Odontogenic Keratocyst Case Study
Odontogenic keratocyst (OKC) is a common developmental odontogenic cyst affecting the maxillofacial region. Multiple OKCs are usually seen in association with nevoid basal cell carcinoma syndrome (NBCCS) but approximately 5% of patients with OKC have multiple cysts without concomitant syndromic presentation. This report represents a case of multiple OKCs in a non-syndromic patient
Key Words: Multiple Odontogenic Keratocyst, Gorlin-Goltz Syndrome, Non-Syndromic
Odontogenic keratocyst (OKC) is a developmental odontogenic cyst with specific histopathologic features and clinical behavior. Several investigators suggested that OKCs must be regarded as a benign cystic neoplasm rather than a cyst.
In the latest WHO classification of odontogenic tumors in 2005, these lesions have been given the name “karatocystic odontogenic tumors” (KCOTs) .
Multiple KCOTs are usually seen with cutaneous, skeletal, ocular and neurologic abnormalities as a component of nevoid basal cell carcinoma syndrome (NBCCS). The features of this syndrome were first described by Gorlin and Goltz in 1960, so it is also recognized as Gorlin- Goltz syndrome .
KCOTs in the jaws arise from the cell rests of dental lamina and usually are seen during the second to fourth decades of life with a slight male predilection . Typically, multiple KCOTs have been known to occur in association with NBCCS, but rarely may they be seen without concomitant syndromic manifestations .
This study reports a case of multiple KCOTs without any syndromic manifestations.
An 11- year-old boy with a complaint of swelling in left side of the upper jaw was referred to our clinic.
Systemic signs and symptoms, past medical history and hematologic tests were within normal limits. The radiographies from chest and skull were unremarkable and no cutaneous abnormality was revealed. In panoramic radiograph, two radiolucencies with corticated border were revealed around the unerupted mandibular left canine and the unerupted maxillary left second molar area. Maxillary second molar was displaced (Figure1).
Panoramic radiograph showing two cyst-like radiolucencies in upper and lower jaws
Regarding the radiographic examination and presence of unerupted teeth and their location, the initial differential diagnosis was dentigerous cyst and the second was KCOT. Other odontogenic cysts and tumors such as adenmatoid odontogenic tumor were considered as other differential diagnoses.
Enucleation of the cystic lesions was performed under local anesthesia and tissue samples were obtained for histopathologic examination. The surgical specimens were sheet- like with cystic appearance. After processing, the tissue samples were sectioned and stained with hematoxylin and eosin (H&E).
The histopathologic examination revealed that the cystic lining of mandibular lesion was corrugated parakeratinized epithelium with uniform thickness of 5-6 peg formation. The cyst wall was composed of a non-inflammatory fibrous connective tissue. All these features established the diagnosis of KCOT for the mandibular lesion (Figure 2a). But the maxillary lesion showed an inflammatory odontogenic cyst appearance with inflammatory cells infiltration in fibro-vascular connective tissue wall. The epithelial lining showed varying degrees of hyperplasia and rete ridge formation (Figure 2b).
Mandibular lesion photomicrograph showing parakeratinized stratified squamous epithelium of uniform 5-6 cell thickness and palisaded basal cell layer without rete pegs (H &E Stain. 10x) b Maxillary lesion photomicrograph showing an inflammatory...
With definitive diagnosis of KCOT in mandibular lesion and absence of any evidence of NBCCS in clinical examinations, multiple sections were cut from the maxillary lesion. These sections were obtained because of the probability of multiple non- syndromic KCOTs.
H & E slides were evaluated carefully and at last a small epithelial lining with characteristic features of KCOT was detected (Figure 2c).
According to these entire features which were correlated with the clinical and radiographic findings, the diagnosis of KCOT was established for both cystic lesions.
OKC is a common developmental odontogenic cyst and its biologic behavior is similar to a benign neoplasm . Therefore, in the latest WHO classification of odontogenic tumors in 2005, it has been given the term keratocystic odontogenic tumor .
