Non-Syndromic Multiple Keratocystic Odontogenic Tumor: A Case Report

A Keratocystic Odontogenic Tumor (KCOT) is a benign odontogenic lesion that is well-known for its histopathological features and invasive clinical behaviors. KCOTs may be non-syndromic or syndromic, and the syndromic type presents a higher risk of recurrence and invasive behaviors. Non-syndromic KCOTs are uncommon and account for only 5% of the cases. The purpose of the present case report is to conduct clinical, radiographic and pathologic evaluations and treat a 37-year-old man with non-syndromic nevoid basal cell carcinoma. Syndromic and non-syndromic keratocysts present more invasive behaviors than the solitary type. The initial symptoms of syndromic keratocysts appear as multiple keratocysts of the mandible and maxilla that are diagnosed by a dentist; as a result, the diagnosis and treatment of the disease is very crucial. DOI : 10.14302/issn.2379-8572.joa-17-1668 Corresponding Author: safoura seifi, Departement of Oral and Maxillofacial pathology, Tel: +98 3229 1408 -9, Fax: +98 3229 1093, Email: sf_seify@yahoo.com. Running title: Non-syndromic multiple keratocystic odontogenic tumor


Introduction
Odontogenic keratocyst is a developmental odontogenic cyst that requires special considerations due to its histopathological features and invasive clinical behaviors. The WHO has recommended the term KCOT to be used as it better reflects the molecular genetic changes in the cyst, which resemble the changes in certain neoplasms [1] .
Multiple odontogenic keratocysts are normally associated with concomitant cutaneous, skeletal, ocular and neurological anomalies as part of nevoid basal cell carcinoma syndrome or the Gorlin-Goltz syndrome, [2] although they may also be associated with the Simpson-Golabi-Behmel syndrome, the orofacial digital syndrome, the Noonan syndrome and the Ehler-Danlos syndrome [2][3][4][5] . Non-syndromic multiple odontogenic keratocysts are uncommon and only about 5% of the patients report this type [2] .
KCOTs originate from the remnant of the dental lamina and normally affect a wide age range but affect men very little [1] . The most common sites involved in odontogenic keratocysts include the posterior mandible body and the ramus areas [1] . Its syndromic type usually occurs in the first decades of life and presents a higher risk of recurrence as well as more invasive behaviors compared to the non-invasive type. Odontogenic keratocysts may turn into invasive neoplasms such as ameloblastoma and squamous cell carcinoma. The syndromic and non-syndromic types of keratocysts are more invasive than the solitary types [6] .
Given that multiple KCOTs are very rare and may be the first symptom of the syndromic condition and since the non-syndromic type is less common, the present study was conducted to perform clinical, radiographic and pathologic evaluations and treatment non-syndromic multiple KCOTs involving the mandible and maxilla in a 37-year-old man.

Case Report
The patient was a 37-year-old man who had undergone periodontal treatment eight years before due to gum disease. He presented to a dentist about two   (Figures 4 and 5). The patient underwent marsupialization in the symphysis and the lateral ramus for treatment and was then followed up with for the purpose of this study for 8 months.No recurrence was seen.

Discussion
Solitary keratocysts are more common than syndromic and non-syndromic multiple odontogenic keratocysts [4] . Habibi  in the Iranian population in 2016 [9] . The case reported in this study was a 37-year-old man with non-syndromic multiple keratocysts in his mandible and maxilla, who findings. Kurdekar [10] and Bartake [11] found the maxilla to be the target area of non-syndromic keratocysts, which is inconsistent with this case report. Swelling, pain, drainage and discharge of pus and delays in tooth eruption have been reported as the causes of presenting with non-syndromic multiple keratocysts. The present case report is consistent with the results obtained by Bartake [11] , Kurdekar [10] and Auluk et al. [3] in terms of the symptoms of involvement, but inconsistent with the results obtained by Parikh [12] .
This case report is on the third case of nonsyndromic multiple keratocysts in the Iranian population as per the review of English articles on the subject in Iran. The non-syndromic type appears to be less common than the syndromic type in the Iranian to the multifocal nature of keratocysts [3] .
The present case report found no symptoms in the skin of the patient's hands and legs and in his appearance in the clinical examination, and also no symptoms in his skull and chest radiographs.
The radiographs in this case report showed seven well-circumscribed unilocular radiolucencies in the mandible and maxilla.
The case report presented by Bartake et al. showed non-syndromic multiple keratocysts and multiple radiolucencies with ill-defined borders in the maxilla [11] . Rai et al. reported multiple radioloucencies with well-defined borders in both the mandible and maxilla [6] . in non-syndromic multiple keratocysts [13] .
Cases of non-syndromic multiple keratocysts are reported mainly to highlight the importance of further examining therapeutic methods. Numerous therapeutic methods are used for the treatment of keratocysts, including simple curettage, enucleation, marsupialization and resection [14] . Some researchers believe that marsupialization reduces inflammatory cytokeratins, especially IL1, in cases of multiple keratocysts [15] . Resection and iliac crest grafts are used in cases where the lesion is too large [14] .
Marsupialization was used to treat the cases of nonsyndromic multiple keratocysts reported and no recurrences were observed over a six-month period.