Brainscape's Knowledge GenomeTM

Browse over 1 million classes created by top students, professors, publishers, and experts.


See full index

OMS Finals > 6 - Pathology > Flashcards

6 - Pathology Flashcards

1
Q

OKC
Epidemiology
Etiology
Presentation

A

Epidemiology

  • Can occur any time in life
  • Peak in the 2nd and 3rd decade of life
  • M>F (Slightly more)

Etiology

  • Originates from the cell rests of the dental lamina with the basal layer of epithelium playing a role

Presentation

  • 25 -40% associated with unerupted tooth (Neville & Damm)
  • Resorption of roots is uncommon
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
2
Q

OKC

Proponents for reclassification as a tumor

A

Greater expression for:

  • Ki 67
  • PCNA (Proliferating cell nuclear antigen)

Loss of tumor supressor genes

  • p16
  • p53
  • MCC
  • LATS2
  • FHIT

85% of OKC associated with NBCCS

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
3
Q

OKC

FNA
Histopathology

A

FNA

  • Immunochemical staing for cytokeratin 10
  • Presence of keratin flakes
  • Protein levels of <4g/100mL

Histopathology

  • 6 - 8 cells thick
  • Stratified squamous epithelium
  • Luminal surface show parakeratotic epithelial cells
  • Basal layer
    • Hyperchromatic
    • Palisading cuboidal or columnar cells
  • Lumin contents
    • Macroscopic “cheesy” debri
    • Microscopic - Keratinaceous debri

***If the cyst is inflammed, parakeratinisation and pallisading of the basal layer are lost. Rete ridges develop. It becomed difficult to diagnose an OKC***

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
4
Q

Histology - Ortho and Para keratinization

A
  • Orthokeratinization
    • As the tissue matures, it forms keratin within its superficial cells
  • Parakeratinization
    • The most superficial layer contain not only keratin but cell nuclei as well
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
5
Q

OKC Recurrence Rates

A

Reasons for recurrence

  • Incomplete removal of original cyst lining
  • Growth of new OKC from satellite cyst or epithelial lining
  • Unrelated OKC

Johnson and Batstone 2013

  • Enucleation - 25%
  • Enucleation with adjunctive measures (Not Carnoy’s) - 20%
  • Enucleation with Carnoy’s - 8% (5% in conclusion)
  • Marsupialisation with adjunctive measures - 15%
  • Resection - 6% (1.8% in conclusion)

Al-Moraissi 2017

  • Marsupialisation - 30%
  • Enucleation - 25%
  • Marsupialisation with enucleation - 15%
  • Enucleation with peripheral ostectomy/cryotherapy - 15%
  • Enucleation with Carnoy’s - 10%
  • Resection - 2.5%
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
6
Q

Carnoys solution

Ingredients

Mechanism of action

Application technique

Bone penetration

Efficacy without chloroform

A

Ingredients - FACE (0.1, 1, 3, 6)

  • Ferric chloride 0.1g/mL
    • Hemostatic & Coagulant agent
  • Acetic acid (Glacial) - 1mL (10%)
    • Breaks cross-links between proteins
    • Coagulation of nucleic acid
  • Chloroform - 3mL (30%)
    • Carcinogenic - 1992
    • Enhances ethanol’s penetration into tissues
  • Ethanol - 6mL (60%)
    • Denatures proteins
    • Coagulative fixative

Voorsmit 1981

  • Penetrates 1.54mm into bone when applied for 5 min

Frerich 1994

  • Rabbit study - Carnoys applied to IAN for various duration
  • 3mins - Did not result in somatosensory evoked potentials in one, slight reduction in another and partial sensory impairment in another
  • At 5 and 10 min - Absent evoked potentials

Dashow 2015

  • Carnoy’s - 10% Recurrence rate
  • Modified Carnoy’s - 35% Recurrence rate
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
7
Q

Giant Cell Granuloma

Definition Epidemiology Pathophysiology

A
  • Central giant cell granuloma represents a benign proliferation of fibroblasts and multinucleate giant cells
  • F > M
  • 10 -20 yr olds (75% occur before the age of 30 yrs old)
  • 70% mandible
    • Usually anterior mandible
  • Aetiology - not known
    • Inflammatory
    • Reactive lesion
    • Neoplasm
    • Endocrine lesion
  • The proliferating cell in this lesion is the fibroblast which is believed to produce cytokines leading to recruitment of monocytes which subsequently transform into multinucleate giant cells (osteoclast origin)
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
8
Q

Giant Cell Granuloma

Classification

A

Chuong Kaban (2002)

  • Classifies the lesion into Aggressive vs Non-aggressive
    Aggressive : 1 Major and 3 Minor criteria
  • Major Criteria
    • Size >5 cm
    • Recurrence
  • Minor (DR CC GP)
    • Root resorption
    • Tooth displacement
    • Cortical bone perforation
    • Cortical thinning
    • Rapid growth
    • Pain/paresthesia
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
9
Q

Giant Cell Granuloma

Workup & Differentials

A

Workup

  • History
    • Cherubism, Noonan, Ramon
  • Exam
    • Aggressive vs Non-aggressive
  • Bloods
    • PTH, Ca, PO4, Alk Phos
  • Img
    • OPG, CT
    • PET - Baseline activity and to monitor for resolution of lesion
  • Bx
    • Multinucleate giant cells within a stroma of blood vessels and fibroblasts
    • Perivascular cuffing of giant cells
    • Extravasated RBC
    • Foci of bone and osteoid formation

Differentials (Giant cell lesions)

  • Infective
    • Mycobacteria - Tb, Leprosy
    • Syphilis
  • Trauma
    • Foreign body reaction - Sutures, Alveogyl, Bone wax
    • Chronic trauma
    • TUGSE
  • Autoimmune ulcers
    • Oro facial granulomatosis
    • Crohn’s Disease
    • Sarcoidosis
    • Amyloidosis
    • Wegners Granulomatosis
  • Metabolic
    • Brown Tumour
    • Paget’s Disease
    • Fibrous dysplasia
  • Pathology
    • Cherubism
    • Ramon syndrome
    • Neurofibromatosis I
    • Aneurysmal bone cyst
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
10
Q

Giant Cell Lesions Management

A

Giant Cell Granuloma

  • Medical
    • Calcitonin (Shreuder 2017)
      Non recommended by FDA due to increased cancer risk
      • 100-200U/day for 18 months (primary treatment)
        100-200U/day for 3 months (adjuvant therapy)
      • 9.1% Recurrence in treatment group
        54% Recurrence in placebo group
    • Steroids (Terry and Jacoway 1998)
      • Triamcinolone 10mg/mL
        • 1mL for every cm of lesion
        • 1x/week for 6 weeks
      • Resolution in 50% of cases
    • Interferon alpha (Kaban 1999)
      • Based on hypothesis GCT is the bone equivalent of infantile haemangioma
      • S/cut injections until osseous cavity filled with bone
    • Denosumab
      • 120mg S/cut weekly for 2 weeks then monthly for 6 months
        Not approved for use in growing skeleton
    • Bisphosphonates
  • Surgical
    • Enucleation and currettage
      • Non aggressive
    • Segmental/Marginal resection
      • Aggressive
        ​

Brown Tumor

  • Medical
    • Restriction of dietary phosphate
    • Phosphate binding agents
    • Vit D
    • Cinacalcet - Calci-mimetic agent that sensitises calcium receptors of PTH cells and therefore reduces PTH level
  • Surgical
    • Parathyroidectomy
    • Renal transplant to restore normal processing of Vit D
    • Soft tissue lesions can be treated with local excision

Cherubism

  • Conservative monitoring
    • Self limiting
  • Surgical
    • Guide eruption of teeth
    • Cosmetic recontouring at cessation of growth and after disease has ceased

Aneurysmal bone cyst

  • Surgical
    • Curettage but very vascular
    • Pre-op embolisation can be considered
    • Defect heals within 6-12 mths without grafting
    • Recurrence 10-20% Usually due to complete removal
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
11
Q