KCOT may be found in any age with peak prevalence between 10 to 40 years old .
The mandible is involved in 60 to 80% of cases with a marked tendency to occur in the posterior body and ascending ramus [5, 7].
Small KCOTs are usually asymptomatic but larger ones may show clinical manifestations like pain, swelling or drainage . The presented case, revealed only the swelling.
Radiologically, KCOTs demonstrate a well defined radiolucent area with smooth and often corticated margins and may be unilocular or multilocular. In 25 to 40% of cases, an unerupted tooth is seen in association with the lesion . Radiographic findings in mandibular lesion showed a unilocular radiolucency in relation with an unerupted canine. In maxilla, a multilocular radiolucent lesion in relation with an unerupted second molar was seen; which had well corticated margins.
In this case, microscopic evaluation of the mandibular lesion showed characteristic features of KCOT inducing a corrugated parakeratinized stratified squamous epithelial lining with palisaded basal cell layer without rete ridge formation .
Maxillary lesion showed histopathologic features of an inflammatory odontogenic cyst, but regarding the possibility of multiple KCOTs in this patient, several sections were prepared to achieve the correct diagnosis.
Finally we revealed characteristic features of KCOT in small area of histopathologic slides and the diagnosis of other cysts, especially inflammatory dentigerous cyst, was ruled out.
Finally, the diagnosis of KCOT was established for both cystic lesions.
Multiple KCOTs are usually considered as a component of Gorlin- Goltz syndrome or NBCCS , orofacial digital syndrome , Ehler- Danlos syndrome , Noonan syndrome  or other syndromes.
Rarely, multiple KCOTs are seen without other syndromic manifestations .
Brannon  reported that 5.8 percent of 312 cases of KCOTs, had multiple cysts without any syndromic manifestations.
NBCCS is recognized by multiple KCOTs, nevoid basal cell carcinomas of the skin, bifid ribs, calcification of the falx cerebri and other features .
However, except for presence of KCOTs, our patient was healthy in clinical examinations and suggestive features of these syndromes such as basal cell carcinoma, skeletal or orofacial defects, stunted growth, bleeding diathesis, hyper-extensible skin and hypermobile joints and other features were not present.
Multiple KCOTs might be the first and the only manifestation of NBCCS without any other features associated with syndrome. However, other symptoms can occur in later decades of life .
Similar cases to the current case have been reported in a few published English articles.
Auluck et al.  discussed a 22 year-old patient with multiple recurrent KCOTs in all four quadrants with a complaint of pus drainage over the previous week without pain or facial swelling. The patient had no any other features associated with NBCCS.
Sholapurkur et al.  presented a 24 year-old case with multiple non-syndromic KCOTs in both jaws with chief complaint of a slow growing swelling since 3 years and drainage since 15 days. The swelling was associated with pain with gradual onset radiating to head on same side. Lesions were cyst-like radiolucencies associated with impacted teeth on panoramic radiograph.
Parikh  reported a 19- year-old case with two KCOTs in both jaws without any other concomitant syndromic features. The complaint was swelling for one year and pain for three months. Panoramic radiograph revealed two radiolucencies with corticated borders associated with impacted teeth.
Bartake et al.  reported a 20- year-old case with multiple recurrent KCOTs without any other noticeable features indicative of Gorlin syndrome. No recurrence occurred after 3- year follow up.
Guruprasad et al.  discussed a 16- year-old patient with multiple KCOTs and a complaint of slow progressing swelling in both jaws without any other features of syndrome.
Also, findings of Habibi et al.  study on Iranian populations showed that 8.1% of 83 cases with KCOTs, were associated with NBCCS and 7.6% of them had recurrence, but none of the cases with multiple KCOTs were non-syndromic.
Therapeutic interventions of KCOT include marsupialization and enucleation, combined with adjuvant cryotherapy with Carnoy’s solution, and marginal or radical resection .
