cm in size.
• More than 75 of cases in adult females in an
  edentulous area posterior mandible biopsy.

21
Focal Osteoporotic Marrow Defect
22
Focal Osteoporotic Marrow Defect
23
Idiopathic Osteosclerosis

• Focal area of increased radiodensity of unknown
  cause.
• Also known as dense bone island, bone eburnation,
  bone whorl, bone scar, enostosis, focal
  (periapical) osteopetrosis.
• Peak age 20s no sex predilection.
• Well defined rounded or elliptic radiodense mass
  3-20 mm.
• Common (5) in mandible (90) first molar, 2nd
  premolar or 2nd molar most are periapical.

24
Idiopathic Osteosclerosis

• Need to rule-out condensing osteitis (pulp
  disease).
• Diagnosis based on history clinical and
  radiographic features pre-existing radiographs
  and periodic reassessment.
• Biopsy if expansion is present.
• Because lesion becomes stable and indolent, not
  treatment necessary.

25
Idiopathic Osteosclerosis
26
Massive Osteolysis

• Also known as Gorham disease, Gorham-Stout
  syndrome, vanishing bone disease and phantom bone
  disease.
• Rare, idiopathic spontaneous progressive
  destruction of one or more bones may represent
  hamangiomatosis of bone.
• Most often in children young adults 50 report
  antecedent trauma.
• Pelvis, humeral head or shaft axial skeleton
  most common sites.

27
Massive Osteolysis

• 30 maxillofacial involvement, usually the
  mandible.
• Signs include mobile teeth, pain, malocclusion,
  midline deviation, pathologic fracture and
  obstructive sleep apnea.
• Intramedullary lucency, loss of lamina dura,
  thinning of cortex.
• Spontaneous arrest radiation/surgery.

28
Massive Osteolysis
29
Pagets Disease of Bone

• Also known as osteitis deformans
• Idiopathic disorder of abnormal anarchic
  resorption and deposition of bone (abnormal
  remodeling).
• Relatively common after age 40 1/100-150
  (increasing to 1/10 by age 90) but most often
  asymptomatic innocuous MgtF white gt blacks or
  Asians.
• Usually polyostotic (multiple bone affected),
  rarely monostotic (single bone)

30
Pagets Disease of Bone

• Lumbar vertebrae, pelvis, skull femur jaw
  involvement in 17, usually the maxilla.
• Bowing of legs, leontiasis ossea, fracture, bone
  and joint pain, pressure neuropathy.
• May cause progressive alveolar ridge expansion,
  spacing of teeth and dental appliances that no
  longer fit the mouth.
• Altered trabecular pattern initially from
  decreased bone density evolves to patchy
  sclerotic areas having a cotton wool appearance.

31
Pagets Disease of Bone

• Alternating resorption and formation of bone
  produces a characteristic mosaicpattern and
  marrow replaced by highly vascular fibrous
  connective tissue.
• Elevation of serum alkaline phosphatase treated
  if elevated by more than 25-50 elevated urinary
  hydroxyproline levels.
• Increased risk of osteosarcoma (1) and giant
  cell tumors of bone.
• Treatment with calcitonin biophosphonate
  plicamycin for refractory cases no longer used

32
Pagets Disease of Bone
33
Pagets Disease of Bone
34
Pagets Disease of Bone
35
Pagets Disease of Bone
Under polarized light, the irregularities of the
bony lamellae are apparent in Paget's (mosaic
bone)
36
Central Giant Cell Granuloma (Lesion)

• A non-neoplastic lesion of the jaws most common
  before age 30 (60) femalesgtmales 70 in
  mandible, especially anterior two-thirds
• Most often asymptomatic or produce painless
  expansion occasionally pain, paresthesia or
  cortical perforation.
• Non-aggressive lesions are most common, produce
  few symptoms and are slow growing.
• Aggressive lesions produce pain, rapid growth,
  cortical perforation and root resorption.

37
Central Giant Cell Granuloma

• Radiographic lesions range from less than 5 mm to
  greater than 10 cm unilocular or multilocular
  differential diagnosis required.
• Histopathologically consist of multinucleated
  giant cells in a stroma of spindle-shaped
  mesenchymal cells usually with numerous small
  vessel and hemorrhageindistinguishable from
  hyperparathyroidism, cherubism and aneurysmal
  bone cyst.

