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Transcript of Tumours of bone clas
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TUMOURS OF BONE
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Bone tumors are very diverse in morphology and biological potential
According to their biological behaviour, they are divided into
benign and malignant lesions
MOST bone tumors are benign lesions
Bone Tumors
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Classification of Bone Tumours
Benign tumor is a localized mass that grows by expansion and, given sufficient time, would cease growing
Benign tumor is a localized mass that grows by expansion and, given sufficient time, would cease growing
Malignant tumor is characterized by uncontrollable growthMalignant tumor is characterized by uncontrollable growth
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Benign neoplasms of bone
Benign neoplasms of bone :
Osteoma
Osteoid Osteoma
Osteoblastoma
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OSTEOMA
A solitary benign neoplasm characterized by a proliferation
of either compact or cancellous bone, usually in an
endosteal or periosteal location
•Essentially restricted to craniofacial skeleton
•Rarely in long bones
A solitary benign neoplasm characterized by a proliferation
of either compact or cancellous bone, usually in an
endosteal or periosteal location
•Essentially restricted to craniofacial skeleton
•Rarely in long bones
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Clinical features Site: Skull (frontal sinus), condyle M>F Presents as slow growing, painless bony mass Paranasal sinus: pain swelling, sinusitis nasal discharge Soft tissue osteoma osteoma mucosae / osteoma cutis Children rarely affected (Gardner’s syndrome)
Gardner’s syndrome:• Multiple osteomas of skull
and mandible• Multiple sebaceous cysts• Desmoid tumors• Skin fibromas• Colonic polyposis
Gardner’s syndrome:• Multiple osteomas of skull
and mandible• Multiple sebaceous cysts• Desmoid tumors• Skin fibromas• Colonic polyposis
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Radiographic findings
Round to ovoid (1-5 cm)
Sharply circumscribed, blastic/ sclerotic mass
Not associated with surrounding bone destruction
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Histology Well circumscribed, non encapsulated Composed of condensed masses of mature lamellar bone Scanty marrow/ prominent fibro fatty marrow Variable quantities of woven bone may be admixed Osteoblastic activity present In any given area- bone appears normal
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Course and treatment
Treatment: excision
Margins of >1 mm are unnecessary
Follow-up: not important
Finding of osteoma in jaw- suspect Gardner’s syndrome
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OSTEOID OSTEOMA
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•A distinctive, benign lesion characterized by a
< 1.5cm mass of abnormal bone (nidus)
•Nidus – richly innervated by nerve fibers,
thereby evoking considerable pain
•A distinctive, benign lesion characterized by a
< 1.5cm mass of abnormal bone (nidus)
•Nidus – richly innervated by nerve fibers,
thereby evoking considerable pain
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Clinical features
Incidence: 3% of primary bone tumors
Age: < 20 yrs
Sex: M:F= 3.1
Location:
<80% in long bones,
10% in spine
Rarely involve skull,
jaw bones, clavicle, sacrum
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Clinical features
Pain: -progressive type, associated with tenderness
-intense at night,
-relieved by aspirin
Swelling and redness: -superficially located lesions
-imitates osteomyelitis
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Radiological features:
Typical findings:
Thick, smooth, convex, homogenous periosteal density
The Nidus:
1. Round to ovoid contour
2. Small size: < 2cm in diameter
3. Blastic center: homogenously dense
4. Thin 1-2 mm peripheral radiolucent zone
Exceptions: radiolucent nidus: osteoid phase13
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Gross :
Early lesions are cherry red in color
More mature are yellow-white
Nidus may fall out of the bone like a pea
Consistency of nidus: soft and granular to
densly sclerotic
Reactive host bone sclerosis
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Histology
Nidus : variable quantitites of osteoid and woven bone
studded with osteoclasts and numerous dilated vessels
Some regions generally show osteoblastic rimming
Woven bone edges of nidus are sharply circumscibed
from surrounding, thickened or irritated host lamellar
trabecular bone
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Variable histologic findings
Increased cellular activity Osteoclasts Peripheral fibrovascular zone Surrounding bone sclerosis Chronic inflammation
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Special stains
Silver stains reveal non myelinated axons within the lesion
Correlates with high level of pain associated with it
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Disease course and treatment
Completely benign
Malignant transformation absent
Complete excision of nidus- curative
Removal of reactive bone not necessary
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OSTEOBLASTOMA
A rare benign tumour, typically larger 1.5cm,
characterized by osteoid and woven bone production
First delineated by Jaffe and Litchenstein in 1956