Syndromes associated with Pathology

A
  • OKC/BCC
    • Gorlin-Goltz
  • Fibrous dysplasia
    • McCune Albright
    • Jaffe Lichtenstein
    • Mazabraud
  • CGCG Multiple
    • Noonan
    • Browns/HyperPTH
    • Ramon/Cherubism
    • NF1
    • Fragile X
    • Jaffe Campanacci
  • Osteoma/Epidermoid cysts
    • Gardner
  • Ulcers
    • Behcets
  • Oral fibroma (Multiple)
    • Cowden’s syndrome
  • Neurofibroma
    ​Schwannoma
    • Neurofibromatosis
  • Neuroma
    • MEN 2B
  • Erythema multiforme
    • Steven-Johnson Syndrome
  • Infantile hemangioma
    • PHACES
  • Capillary malformations
    • Sturge Weber
    • HHT
    • Peutz Jagher
    • Klippel Trenaunay
    • Proteus
  • AVM
    • Sturge Weber
  • Cafe au Lait
    • McCune Albright
    • NF 1
  • Melanin lesions
    ​Macules/Naevi
    • Peutz Jagher
    • Addisons
  • Sarcoidosis
    • Lofgren
    • Heerfordt
  • Sialadenitis
    ​Mucocoeles in children
    • HIV
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
12
Q

Vascular Anomaly Classification

A

2018 - International society for study of Vascular Anomalies

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
13
Q

Vascular Tumors

Benign

A

ISSVA Classification

  • Tumors and Malformations
  • Tumors
    • Benign
      • Infantile Hemangioma
      • Congenital Hemangioma
        • RICH
        • NICH
      • Pyogenic Granuloma
        Lobular capillary hemangioma
    • Locally aggressive
      • Kaposi sarcoma
      • Hemangioendothelioma
    • Malignant
      • Angiosarcoma

Infantile Hemangioma

  • Epideiology
    • 4-10% (Fonseca)
      More common in Caucasians
    • 60% found in the H&N region
  • Clinical course
    • IH appears during first month
    • Growth peaks at 5 - 6 month but continues until 12 months
    • Regresses at 10% per year
  • Histopathology
    • Proliferation
      • Rapidly dividing endothelial cells that form tightly packed sinusoidal channels
    • Involuting
      • Decreased proliferation
      • Increased apoptosis
      • Increase in stromal cells
    • Involuted
      • Few tiny capillary feeding vessels
      • Islands of fibrofatty tissue admixed with dense collagen and reticular fibres
  • Associated anomalies
    • PHACE
      • Posterior fossa anomalies
      • Hemangiomas
      • Arterial abnomalities
      • Cardiac defects and Coarctation
      • Eye abnormalities
      • Sternal non-union

Congenital Hemangioma

  • Two subtypes - RICH and NICH
  • RICH
    • Presentation
      • Red violaceous colour
      • Central telangiectasia
      • Peripheral pale halo
      • Transient thrombocytopenia
        Consumptive coagulopathy
    • Natural history
      • Proliferates and involutes rapidly during the first few weeks and months post partum
  • NICH
    • Presentation
      • Ovoid, macular and raised
      • Light gray with prominent coarse telangiectasia
      • Well circumscribed lesion
    • Natural history
      • Expands during adolescence

Pyogenic granuloma
Lobular capillary hemangioma

  • fsdfs
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
14
Q

Vascular Tumor

Locally aggressive & Malignant

A

ISSVA Classification

  • Tumors and Malformations
  • Tumors
    • Benign
      • Infantile Hemangioma
      • Congenital Hemangioma
        • RICH
        • NICH
      • Pyogenic Granuloma
        Lobular capillary hemangioma
    • Locally aggressive
      • Kaposi sarcoma
      • Hemangioendothelioma
    • Malignant
      • Angiosarcoma

Locally aggressive

  • Kaposi Sarcoma

Malignant

  • Angiosarcoma
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
15
Q

Vascular Malformation

Lymphatic and Capillary

A
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
16
Q

Vascular Malformation

Venous and Arterial

A
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
17
Q

Fibro-osseous lesions

A

Fibro-osseous lesions

  • A group of lesions in which normal bone is replaced by fibrous connective tissue initially and eventually infiltrated by osteoid and or cemmentoid tissue
  • This is a descriptive histological term and is non-specific in diagnosis
  • Classification - Eversole 2008
    • I - Bone dysplasia
      • Paget’s disease
      • Fibrous dysplasia
        • Monostotic
        • Polyostotic
        • Craniofacial
      • Segmental odontomaxillary dysplasia
    • II - Cemento-osseous dysplasia
      • Periapical
      • Focal
      • Florid
    • III - Inflammatory/Reactive
      • Primary Osteomyelitis
        • Focal sclerosing osteomyelitis
        • Diffuse sclerosing osteomyelitis
        • Periostitis ossificans - Garres Osteomyelitis
    • IV - Metabolic
      • Hyperparathyroidism
    • V - Neoplasia
      • Ossifying fibroma
      • Cemento-ossifying fibroma
      • Juvenile ossifying fibroma
        • Trabeculae
        • Psammomatoid
      • Cementoblastoma
      • Familial gigantiform cementoma - Subtype of COD

​
* WHO 2017
WHO re-classifies many of the lesions into differenct categories
* Fibro-osseous lesions
* Fibrous dysplasia
* Cemento-osseous dysplasia
* Familial gigantiform cementoma
* Ossifying fibroma
* Benign Mesenchymal Odontogenic Tumour
* Cemento-ossifying fibroma
* Cementoblastoma
* Odontogenic fibroma
* Odontogenic myxoma
* Giant Cell Lesions
* Central giant cell granuloma
* Peripheral giant cell granuloma
* Cherubism
* Aneurysmal bone cyst
* Simple bone cyst

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
18
Q

Fibrous Dysplasia

A
  • Epidemiology
    • <20 yrs of age
  • Pathophysiology
    • GNAS mutation
    • Affects
      • Osteoblasts - fibrous dysplasia
      • Melanocyte - Cafe au lait spots
      • Endocrine cells - Endocrinopathies (40%)
    • Age at which the mutation occurs determines the severity of disease
      • < 6 weeks - Severe
      • >6 weeks - Less severe
      • Near birth - Monostotic
  • Presentation / Progression
    • Painless asymptomatic deformity
    • Neuropathies can occur due to entrapment of nerves
    • Lesions tend to burn out after puberty
    • Presentation
      • Monostotic 85%
      • Polyostotic 15%
    • Associated syndromes
      • Jaffe-Leichtenstein - Cafe au lait
      • McCune Albright - Cafe au lait + Endocrinopathies
      • Mazabraud - Soft tissue myxomas (Typically cardiac)
        ​
  • Histology
    • Irregular trabeculae of woven immature bone with fibrous stroma
    • Chinese characters
      ​
  • Treatment
    • Small lesions - Excised fully
      • Can re-occur in younger patients
    • Symptomatic treatment
      • Function
        • Neuropathies
        • Restriction of mouth opening
      • Esthetic
        • Deformity
        • Orbital dystopia
      • Medical
        • Bisphosphonates for pain
          ​
  • Malignant transformation and associated pathology
    • XRT contraindicated due to risk of post XRT sarcomas
    • Kaban - 1/70 underwent transformation into Osteosarcoma
    • Aneurysmal bone cysts can occur within this lesion due to hypervascularity during the proliferative phase
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
19
Q

Cemento-osseous dysplasia

A
  • Epidemiology
    • F>M : 10>1
    • African americans
    • 30 - 50 yrs of age
    • Most common fibro-osseous lesion
  • Pathophysiology
    • ?Local trauma
    • Arises from bone adjacent to PDL
      ​
  • Classification
    • Periapical
      • <1cm
      • Can be multiple
      • Typically involving anterior mandibular teeth
    • Focal
      • Single, edentulous areas
    • Florid
      • >2 quadrants, symmetrical
    • Familial gigantiform cementoma
  • Presentation / Progression
    • Asymptomatic
    • Vital teeth
    • Can become secondarily infected
    • Typically occurs above the IAN
    • Initially radiolucent and become radiopaque over time
      ​
  • Histopathology
    • Lesion poorly defined compared to COF
    • More cementum and less woven bone compared to COF
    • Cementicles
    • Ginger roots calcifications with rounded projections (sharp projections in Fibrous Dysplasia) in a fibrous connective tissue stroma
      ​
  • Treatment
    • No treatment generally required
    • Avoid surgical procedures as it can less to osteomyelitis
    • Antibiotics + Saucerization and debridement if infected
      ​
  • Associated pathology
    • 10% of Florid cases progresses to osteomyelitis
    • Associated with simple bone cysts
20
Q