For unerupted permanent teeth in children, conservative treatment should be done first, because an aggressive operation can cause adverse effects on teeth development and its eruption. Marsupialization followed by enucleation has the lowest recurrence rate among the conservative treatments .
KCOTs related to NBCCS have more aggressive behavior and higher recurrence rates than non- syndromic ones.
This characteristic is due to a high proliferation rate of the epithelial linings in the syndromic cases .
There is a little information about the relationship between cell proliferative markers and the recurrence rate of KCOTs. The study performed by kuroyanagi et al.  showed that ki- 67 expression, at time of diagnosis, may act as a prognostic marker. In order to prevent the recurrence of the tumor, ki- 67 labeling index consideration can be helpful in adjunctive surgical procedures.
Since our patient lived in a small village, following up and checking the recurrence of the lesions was impossible.
In any patient with a KCOT, the presence of multiple KCOTs should be considered. Therefore, careful histopathologic examination for any other existing lesion should be done. Moreover, a complete clinical examination and long- term follow up must be performed to detect any other features associated with NBCCS.
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Odontogenic keratocyst in anterior Mandible: An interesting case report
U Punitha Gnanaselvi, D Kamatchi, Keerthana Sekar, B Surya Narayanan
Department of Dental surgery, KAPV Government Medical College, Trichy, Kerala, India
|Date of Web Publication||18-Oct-2016|
Dr. U Punitha Gnanaselvi
Department of Dental surgery, KAPV Government Medical College, Trichy, Kerala
Source of Support: None, Conflict of Interest: None
Odontogenic keratocyst (OKC) is a common developmental odontogenic cyst affecting the maxillofacial region that arises from the dental lamina. The OKC is distinctive among jaw cysts given its tendency toward recurrence and aggressive clinical behavior. This article presents a case of OKC in the anterior mandible and a review of diagnostic aids and different treatment modalities.
Keywords: Anterior mandible, enucleation, odontogenic keratocyst
|How to cite this article:|
Gnanaselvi U P, Kamatchi D, Sekar K, Narayanan B S. Odontogenic keratocyst in anterior Mandible: An interesting case report. J Indian Acad Dent Spec Res 2016;3:22-4
|How to cite this URL:|
Gnanaselvi U P, Kamatchi D, Sekar K, Narayanan B S. Odontogenic keratocyst in anterior Mandible: An interesting case report. J Indian Acad Dent Spec Res [serial online] 2016 [cited 2018 Mar 11];3:22-4. Available from: http://www.jiadsr.org/text.asp?2016/3/1/22/192466
The term odontogenic keratocyst (OKC) was first used by Philipsen in 1956. It is one of the most aggressive odontogenic cysts of the oral cavity. OKC is known for its rapid growth and its tendency to invade the adjacent tissues including bone., KeratoCystic Odontogenic Tumor(KCOT) in the jaws arise from the cell rests of dental lamina and are usually seen during the second to fourth decades of life with a slight male predilection. The majority of patients are in the age ranges of 20-29 and 40-59 years, but cases ranging from 5 to 80 years have been reported. A total of 70% to 80% keratocysts are most commonly found in the lower jaw in the angle between jaw and mandibular branch and in the maxilla in the area of the third molar.,, Growth is chiefly in the anteroposterior dimension, and the lesions may attain remarkable size without significantly deforming the jaw skeleton. The particular tendency to rapid growth is due to the higher activity of the epithelial cells of the cyst lining, stimulating osteolytic activity of prostaglandin substances in the cell population of the cyst lining and the higher accumulation of hyperkeratotic scales in the lumen of the cyst, resulting in greater difference in hydrostatic pressure.
