|Year : 2013 | Volume
| Issue : 1 | Page : 28-32
Massive keratocystic odontogenic tumor of mandible crossing the midline in 11-year child: An unusual case report and its management
AN Sulabha1, Sameer Choudhari2, Umesh Kenchappa2, Shrishal Totad3
1 Department of Oral Medicine and Radiology, Al-Ameen Dental College and Hospital, Bijapur, Karnataka, India
2 Department of Oral and Maxillofacial Surgery, Al-Ameen Dental College and Hospital, Bijapur, Karnataka, India
3 Department of Conservative and Endodontics, Al-Ameen Dental College and Hospital, Bijapur, Karnataka, India
|Date of Web Publication||6-Apr-2013|
A N Sulabha
Department of Oral Medicine and Radiology, Al-Ameen Dental College and Hospital, Athani Road, Bijapur-586108, Karnataka
Source of Support: None, Conflict of Interest: None
Introduction: Odontogenic keratocyst (OKC) is now designated by World Health Organization (WHO) as keratocystic odontogenic tumor (KCOT). The OKC involves approximately 11% of all the cysts in jaws. OKC possesses tumor-like characteristics because of its clinical behavior. Incidence of occurrence of this lesion in nonnevoid basal cell carcinoma syndrome patients before ten is low. Case Report: We report a massive OKC in the anterior region of mandible in a child. Combination of age, sex, size of the lesion, its location, and rapid growth in the present case makes it different from other KCOTs. Our management plan aimed to preserve the natural dentition, shape, function, and continuity of mandible. Discussion: An aggressive treatment modality like enucleation in combination with Carnoy's solution application, as done in the present case might be considered as a viable treatment modality for massive KCOT. The present paper also highlights brief discussion concerning the management of OKC.
Keywords: Carnoy′s solution, child, odontogenic keratocyst
|How to cite this article:|
Sulabha A N, Choudhari S, Kenchappa U, Totad S. Massive keratocystic odontogenic tumor of mandible crossing the midline in 11-year child: An unusual case report and its management. Dent Hypotheses 2013;4:28-32
|How to cite this URL:|
Sulabha A N, Choudhari S, Kenchappa U, Totad S. Massive keratocystic odontogenic tumor of mandible crossing the midline in 11-year child: An unusual case report and its management. Dent Hypotheses [serial online] 2013 [cited 2019 Dec 11];4:28-32. Available from: http://www.dentalhypotheses.com/text.asp?2013/4/1/28/110179
| Introduction|| |
Ever since Philipsen described the odontogenic keratocyst (OKC) in 1956, the lesion has continued to raise a considerable clinical interest because of its unusual growth pattern and tendency to recur after surgical removal.  OKC is now designated by World Health Organization (WHO) as keratocystic odontogenic tumor (KCOT) and is defined as a benign uni- or multicystic, intraosseous tumor of odontogenic origin, with a characteristic lining of parakeratinised strafied squamous epithelium and potential for aggressive infiltrative behavior. WHO recommends the term KCOT as it better reflects its neoplastic nature. ,
KCOTs are relatively common developmental cysts known for its rapid growth and its tendency to invade the adjacent tissue including the bone. , It is one of most aggressive, odontogenic cyst of the oral cavity. KCOTs are generally thought to be derived from either the epithelial remnants of the tooth or basal cell layer of the surface epithelium. Some authors suggest that as many as half of KCOTs are related to the nevoid basal cell carcinoma syndrome (NBCCS) or Gorlin syndrome. These lesions grow to sizes larger than any other odontogenic cyst. They more often penetrate the bone rather than expanding and growing in anterior to posterior direction. Despite this aggressive growth, they often remain asymptomatic.  Unfortunately there is no consensus on uniform treatment plans and the recommended surgical management varies from marsupialization and enucleation to enbloc resection. 
| Case Report|| |
An 11-year-old child reported to the Department of Oral Medicine and Radiology with complaint of painless swelling of lower jaw since 8-10 weeks. The swelling progressively increased to reach the present size.
Extraoral examination revealed considerable asymmetry of face due to swelling in lower jaw making the mandible prognathic [Figure 1]. Intraoral examination revealed a single, diffuse swelling extending from right first molar to left first molar [Figure 2]. Swelling was not tender, hard in consistency with expansion of cortical plates from right first molar to left first molar and more prominent in right and left premolar regions. There was no mobility of permanent teeth expect in deciduous right and left molars. Displacement of lower anterior teeth and right second premolar was noted. All permanent teeth were vital.