Familial Gigantiform Cementoma

A
  • Epidemiology
    • Rare hereditary form of COD
    • M=F
    • No ethnic predilection
    • Occurs in first decade of life
      ​
  • Pathophysiology
  • Presentation / Progression
    • Multiple quadrants affected
    • Rapid regrowth of recontoured lesion
    • Sclerotic lesion has high rate of infection - Osteomyelitis
    • Expansile lesion
      ​
  • Associated Pathology
    • Uterine adenomas
21
Q

Cemento ossifying fibroma

A
  • Epidemiology
    • F>M : 5:1
    • 30 - 40 yrs of age
    • Mand > Max
      ​
  • Pathophysiology
    • Associated with HRT2 mutation (Tumor suppressor gene)
      ​
  • Presentation / Progression
    • Asymptomatic lesion
    • Well defined lesion
      ​
  • Histology
    • Capsulated
    • Mainly avascular fibrous connective tissue with bits of woven bone and cementum like material
    • Small trabeculae with peripheral osteoid and osteoblastic rimming
      ​
  • Treatment
    • Enucleate if small and has not lost its capsule
    • Resect large/recurrent lesions affecting function - nose
      • 5mm margin
        ​
  • Associated pathology
    • Aneurysmal bone cyst occur within lesion
22
Q

Cemento ossifying fibroma - Juvenile ossifying fibroma

A

Subtype of COF but occurs within the first two decades of life
More aggressive form of COF

  • Epidemiology
    • Occurs within the first two decades of life
      ​
  • Classification
    • Psammatoid (4x more common)
      • 20 yr olds
      • More common in orbital/sinonasal
    • Trabeculae
      • Young children
      • More common in jaws
        ​
  • Histology
    • Both subtypes do not have a capsule - High recurrence rate with enucleation
    • Psammatoid
      • Concentric lamellar round/ovoid ossicles
      • Within myxoid fibrous connective tissue
    • Trabecular
      • Strands of osteoid
      • Osteoblasts and giant cells
        ​
  • Treatment
    • Chrcanovic 2019
      • Enucleation: 40% recurrence rate
      • Enucleation + adjuncts: 15 - 30% recurrence rate
      • Marginal: Up to 10%
      • Segmental: 0%
    • Consideration
      • Proximity to vital structures
      • Patients wishes
      • Patients age
23
Q

Paget’s Disease

A

Osseous dysplasia characterised by rapid turnover and remodelling of bone throughout the skeleton

  • Epidemiology
    • 1:100 in over 45 yrs of age
    • 10% in over 90 yrs of age
    • M>F : 3:2
      ​
  • Pathophysiology
    • Defective function of the OPG/RANK/RANKL
    • Dysregulation of Osteoclastgenesis
      ​
  • Presentation / Progression
    • Lion Facies - leontiasis ossea
    • 3 Phases
      • Resorption - Bone softened
      • Vascular - Bone distortion and increase in size
      • Sclerotic - Bone mineralised in deformed state
    • Monostotic and Polyostotic
    • Presentation
      • Bone pain common - may be asymptomatic
      • Enlarging calvarial or jaw bones
      • Cranial nerve palsies
      • Bowing of long bones - weakened bones
      • Splaying of teeth
    • Imaging
      • Bone scan
        Abraham Lincoln sign - Blackbeard sign
        ​
  • Histology
    • Irregular bone with basophilic reversal lines + trabeculae line by osteoid
    • Fibrous dysplasia vs Paget’s disease
      Histo in Paget’s:
      • Osteoclast and osteoblast on the same piece of bone
      • Bone more mature
      • Reversal lines
  • ​​Treatment
    • ​Medical
      • ​Reduce bone resorption
        • Bisphosphonates
          • Reduces bone pain
          • Fracture risk
      • Hypocalcemia
        • Calcitonin
      • Pain management
        • Paracetamol
        • NSAIDs
    • Surgical
      • Recontouring of bone
        • Hypervascular bone in early phases
      • OMS Issues
        • Hypervascular bone
        • Dental extraction difficult due to hypercementosis
        • Abnormal bone healing
        • Monitoring due to malignant transformation​
    • Physiotherapy
      • Aims to strengthen muscles and control bone pain
  • Malignant transformation
    • 1% lifetime risk
24
Q

Osteoblastoma / Osteoid osteoma

A

Benign neoplasm of the bone - arising from osteoblasts
Very similar to Osteoid osteoma
Osteoblastoma > 2cm
Osteoid osteoma < 2cm

  • Epidemiology
    • <30 yr olds
    • M:F - 2:1
    • Mand > Max
      ​
  • Pathophysiology
    • FOS gene rearrangement
      • Function in regulating the development of cells destined to form and maintain the skeleton
        ​
  • Presentation / Progression
    • Dull aching pain
      • Worse at night
      • Osteoid osteoma - Nocturnal pain relieved by NSAIDs
    • Gait disturbances
    • Neuropathy if lesions affect vertebrae and foramina
    • Dental displacement and root resorption
  • Histology
    • Osteoid osteoma
      • Discrete central nidus <1cm with diffuse peripheral sclerosis
      • Nidus is usually distinct well circumscribed cavity surrounded by dense reactive bone of varying thickness
      • Typically cherry red in colour and can be shelled out from surrounding reactive bone
    • Osteoblastoma
      • Immature bony trabeculae are lined by plump large osteoblasts. Some of these trabeculae may have extensive ossification
      • Highly vascularized connective tissue
      • The stroma contains widely dilated capillaries and areas of large dilated sinusoids
      • Low mitotic activity - slow growth
        ​
  • Treatment
    • Medical
      • Chemotherapy and Radiotherapy have been controversial
      • Post XRT sarcoma has been documented
    • Surgery
      • Currettage - 24% recurrence
      • Resection with 5mm margins - curative
      • New technology
        • Radiofrequency ablation for spinal lesions
25
Q

Cementoblastoma

A
  • Epidemiology
    • < 20 yrs of age
    • M > F
    • 80% Mandible
    • 50% involve the first lower molar
      ​
  • Pathophysiology
    • Cementoblasts slowly lays down cementum and obliterates the surrounding periodontal ligament space and bone
      ​
  • Presentation / Progression
    • Pain on palpation or biting
    • Vital tooth
    • Slow growing
    • Expansile lesion
    • Imaging
      • Calcified opacity
      • Mass blends with root of tooth
        • cf Condensing Osteitis - Sclerosis of bone does not resorb and obscure root outline
      • Peripheral radiolucent zone
        ​
  • Histology
    • Very similar in appearance to Osteoblastoma
      Cementoblastoma is intimate contact with root
    • Dentine like material
    • Irregularly placed lacunae and prominent reversal lines
    • Mineralised material rimmed by cementoblasts
    • Multinucleate giant cells often present
      ​
  • Treatment
    • Extraction + excision of lesion
    • RCT + Root amputation
    • Recurrence rate 12% - Chrcanovic
26
Q

Osteoma
Gardner’s Syndrome

A
  • Epidemiology
    • 20 - 50 yrs of age
    • M>F
    • Mand > Max
    • Associated with Gardner’s Syndrome
  • Pathophysiology
    • Unknown cause
    • ?Trauma
      ​
  • Presentation
    • Painless
    • Asymptomatic
    • Firm lump of bone
    • Jaw osteomas typically affects
      • mandibular body and condyle
    • Slow growing
    • Displacement of teeth
  • Histology
    • Compact osteoma - Normal appearing dense bone with minimal marrow
    • Cancellous osteoma - Bony trabeculae and fibrofatty tissue
      ​
  • Treatment
    • Surgical excision often done for diagnosis
    • Asymptomatic cases can be monitored
    • Recurrence is rare
      ​
  • Malignant transformation
    • No malignant transformation
      ​