38
Central Giant Cell Granuloma

• R/O other causes for giant cell lesions
• Treatment by thorough curettage recurrence rate
  15-20 resection for larger lesions.
• Investigational treatment of repeated intrabony
  lesional injection of triamcinolone or daily
  calcitonin injection (or nasal spray) for one
  year as alternatives to deforming surgery.

39
Central Giant Cell Granuloma
40
Central Giant Cell Granuloma
41
Giant Cell Tumor

• Considered a true neoplasm usually found in long
  bones.
• Although it can be histopathologically
  indistinguishable from central giant cell
  granuloma, the biologic behavior of GCT different
  from that of CGCG has a higher recurrence rate
  and 10 rate of metastasize.

42
Cherubism

• Rare development condition of the jaws, generally
  AD (4p16.3) with high penetrance but variable
  expressivity.
• Onset usually 2-5 progresses until puberty,
  becomes stable, then slowly regresses.
• Mandibular involvement of posterior areas results
  in bilateral symmetrical multilocular expansion
  maxillary involvement usually in tuberosity
  areas.
• Involved bone produces widening and distortion of
  alveolar ridges, tooth displacement, altered
  eruption, impaired mastication speech
  difficulties.

43
Cherubism

• Clavicle, ribs and humerus reported as rare
  extragnathic sites.
• Microscopically similar to CGCG eosinophilic
  cuff-like deposits around small vessels a helpful
  finding. Long-standing lesions become more
  fibrous with fewer giant cells.
• Prognosis variable return to normalcy for many
  some with significant deformity dental
  management sometimes difficult.

44
Cherubism
45
Cherubism
46
Cherubism
47
Simple Bone Cyst

• Also known as traumatic bone cyst, hemorrhagic
  bone cyst, solitary bone cyst idiopathic bone
  cavity.
• A benign, empty or fluid-filled cavity within
  bone devoid of epithelial lining.
• Trauma might play role in some cases but other
  theories are offered.
• Majority occur in long bones, ages 10-20
  mandible 60 of jaw lesions in males.

48
Simple Bone Cyst

• Usually asymptomatic 20 have swelling of jaw.
• Well-delineated radiolucency usually in
  premolar-molar region, often scalloped upward
  between the roots of teeth rarely multilocular.
• Pulp test teeth can be associated with
  cemento-osseous dysplasia.
• Diagnosis based on clinical, radiographic and
  surgical findings.
• Gnathic lesions treated by surgical exploration
  and follow-up.

49
Simple Bone Cyst
50
Aneurysmal Bone Cyst

• A non-neoplastic lesion of uncertain etiology
  that may mimic the growth and behavior of a
  neoplasm of bone. It is not a true cyst.
• Trauma, vascular malformation or an associated
  neoplasm that disrupts the normal hemodynamics of
  bone have been proposed as causes.
• An intraosseous accumulation of variable-sized
  blood-filled spaces surrounded by cellular
  fibrous CT reactive bone.

51
Aneurysmal Bone Cyst

• Most common in shaft of long bone age lt30
  gnathic lesions account for 2 with a mean age of
  20 years most often in posterior mandible.
• Rapid swelling, pain often reported.
• Unilocular or multilocular radiolucency with
  cortical expansion ballooning blow-out
  distension of bone contour sometimes radiopaque
  foci or small trabaculae seen.

52
Aneurysmal Bone Cyst

• At surgery lesion often describe as a
  blood-soaked sponge.
• 20 of jaw lesions associated with other pathosis
  (fibro-osseous lesion of CGCG).
• Curettage or enucleation recurrence variable
  (8-60) resection rarely needed.

53
Aneurysmal Bone Cyst
54
Aneurysmal Bone Cyst
55
Fibro-osseous Lesions of the Jaws

• Fibrous dysplasia
• Cemento-osseous dysplasia
• a. Focal cemento-osseous dysplasia
• b. Periapical cemento-osseous dysplasia
• c. Florid cemento-osseous dysplasia
• Ossifying fibroma

56
Fibrous Dysplasia

• Developmental condition of replacement of normal
  bone by an excessive proliferation of cellular
  fibrous connective tissue intermixed with
  irregular bone trabeculae.
• Sporadic condition attributed to mutation of GNAS
  1 gene (guanine nucleotide-binding, ?-stimulating
  activity peptide) clinical severity determined
  by time when the mutation occurs early embryonic
  mutation produces polyostotic disease with other
  abnormalities postnatal monostotic disease.