Panoramic view revealed large, multilocular radiolucency with well defined scalloped borders extending from mesial surface of right first molar to mesial surface of left first molar [Figure 3]. Thinning of inferior border of mandible was noted. Multiple teeth (right permanent canine, first premolar and left second premolar) in various stages of root formation were seen to be floating and displaced within radiolucency. Root resorption was seen only with the deciduous molars (right first molar and left second molar), with the displacement of lower anterior permanent teeth. Inferior alveolar canal was not clearly visible, with possibility of its displacement to inferior border of the mandible. Occlusal view showed the expansion of cortical plates. Aspiration was positive for the thick yellowish fluid and it demonstrated low soluble protein content (<3.75 g/100 ml). General physical examination did not reveal any syndromic features and a provisional diagnosis of KCOT were made.
Under all aseptic precaution, preoperatively root canal therapy was done for 31, 32, 33, 41, 42, 43, and 45 teeth. Under general anesthesia, patient was taken for enucleation in combination with Carnoy's solution. Crevicular incision was given from right first molar to left first molar along with releasing incision. The purpose of this was to get the good exposure of the lesion, and to maintain the vascularity and anatomy of the gingiva as permanent teeth were retained. Drill holes were made on buccal cortical plate and cyst was exposed. The cyst was carefully enucleated along with all the teeth impacted in the lesion [Figure 4].
Chemical curettage was done by applying Carnoy's solution to the enucleated cavity. Care was taken to avoid its contact with soft tissue and nerves. Deciduous right first molar and left second molar, and deciduous canine were extracted. Apextomy of root canal treated teeth was done to ensure complete sealing of the apex. Reconstruction plate fixation was done to achieve stability, contour, and continuity of chin. Histopathological examination of surgical specimen revealed thin parakeratinised stratified squamous epithelial lining with palisading of basal cell layer. Epithelium was of uniform thickness without rete ridges thrown up into folds and surface corrugations. Cyst wall mainly consist of plump fibroblast with scanty inflammatory cells, chiefly lymphocytes confirming the diagnosis of KCOT [Figure 5].
Postoperative healing was uneventful. Follow-up of 1 year showed excellent osseous regeneration [Figure 6], with marked improvement in facial profile in lower one-third of face [Figure 7] and further follow-up was strongly recommended to the patient.
| Discussion|| |
Mikulicz in 1876 first described the KCOT as a part of familial condition affecting the jaws, but the term OKC was first introduced by Philipsen in 1956.  Three quarters of all OKCs occur in the mandible, especially in the posterior body and ramus, anterior mandible is an uncommon site with the lesion crossing the midline being a unique occurrence. , The present case is a massive multilocular lesion in anterior mandible crossing the midline extending from right permanent first molar to left permanent molar.
The age distribution appears to be bimodal. There appeared to be two incidence peaks between 25-34 years and 55-65 years of age.  OKCs are usually seen in children demonstrating the NBCCS.  The present case was seen in healthy female child, was rapidly growing, massive in size and located in the mandibular anterior region crossing the midline which is very unusual. Rapid growth of the lesion noted within 8-10 weeks in the present case may be due to the aggressive nature of lesion, as reported earlier by many authors particularly in young children. 
OKCs have tendency for intraosseous growth, more often in a longitudinal than in transverse direction, without the obvious expansion of cortex. The lesion demonstrates a well defined unilocular or multilocular radiolucency with smooth and often corticated margin. , The present case demonstrated cortical plate expansion which is unusual.
In the selection of most appropriate treatment for OKCs, one must take into consideration both, patient and lesion characteristics.  The goal of treatment should involve eliminating the potential for recurrence while minimizing surgical morbidity. Eyre and Zakrzewska in 1985 stated the following treatment options for KCOT:  (i) Enucleation: With primary closure, packing, chemical fixation, and cryosurgery. (ii) Marsupialization: Only, followed by enucleation. (iii) Resection.
Morgan and colleagues  categorized surgical treatment methods for KCOT as conservative or aggressive. Conservative treatment is a cyst oriented, and thus includes enucleation with or without curettage or marsupialization. Its advantage is preservation of anatomical structures (including the teeth), which is advocated because KCOTs commonly present in the younger patient. It has been asserted that conservative approach is applicable not only to all age groups, but also to patients with NBCCS. ,
Some surgeons believe that the cyst can be properly treated with enucleation if the lesion is removed intact. However, simple enucleation may result in unnecessarily high recurrence rate, as complete removal of KCOT can be difficult because of the thin friable epithelial lining, limited surgical access, skill and experience of the surgeon, cortical perforation, and desire to preserve the adjacent vital structures. Great care must therefore be taken to ensure complete removal of the cyst lining without leaving behind remnants attached to adjacent bone or soft tissue mucosa overlying the lesion.  The use of cryotherapy using a liquid nitrogen spray in combination with enucleation has shown to reduce the recurrence. However, paresthesia and anesthesia have been noted with their use. 