A rare benign tumour, typically larger 1.5cm,
characterized by osteoid and woven bone production
First delineated by Jaffe and Litchenstein in 1956
It was also called as giant osteoid osteoma
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Osteoblastoma
Incidence: Rare- 1 % of bone tumors
15%- facial bones(mandible)
36%- skull
30%- long bones
Age: < 30 yrs (median: 18yrs)
M:F= 2:1
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Signs and symptoms
Local bony expansion with mild, dull, persistent pain,
unresponsive to aspirin
Potential for progressive growth- swelling of jaw,
pathologic fractures and loosening of teeth
Temporal bone: facial paralysis, hearing loss,
Neurologic disorders secondary to spinal tumors
Alkaline phosphatase may be increased
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Radiographic features
Well defined, mixed radiolucent- radiopaque mass
Size: avg: 3.55cm
Commonly expands bone in
a fusiform manner often
Surrounded by thin rind of benign host bone sclerosis
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Gross
Reasonably well circumscribed
Tumour tissue is usually red , granular and friable
Some lesions are;
Whitish yellow, gritty and hard
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Histological features
Well circumscribed, nonencapsulated
Irregular trabeculae in loose fibrovascular stroma
Matrix is variably calcified
Bone shows prominent reversal lines
Trabeculae and broad osteoid seams- rimmed by plump
osteoblasts which tend to aggregate
Osteoblasts- small/large vesicular nuclei, abundant cytoplasm
Osteoclasts are usually present
Stroma is loosely fibrous with numerous dilated capillaries 24
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Histologic features Conventional osteoblastoma: Osteoid and woven bone production
– Thick osteoid seams cover the surfaces of the woven bone
Prominent osteoblastic rimming Reversal lines Loose intertrabecular stroma Richly vascular Sprinkling of osteoclasts
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Variants of osteoblastoma
Pseudomalignant ( bizzare) osteoblastoma Totally benign entity Bizzare nuclei Lacks mitotic activity Mimicks malignancy
Bizzare nuclear appearance
degenerative change
“pseudomalignant”
Bizzare nuclear appearance
degenerative change
“pseudomalignant”26
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Malignant osteoblastoma
Features of conventional osteoblastoma Blue spiculated bone Foci of sheets of plumper osteoblasts Greater nuclear pleomorphism Presence of mitotic activity along with atypical mitoses Age> 70 yrs Local recurrence Absence of metastases
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Course and treatment
Conventional form: Conservative surgical treatment – usually entire lesion is
removed by curretage Response to treatment is nearly always good May recur over a period of 1-10 years Radiation therapy contraindicated Osteoblastoma osteosarcoma
Aggressive forms: En block resection Follow – up is important
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Also referred as osteogenic sarcoma is the 3rd most common cancer in adolescence, occurring less frequently than lymphomas and brain tumors. It is
thought to arise from a primitive mesenchymal bone forming cell and is characterized by production of osteoid
Introduction to Osteosarcoma
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Etiopathogenesis
Environmental factors
Chromosomal abnormalities
Genomic alterations
Tumor suppressor gene dysfunction
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Etiopathogenesis
General risk factors:Rapid bone growth ( adolescent growth spurt)
In jaws, incidence seen 1 or 2 decades after adolescenceExcludes rapid bone growth as causative agent
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Environmental factors
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Syndromes associated
Reported in Rothmund-thompson syndromeLi-fraumeni syndromeHereditary retinoblastoma
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Genomic alterations
• Loss of heterozygosity of 10q21.1 -- most common osteosarcoma.
• Loss of chromosomes 9, 10, 13, and 17 • Gain Of chromosome 1.
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Tumor suppressor gene dysfunction
p53 gene:
Well-known tumour-suppressor genesThe p53 mutated in 22% of osteosarcomasPrototypical condition --Li-fraumeni syndrome -- High-grade conventionalRetinoblastoma (Rb) genes :critical for cell-cycle control Inherited mutation ->retinoblastoma syndrome > multiple malignancies including osteosarcomaLOH – poor prognosis
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Classification of osteosarcoma
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Based on etiopathogenesis
Osteosarcoma classified in to
2 groups
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Classification based on location - Fletcher
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Clinical features
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Osteosarcoma – clinical features
Extragnathic Affects most rapidly growing parts of
skeleton Metaphyseal growth plates of
Humerus, Femur and Tibia Males > females Bimodal 10-20yrs, > 50yrs
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• Comprises 6.5% • Mean age according to Garrington et.al – 34 – 36yrs• Men : female == 1. 1 : 1• Swelling rather than pain – common• Often succeed tooth extraction• Maxilla and mandible -equally involved.
