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Mona Shah

on 19 March 2013

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Transcript of CASE REPORT ON OKC

Dr. Xu-Xia Wang, PhD, DDS.
He is an associate professor at Department of Oral and Maxillofacial Surgery
Dr. Jung Zhang, MD, is a professor at Department of Orthodontics.
Dr. Feng-Cai Wei, MD is professor at Department of Oral Maxillofacial Surgery.
All at Shandong University School of Stomatology, in Jinan, Shandong, People's Republic of China. Review of Case Report by; Mona Shah and Sameer. ABSTRACT: The purpose of this paper was to present a family case report of nevoid basal cell carcinoma syndrome with multiple odontogenic keratocysts.
Multiple odontogenic keratocycts (OKCs) are principle features of Nevoid Basal Cell Carcinoma Syndrome (NBCCS) also reffered as Gorlin-Goltz syndrome.
NBCCS is a genetic disorder transmitted by an autosomal dominant gene with variable expressivity.
Common Findings include; The cysts of the jaws and certain appearance of the face, such as:
large calvaria (increased size of skull cap)
high-arched eyebrows
broad nasal root
mild hypertelorism (Increase distance between eyes).
Before-therapy and after-therapy diagnosis is therefore important. Genetic counseling and examination may also be indicated.
The features identified by these combined clinical, imaging, and histologic findings are described, along with a brief mention of the family history and a review of the literature. INTRODUCTION The odontogenic keratocyst (OKC) is a jaw cyst with an aggressive clinical behavior.
high recurrence rate.
The cyst may occur in all ages, but with a peak incidence in the second and third decade of life.
Most OKCs present as solitary lesions.
Multiple primary cysts may occur, the most common association being with nevoid basal cell carcinoma syndrome (NBCCS).
The purpose of this paper was to report similar clinical findings in a son and mother with multiple odontogenic keratocysts, where the findings on plain films, computed tomography (CT), and histopathologic examinations are com- pared and contrasted. CASE REPORT PATIENT PROFILE PRESENTING COMPLAIN:
A 13-year-old Chinese male presented for orthodontic treatment to the Department of Orthodontics in School of Stomatology at Shandong University, Jinan, Shandong, People’s Republic of China.

Family history revealed that his mother once underwent an operation to excise multiple cysts in the jaws and associ- ated teeth at the age of 32 years.

An intraoral examination revealed poor occlusion, malalignment of existing teeth, and 5 unerupted permanent teeth in the right maxilla and the mandibular anterior region.