57
Fibrous Dysplasia

• Monostotic accounts for 80-85 of cases jaws are
  common location maxilla gt mandible maxillary
  lesions sometimes part of wider craniofacial
  fibrous dysplasia.
• Radiographic poorly demarcated ground-glass
  appearance of affected bone causing expansion in
  long bones often radiolucent, multilocular
  expansile.
• PDL may be narrow and lamina dura indistinct
  inferior alveolar nerve canal displaced
  superiorly.

58
Fibrous Dysplasia

• Polyostotic (PFD) involves two or more bones.
• Jaffe-Lichtenstein syndrome consists of PFD with
  café au lait pigmentation.
• McCune-Albright syndrome consists of PFD, café au
  lait pigmentation and multiple endocrinopathies
  such a sexual precocity, pituitary adenoma or
  hyperthyroidism.

59
Fibrous Dysplasia

• Histopathologically consists of trabeculae of
  woven bone in a cellular, fibrous stroma (Chinese
  characters).
• Excision or resection of small jaw lesions
  extensive or diffuse lesions may require repeated
  recontouring for cosmesis 25-50 regrowth after
  shave-down.
• Occasional development of osteosarcoma in bone
  affected by FD.

60
Fibrous Dysplasia
61
Fibrous Dysplasia
62
Fibrous Dysplasia
63
Fibrous Dysplasia
64
Cemento-Osseous Dysplasias

• Non-neoplastic fibro-osseous alteration of bone
  in the tooth bearing areas of the jaws.
• Three distinct radiographic/clinical patterns
  that share a common histopathologic appearance.
• Cellular connective tissue containing a mixture
  of woven bone, lamellar bone and cementum-like
  particles ratio of fibrous tissue decreases over
  time as it becomes sclerotic by fusion of
  calcified elements.

65
Cemento-Osseous Dysplasias
This high power photomicrograph demonstrates the
production of bone and/or cementum in a highly
cellular connective tissue stroma. It is not
always possible histologically to distinguish
between the production of bone and cementum.
However, cementum (C) is usually deposited within
the stroma in a droplet pattern.
66
Cemento-Osseous Dysplasias (Focal)

• Focal cemento-osseous dysplasia occurs most
  frequently in young to middle aged white females
  (91,FM).
• Solitary radiolucent to radiopaque lesion, less
  than 1.5 cm, if opaque with thin radiolucent rim
  most often in posterior mandible well-defined
  with slight irregular borders.
• Easily fragmented gritty tissue that can be
  curetted but does not separate easily from the
  surrounding bone.
• Monitor once diagnosis established by biopsy.

67
Focal cemento-osseous dysplasia
68
Cemento-Osseous Dysplasias (Periapical)

• Periapical cemento-osseous dysplasia occurs most
  frequently in black females (70) 30-50 y.o.
• FM, 141.
• Asymptomatic, anterior mandible, typically
  multifocal, periapical to vital teeth. Must pulp
  test teeth.
• Lucent gt opaque over time lt 1cm PDL intact,
  opacity not fused to tooth.
• No treatment necessary.

69
Periapical cemento-osseous dysplasia
70
Florid Cemento-Osseous Dysplasia
71
Cemento-Osseous Dysplasias (Florid)

• Florid cemento-osseous dysplasia occurs most
  frequently in middle aged to older black females
  (90).
• Multifocal involvement but not limited to the
  anterior mandible often bilaterally symmetric.
• Most often asymptomatic pain, swelling or signs
  of osteomyelitis.
• Lucent/opaque sometimes associated with simple
  bone cyst.
• No treatment for asymptomatic lesions
  exploration of simple bone cysts avoid surgical
  exposure of affected sclerotic areas symptomatic
  areas are treated the same as for osteomyelitis.