Gardner’s Syndrome

  • Epidemiology
    • 1: 10 000
    • APC gene - Autosomal dominant​
      Adenomatous polyposis coli gene
  • Pathophysiology
    • Form of Familial adenomatous polyposis
      • Characterizd by multiple colorectal polyps and other benign and malignant lesions
    • Mutation on the APC gene - Tumor suppressor gene
      • Monitors cell growth and division
        ​
  • Presentation
    • GI
      • Intestinal polyps
      • 50% will have colon cancer by age 30
      • PR bleeding, Anaemia
    • Osteomas
      • 90% will have multiple osteomas
      • Most commonly skull, paranasal sinuses and mandible
    • Dental
      • Odontomas
      • Supernumerary teeth
      • Impacted teeth
    • Skin
      • Epidermoid cysts
      • Lipomas
      • Neurofibromas
      • Desmoid tumors
        ​
  • Differentials for Intestinal Polyps
    • Turcot syndrome
    • Cowden syndrome
    • Peutz Jegher syndrome
27
Q

Desmoplastic Fibroma

A

Bony counterpart to Desmoid Tumours

  • Epidemiology
    • <20 yrs of age
    • Mand > Max
  • Pathophysiology
    • Unknown etiology
      ​
  • Presentation / Progression
    • Painless swelling
    • Can cause dental displacement and root resorption
    • 1/4 unilocular, 3/4 multilocular
    • Expansile with cortical thinning
      ​
  • Histology
    • Identical to soft tissue Desmoid tumour
    • Small elongated fibroblasts
    • Abundant collagen in dense interlacing bundles
    • No bone formation
      ​
  • Treatment
    • Currettage - 30% Recurrence
    • Resection - 5% Recurrence
28
Q

Histopathology - Ghost cells

A
  • Ghost Cells
    • Pale eosinophillic epithelial cells which have lost their nucleus but retain the cell outline
    • Eosin refers to staining of the cell not Eosinophil
    • Stains (Pink)
      ​
  • Associated Pathology
    • Calcifying odontogenic cyst / Gorlin’s cyst / Calcifying cystic odontogenic tumor
    • Dentinogenic ghost cell tumour
    • Ghost cell odontogenic carcinoma
    • Ameloblastic spectrum with ghost cells - Less ghost cells than DGCT
29
Q

Histopathology - Clear cell

A
  • Clear cells
    • Some secretory cells in the epithelium appear as clear cells and are one of the components of eccrine sweat glands
    • The cytoplasm contains large amounts of glycogen and mitchondria
    • Melanocytes appear as clear cells when in the stratum basale
    • Langerhans cells appear as clear cells in the stratum spinosum
  • Associated Pathology
    • Calcifying odontogenic epithelial tumor / Pindborg tumor
    • Mucoepidermoid carcinoma - 10% of tumor population
    • Clear cell odontogenic carcinoma
30
Q

Calcifying odontogenic cyst / Dentinogenic ghost cell tumor / Ghost cell odontogenic carcinoma

A

Spectrum of lesions involving odontogenic epithelium containing ghost cells
Lesions may subsequently calcify

  • Classification
    • 95% Cystic - Calcifying odontogenic cyst / Calcifying cystic odontogenic tumor
      • Intraosseous 75%
      • Extraosseous 25%
    • 5% Tumor - Dentinogenic ghost cell tumor
    • Malignant - Ghost cell odontogenic carcinoma
  • Calcifying odontogenic cyst - Gorlin’s cyst
    • Epidemiology
      • Most common in 30 yrs of age
        Younger if associated with unerupted teeth and odontomes
        20% associated with odontomes
      • Posterior mandible most common site
        ​
    • Pathophysiology
      • Unknown cause
        ​
    • Presentation / Progression
      • Associated with unerupted teeth
      • Associated with root resorption
      • Most common in incisor and canine region with unerupted teeth
      • Imaging
        • Unilocular, well defined
        • Radio-opaque flecks
          ​
    • Histology
      • 4-10 cell thick odontogenic epithelium
      • Resemble ameloblastoma
        • Basal pallisading columnar cells with reverse polarity
        • Stellate reticulum like epithelium
      • Ghost cells within epithelium
        • Eosinophilic
        • Large and pale
        • Can coalesce and become calcified
          ​
    • Management
      • Slow growing
      • Recurrence is rare
      • Enucleate
        ​
    • Malignant Transformation
      • Rarely carcinoma arise in Calcifying odontogenic cyst
        ​
  • Dentinogenic Ghost Cell Tumor
    • Solid variant of COC
    • More often Extraosseous than Intraosseous
    • Difficult to distinguish from COC radiographically
      ​
    • Histology
      • Ameloblastomatous islands of epihelium in mature stroma
      • Have nests of ghost cells preent within epithelium - differentiates it from a peripheral ameloblastoma
      • Aberrant keratinisation may be found in the form of ghost cells with dysplastic dentine
        ​
    • Treatment
      • Can be aggressive - therefore wide local excision
        ​
  • Ghost cell odontogenic carcinoma
    • Dentinogenic ghost cell tumor that undergoes malignant transformation
    • Histology
      • Both epithelial and ectomesenchyme components are present but only epithelial component is malignant
        • Cellular pleomorphism
        • Increased mitotic activity
        • Invasion of surrounding tissues
      • Diagnosis based on identifying benign features of COC or DGCT with malignant features
        ​
    • Treatment
      • Wide local excision
      • Recurrence common
      • 75% survival at 5 years
31
Q

WHO 2017 - Benign Odontogenic Tumors

A
  • Ectodermal origin
    • Ameloblastoma
      • Coventional
        • 6 Histological variants
      • Unicystic
        • Ackerman classification
      • Peripheral
    • Squmous odontogenic tumor
    • Calcifying epithelial odontogenic tumor
    • Adenomatoid odontogenic tumor
      ​
  • Ectomesenchyme origin
    • Ameloblastic fibroma
    • Dentinogenic ghost cell tumor
    • Odontoma
      • Complex / Compound
    • Primodial odontogenic tumor
      ​
  • Mesenchye origin
    • Odontogenic fibroma
    • Odontogenic myxoma
    • Cemento-ossifying fibroma
      • Juvenile ossifying fibroma
    • Cementoblastoma
32
Q

WHO 2017 - Malignant Odontogenic Tumors

A
  • Odontogenic carcinomas
    • Ameloblastic carcinoma
    • Ghost cell odontogenic carcinoma
    • Clear cell odontogenic carcinoma
    • Primary osseous carcinoma
    • Sclerosing odontogenic carcinoma
      ​
  • Odontogenic carcinosarcoma
  • Odontogenic sarcoma
33
Q

Oral potentially premalignant disorders - Conditions

A
  • Pre-malignant conditions
  • Discoid lupus erythematosis
    • Spectrum condition of Lupus erythematosus
    • Autoimmune mediated
    • Pathophysiology
      • Autoimmune condition to unknown antigen
      • Sun exposure can trigger these lesions
    • Presentation / Prognosis
      • Purplish red flat or raised patches
      • Increasing amounts of white scale
      • Darker skinned patients often have severe scarring
  • Fanconi anaemia
    • Rare genetic disease of FA genes resulting in impaired response to DNA damage
    • Pathophysiology
      • Autosomal recessive
      • FA genes - Responsible for DNA repair
    • Presentation / Progression
      • Bone marrow failure - Variable anaemia
      • AML
      • Short stature
      • Endocrinopathies
      • Cafe au lait spots
  • Dyskeratosis congentia
    • Congenital disorder of variable phenotype
    • Shot telomeres - manifests as premature ageing with bone marrow failure
    • X-Linked recessive
    • Pathophysiology
      • Mutation DKC gene on the X chromosome
      • Short telomeres
    • Presentation / Progression
      • Variable phenotype but typically
        • Nail dystrophy
        • Leukoplakia
        • Abnormal pigmentation
      • Myelodysplastic syndrome is associated with this condition
  • Xeroderma pigmentosum
    • Autosomal recessive
    • Genetic disorder where there is a decreased ability to repair DNA caused by UV light
    • Pathophysiology
      • Autosomal recessive mutation on NER (Nucleotide excision repair) enzyme
    • Presentation / Progression
      • Corneal ulcerations
      • Xeroderma (Dry skin)
      • Hyperpigmentation
      • Blistering and freckling on minimum sun exposure
  • Plummer Vinson syndrome
    • Rare disease characterized suspected to be due to nutritional deficiencies
    • Pathophysiology
      • Possible nutritional deficiency
        • Treated with Iron supplementation
      • Possible genetic factors
    • Presentation / Progression
      • Occurs in peri-menopausal women
      • Characterized by:
        • Difficulty swallowing
        • Angular cheilitis
        • Oesophageal webs
        • Iron deficiency anaemia
        • Glossitis
  • Epidermolysis bullosa
    • Rare group of medical conditions that results in easy blistering of skin and mucous membranes
    • Pathophysiology
      • Mutation can be due to 16 different genes
      • Lack of protein anchors that hold the skin together
        Leads to shearing of skin
      • Chronic skin damage leads to increased risk of malignancies
    • Presentation / Progression
      • Classified into four types
        • Epidermolysis bullosa simplex
        • Junctional Epidermolysis bullosa
        • Dystrophic Epidermolysis bullosa
        • Kindler syndrome
      • Blistering of skin and mucous membranes
  • Graft vs Host disease
    • Immune reaction from donor’s tissue towards the host
      • The opposite of Transplant rejection
    • Typically associated wtih bone marrow and stem cell transplants
    • In the oral cavity, chronic graft vs host disease manifests as Lichen reaction with a higher risk of malignant transformation
34
Q

