primordial odontogenic tumor

SUBMITTED BY: Lekshmy Jayan

I MDS

Department of Oral Pathology

GUIDED BY: Dr. Jayant

ODONTOGENIC TUMOR

Group of neoplasm and tumor- like malformations arising from cells of odontogenic apparatus and their remnants.

In simple terms, odontogenic tumors arise from odontogenic or tooth forming apparatus.

Origin of Odontogenic Tumors

Ectodermal

Dental lamina( Cell

rests of Serre)

Enamel organ

REEHERS( Cell

rests of Malassez)

Mesenchymal

Dental papilla

Dental sac

• Malignant Odontogenic Tumors

Ameloblastic carcinoma

Primary intraosseous carcinoma

Clear cell odontogenic carcinoma

Sclerosing odontogenic carcinoma

Ghost cell odontogenic carcinoma

Odontogenic carcinosarcoma

Odontogenic sarcoma

Benign Odontogenic Tumors

Ameloblastoma

Squamous odontogenic tumor

Calcifying epithelial odontogenic tumor

Adenamatoid odontogenic tumor

Ameloblastic fibroma

Primordial odontogenic tumor

Dentinogenic ghost cell tumor

Odontoma

Odontogenic fibroma

Odontogenic myoma

Cementoblastoma

Cemento-ossifying fibroma

Odontogenic

carcinoma with

dentinoid

Odontogenic Cysts

Dentigerous cyst

Odontogenic keratocyst

Lateral periodontal and botryoid odontogenic cyst

Gingival cyst

Glandular odontogenic cyst

Calcifying odontogenic cyst

Nasopalatine cyst

Orthokeratinized odontogenic cyst

(Wright et al : Update from the 4th Edition of the World Health Organization Classification of Head and Neck Tumours: Odontogenic and Maxillofacial Bone Tumors, 2017)

First described by Mosqueda et al, 2014

Found 6 cases of tumors that did not fulfil the criteria of any described odontogenic tumor

Primordial odontogenic tumor is an intraoral mixed odontogenic tumor consisting of dental papilla like tissue covered with cuboidal to columnar epithelium that resembles inner enamel epithelium of enamel organ

(Mikami et al : Primordial odontogenic tumor: A

case report with histopathological analysis. 2017)

Soft tissue odontoma with massively enlarged dental papilla

Soft tissue odontoma with monstrous papillomegaly

(Slater et al: Primordial Odontogenic Tumor: Report of a Case:2015)

Still not clear

But may possibly originated from mesenchyme of an abortive tooth germ that failed to produce a dental organ

The mesenchymal tissue may have stimulated the epithelium to proliferate around it

Mimics early/ primordial stages of tooth development

(Ronell Bologna- Molina et al: Primordial odontogenic tumor: an immunohistochemical profile: 2017)

Novel lesion

Only 9 cases reported so far

Age= 3-19years

Sex =

Mandible : Maxilla = 6:1

Posterior molar region

All cases except one has been reported in left side

Asymptomatic expansile lesion

Average size= 56mm (25-90 mm)

Seen in association with unerupted teeth

Aggressive lesion

( Toshinori Ando et al: A case report of priomordialodontogenic tumor: A new entity in WHO classification 2017: 2017)

Age/

Gender

Location Clinical findings Radiographic findings Treatment Follow

up

1.

18yr/M

Left posterior mandible from

the first molar to the angle of

the mandible

Asymptomatic buccal

swelling clinically

evident for 6 months

RL, UL, well defined,

45 9 40 mm, enclosing

the crown of the third

molar

Enucleation together

with embedded third

molar

20

years,

NED

2.16yr/M Left posterior mandible from


the mandible

Asymptomatic, buccal

and inferior mandibular

cortical bone expansion.

Clinically evident for 4

months




molar


with embedded third

molar

LFU

3.

16yr/M

Left posterior mandible from


the mandible

Asymptomatic buccal

swelling. Clinically

evident for 1 year




molar


with embedded third

molar

10

years,

NED

4. 3yr/F Left posterior mandible from


the mandible

Asymptomatic buccal

and lingual bony

expansion. Clinically

evident for 22 months

RL, biloculated, well

defined, 90 9 70 mm,

enclosing the crowns of

the second deciduous

and first permanent

molars. Root resorption

of the first deciduous

molar


with first and second

deciduous molars

and tooth 19

9 years,

NED

Age/

Gender

Location Clinical features Radiographic features Treatment Follow

up

5. 13yr/F Left posterior mandible

from the second premolar

to the upper third of the

ascending ramus

Asymptomatic

buccal swelling.

Clinically evident

for 4 months

RL, biloculated, well defined,

80 9 50 mm, enclosing the

third molar

Enucleation with the

third molar and

extraction of the first

and second molars

3 years,

NED

6.

3yr/F

Left posterior maxilla from

the first deciduous molar

up to the floor of the

maxillary sinus and

tuberosity

Asymptomatic

buccal and palatal

bony swelling.

Clinically evident

for 3 months

RL, UL, well defined, 35 9 30

mm, displacing the second

deciduous and first permanent

molars


with the second

deciduous and first

permanent molars

6

months,

NED

7.