72
Familial Gigantiform Cementoma

• Uncommon hereditary AD disorder of gnathic bone
  in which massive sclerotic masses of disorganized
  mineralized tissue develop.
• Begin as multiple periapical lucencies which
  expand to produce a mixed radiolucent-radiopaque
  pattern.
• Anemia and multiple polypoid adenomas of the
  uterus reported in some affected females.
• Extensive resection reconstruction may be
  needed to achieve acceptable cosmesis.

73
Ossifying Fibroma

• A true neoplasm of an admixture of fibrous tissue
  bony trabeculae, cementum-like spherules, or
  both.
• Most frequent in third-fourth decade female
  predilection.
• Premolar-molar region of mandible most frequent
  site.
• Small lesions asymptomatic larger lesions
  produce painless swelling.
• Well defined radiolucency, often with a sclerotic
  border, containing varying amounts of opacity.
  Larger lesions produce downward bow of the
  inferior cortex of mandible.
• Enucleation resection for large lesions.

74
Ossifying Fibroma
75
Ossifying Fibroma
76
Ossifying Fibroma
77
Juvenile Ossifying Fibroma

• Differs from common OF by age (younger), location
  (maxilla) biologic behavior (sometime
  aggressive and/or recurrent).
• Two microscopic types trabecular psammomatoid
  age at diagnosis 11 and 22 respectively.
• Maxilla often with impingement on sinuses, orbit,
  nose and cranium.

78
Juvenile Ossifying Fibroma

• Richly cellular fibrous tissue showing strand of
  osteoid or psammomatoid ossicles.
• Growth of individual tumors varies from slow to
  rapid tends to be rapid in younger patients.
• Complete curettage for small lesions resection
  for larger, aggressive lesions recurrence 30-58.

79
Osteoma

• Benign tumors (neoplasm?) of mature compact or
  cancellous bone that are restricted to the
  craniofacial skeleton.
• May be periosteal or endoseal perioseal lesions
  produce slowing growing mass endosteal lesions
  asymptomatic or slow enlargement.
• Condylar lesion produces lobulated mass
  deviation of midline.
• Radiographically a circumscribed sclerotic mass
• Symptomatic or deforming lesions removed by
  conservative excision.

80
Gardner Syndrome

• Rare inherited AD disorder (gene on chromosome 5)
• Within the spectrum of familiar colorectal
  polyposis
• 1 in 8,300-16,000
• Osteomas of any bone, skull, paranasal sinuses
  and mandible common.
• Supernumerary teeth in 20
• Epidermoid cysts of skin, desmoid tumor of skin,
  adenomatous colonic polyps gt colon cancer,
  pigmented lesions of ocular fundus, 100-fold
  increase in thyroid cancer in females.
• Colonectomy elective prophylactic thyroidectomy,
  elective removal of osteomas cysts and other
  tumors.

81
Osteoblastoma Osteoid Osteoma

• Rare neoplasms of bone (lt1) are microscopically
  identical osteoblastomas most frequent in
  vertebral column, sacrum, calvarium, long bones
  small bones of hands/feet osteoid osteoma most
  often femur, tibia phalanges.
• Osteoid osteoma produce prostaglandin gt pain
  relieved by aspirin not osteoblastoma.
• In jaws, slight mandible predilection in
  posterior area 85 lt age 30.
• Painful radiolucent/opaque lesion often 2-4 cm.
• Local excision or curettage 50 chance for
  recurrence of aggressive lesions.

82
Osteoblastoma Osteoid Osteoma
83
Osteoblastoma Osteoid Osteoma
84
Cementoblastoma

• Rare neoplasm thought by some to be identical
  with osteoblastoma differing only by its
  attachment to the root of a tooth. lt1 of
  odontogenic tumor.
• 75 in mandible 90 molar or premolar with 50
  involving first molar.
• Children and young adults 50 ltage 20 75 lt
  age 30.
• Pain reported by 2/3
• Opaque mass fused to root surrounded by thin
  radiolucent rim.
• Extraction/removal of mass removal of mass, root
  amputation endodontics.

85
Osteosarcoma

• Most common primary malignancy of bone
  (non-hematopoietic).
• Most are intramedullary small number are
  peripheral (juxtacortical), periosteal or
  parosteal.
• Most often between age 10-20 and located in
  distal femur or proximal tibia secondary group gt
  age 50 associated with Pagets disease located in
  axial skeleton.

