Svrha ovoga rada bila je prikazati nastanak odontogenih keratocista (OKC) i keratocistama sličnih lezija u oboljelih od Gorlin-Goltzova sindroma s obzirom na dob i spol bolesnika te na mjesto nastanka OKC-a (donja čeljust, gornja čeljust, meka tkiva i maksilarni sinusi). Od godine 1965. do 1998. u 30 je bolesnika s Gorlin-Goltzovim sindromom ustanovljeno 58 OKC-a koji su potvrđeni i histopatološkom dijagnozom. Bolesnici su bili u dobi od 10 do 90 godina, prosječne dobi 33,71. Prosječna dob muških bolesnika bila je 35,53 godina života, a ženskih 31,12 godina života. OKC je u sklopu Gorlin-Goltzova sindroma češće dijagnosticiran u muškaraca (58,62%) nego u (41,38%). Najčešća dob u kojoj je OKC nađen u bolesnika s Gorlin-Goltzovim sindromom bila je između 21. i 30. godine. S obzirom na lokalizaciju, većina OKC-a nalazila se u donjoj čeljusti (60,34%), zatim u gornjoj čeljusti (15,52%), u mekim tkvima (13,79%) te u maksilarnim sinusima (10,34%).The aim of this study was to determine the appearance of odontogenic keratocysts (OKC) and keratocyst-like lesions ( KLL) in patients with Gorlin-Goltz syndrome, according to age and gender of participants, and in relation to the place of origin (mandible, maxilla, soft tissues, which include the mucosa of the alveolar ridge, skin of the nose and face, parotid gland together with maxillary sinuses). From 1965 until 1998, 58 OKC were found in 30 patients with Gorlin Goltz syndrome and confirmed by histopathologic analysis. Patients were aged from 10 to 90 years. Average age of the patients with Gorlin-Goltz syndrome was 33.71 years. Average age in males was 35.53 years and in females 31.12 years. OKC connected with Gorlin Goltz syndrome were more frequently found in males (58.62%) compared to the females (41.38%). The peak incidence of OKC in patients with Gorlin-Goltz syndrome was between ages 21-30 years. According to the location, OKC were found predominantly in the mandible (60.34%), the maxilla (15.52%), soft tissues (13.79%), and in maxillary sinuses (10.34%)