Osteosarcomas of jaw
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Clinical presentation
Other complaints
Paresthesia
Displacement of teeth
Trismus
Hypoesthaesia or paresthaesia in the
case of the mandibular tumours
Lack of healing
swelling at the site of tooth exraction
Epistaxis
Eye problems,
Nasal obstruction
Weight loss
Other complaints
Paresthesia
Displacement of teeth
Trismus
Hypoesthaesia or paresthaesia in the
case of the mandibular tumours
Lack of healing
swelling at the site of tooth exraction
Epistaxis
Eye problems,
Nasal obstruction
Weight loss
• 90% high grade osteosarcoma -- soft tissue extensions
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Difference
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Gross
Large mass extending into the soft tissuesFish flesh appearanceBoth distal and proximal margins are well definedSoft , fleshy/ gritty - depending on osteoid production
Well circumscribed, lobulated, glistening grayish white
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Radiologic and Histologic features
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Radiologic appearance
Varied appearance, often mixture of radiolucency with opacityNakayama et al described four radiographic patterns•Osteolytic•Osteolytic dominant•Osteogenic dominant •Osteogenic.
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Hypothesis for types of ossificationTumor stage Radiologic features Histologic features
Early -Amorphous
ossification
Intermediate -Mixture of
amorphous and lamellar
ossification
Advanced - Lamellar
ossification
BRAZILIAN JOURNAL OF OTORHINOLARYNGOLOGY 71 (2) PART 1 MARCH/APRIL 2005
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Radiologic presentationsIn about 80% - one or more of following type seen
1.Sunray/ Sun burst 2.Codman’s Triangle3.Widening of PDL space
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Sunburst appearance
If invades periosteum, thin spicules of new bone formed
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Codman’s triangle
Destruction of cortex by tumor
Lifts up periosteum
Reactive new bone formation at junction
CODMANS
TRIANGLE
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Widening of PDL
Garrington et al1 mentioned that
•Radiological evidence of a symmetrically
widened periodontal ligament space is a
significant early finding in OSJ
•Absence of lamina dura
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Histologic variants
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Classification based on location - Fletcher
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Osteoblastic variant
Approx, 50% of all conventional type•Osteoid – fine , lace like between tumor cells•Amount of mineralisation – variable•Tumor osteoid surrounded by fibroblast like cells•Less commonly organized into trabeculae•Tumor cells – relatively uniform -- spindle shaped -- bizarre, hyperchromatic nucleus
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Chondroblastic variants
prevalence – 25%, most common variant in jaw•Cartilage - in form of lobules•Towards periphery –hypercellular, spindling•Center of lobule/ detween spindle cells – osteoid•Cells within lacunae – marked anaplasia
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Fibroblastic variant
• Least common, Approx. 25% • Pure spindle cell growth with only small foci of matrix• Arranged in herring bone pattern ( resembles fibrosarcoma) Plump cells with pink cytoplasm resembling epithelial cell• In approx. 1/4th O.S benign giant cells are scattered
throughout
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Small cell osteosarcomaSmall cell osteosarcoma Ewing sarcoma
osteoid/ bony matrix
Small foci of chondroid
Fibrinous deposition amongst tumor
Marked cellularity with little stroma
Tumor cells – uniformly bland and
undifferentiated, small hyperchromatic,
round to oval
Mitotic index - low
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Telangiectatic osteosarcoma
• Approx 4% of all O.S, most lethalCriteria used for diagnosis:1. Pure lytic, destructive lesion usually in metaphysis of a long bone of a young pt.1. Gross – cavity separated by septa or a cavity containing
blood clot2. Microscopically – septa separate spaces and simulates the
appearance of an aneurysmal bone cyst.
Lining cells are pleomorphic
Blood soaked sponge
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Pic of T.OS and Aneurysmal bone cystTelangiectatic variant Aneurysmal Bone Cyst
Lining cells -- AnaplasticLoosely arranged spindle cells
No tumor osteoid
Lack of atypia
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Histology of periosteal variant
• Malignant chondroid
• Anaplastic spindle cells
• Osteoid with or without
calcification
• Mitotic figures
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Features Paraosteal periosteal High grade surface
osteosarcoma
Clinical
features
Lobulated nodule attached
to cortex by short stalk
Lobulated nodule Least common, worst
prognosis
Radiograp
h
Radiodense and lobulated
mass attached by a broad
base to underlying bone
No periosteal elevation/
bone formation
Histologic
features
Fibroblastic differentiation
Well developed bony
trabeculae
Malignant chondroid , less
osteoid
Sheets of pleomorphic
spindle cells, osteoid
matrix, lacks cartilage
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Staging and grading of osteosarcoma
Universally accepted staging system not commonly used•Stage I – Low – grade lesion•Stage II – High- grade lesion•Stage III – Metastatic diseaseSub Stages:A.Intramedulary lesionB.Local extramedullary spreadSite of primary:I.Distal extremity – Best prognosisII.Distal Femur – Intermediate prognosisIII.Axial Skeleton – Worst prognosis
• Shafer, Hine, Levy. Shafer’s textbook of Oral Pathology 6th
edition
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Routine protocol
1. Initial chemotherapy of about 5 inductions2. Surgery (Resection of involved segment of bone)3. Additional 2 – 3 inductions postoperative chemotherapy
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Historical Background First described by James Ewing in 1921
Angervall and Enzinger in 1975 described the extra
skeletal form tumor
Termed as Ewing’s sarcoma of Soft tissue
Ewing’s Sarcoma/PNET
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Exact origin of cells questioned ???