A 31-year-old male patient reported to the Department of Dental Surgery in MGM Government Hospital, Trichy, with the chief complaint of pus discharge in the chin region for the past 4 months. Patients’ general condition was normal. On extraoral examination, a diffuse swelling was noted in the lower jaw region extending right side of the parasymphysis to the left side. The swelling is soft, fluctuant, tender on palpation and extraoral sinus opening present on the left parasymphysis region with pus discharge. On intraoral examination, there is a diffuse swelling extending from distal surface of 35 to the distal surface of 45 with the obliteration of the vestibule. Retained deciduous teeth 73 and 83 were noted; 33 and 43 were clinically missing. Grade II mobility was present in 35, 34, 32, 31, 41, 42, and 44. Gross displacement of 34, 35, and 44 was also noted [Figure 1].
|Figure 1: (a and b) Clinical presentation: extraoral and intraoral images|
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Panoramic radiograph revealed multilocular radiolucent area extending from 37 to 47 region with well-demarcated borders. Thinning of cortical bone along with cortical expansion in mid-symphysis region was noted. Impacted 33 and 43 was noted. Resorption of root 34 was also noted [Figure 2]. CT scan mandible revealed an osteolytic lesion with buccal and lingual cortical plate expansion, and there is discontinuity of cortical plate in left parasymphysis region [Figure 3].
Fine Needle Aspiration Cytology(FNAC) was performed using 24-gauge needle attached to a 10-mL syringe. Aspirate was creamy white suspension with keratin flakes. Smears showed scattered mature and degenerated squamous cells and rare inflammatory cells in a necrotic background.
Intraosseous biopsy was done to obtain a slice of soft tissue along with the overlying bone. The histopathologic report revealed a cystic lining with orthokeratin layer, stratified squamous cells of 6-8-cell thickness, and focal granular layer was noted. Stroma was congested. No daughter cyst or epithelial detachment noted. The histological findings were suggestive of OKC [Figure 4].
Under general anesthesia, through intraoral incision, the mass was exposed. Enucleation of the lesion and removal of the involved teeth were accomplished. Soft tissues adhering to the capsule of the lesion in the buccal fenestration were also removed [Figure 5]. Then, peripheral ostectomy of the whole surgical bed was completed, followed by a single application of Carnoy's solution. The thinned out inner cortical lining of the bone was removed. The lesion contained white, cheesy material. The multilocular cystic lesion was sent for histopathologic examination.
Histopathologic evaluation revealed cystic lining with hyperplastic squamous cell with mild hyperparakeratosis and acanthosis. Stroma was congested with focal inflammatory cell infiltration. The fibrous connective tissue wall exhibited cholesterol clefts and “daughter cysts,” which are considered characteristic of OKC found [Figure 6].
OKC is a common developmental odontogenic cyst, and its biologic behavior is similar to a benign neoplasm. Therefore, in the latest WHO classification of odontogenic tumors in 2005, it has been given the term keratocystic odontogenic tumor. OKCs are generally thought to be derived from either the epithelial remnants of the tooth germ or the basal cell layer of the surface epithelium.,
KCOT may be found in any age, with peak the prevalence between the age 10 and 40 years. The mandible is involved in 60% to 80% of cases with a marked tendency to occur in the posterior body and ascending ramus. However in this case, the lesion was present in anterior mandible associated with impacted canine and involving contralateral jaw bone. Small OKCs are usually asymptomatic, but larger ones may show clinical manifestations such as pain, swelling, or drainage. Radiologically, KCOTs demonstrate a well-defined radiolucent area with smooth and often corticated margins and may be unilocular or multilocular. In 25% to 40% of cases, an unerupted tooth is seen in association with the lesion. The diagnosis of OKC is based on the histopathologic features. The radiographic findings, although often highly suggestive, are not diagnostic.
Therapeutic interventions of KCOT include marsupialization and enucleation, combined with adjuvant cryotherapy with Carnoy’s solution, and marginal or radical resection. OKCs/KCOTs are characterized by high tendency to postoperative recurrence (30%-60%). Causes of high recurrence rates include incomplete removal, remnants of dental lamina, and presence of daughter/satellite cysts within the cyst wall. Because recurrence may be long delayed in this lesion, follow-up of any case of OKC with annual radiographs is essential for at least 5 years after the surgery.
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[Figure 1], [Figure 2], [Figure 3], [Figure 4], [Figure 5], [Figure 6]