WHO 2017 - Maxillofacial Cysts

A
  • See attached image
    • Odontogenic vs Non-odontogenic
    • Developmental vs Acquired
35
Q

WHO 2017 - Cysts - Odontogenic

A
  • Developmental
    • Dentigerous Cyst
      • Classification
        • Central - Cyst surrounds crown
        • Lateral - Grows on one side (Mesioangular lower 8)
        • Circumferential - Encloses whole tooth
      • Presentation / Progression
        • Encloses crown and attaches to CEJ
        • Asymptomatic
        • Can resorb adjacent roots
      • Pathophysiology
        • Alteration of reduced enamel epithelium (after tooth development) leads to cystic formation. Due to fluid between REE and Tooth
      • Histology
        • Non keratinised stratified squamous epithelium
        • 4-6 cells thick
        • Loosely arranged fibrous connective tissue wall
      • Treatment
        • Enucleation
    • OKC - Parakeratinised
    • OKC - Orthokeratinised
    • Lateral periodontal cyst / Botryoid Cyst
      • Botryoid cyst is considered the multicystic variant of LPC
      • Presentation / Progression
        • M:F - 2:1
        • 50 - 70 yr olds
        • Occurs in the PDL space
        • Asymptomatic
        • Vital Teeth
        • Displaced teeth
        • Can resorb roots
      • Pathophysiology
        • Unclear etiology
        • May arise from Rests of Serres (Dental lamina remnants)
      • Histology
        • Thin walled - 1 to 2 cells thick
        • Non keratinised SSE
        • Glycogen rich clear cells may be interspersed in the lining or seen within fibrous connective tissue
        • Localised areas of thickening along epithelium
      • Treatment
        • Enucleation (Chrcanovic - 2019)
          • Lateral periodontal - 2.5%
          • Botryoid - 22%
            • Consider adjuncts with enucleation
    • Gingival cyst
      • Soft tissue counterpart to Lateral periodontal cyst
      • Presentation / Progression
        • 50 - 60 yr olds
        • Blue grey lesion
        • Less than 5mm
        • May cause cupping of the alveolar bone underneath
      • Pathophysiology
        • Derived from Rests of Serres (Dental Lamina)
      • Histology
        • Similar to Lateral periodontal cyst
        • Thin cyst - 1 to 3 cells
        • Small nests of glycogen rich clear cells in surrounding connective tissue
        • Can contain plaque like thickening along cavity wall
      • Treatment
        • Simple excision
    • Calcifying odontogenic cyst
      • Part of the spectrum along with Dentinogenic ghost cell tumor and Ghost cell odontogenic carcinoma
      • Presentation / Progression
        • 30 yr olds
          • Presents younger if associated with teeth / odontomes
        • Rarely undergoes malignant transformation
        • 75% Intraosseous, 25% Extraosseous
        • Can have radio-opaque flecks
        • Associated with underupted teeth and resorption
          • 20% associated with odontomes
      • Pathophysiology
        • Unknown cause
      • Histology
        • Ameloblast cells form cyst lining - Can look like ameloblastoma
        • Ghost cells present within epithelium
          • Eosinophillic epithelial cells which have lost their nucleus
        • Large and pink
        • Can coalesce and become calcified
      • Treatment
        • Enucleate
    • Glandular odontogenic cyst
      • Classified as odontogenic origin. Can show salivary features
        Likely indicates pluripotent nature of odontogenic behaviour
      • Presentation / Progression
        • 50 yr old
        • Mand > Max
          • 80% anterior mandible
        • Behaves like ameloblastoma - aggressive behaviour with recurrence
        • Imaging
          • Uni or multilocular
          • Well defined cortical rim
      • Pathophysiology
        • Mutated cell from epithelial origin - undergoes maturation to a glandular differentiation
      • Histology
        • Squamous cuboidal cells
        • Intra-epithelial microcysts filled with mucin
        • May contain ciliated and goblet cells
      • Treatment
        • Enucleation - 22%
          • Adjuncts recommended with enucleation
        • Recurrence up to 8 yrs
    • Eruption cyst​
      • Presentation / Progression
        • Bluish swelling that occurs over soft tissue of an eruptin tooth
        • Considered superficial dentigerous cyst
      • Histology
        • Non keratinised squamous epithelium
      • Treatment
        • Marsupialization and decompression of cyst
        • Monitor - can burst spontaneously
  • Acquired
    • Radicular cyst
      • Presentation / Progression
        • Most common cyst
        • M > F
        • Associated with the apex of Non-vital teeth
      • Pathophysiology
        • Develops from from pre-existing Apical granuloma
      • Histology
        • Focus of chronically inflammed tissue at the apex of non-vital teeth
        • Central necrosis
        • May be cholesterol clefts or giant cells in wall due to RBC breakdown
        • Inflammatory infiltrate
        • Non keratinised SSE
        • 10% Contain Rushton bodies - Proteinaceous material (Hyaline bodies)
      • Treatment
        • Enucleate + Treat non vital dentition
    • Paradental cyst
      • Inflammed Lateral Dentigerous cyst
      • Presentation / Progression
        • Pain and tendernes with infective hx
      • Pathphysiology
        • Aleration of Reduced enamel epithelium leading to cystic formation.
          Infection of cyst
        • Can also originate from proliferation of Rests of Malassez from PDL
      • Histology
        • Non keratinised SSE
        • Variable thickness
        • Fibrous wall
        • Inflammatory cells
      • Treatment
        • Enucleation
    • Buccal bifurcation cyst
      • Presentation / Progression
        • Occurs in children
        • Associated with lower 6s
        • 1/3 are bilateral
        • Imaging
          • Well circumscribed radiolucency
          • Distinct U shaped lesion
      • Pathophysiology
        • Triggered by infection or food debri impaction
      • Histology
        • Identical to Radicular cyst on histology
        • Non keratinised SSE
        • May be cholesterol clefts or giant cells in wall due to RBC breakdown
        • Inflammatory infiltrate
        • Non keratinised SSE
        • 10% Contain Rushton bodies - Proteinaceous material (Hyaline bodies)
      • Treatment
        • Enucleate leaving the tooth
36
Q