86
Osteosarcoma

• Osteosarcoma of jaws accounts for 6-8 of all
  osteosarcomas mean age of 33
• Maxilla mandible.
• Swelling and pain, loosening of teeth,
  paresthesia and nasal obstruction most common in
  jaw lesions.
• Osteoblastic, chondroblastic and fibroblastic are
  the most common microscopic subtypes.

87
Osteosarcoma

• Radiographic lesions of jaws vary from lucent to
  opaque, often ill-defined spiking or roots,
  widening of PDL (common) osteophytic reaction
  (sun-ray) is seen in 25.
• Prognosis linked to the initial attempt at
  complete removal.
• Metastasis to regional nodes, lung and brain gt
  for long bones than jaws.

88
Osteosarcoma

• For extragnathic sites, pre-op (neoadjuvant)
  chemotherapy followed by radical excision and
  post-op (adjuvant) chemotherapy has increased
  survival to more than 4 years for 80.
• Data for jaws sparse estimated 30-50 survival.
  Better outcomes for juxtacortical tumors compared
  to intramedullary lesions.
• 0.2 risk of post-radiation sarcoma for dose or
  7000 cGy.

89
Osteosarcoma
90
Osteosarcoma
91
Osteosarcoma
92
CAT scan osteosarcoma
93
Weber-Ferguson procedure for osteosarcoma of the
maxilla
94
(No Transcript)
95
Chondrosarcoma

• Malignant neoplasm of bone (10) characterized by
  the formation of cartilage.
• Jaw lesions are rare accounting for 1-3 of all
  chondrosarcomas.
• Extragnathic lesions occur predominately in those
  gt age 50 ileum, femur humerus most common.
• Jaw lesions most often in maxilla reported over
  a wide age range, average age of 41.6 and 20 lt
  age 20.

96
Chondrosarcoma

• Most often produces a painless mass may be
  associated with loosening of teeth, epistaxis,
  nasal obstruction and visual disturbance.
• Lesions grades from I-IV influence on outcome
  most jaw lesion grade I or II.
• Size, location and grade influence outcome
  average 5 year survival of 67.5 with late
  recurrence metastasis reducing long-term
  survival to less than 44.

97
Chondrosarcoma
98
Ewings Sarcoma

• Primary malignancy of bone of uncertain
  histogenesis with recent evidence of
  neuroendocrine origin.
• 85-90 show reciprocal translocation t(1122)
  (q24q12).
• 6-8 of primary malignancies of bone long bones,
  pelvis and ribs jaw or craniofacial lesions are
  very uncommon (1-2).
• Peak incidence in second decade (50) 80 less
  than age 20 male slightly gt females.

99
Ewings Sarcoma

• Rare in blacks.
• Pain, paresthesia and swelling with loosening of
  teeth.
• Ill-defined lytic lesion onion-skin periosteal
  reaction of long bones rare in jaws.
• Small round cells PAS positive diastase labile
  angiotropism and necrosis MIC2 glycoprotein
  detected by immunoperoxidase (CD99).
• Combined surgery, multidrug chemo radiotherapy
  5-year survival 40-80 pelvis worst.

100
Ewings Sarcoma
101
Ewings Sarcoma
Angiotropism--small round cells, vital on left
adjacent to blood vessel and necrotic on right
Onion-skin radiographic change
102
Metastatic Tumors to the Jaws

• The jaws are regarded as an uncommon site for
  metastasis although a study of autopsies revealed
  10/62 (16) carcinomas involved the mandible
  microscopically though negative radiographically.
• 80 of reported case are in mandible.
• Symptoms include pain, loosening of tooth, a mass
  or paresthesia (numb-chin syndrome).
• Lucent lesion, well or ill defined (moth-eaten).

103
Metastatic Tumors to the Jaws

• Involvement of alveolus may mimic periodontal or
  periapical disease occasional widened
  periodontal ligament space noted.
• Metastatic carcinomas (especially breast
  prostate) may stimulate new bone formation with a
  resultant radiopaque or mixed lesion.
• Most common sites of primary are breast, lung,
  thyroid, prostate kidney.
• Stage IV primary (not oral cancer) with a usually
  survival of less than one year.

104
Metastatic Tumors to the Jaws
105
Metastatic Tumors to the Jaws
?
?
?