Ewing believed cells were derived from marrow
endothelium
Tumor arose from undifferentiated cells of the
reticuloendothelial system
Neuroectodermal in origin and derived from cells
of the neural crest cells
HISTOGENESISES/PNET
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Cytogenetic abnormality i,e t(11;22) found in 80-90% of cases.
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AGE: Peak prevalence- 2nd or 3rd decade 80% affected <20yrs at diagnosis 50% tumor detected during 2nd decade
SEX: Slight male predilection (Neville) Whites mostly affected with blacks almost never developing
this tumor
Clinical featuresES/PNET
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SITE: Long bones, pelvis and ribs
Jaw involvement seen in 1 to 2 % cases (Gnathic or craniofacial bones)
Tumor of jaw present as swelling, pain, loose teeth & paresthesia.
Mandible affected twice as maxilla
ES/PNET
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Anywhere in the body Most common
– Deep soft tissues of extremities mostly upper thigh or buttock
– Upper arm
– Shoulder
– Para vertebral chest wall
Tumor attached to a major nerve shows signs and symptoms of diminished neurologic functions
Sites for Extraskeletal ES/PNET
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Rapidly growing, deeply located mass measuring 5-10 cm
Painful in one-third of cases
Progressive sensory or motor disturbances
Preoperative period is 1 year
Fever, leukocytosis and elevated ESR may be sometimes misleading
Clinical presentation ES/PNET
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Macroscopic findings: Multilobulated, soft and
friable
Rarely > 10 cms
Gray-yellow or gray-tan appearance
Areas of necrosis, cyst formation or hemorrhage
No calcification72
ES/PNET
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Marked cellularity with little stroma
Solidly packed, lobular pattern of uniform round cells
Cells have uniform, small, hyperchromatic, round or ovoid nucleus 10-15μm with distinct nuclear membrane
Cytoplasm ill-defined with scanty & pale staining Mitotic index low
Multinucleated giant cells are usually not present Intracytoplasmic glycogen highlighted by PAS stain
Filigree pattern
Histopathology
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ES
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MONOTONOUS PROLIFERATIONS OF ROUND CELLS
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COMPRESSED BLOOD VESSEL
ES
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CELLS WITH CLEARED OUT CYTOPLASM
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FILIGREE PATTERN
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CD 99 POSITIVITY
ES/PNET
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Form diffuse sheets, lobules, nests, or alveoli of small round
cells with hyperchromatic round to oval nuclei
Cytoplasm indistinct except in areas where cells are more mature
Elongated hair like cytoplasmic extensions coalese to form
rosettes (Homer-Wright rosette)
Focal lack of cohesiveness may mimic rhabdomyosarcoma (Peudoalveolar pattern)
Dense chromatin clumping and mitoses frequent
PNET
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Distinctive lobular architecture
PNET
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HOMER-WRIGHT ROSETTES
PNET
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Pseudo alveolar pattern
PNET
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EWING SARCOMA SHOWS: Densely packed uniform cells with round or oval nuclei Finely dispersed chromatin Narrow rim of cytoplasm & sparse organelles Rare cytoskeletal microfilaments & pools of glycogen
PNET: Some degree of neural differentiation in the form of dense-core
neurosecretory granules, neuritic cell processes, synaptic-like junctions, and microtubules
Ultrastructure
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ES/PNET
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ES/PNET currently treated by neoadjuvant chemotherapy with surgical resection followed by adjuvant chemotherapy
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Treatment
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References
Shafer, Hine, Levy. Shafer’s textbook of Oral Pathology 6th edition
Neville, Damm, Allen, Bouquot. Oral and maxillofacial pathology, 3rd edition
Joseph M Mirra ‘Bone tumours: Clinical, radiological and pathological correlation’ Volume 1
Marx RE and Stern D ‘Oral and maxillofacial pathology: A rational for diagnosis and treatment’
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K. Krishnan Unni and Carrie Y Inwards ‘ Dahlin’s Bone Tumours’ 6th edition
R A Cawson, E W Odell – Cawson’s essentials of oral pathology and oral medicine – 7th edition
R.A Cawson. Lucas Pathology of tumours of oral tissues 5th edition
Eversole R.Benign Fibro-Osseous Lesions of the Craniofacial Complex - A Review. Head and Neck Pathol.2008; 2 :177–202
References
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Thank you