Odontogenic Keratocyst

Definition

Odontogenic cyst characterized by corrugated surface and basal palisading, sometimes associated with the nevoid basal cell (Gorlin's) syndrome.

Clinical Features

≈10% of cysts of jaw
Divided into:
- solitary or primordial (90%)
- multiple (10%):
  - component of nevoid basal cell carcinoma syndrome (Gorlin's syndrome):
    - together with:
      - cutaneous nevoid basal cell carcinomas
      - numerous skeletal abnormalities1,2
      - rarely, keratocysts of similar appearance in various skin locations 3
    - transmitted by autosomal dominant gene having high penetrance and variable expressivity4,5
    - mutation of the human homologue of Drosophila patched (PTC) gene is thought to be molecular defect 6
- Mean age 40 years
- Swelling and pain more common with than with dentigerous cyst

Gross Pathology

Most frequently:
- third molar region of mandible (Fig. 1
  Fig. 1: Radiograph demonstrating portion of maxillary odontogenic keratocyst in a 15-year-old with a 2-week history of facial swelling.
  )
- multilocular

Histopathology

Cavity:
- contains cheesy material consisting of keratinous debris
- white, subtly wrinkled surface
Epithelial lining characterized by:
- regimentation of basal layer
- wavy, corrugated, or mildly verrucous surface of parakeratotic squamous epithelium:7–9
- some propose separation from cystic lesions exhibiting orthokeratinized lining on basis of lesser degree of aggressiveness with latter 10,11

Diagnosis

Usually diagnosed on histologic features 12,13

Differential Diagnosis

Select up to 2 differential diagnoses to compare with Odontogenic Keratocyst

Other epithelium-lined cysts of maxilla or mandible14–18

Prognosis

Recurrence rate up to 60%:
- budding of epithelial lining within cyst wall 19

References

1 Gorlin RJ, Vickers RA, Kelly E, Williamson JJ. The multiple basal cell nevi syndrome. Cancer. 1965;18:89–104.

2 Rayner CRW, Towers JF, Wilson JSP. What is Gorlin's syndrome? The diagnosis and management of the basal cell naevus syndrome, based on a study of thirty-seven patients. Br J Plast Surg. 1977;30:62–67.

3 Hamel AF, den Dunnen AFA, Suurmeiger AJH. The cutaneous keratocyst: a rare hallmark of the nevoid Basal cell carcinoma syndrome. Int J Surg Pathol. 2003;11:36.

4 Donatsky O, Hjörting-Hansen E, Philipsen HP, Fejerskov O. Clinical, radiologic, and histopathologic aspects of 13 cases of nevoid basal cell carcinoma syndrome. Int J Oral Surg. 1976;5:19–28.

5 Gorlin RJ, Pindborg JJ, Cohen MM. Syndromes of the head and neck. 2nd ed.. New York: McGraw-Hill; 1976;.

6 Zedan W, Robinson PA, High AS. A novel polymorphism in the PTC gene allows easy identification of allelic loss in basal cell nevus syndrome lesions. Diagn Mol Pathol. 2001;10:41–45.

7 Kramer IRH, Pindborg JJ, Shear M. Histological typing of odontogenic tumours. In World Health Organization: International Histological Classification of Tumours. 2nd ed.. Berlin: Springer-Verlag; 1992;.

8 Kramer IRH, Toller PA. The use of exfoliative cytology and protein estimations in preoperative diagnosis of odontogenic keratocysts. Int J Oral Surg. 1973;2:143–151.

9 Wysocki GP, Sapp JP. Scanning and transmission electron microscopy of odontogenic keratocysts. Oral Surg Oral Med Oral Pathol. 1975;40:494–501.

10 Li T-J, Kitano M, Chen X-M, Itoh T, Kawashima K, Sugihara K, et al. Orthokeratinized odontogenic cyst: a clinicopathologic and immunocytochemical study of 15 cases. Histopathology. 1998;32:242–251.

11 Wright JM. The odontogenic keratocyst. Orthokeratinized variant. Oral Surg Oral Med Oral Pathol. 1981;51:609–618.

12 Brannon RB. The odontogenic keratocyst. Oral Surg Oral Med Oral Pathol. 1976;42:54–72.

13 Hodgkinson DJ, Woods JE, Dahlin DC, Tolman DE. Keratocysts of the jaw. Cancer. 1978;41:803–813.

14 Regezi JA, Courtney RM, Batsakis JG. The pathology of head and neck tumors. Part 12. Cysts of the jaws. Head Neck Surg. 1981;4:48–57.

15 Gardner DG, Sapp JP, Wysocki GP. Odontogenic and “fissural” cysts of the jaws. Pathol Annu. 1978;13(Pt 1):177–200.

16 Gorlin RJ. Potentialities of oral epithelium manifest by mandibular dentigerous cysts. Oral Surg Oral Med Oral Pathol. 1957;10:271–284.

17 Moursh F. A roentgenographic study of dentigerous cysts. Oral Surg Oral Med Oral Pathol. 1964;18:466–473.

18 Shear M. Cysts of the oral region. Bristol: John Wright & Sons, Ltd.; 1976;.

19 Stoelinga PJW, Cohen MM, Morgan AF. The origin of keratocysts in the basal cell nevus syndrome. J Oral Surg. 1975;33:659–663.

Last updated: 29 Oct 2006