WHO 2017 - Cysts - Non-Odontogenic

A

Non-Odontogenic Cysts

  • Developmental
    • Lymphoepithelial cyst
      • Presentation / Progression
        • OKC with lymphoid tissue in wall
        • Occurs at the sites of lymphoid tissue
          • Commonly FOM, lateral & posterior tongue and tonsil pillars
      • Pathophysiology
        • Arises from entrapment of epithelium within lymph nodes or lymphoid tissue during development
        • Blind ended epithelial invagination into lymphoid tissue that may lead to a tract that become filled with keratin
      • Histology
        • Lined by parakeratinised stratified squamous epithelium (Similar to OKC)
        • Epithelial lining surrounded by discrete lymphoid component
        • Cyst wall contains variable proportions of lymphocytes, macrophages and plasma cells
        • Cyst lining may be continuous with surface epithelium
      • Treatment
    • Thyroglossal duct cyst
      • Presentation / Progression
        • Most common developmental cyst of the neck
        • 7% of the population
        • Any age
        • Midline lesion but 20% para-median
        • Moves with tongue on protrusion
        • Imaging
          • Thyroid scan - Iodine 131
            • Determines presence of thyroid tissue in neck or lingual thyroid
          • US - can show tract and lesion
      • Pathophysiology
        • Remnant of epithelial cells from the descent of thyroid gland
          • Thyroid develops around Foramen cecum - 3 to 4 weeks
          • Descends - 4 to 8 weeks
          • Involution of duct - 8 to 10 weeks
          • Distal portion may persist at the pyramidal lobe in up to 50% of the population
      • Histology
        • Lining of psudostratified columnar epithelium
        • Frequently contain thyroid follicles and thyroid colloid
      • Treatment
        • Sistrunk procedure
          • Excison of cyst and entire tract all the way up to tongue
          • Centre of hyoid bone usually invovled in excision
            Can excise lesion from periosteum as an alternative
          • Ligation of duct as far proximal as possible
        • Recurrence is rare
    • Branchial cleft cyst
      • Presentation / Progression
        • Also known as cervical lymphoepithelial cyst
          Contains epithelial cells and lymphoid tissue/germinal centres
        • 20 - 30 yr old
        • II branchial arch - 95%
          • Bailey Classification for II arch cysts
            • Based on depth
            • I - Deep to platysma
            • II - Superficial to Carotidy body
            • III - Passing between Carotid arteries
            • IV - On pharyngeal wall
          • I - 5%
        • Incidental finding or activation of activation of lymphoid tissue during infection
        • Branchial arches cleft cyst locations:
          • I - Near angle of mandible. Can extend to auricle
          • II - Located anterior to SCM (Inf and mid third)
          • III - Extend from SCM (Inf and mid third) to piriform fossa
          • IV - Parallels the course fo the recurrent laryngeal nerve. Begins at the piriform fossa and extend to the thyroid
      • Pathophysiology
        • Two theories
          • Cleft between branchial arches fails to completely involute
          • Cystic transformation of cervical lymph nodes
      • Histology
        • Lined by SSE
        • Lymphocytes and germinal centres
        • Same as lymphoepithelial cyst - cystic degeneration of lymph node
      • Treatment
        • Sclerotherapy with Picibanil
        • Surgery
          • Excision and ligation of residual tract
    • Dermoid cyst
      • Presentation / Progression
        • Dermoid cyst contains adnexal structures
          cf Epidermoid cyst (Sebaceous cyst) - Epithelial cells
        • Most common site is the lateral brow
        • Asymptomatic swelling
      • Pathophysiology
        • Improper fusion of skin layers
      • Histology
        • Line by SSE
        • Fibrous connective tissue wall
        • Contain adnexal structures
          • Hair follicles
          • Sebaceous glands
          • Sweat glands
        • Teratoid variety includes elements of all germ cell layers
          • Teeth
          • Bone
          • Muscle
      • Treatment
        • Surgical excision
        • Recurrence is rare
  • Acquired
    • Simple bone cyst / Solitary bone cyst
      • Presentation / Progression
        • Not a true cyst - Lacks epithelial lining
        • Occurs in younger patients
        • Asymptomatic
        • No bony expansion
        • Vital dentition
        • Radiolucent lesion with sclerotic bone that scallops around the teeth
      • Pathophysiology
        • FITH
        • Fibro-osseous lesion resolving
        • Ischaemia - Necrosis leading to interruption of venous drainage leading to increased intramedullary pressure which triggers osteoclastic bone resorption
        • Trauma - Intramedullary hemarrhage which resolves but is not replaced with bone
        • Hormones - changes during teenage years cause disturbances in remodelling of trabecular bone
      • Histology
        • Bone cavity with no epithelial lining
        • Serosanguinous material in the lumen
      • Treatment
        • Exploration and currettage the bone wall to stimulate bleeding and bone infill
    • Nasopalatine duct cyst
      • Presentation / Progression
        • Most common non-odontogenic cyst
          1% of population
        • M>F
        • 30 - 50 yrs of age
        • Asymptomatic soft swelling of the anterior hard palate
        • Vital teeth
        • Root resorption is rare
        • Fluctuating painful swelling can occur due to secondary infection
        • Imaging
          • Heart shaped appearance
          • Occurs between the central incisors
          • 7mm cut-off (Roper-Hall 1938)
      • Pathophysiology
        • Nasopalatine ducts runs either side of the nasal septum and exite at the incisive canal
        • Epithelial remnants of nasopalatine duct that have failed to degenerate can form cysts
        • Infection or trauma can stimulate cyst formation
      • Histology
        • Epithelial lined cyst
          • Stratified squamous or Cuboidal or Columnar
          • Pseudostratified columnar
          • Higher up in canal - More features of respiratory epithelium
        • Can have nerves, arteries, veins and minor salivary gland
      • Treatment
        • Enucleate
        • Recurrence is very low
    • Median palatal fissure cyst
      • Presentation / Progression
        • Fissural cyst due to the entrapment of epithelium along fusion line of lateral palatal shelves
        • Firm fluctuant swelling in midline of hard palate and posterior incisive canal
        • Can be difficult to distinguish from a posteriorly located Nasopalatine duct cyst
      • Pathophysiology
        • Due to the entrapment of epithelium along fusion of the lateral palatal shelves
      • Histology
        • Stratified squamous epithelium
        • No nerves, arteries or veins
      • Treatment
        • Excision
        • Recurrence low
    • Nasolabial cyst
      • Presentation / Progression
        • 30 - 40 yr olds
        • 10% bilateral
        • M < F - 1:4
        • Nasolabial swelling
          • Anywhere from alar crease to labial vestibule
        • Asymptomatic
        • Fluctuant
      • Pathophysiology
        • Two theories
          • Entrapped epithelial remnants at the junction of maxillary medial and lateral nasal processes
          • Ectopic epithelium of the Nasolacrimal duct
            • Deep to muscles of facial expression
            • May have respiratory epithelium
      • Histology
        • Line by pseudostratified columnar epithelium +/- goblet cells
        • Fibrous connective tissue wall
      • Treatment
        • Extracapsular excision
          • Can be performed trans-cutaneously or trans-orally if close to nasal floor
          • Part of the nasal mucosa may require excision
    • Mucous retention cyst
      • Presentation / Progression
        • True cyst - cf Mucous extravasation cyst
        • Asymptomatic swelling
        • Painful if infected
      • Pathophysiology
        • Obstruction of salivary duct that causes pooling of saliva and distension of the duct and or gland
        • True cyst - lined by epithelium
      • Histology
        • Lined by cuboidal, columnar, squamous cells
        • Mucoid secretions
        • Dilated ducts
      • Treatment
        • Excise if isolated
        • If within major gland - may require gland removal
    • Surgical ciliated cyst
      • Presentation / Progression
        • 30 - 40 yr olds
        • Occurs post trauma or surgery
        • Asymptomatic swelling
      • Pathophysiology
        • Occurs post trauma or surgery
        • Respiratory epithelium is trapped in foreign tissue in surgical site
      • Histology
        • Lined by respiratory epithelium
          • Pseudostratified columnar epithelium
      • Treatment
        • Excision of cyst
37
Q