large calvaria, a broadened nasal root, high- arched eyebrows, and mild hypertelorism. INVESTIGATIONS Patient's Panoramic Radiagraph was taken.
This was followed by a CT scan.
They were compared to the panoramic radiograph and computed tomography (CT) scan of the patient's mother. That showed multiple cystic lesion in the maxilla and mandible.
[ Following diagram shows Panoramic Radiograph of son (a) and mother (b) ] Patient's CT and Panoramic Radiograph revealed Multiple cystic lesions in the jaws associated with unerupted teeth.
They were on the right maxillary posterior area (a) and the mandibular anterior region (b).
The teeth were impacted to the edge. There was strong ring enhancement.
The well-defined border of the right maxillary sinus on the Waters’ view of the CT scan suggested a cystic lesion.
MANAGEMENT Surgical excision was done with peripheral osseous curettage was planned under a general anesthetic.
The cyst contained a white, cheesy material and contained the crown of a retained tooth within its lumen (Figure 3a).
The cyst epithelium proliferated formed a new cyst in the host tissue
but typical histological features were retained (Figure 3b).
The lesions were histologically reported as keratocysts and parakeratinized. Other relevant investigations were done, but no other associated symptoms were noticed.
Chromosomal analysis showed no abnormality, as compare to the mother whose chromosomal study of peripheral blood showed 46, XX.
Mother's pathology demonstrated Odontogenic and parakeratinized keratocysts. DISCUSSION The incidence of OKC among all cysts is 8%.
The OKCs have a tendency of recurrence and aggressive behavior which makes them more significant.
Some authors suggest half of OKCs are related to NBCCS
Multiple OKCs are a well-recognized feature of NBCCS.
Autosomal Dominant mode of inheri- tance.
All the clinical manifestations of NBCCS do not have to be present for a diagnosis.
Some authors think that the presence of multiple keratocysts (even just keratocysts) associated with family history is sufficient to establish NBCCS. Majority of authors make the diagnosis if 2 major criteria or 1 major and 2 minor criteria are presented.
Major Criterieas include:
OKCs of the jaws.
Nevoid basal cell carcinomas,
Palmar and plantar pits,
Cerebral falx calcifications,
Family history,
And Skeletal anomalies.
Minor Criterias inclue:
Ovarian calcification Early diagnosis and treatment is essential
Family detection and genetic counseling, are important to recognize when a patient has multiple OKCs, because lifelong monitoring is essential for patient management.
With respect to this patient, the authors can anticipate a high risk of recurrence because of the multiple lesions, pathologic findings of parakeratinized keratocysts, and the family history. LIMITATIONS
Although incidence of OKC was mentioned, there was no mention of
The demographic spread of OKZ,
Incidence in different races, for example 67 times more prevalent in caucasians than African-Americans.
Reference: http://www.jaypeejournals.com/eJournals/ShowText.aspx?ID=1435&Type=FREE&TYP=TOP&IN=_eJournals/images/JPLOGO.gif&IID=122&isPDF=NO.
Association of common sites and gender.
No reference was made to previous medical and dental history.
No differential diagnosis was given.
No reference was made to other cysts or conditions that may resemble OKC and hinder correct diagnosis.
No light was shed on Non NBCCS- associated OKCs.
Unclear as to what other investigations were carried out as they weren't mentioned. Was a biopsy taken?
CT scan of the mother could've been shown as since they mentioned comparing it.
Radiographic findings could've been explained more.
Histopathology slide could have been shown.
Other treatment options werent mentioned. Although the best treatment option is sugery in this case, why werent conservative treatment options such as marsupialization, and enucleation.
Management after sugery wasn't outlined properly.
Duration between follow ups not outlined specific to the patient was not outlined.
If recurrance occurs what treatment strategies are the hoping to take.
No reference to similar cases made. REFERENCES 1.BroumeRM.Theodontogenickeratocyst:Histological features and their correlation with clinical behavior. Br Dent J 1971;131: 249-59.2. StoelingaPJW,BronkhorstFB.Theincidence,multiple presentation, and recurrence of aggressive cysts of the jaws. J Craniomaxillofac Surg 1988;16:184-95.3. Voorsmit RACA. The Incredible Keratocyst [disser- tation]. Nijmegen, The Netherlands: The Catholic University of Nijmegen; 1984.4. Philipsen HP. Om keratocyster (kolesteatom) I kaek- berne. Tandlaegegebladet 1956;60:963-81.5. Browne RM. The odontogenic keratocyst. Clinical aspects. Br Dent J 1970;128:225–31.6. Brannon RB. The odontogenic keratocyst: A clini- copathological study of 312 cases. Part I. Clinical features. Oral Surg 1976;42:54-71.7. Gorlin RJ, Pindgorg JJ, Cohen MM. Multiple nevoid basal carcinoma syndrome. In: Gorlin RJ, ed. Syn- dromes of Head and Neck. 2nd ed. New York, NY: McGraw-Hill; 1976:520-6.8. Gorlin RJ, Goltz RW. Multiple nevoid basal-cell epi- thelioma, jaw cysts, and bifid rib: A syndrome. N Engl J Med 1960;262:908-12.9. Totten JR. The multiple nevoid basal cell carcinoma syndrome: Report of its occurrence in four generations of a family. Cancer 1980;46:1456-62.10. Kimonis VE, Goldstein AM, Pastakia B, Yang ML, Kase R, DiGiovanna JJ, et al. Clinical manifestations in 105 persons with nevoid basal cell carcinoma syn- drome. Am J Med Genet 1997;69;299-308. THANK YOU ANY
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