Božidar Pavelić

Davor Katanec

Karl Donath

Kruna Valter

Sonja Levanat

Vanja Vučićević-Boras

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A S CActa Stomatol Croat, Vol. 38, br. 1, 2004. 23Incidence of OdontogenicKeratocysts in Patients withGorlin-Goltz Syndrome accordingto Age, Gender and LocationSummaryThe aim of this study was to determine the appearance of odonto-genic keratocysts (OKC) and keratocyst-like lesions ( KLL) in patientswith Gorlin-Goltz syndrome, according to age and gender of partici-pants, and in relation to the place of origin (mandible, maxilla, softtissues, which include the mucosa of the alveolar ridge, skin of the noseand face, parotid gland together with maxillary sinuses). From 1965until 1998, 58 OKC were found in 30 patients with Gorlin-Goltz syn-drome and confirmed by histopathologic analysis. Patients were agedfrom 10 to 90 years. Average age of the patients with Gorlin-Goltzsyndrome was 33.71 years. Average age in males was 35.53 years andin females 31.12 years. OKC connected with Gorlin-Goltz syndromewere more frequently found in males (58.62%) compared to the females(41.38%). The peak incidence of OKC in patients with Gorlin-Goltzsyndrome was between ages 21-30 years. According to the location,OKC were found predominantly in the mandible (60.34%), the maxil-la (15.52%), soft tissues (13.79%), and in maxillary sinuses (10.34%).Key words: odontogenic keratocysts, Gorlin-Goltz syndrome.Boæidar PaveliÊ1Kruna Valter2Vanja VuËiÊeviÊ-Boras3Davor Katanec4Sonja Levanat5Karl Donath61Department of DentalPathology School of Dental MedicineUniversity of Zagreb2Private Dental Practice,Zagreb3Department of Oral MedicineSchool of Dental MedicineUniversity of Zagreb4Department of Oral SurgerySchool of Dental MedicineUniversity of Zagreb5Department of MolecularMedicineInstitute “Ruer BoπkoviÊ”,Zagreb, Croatia.6Institute of Oral PathologyUniversity of Hamburg,GermanyActa Stomat Croat2004; 23-25ORIGINAL SCIENTIFICPAPERReceived: January 3, 2004Address for correspondence:Boæidar PaveliÊDepartment of DentalPathologySchool of Dental MedicineGunduliÊeva 5, 10000 ZagrebCroatiatel: 4802 126/136fax: 4802 159e-mail:bozidar.pavelic@zg.hinet.hrA S C Acta Stomatol Croat, Vol. 38, br. 1, 2004.24B. PaveliÊ et al. Incidence of Odontogenic Keratocysts...IntroductionOdontogenic keratocysts (OKC) are classified asdevelopmental epithelial cysts which compriseapproximately 11% of all cysts of the jaws (1, 2).One of the most characteristic features of OKC isthe high recurrence rate(3). OKC may occur as asporadic OKC or together with Gorlin-Goltz syn-drome (nevoid basal cell carcinoma syndrome). Gor-lin-Goltz syndrome is characterized by multiplebasal cell carcinomas, odontogenic keratocysts ofthe jaws, palmar and/or plantar pits, skeletal anom-alies (most frequently seen on vertebral bones andribs), and intracranial calcifications (4-6). Gorlin-Goltz syndrome is an autosomal dominant inheri-tance disorder, and probably a result of abnormali-ties located on the chromosomes 9 (7). Recently itwas reported that the loss of human patched (ptch),a tumor suppressor gene, could be the possible molec-ular origin of OKC (8). Most frequently it becomesapparent during the second or third decade of life(9). OKC are usually the first sign of Gorlin-Goltzsyndrome, and can occur also in patients under theage of ten. It has been hypothesized that OKC arefrequently found earlier in patients with Gorlin-Goltzsyndrome compared to sporadic OKC (10). In onepatient, 1-30 cysts may develop (11). Therapy mayinclude various types of surgical procedures, oftenwith high recurrence rate or some other early post-operative complication (12, 13). The most effec-tive way to follow-up the bone healing and tissuedensity after surgical procedure is by radioviso-graphical (RVG) densitometry (14, 15). Materials and methodsIn this study, 30 patients with 58 OKC and Gor-lin-Goltz syndrome were included. The patients wererecruited from the Institute of Oral Pathology inHamburg, Germany and from the School of DentalMedicine (Department of Dental Pathology, OralMedicine, Pediatric Dentistry and Oral Surgery) inZagreb, Croatia, between the years 1965-1998. Allthe OKC were confirmed histopathologically. Theywere subdivided according to the age and genderof the participants and location in the orofacial sys-tem. According to the location OKC were dividedinto four groups: in the mandible (anteriorly and pos-teriorly), in the maxilla (anteriorly and posterior-ly), in soft tissues which comprises the mucosa ofthe alveolar ridge, parotid gland, skin of the noseand face, and in the maxillary sinus (either left orright). OKC located anteriorly were those situatedbetween canines, and those located posteriorly weresituated distal to the first premolars.ResultsAge and genderThe patients included in this study were agedfrom 10 to 90 years. The average age of patients withGorlin-Goltz syndrome at the time when OKC werediagnosed was 33.71 years. In males the average agewas 35.53 years and in females 31.12 years. Out of58 diagnosed OKC, 34 (58.62%) were found inmales and 24 (41.38%) in females. Male to femaleratio was 1.4:1.The highest incidence of OKC in all patients withGorlin-Goltz syndrome was determined betweenages 21-30 years (Table 1).LocationOut of 58 diagnosed OKC in patients with Gor-lin-Goltz syndrome, 35 (60.34%) were located in themandible, 9 (15.52%) in the maxilla. In the mandible,two OKC were situated anteriorly, and 33 (94.29%)posteriorly. In the maxilla, one OKC was found ante-riorly, and 8 (88.89%) were found posteriorly. 