WHO 2017 - Benign Tumors - Epithelial

A

Benign Odontogenic Tumours - Epithelial

  • Ameloblastoma
    • Classification
      • Cystic - Ackermann Classification
        • Luminal - 10%
        • Intraluminal - 40%
        • Intramural - 50%
      • Conventional - Histological Classification
        • Follicular
        • Plexiform
        • Desmoplastic - can appear radio-opaque (Typically ant mand)
        • Granular
        • Acanthomatous
        • Basal cell
      • Peripheral
      • Metastasising
    • Presentation
      • 37% of all odontogenic tumors
      • 1 in 1 million per year
      • 80% mandible
      • 30 - 40 yr olds
      • Asymptomatic
      • Bony expansion
      • Resorbs teeth and leads to mobility
    • Pathophysiology
      • Arises from Rest of Serres (Dental lamina)
      • BRAF mutation in 80%
      • Over expression in BCL2 and RAS - excess signalling in MAPK pathway
      • Maxillary ameloblastoma can have mutations in SHH pathway
    • Histology
      • Islands of epithelium in FCT stroma
      • Ameloblast like cells with reverse polarity (towards centre of island)
      • Centre stellate reticulum with cystic degeneration
      • Histological subtypes
        • Follicular - Cysts
        • Plexiform - Follicular with no cysts
        • Acanthomatous - Forming keratin
        • Granular cell - Granular cells instead of stellate reticulum
        • Basal cell - Basal cell instead of stellate reticulum
        • Desmoplastic - Stands compressed by dense FCT
    • Treatment
      • High rate of recurrence up to 80%
      • Unicystic - Treat as cyst except for intramural
      • Conventional - Treat as tumor
        • Marx - up to 8mm from radiographic margin - Average 4.5mm
          Therefore 1.5cm margin
        • Engelbrecht - Perineural invasion
      • Recurrence - 50% in first 5 yrs - Can be up to 30 years
  • Adenomatoid odontogenic tumor
    • Classification
      • Follicular - 75% - Intraosseous lucency associated with the crown of unerupted tooth
      • Extra-follicular - 20% - Intraosseous lucency between roots of teeth
      • Peripheral 2% - Extraosseous
    • Presentation / Progression
      • 20 - 30 yr olds
      • M:F - 1:3 (2/3rd female)
      • 2/3 in maxilla
      • 2/3 associated with impacted canine
      • 2/3 radiolucent
      • Asymptomatic
    • Pathophysiology
      • Thought to arises from HERS / Rests of Malassez
      • Arises mid root level and encloses crown
        cf. Dentigerous cyst - arises from CEJ
    • Histology
      • Well encapsulated, thick capsule
      • Odontogenic epithelium in whorls, islands, rosettes
        Made from columnar ameloblast like ells not truly glandular
      • Central space can have stellate reticulum like material
    • Treatment
      • Enucleations
      • Recurrence very rare
  • Squamous odontogenic tumor
    • Presentation / Progression
      • Wide age range - Mean 30 yr olds
      • M:F - 2:1
      • Benign tumor showing terminal squamous differentiation
      • Asymptomatic
      • Gingival swelling
      • Associated with mobility of teeth
      • Imaging
        • Triangular defect between roots
        • Vertical periodontal bone loss
        • Poorly defined margin
        • Uni or multilocular
    • Pathophysiology
      • Originates within the PDL associated with the lateral root surface
      • Arises from Rests of Seres (Dental Lamina) or Rests of Malassez (HERS)
    • Histology
      • Squamous islands in a dense fibrous connective tissue stroma
      • Can look like well differentiated SCC
      • Peripheral cells do not show reverse polarity seen in Ameloblastoma
    • Treatment
      • Conservative local excision or currettage
      • Reccurance is rare
  • Calcifying epithelial odontogenic tumor
    • Presentation / Progression
      • 30 - 50 yrs old
      • Mand > Max
      • Chrcanovic 2017
        • 90% Central
        • 10% Peripheral
      • Asymptomatic
      • Slow growing
      • Bony expansion
      • Imaging
        • Uni or multilocular
        • Variable calcification - fine flecks - Honeycombed appearance
    • Pathophysiology
      • Arises from Stratum intermedium of enamel organ
      • Associated with PTCH1 gene - Not associated with Gorlin Goltz Syndrome
    • Histology
      • Poorly encapsulated
      • Cystic spaces filled with amyloid like material
        • Apple green birefringence - Congo red stain
      • Polyhedral cells - Plump, pink eosinophilic cytoplasm
      • Spherical calcifications - Leisegang rings (Tree trunk rings)
    • Treatment
      • Locally aggressive
      • Conservative local resection with a narrow rim of surrounding bone
        • Currettage - Recurrence rate 15%
38
Q

WHO 2017 - Benign Tumors - Mixed

A

Benign Tumors - Mixed

  • Ameloblastic Fibroma
    • Presentation / Progression
      • True mixed tumors
      • < 20 yrs old
      • M:F - 3:2
      • Asymptomatic, Slow growing
      • Bony expansion
      • Mandibular molars, 70% posterior mandible
      • Most cases are associated with unerupted teeth
      • Imaging
        • Usually unilocular
    • Pathophysiology
      • Characterised by proliferation of both odontogenic epithelium and mesenchyme without hard tissue formation
    • Histology
      • Well encapsulated
      • Thin walled (1-2cells thick)
      • Cuboidal or columnar cells
      • Epithelium has two patterns
        • Long narrow cords in an anastamosing arrangement
        • Small, discrete islands that resemble follicular stage of devleoping enamel organ
      • May contain epithelial islands like ameloblastoma
    • Treatment
      • Less aggressive than ameloblastoma
      • More conservative as lesion tends to occur in kids
      • Excision with a rim of normal bone
      • Chrcanovic 2018
        • Recurrence 20%
        • 50% change to ameloblastic fibrosarcoma - lifelong follow-up
  • Dentinogenic ghost cell tumor
    • Presentation / Progression
      • Solid variant of COC
      • More often Extraosseous than Intraosseous
      • Difficult to distinguish from COC radiographically​
      • 50 yr old
    • Pathophysiology
      • Arises from epithelial remnant that were trapped within bone
    • Histology
      • Ameloblastomatous islands of epihelium in mature stroma
      • Have nests of ghost cells preent within epithelium - differentiates it from a peripheral ameloblastoma
        • Aberrant keratinisation may be found in the form of ghost cells with dysplastic dentine
    • Treatment
      • Can be aggressive - therefore wide local excision
        ​
  • Odontoma
    • Classification
      • Complex - Disorganised mass of enamel, dentine and cementum
        Usually occur posteriorly
      • Compound - Multiple denticles
        Usually occur in the maxilla
    • Presentation / Progression
      • Most common odontogenic tumor
      • < 20 yrs old
      • Mand > Max
      • Often replaces missing teeth
    • Pathophysiology
      • Harmatoma
      • Most simple form of a compound odontome is a supernumerary
      • Exact etiology unknown
    • Histology
      • Complex
        • Disorganised mass of enamel, dentine and cementum
        • Fibrous connective tissue capsule
        • Ghost cells may be present in 20%
      • Compound
        • Denticles present
        • Similar to complex but much more complete
    • Treatment
  • Primordial odontogenic tumor
    • Presentation / Progression
      • Very rare - 10 Case reports
      • Occurs in young patients
      • Asymptomatic swelling
      • Imaging
        • Well defined unilocular radiolucency
    • Pathophysiology
      • Unknown
    • Histology
      • Similar to ameloblastic fibroma
      • Single layer of columnar epithelium covering periphery of tumor resembles inner enamel epithelium with a core of dental papilla-like tissue
      • There is no signs of hard tissue formation
    • Treatment
      • Enucleate
39
Q