8yr/F Left posterior maxilla from

second premolar upto the

floor of the maxillary sinus

and tuberosity

Asymptomatic left

maxillary buccal

and palatal

swelling

well-defined RL associated

with an unerupted first

deciduous molar, which

induced buccal cortical plate

expansion and elevation of the

floor of left maxillary sinus,

and also slightly displaced the

first premolar toward palatal

side


with the deciduous

first molar

16

months,

NED

8. 5yr/M Right posterior mandible

from deciduous first molar

to permanent first molar

Asymptomatic

buccal swelling

Well-defined homogenous

radiolucency pushing the

unerupted tooth to the base of

the mandible.


with second deciduous

molar

7

months,

NED

White, solid spherical mass

Glossy and multilobulated

If enucleated along with the involved tooth then it is also seen

Loose and myxoid fibrous tissue

Scattered stellate and fusiform fibroblast- forms central area of tumor

Cell-rich ectomesenchymatous tissue or myxoid tissue

Periphery of lesion is covered by columnar or cuboidal epithelium

Resemble INNER ENAMEL EPITHELIUM of developing tooth

No odontoblastic differentiation

REE overlying enamel surface of embedded teeth- no direct continuity

Independent ectomesenchymal proliferation??

Induce proliferation of adjacent dental lamina to surround it !!

(Mosqueda- Taylor et al ; Primordial odontogenic tumor: Clinicopathological analysis of 6 cases of previously undescribed entity: 2014)

CK 14- positive in all epithelial layers

CK 19 positive in columnar epithelium

Vimentin strongly positive in mesenchymal tumor cells

Moderately positive in all epithelial layers

Amelogenin -positive in cuboidal and columnar epithelium

MOC-31- found in localised areas in epithelium

SYNDECAN-1( CD138)- vary from entirely negative zones to focally positive areas in epithelium

Strong stromal expression

PITX2- Weak immunostaining in mesenchymal cells

Weak to moderate positivity in endothelial cells

Focal moderate positivity in epithelium

CD34- strong positivity in mesenchymal tumor cells in contact or closer to epithelial layers and blood vessels

Indicate presence of embryonic fibroblast

(Ronell Bologna et al: Primordial odontogenic tumor: A immuohistochemical profile, 2017)

Dentigerous cyst

Ameloblasticfibroma

Cemento-ossifying fibroma

Odontogenic myxoma

Hyperplastic dental follicle

Loose collagenous fibrous connective tissue with myxoid areas

Small odontogenic epithelial nests

Lined on inner surface by epithelium derived from Enamel Organ

Epithelium never covers external surface

Doesn’t show expansile growth as big as lesions reported with POT

Highly cellular mesenchymal tissue

Surface has single layer of odontoblasts adjacent to enamel organ

Represent primordial ectomesenchymal odontogenic proliferation with features closely resembling dental papilla

(Mosqueda- Taylor et al ; Primordial odontogenic tumor: Clinicopathological analysis of 6 cases of previously undescribed entity: 2014)

Only

difference

Enucleation of lesion by separating it from the adjacent bone along with the impacted tooth

No recurrence has been reported so far

Primordial odontogenic tumor (POT) was first reported by Mosqueda-Taylor et al in 2014.

Classified into benign mixed epithelial and mesenchymal odontogenic tumour in the 4th edition of the World Health Organization (WHO) classification of Head and Neck tumours in 2017.

Radiographic appearance and histopathological features

However, there are only eight reported cases so far, therefore, pathogenesis and the biological properties of this tumor are not well understood.

Reported case of 5 year old patient with POT

5-year-old Japanese boy

Chief complaint of delayed eruption of the second deciduous molar in the right mandible

No relevant medical or family history

Intraoral examination revealed mobility of the adjacent first deciduous molar, and buccal swelling in the affected area of the mandible with no pain

Normal overlying mucosa

Surgical excision of the lesion was performed under general anesthesia

The tumor was detached from adjacent bone, and surgically enucleated in one piece

Vimentin

CK 18

Mitotic activity of tooth germ

CD 34

All these support that the mesenchymal

component of POT is derived from

dental papilla of primordial tooth germ

10-20th week- cap to late bell stage

FOR CASE REPORT

TOPIC ITEM CHECKLIST ITEM DESCRIPTION REPORTED

ON PAGE

TITLE 1 The area of focus and “case report” should appear in the title

KEYWORDS 2 Two to five key words that identify topics in this case report

ABSTRACT 3a Introduction – What is unique and why is it important?

(UNSTRUCTURED) 3b The patient’s main concerns and important clinical findings

3c The main diagnoses, interventions, and outcomes

3d Conclusion—What are one or more “take-away” lessons?

INTRODUCTION 4 Briefly summarize why this case is unique with medical literature

references.

PATIENT INFORMATION 5a Demographic information

5b Main concerns and symptoms

5c Medical, family, and psychosocial history including genetic information

5d Relevant past interventions and their outcomes

CLINICAL FINDINGS 6 Relevant physical examination (PE) and other clinical findings

TIMELINE 7 Relevant data from this episode of care organized as a timeline (figure or table) X

DIAGNOSTIC

ASSESSMENT

8a Diagnostic methods (PE, laboratory testing, imaging, surveys)

8b Diagnostic challenges

8c Diagnostic reasoning including differential diagnosis

8d Prognostic characteristics when applicable

THERAPEUTIC

INTERVENTION

9a Types of intervention (pharmacologic, surgical, preventive)

FOLLOW UPS AND

OUTCOMES

10a Clinician and patient-assessed outcomes when appropriate -

10b Important follow-up diagnostic and other test results X

DISCUSSION 11a Strengths and limitations in the approach to this case X

11b Discussion of the relevant medical literature

primordial odontogenic tumor

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