Many authors marked marsupialization as definitive treatment for OKC.  Marsupialization relieves the pressure from cystic fluid, allowing reduction of cystic space, and facilitating bone apposition to the cystic walls. Patient must be cooperative and stent may need to be placed in the cyst to maintain its opening, and it requires the irrigatation of the cyst at least twice a day. Major disadvantage of marsupialization is compliance required by the patient for a prolonged period of time and its failure to eradicate the lesion will subsequently require enucleation or resection. On the other hand, enucleation has advantage of providing a complete specimen for histopathology. ,
Aggressive treatment addresses the neoplastic nature of KCOT and includes peripheral osteotomy, chemical curettage with Carnoy's solution, or Enbloc resection.  Aggressive modalities have generally been recommended for NBCCS cases, large KCOTs, and recurrent lesions. 
Multidisciplinary sequential treatment (MST) approach consists of decompression, scaling of the capsule wall of KCOT, cutting and grinding of sclerotin of bone cavum, and cauterizing bone cavum by using carbolic acid. 
Recurrence rate ranges from 2.5 to 62 %  in KCOTs and they occur due to incomplete removal of the original cyst's lining, thin friable cystic lining, growth of the new OKC from small satellite cyst of odontogenic epithelial cell rests left behind by surgical treatment, or by development of an unrelated OKC in an adjacent region of jaw which is interpreted as a recurrence.  Resection despite a recurrence rate of nil is not significantly better at elimination recurrences than enucleation plus Carnoy's solution or marsupialization plus cystectomy.
In the present case a massive OKC was seen in anterior region of mandible crossing the midline. The things that make this case different from others are combination of age, sex, size of the lesion, its location, and its rapid growth. Considering young age of the patient, subsequent growth of the mandible, enucleation in combination with Carnoy's solution was performed to minimize invasiness and recurrence. The advantages of the technique in the present case are complete eradication of the lesion; preservation of the permanent dentition; potential for recurrence is less; preservation of continuity of mandible, its function, and its shape; and excellent healing was noted after 1 year follow-up.
| Conclusion|| |
Although the literature contains many reports regarding management of KCOT, debate still exists as to the most effective treatment for this lesion. It may be concluded that an aggressive treatment modality like enucleation in combination with Carnoy's solution application as done in the present case might be considered as viable treatment modality for massive KCOT. Preoperative endodontic therapy as in the present case can be helpful in the retention of the permanent dentition; which not only improves the esthetics and speech, but also has psychological implications that helps in regaining the self-confidence of the child. Long term follow-up is an essential aspect of KCOT treatment plan as recurrences are more.
| References|| |
|1.||Zahrani AA. Odontogenic keratocyst: A case report in five-year-old boy. Saudi Dent J 1994;6:27-30. |
|2.||Rajkumar CG, Hemalath M, Shashikala R, Sonal P. Massive keratocystic odontogenic tumor of the mandible: A case report and review of literature. Indian J Dent Res 2011;22:181, 205-9. |
|3.||Madras J, Lapointe H. Keratocystic odontogenic tumour: Reclassification of odontogenic keratocyst to tumour. J Can Dent Assoc 2008;74:165-165h. |
|4.||Singh M, Gupta KC. Surgical treatment of odontogenic keratocyst by enucleation .Contemp Clinc Dent 2010;1:263-7. |
|5.||Oda D, Rivera V, Ghanee N, Kenny EA, Dawson KH. Odontogenic keratocyst: The northwestern USA experience. J Contemp Dent Pract 2000;1:60-74, 1-10. |
|6.||Godhi SS, Kukreja P. Keratocystic odontogenic tumor: A review. J Maxillofac Oral Surg 2009;82:127-31. |
|7.||Stoelinga PJ. Long term follow-up on keratocysts treated according to a defined protocol. Int J Oral Maxillofac Surg 2001;30:14-25. |
|8.||Blanas N, Freund B. Treatment of odontogenic keratocyst by enucleation with adjunctive therapy has lower recurrence rates. Oral Surg Oral Med Oral Pathol Oral Radiol Endod 2000;90:553-8. |
|9.||Dowling PA, Fleming P, Saunders ID, Gorlin RJ, Napier SS. Odontogenic keratocyst in a 5-year-old: Initial manifestations of nevoid basal cell carcinoma syndrome. Pediatr Dent 2000;22:53-5. |
|10.||Morgan TA, Burton CC, Qian F. A retrospective review of treatment of odontogenic keratocyst. J Oral Maxillofac Surg 2005;63:635-9. |
|11.||Pogrel MA, Jordan RCK. Marsupialization as a definitive treatment for the odontogenic keratocyst. J Oral Maxillofac Surg 2004;62:651-5. |
|12.||Giuliani M, Grossi GB, Lajolo C, Bisceglia M, Herb KE. Conservative management of large odontogenic keratocyst: Report of case and review of literature. J Oral Maxillofac Surg 2006;64:308-16. |
[Figure 1], [Figure 2], [Figure 3], [Figure 4], [Figure 5], [Figure 6], [Figure 7]