WHO 2017 - Benign Tumors - Mesenchyme

A

Benign Tumors - Mesenchyme

  • Odontogenic Fibroma
    • Classification
      • Central
      • Peripheral
        • Appears like pyogenic granuloma
        • Histologically has odontogenic rests of epithelium
    • Presentation / Progression
      • 40 yrs of age
      • M:F - 1:2
      • Most common sites are anterior maxilla and posterior mandible
      • Displaces teeth
      • Resorbs dentition
      • Imaging
        • Typically well defined unilocular radiolucency
          Larger lesions can be multilocular
    • Pathophysiology
    • Histology
      • 2 Types - Epithelium rich and Epithelium poor
        Does not affect behaviour or prognosis
        • Epithelium Rich
          • Non infiltrating connective tissue lesion resembling dental follicle
          • Stellate fibroblasts arranged in a whorled pattern
          • Fine collagen fibrils and considered ground substance
        • Epithelium Rich
          • Fairly cellular fibrous connective tissue
          • Varies from myxoid to densely hyalinised
          • Strands or nests of odontogenic epithelium
          • Focal deposits of ODAM (odontogenic ameloblast associated protein)
    • Treatment
      • Enucleation
      • Recurrence uncommon
  • Odontogenic Myxoma
    • Presentation / Progression
      • Benign locally aggressive
      • 30 yrs of age
      • F > M
      • Mand > Max - 2:1
      • Teeth not displaced or resorbed
      • Imaging
        • Multilocular soap bubble appearance
        • Radiolucent wispy trabeculae
        • Scallops around teeth
    • Pathophysiology
      • Dervied from ectomesenchyme of tooth germ ro mesenchyme of jaw
    • Histology
      • Very cellular
      • Sparse stellate/spindle cells in myxoid/mucoid extracellular matrix
        Myxoid stroma contains:
        • GAGs, Hyaluronic acid and chondroitin sulfate
      • Poorly encapsulated
      • Myxoma cells have antibodies directed against vimentin (intermediate filament found in mesenchymal cells)
    • Treatment
      • Chrcanovic 2018
        • Currettage - 30%
        • Enucleation - 13%
        • Enucleation + Peripheral ostectomy - 7%
        • Resection - 3%
  • Cemento-ossifying fibroma
    • Epidemiology
      • F>M : 5:1
      • 30 - 40 yrs of age
      • Mand > Max
    • Pathophysiology
      • Associated with HRT2 mutation (Tumor suppressor gene)​
    • Presentation / Progression
      • Asymptomatic lesion
      • Well defined lesion​
    • Histology
      • Capsulated
      • Mainly avascular fibrous connective tissue with bits of woven bone and cementum like material
      • Small trabeculae with peripheral osteoid and osteoblastic rimming​
    • Treatment
      • Enucleate if small and has not lost its capsule
      • Resect large/recurrent lesions affecting function - nose
      • 5mm margin​
    • Associated pathology
      • Aneurysmal bone cyst occur within lesion
  • Cementoblastoma
    • Presentation / Progression
      • Only true neoplasm of cementum
      • Associated with tooth root - cf Osteoblastoma (Distinguishing feature is that the lesion is fused to the tooth root)
      • 10 - 20 yr olds
      • M > F
      • 80% Mand
        • 50% lower 6s –> lower 5s
      • Pain on palpation or biting
      • Vital tooth
      • Bony expansion
      • Imaging
        • Calcified opacity - Initially not calcified
        • Peripheral radiolucent zone
        • Mass blends with the root - no obvious root outline
    • Pathophysiology
    • Histology
      • Dentine like material
      • Irregularly placed lacunae and prominent reversal line
      • Mineralised material rimmed by cementoblasts
      • Fragments of cementum fuse at edges
    • Treatment
      • Continuous growth so need tooth removal
      • Root amputation and excision of mass + RCT
      • Chrcanovic 2017 - Recurrence rate 12%
40
Q

Ankyloglossia

A
  • ADA Statement Paper 2020
  • Definitions
    • Frenotomy / Frenulotomy
      • Division of frenum without suture or revision of remaining tissues
    • Frenuloplasty
      • Division of a frenum and closure of the mucosa with sutures
    • Frenectomy
      • Excision of frenum
        ​
  • Diagnosis
    • Needs both Functional Limitation + Anatomical Restriction
    • Function Limitation
      • Difficulty in breastfeeding
      • Speech impediment
      • Swallowing
        ​
  • Assessment
    • Hazelbaker
      • 0-14 scale (14 normal, <11 function impaired)
      • Objective assessment whihc include both functional and appearance
        ​
  • Management
    Non-surgical management trailed first
    • Non-Surgical
      • Feeding positioning
      • Latch optimization
      • Nipple shields
      • Supply support
      • Speech path
        • Transition to solids
        • Speech
        • Patient education
    • Surgical Management
      • Frenotomy / Frenectomy
      • Cold steel
        • Recommended as lasers can include eye exposrue, inhalaton of smoke and aspiration of coolant
          ​
  • Contraindications
    • Orofacial malformations
      • Cleft palate
      • Pierre robin sequence
    • Bleeding disorders
    • Neuromuscular conditions
    • Vitamin K deficiency
      ​
  • Complications
    • Acute
      • Deep ulceration
      • Bleeding, Hematoma
      • Airway compromise
      • Swelling
      • Restricted tongue movement
      • Iatrogenic injury
    • Chronic
      • Recurrent ankyloglossia
      • Sub-mandibular salivary duct injury
      • Oral aversion
      • Ranula
      • Tongue paresthesia and infection
41
Q

Langerhan Cell Histiocytosis

A

Langerhan Cell Histocytosis

  • Langerhan cell
    • Mononuclear cell of bone marrow origin that belongs to the dendritic cell family
    • Functions as an Antigen presenting cell
    • Lies within the basal layer of the epidermis
  • Histiocytosis
    • Two groups of immune cells - Macrophages and Dendritic cells
      ​
  • Epidemiology
    • 1-2 per million
    • Average age of presentation 25 yo old
      Peak incidence in below 4 years of oaage
      • The early the diagnosis - the poorer the outcome
      • Children below the age of 4 tend to have multi - have 4L
        ​
  • Classification
    • Single System
      • Uniforacl or Multifocal
        ​
  • Pathophysilogy
    • Unclear aetiology
      • Multisystem
42
Q

Salivary Gland - Surgical Definitions

A
  • Extracapsular dissection
    • Excision of lesion with a thin cuff of partoid tissue
  • Superficial Parotidectomy
    • Partial/Limited - Superficial parotidectomy but tracing some of the branches
    • Complete - Superficial parotidectomy but tracing all of the branches
  • Total Parotidectomy
    • Removing the whole gland but sparing the facial nerve
  • Radical Parotidectomy
    • Removal of the gland + Nerve
  • Extended Parotidectomy
    • Gland + Nerve + Other structures
43
Q

Salivary Gland - Malignant Epidemiology

A
  • Spiro 1986
    • ​MSK data over 35 yrs
      Retrospective rev of 2807 pts
    • Malignant Epidemiology
      • Minor glands 85%
      • Sublingual
      • Submandibular 50%
      • Parotid 25%
        ​
  • Pleomorphic Adenoma
    • 1.5% at 5 years
    • 9.5% at 10 - 15 years
      ​
  • Warthins Tumor - Papillary cystadenoma lymphomatosum
    • 0.1 - 0.5% of cases
44
Q

Salivary Gland - Workup

A
  • Biopsy
    • FNA
      • No evidence for tumor seeding after FNA
      • Schmidt 2011
        • Meta analysis - 64 studies
        • Sensitivity
          • Neoplasm - 96%
          • Malignancy - 80%
        • Specificity
          • Neoplasm - 98%
          • Malignancy - 96%
        • Inadequacy rate
          • 8%
        • False negative
          • 21%
        • Limitations
          • Large variation in studies
          • Accuracy affected by experience of cytopathologists
            ​
    • Core Biopsy
      • Indications
        • Tissue architecture is required
        • Additional testing more accurate
          • Flow cytometry
          • Immunohistochemistry
      • Risks
        • Potential tumor spillage
        • Facial nerve injury - 0.2%
        • Scarring, Fistula, Hematoma
      • Schmidt 2011
        • Sensitivity - 96%
        • Specificity - 99%
        • Inadequacy rate
          • 1%
        • False negative
          • 5%
          • When used in conjunction post FNA this decreases to 1%
            ​
  • Imaging
    • CT
      • Sensitivity - Close to 100%
        • Can detect a lesion but poor at distinguishing between benign vs malignant
        • Poor at identifying spread into adjacent tissue
        • Most useful at evaluating calcific lesions
          ​
    • MRI
      • Sensitivity - Close to 100%
      • Indications
        • Soft tissue and Neural invasion
        • Early bone marrow changes
        • Facial nerve localisation
          • 65% - Superficial to RMV
          • 15% - Deep to RMV
          • 10% - Entwined with RMV
            ​
    • Ultrasound
      • Indications
        • Monitoring
        • Can be useful in characterising the appearance and location of a lesion
        • Used in conjunction for FNA or Core Biopsy
      • Limited in assessing deep anatomical structures
    • Electromyography
      • Useful in evaluating perineural invasion by malignant tumors
      • Up to 40% of facial nerve can be affected without clinical weakness
        ​
    • Sialography
      • Useful in evaluating intra and extra ductal system
      • Limited role for evaluating autoimmune conditions given serum biomarkers
        • Previously used to differentiated between obstructive and autoimmune conditions
          ​
  • Blood Test
    • Autoimmune Screen
      • Rheumatoid Factor
      • ANA
      • Anti ss DNA - A (Anti-Ro)
      • Anti ss DNA - B (Anti-La)
      • ESR
      • IgG
45
Q
A

OMS Finals (18 decks)

Brainscape helps you reach your goals faster, through stronger study habits.
© 2024 Bold Learning Solutions. Terms and Conditions