Oral Pathology Short Case 3 - The radiolucent lesion in the mandible in a 13-year-old male

 Answer : Odontogenic keratocyst

Epidemiology

• Developmental cyst

• 2 peaks – 2nd and third decades and 50-70 years

• Male predilection

• 5 % with Gorlin Goltz syndrome

• PTCH 1 gene – mutation – activation of SHH pathway – aberrant proliferation

• 80 % in mandible (Body and ramus), posterior maxilla mostly associated with Gorlin Goltz syndrome

• Mechanism of expansion: Radicular/Dentigerous cysts – hydrostatic pressure, OKC- Epithelial cell proliferation

Table 2: Gorlin goltz features – autosomal dominant- characteristic facies with frontal and temporal bossing –head circumference >60cm)

Major criteria	Minor criteria
Multiple basal cell ca occur before 20 years OKC confirmed histologically >3 palmer and planter pits  Bilamellar calcification of falx Rib abnormalities that include bifid, fused or significant splaying 1st degree relative with naevoid basal cell ca syn.	Cutaneous defects Osseous anomalies Ophthalmic anomalies Neurological anomalies Sexual anomalies
A diagnosis can be made if the patient has: 1. Two major criteria 2. One major and two minor criteria 3. One major criterion and genetic confirmation

Histopathology 

• Ininflmed fibrous wall lined by a folded, thin, regular, parakeratinized, epithelium 5-8 cell layers thick without rete ridges 

• Parakeratinized surface is corrugated 

• Small satalite layer cyst in the solid islands the wall may have budding of the basal layer 

Treatment :

• Small lesions – Enucleation

• Surgical resection – Large lesions 

• Overall recurrence 25 %

• Recurrence rate with enucleation after carnoys solution – 8 %

• Sporadic and syndromic – Management is the same

• What is the healing mechanism of the marsupialization – Squamous metaplasia 

Table 3 PTCH gene mutation

Germline	Gorlin goltz syndrome
Sporadic	Isolated OKC

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