8(13.79%) KLL were found in the soft tissues whichinclude mucosa of the alveolar ridge, parotid gland,skin of the nose and face. Six (10.34%) OKC weredetected in the maxillary sinus, with unequal dis-tribution of one in the left sinus and 5 (83.83%) inthe right sinus (Table 2).DiscussionOdontogenic keratocysts probably arise from anyof the primordial epithelia (the dental lamina or itsremains) or, as originally believed, from the enam-el organ before tooth formation. The histologicalappearances of the lining of keratocysts are charac-teristic: a flat basement membrane, elongated pal-isated basal cells, corrugated parakeratotic surface,B. PaveliÊ et al. Incidence of Odontogenic Keratocysts...A S CActa Stomatol Croat, Vol. 38, br. 1, 2004. 25with or without keratin layer in the lumen. Mostcysts in the orofacial region, with keratin in thelumen, were often diagnosed as keratocysts, whichlead to misunderstanding and consequently wrongdiagnosis. Thus these lesions are similar to OKC,but they are not the same. Therefore, in this studyall the cysts found in soft tissues were called kera-tocyst like lesions.Sabbia et al. (13) and Addessi et al. (14) report-ed that in 90% of patients, nevoid basal cell carci-noma syndrome is associated with recurring OKC.Lo Muzio et al. (5) reported that OKC were the firstsymptom of Gorlin-Goltz syndrome in 78% of theircases. The same authors also underlined that OKCoccur earlier in patients who have nevoid basal cellcarcinoma syndrome when compared to the patientswho do not have the above mentioned syndrome.Previous investigations showed that connectionbetween OKC and Gorlin-Goltz syndrome is high-er than 5%. Payne (15) reported that the incidenceof OKC together with Gorlin-Goltz syndrome is7.4%, Radden and Reade (16) found connectionbetween OKC and Gorlin-Goltz syndrome in 6%of patients and Hodgkinson (17) in 8.8% of studiedcases. Lam and Chan (18) reported that multiplecysts occurred in 9% out of 69 ethnic Hong KongChinese patients, and only three cases were diag-nosed as Gorlin-Goltz syndrome. Oda et al. (19)found Gorlin-Goltz syndrome in 5% of their patientswith OKC, with a higher distribution in the first andsecond decades. Results of our study show that thehigher distribution of OKC together with Gorlin-Goltz syndrome occured during the second and thirddecade, but also with a second peak during the sixthdecade of life in the studied patients. Dowling etal. (20) stated that although the occurrence of OKCin Gorlin-Goltz syndrome is usually during the sec-ond or third decades of life, it should be noted thatit can occur earlier as seen in their patient, who was5 years old, which was the first manifestation ofnevoid basal cell carcinoma. Lo Muzio et al.(10)found that the youngest patient with OKC and Gor-lin-Goltz syndrome was eight years old. Results ofour study show that the youngest patient with OKCtogether with Gorlin-Goltz syndrome was ten yearsold.According to gender, male preponderance wasfound in this study and male to female ratio was1.4 : 1. We also found that number of OKC in everypatient with Gorlin-Goltz syndrome varied a lot.Seventeen patients had one OKC together with Gor-lin-Goltz syndrome, and nine patients had two OKCat the same time. In one patient, 12 OKC were diag-nosed during the period of 12 years, and two OKCoccurred simultaneously four times. In two patients,three OKC were diagnosed at the same time. Inanother patient in whom 5 OKC were found, twoof them were diagnosed at the same time on the leftside of the mandible and KLL on the skin of thenose. On the basis of these data we can concludethat in patients with Gorlin-Goltz syndrome, multi-ple OKC occur frequently at the same time, or thatthey can be diagnosed simultaneously. Therefore, inevery patient in whom one OKC is diagnosed, it isreasonable to perform additional investigations inorder to exclude coexistence of another OKC.This study also highlights the need to considerGorlin-Goltz syndrome as a possible diagnosis in allpatients with odontogenic keratocyst. Last, but notleast, in every patient with Gorlin-Goltz syndromeit is advisable to make further familial investigations.

Incidence of Odontogenic Keratocysts in Patients with Gorlin-Goltz Syndrome according to Age, Gender and Location

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Incidence of Odontogenic Keratocysts in Patients with Gorlin-Goltz Syndrome according to Age, Gender and Location

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