Multiple keratocystic odontogenic tumors in a non-syndromic minor patient: Report of an unusual case (original) (raw)

Case report: multiple keratocystic odontogenic tumour in a non-syndromal pediatric patient

European Archives of Paediatric Dentistry. Official Journal of the European Academy of Paediatric Dentistry.

Keratocystic odontogenic tumour (KCOT) is an odontogenic tumour which stems from the odontogenic organs mostly localised in the lower jaw, particularly posterior body and ascending ramus of the mandible. The majority of these tumours are single lesions. When detected in the jaw in multiple forms, these cysts are seen in association with Gorlin Goltz/Basal cell naevus syndrome. However a few cases of non-syndromal multiple keratocystic odontogenic tumour have been reported in the literature.

Non-syndromic multiple odontogenic keratocysts associated with dental anomalies: A report of unusual case and its management

Journal of Indian Academy of Oral Medicine and Radiology, 2015

Single odontogenic cysts are very well documented in the literature. Multiple odontogenickeratocysts (OKCs) are principle features of nevoid basal cell carcinoma syndrome (NBCCS; Gorlin syndrome). NBCCS is a genetic disorder transmitted by an autosomal dominant gene w expressivity, which is important to recognize when a patient has multiple OKCs. However, a case of multiple odontogenickeratocysts unassociated with any syndrome is reported here so as to add to the growing number of such cases in the literat possibility of this case being a partial expression of the Gorlin-Goltz syndrome is discussed.

Treatment of Keratocystic Odontogenic Tumors in Nevoid Basal Cell Carcinoma Syndrome

The Journal Of Korean Association of Maxillofacial Plastic and Reconstructive Surgeons, 2014

Nevoid basal cell carcinoma syndrome (NBCCS) is inherited as an autosomal dominant trait with variable conditions, including multiple basal cell carcinoma, numerous keratocystic odontogenic tumors (KOTs) in the jaws, ectopic calcification of the falx cerebri, bifid ribs, macrocephaly, kyphoscoliosis, cleft palate, frontal and temporal bossing, mild ocular hypertelorism, mild mandibular prognathism, vertebral fusion, and so on. A 16-year-old boy visited the Dong-A University Medical Center, requiring diagnosis and treatment of multiple cystic lesions. He presented with many conditions related to NBCCS, including multiple KOTs, bifid rib, cleft lip, frontal bossing, mild ocular hypertelorism, and mild mandibular prognathism. No characteristic cutaneous manifestations (nevoid basal cell carcinoma) were observed in this patient. We report on a case of multiple KOTs associated with NBCCS with a review of the literature.

Multiple Orthokeratinized Odontogenic Cysts: A Case Report

Head and Neck Pathology, 2014

The purpose of this report is to document the clinical, radiographic, pathological and molecular findings of the first case of multiple orthokeratinized odontogenic cysts (OOCs). Multiple odontogenic keratocysts are one of the major features of nevoid basal cell carcinoma syndrome (NBCCS), and loss of heterozygosity in the PTCH gene, the culprit gene for NBCCS, has recently been found in sporadic OOC cases. Therefore, in this presenting case, we also investigated the possibility that this patient might also have NBCCS, by comparing the available clinical information and the molecular findings of this case to the diagnostic criteria for NBCCS (as proposed by the First International Colloquium on NBCCS in 2011). However, this patient with multiple OOCs showed no evidence of having NBCCS. This conclusion supports the findings from previous case series based on sporadic cases that OOC does not appear to be associated with NBCCS.

Non-syndromic odontogenic keratocysts: A rare case report

National Journal of Maxillofacial Surgery, 2013

Odontogenic keratocysts (OKCs) are the most common form of cystic lesions affecting the maxillofacial region. [1] They are clinically aggressive lesions which are thought to arise from the dental lamina or its remnants. [2] The OKC was first described in 1876, [3] and named by Phillipsen in1956. [4] It is one of the most aggressive odontogenic cysts of the oral cavity. OKC is known for its rapid growth, [5-7] and its tendency to invade the adjacent tissues including bone. [3,7-9] It has a high recurrence rate, [9-11] and is associated many a times with nevoid basal cell carcinoma syndrome (NBCCS). [12] Usually, multiple OKCs occur as a component of NBCCS with concomitant cutaneous, skeletal, ophthalmic, and neurologic abnormalities. Gorlin and Goltz first described the spectrum of features associated with this syndrome in 1960; hence, it is also called Gorlin-Goltz syndrome. [13] Multiple OKCs have been known to occur in non-Syndromic cases, though it is very rare. [14] These multiple lesions may be the first manifestation of the NBCCS or otherwise it may be because of the multifocal nature of OKCs. [15,16] We discuss the possibility that the current case is a partial expression of NBCCS and briefly review the current trends in treatment of recurrent OKCs associated with this syndrome.

Multiple Odontogenic Keratocysts

World Journal of Dentistry, 2012

ABSTRACTOdontogenic keratocyst is a cyst of odontogenic origin that exhibits a keratinized epithelial lining. Odontogenic keratocyst has been the topic of numerous investigators and is known for its potentially aggressive behavior and significant rate of recurrences.1OKC cyst often occurs as a solitary lesion at the angle of the mandible, however in some instances, multiple such cysts may occur in association with a syndrome called “Nevoid basal cell syndrome”.1These patients have propensity to develop multiple neoplasm including basal cell carcinoma and medulloblastoma. Hence early diagnosis and treatment is of utmost importance in reducing the severity of long-term sequelae of this syndrome. Here we present a case of multiple odontogenic keratocysts with nevi and narrowed ribs which has profound relevance to specialist in Oral Medicine and Radiology and Oral and Maxillofacial Surgery.

Peripheral odontogenic keratocyst associated with nevoid basal cell carcinoma syndrome: a case report

Oral surgery, oral medicine, oral pathology and oral radiology, 2014

Peripheral odontogenic keratocyst (POKC) is a rare gingival cyst showing histologic features identical to those of keratocystic odontogenic tumor. A rare case of POKC associated with nevoid basal cell carcinoma syndrome (NBCCS) is presented. A 24-year-old woman with NBCCS presented with a pigmented papule, 3 mm in size, involving the lingual gingiva of the right canine area of the mandible. Based on a clinical diagnosis of benign pigmentation, an excisional biopsy was performed, and a histopathologic diagnosis of POKC was rendered. The lining cells were positive for the proteins GLI2, BCL2, keratin 8, keratin 17, and mTOR. TP53 and Ber-EP4 were also weakly positive. Gene mutational analysis on a buccal swab sample revealed 2 missense mutations in the PTCH1 gene. This case is a distinctive example of a genuine soft tissue counterpart of keratocystic odontogenic tumor, in which an aberrant PTCH1-GLI pathway played a considerable role in the pathogenesis.