Dentomaxillofacial Radiology (2002) 31, 333 338 2002 Nature Publishing Group.

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REVIEW

The glandular odontogenic cyst: Clinical and radiological features; review of the literature and report of nine cases
C Noke*,1 and EJ Raubenheimer2
1 2

Departments of Oral and Maxillofacial Radiology, Faculty of Dentistry, Medical University of Southern Africa, South Africa; Department of Oral Pathology, Faculty of Dentistry, Medical University of Southern Africa, South Africa

Nine cases with glandular odontogenic cysts (GOC's) are presented bringing the total number reported in the literature to 54. Our study conrmed that most GOC's occur in the mandible, whereas maxillary lesions present only in the globulo-maxillary region. The radiological features were found to be non-distinctive and presented as well-dened radiolucencies with uniand multilocular appearances. Most of the mandibular GOC's were unilocular, involved the symphysis region and only one extended into the ramus. All GOC's larger than 6 cm in diameter showed perforated margins radiologically. Our two multilocular GOC's demonstrated microscopic features supporting their inltrative radiological appearance. The invasive clinical and radiological features of GOC support the notion of a possible histo-pathologic overlap between GOC and low-grade central mucoepidermoid carcinoma of the jaw. Dentomaxillofacial Radiology (2002) 31, 333 338. doi:10.1038/sj.dmfr.4600730 Keywords: odontogenic cysts; radiography, dental; jaw cysts; review literature Introduction Two multilocular mandibular cysts were originally described by Padayachee and Van Wyk1 who speculated on the possibility of salivary gland origin and proposed the term sialo-odontogenic cyst. Histological characteristics, which supported their choice of terminology, were mucinous material within the cystic spaces and epithelial thickening or plaques in the epithelial lining. One of their cysts recurred. The histological characteristics led to the association of the cysts with the central mucoepidermoid tumour. Gardner2 reported eight cases in 1988 involving both the maxilla and mandible, which occurred over a wide age range in both genders and recurred if not excised adequately. One of their cases was associated with an ameloblastoma. Radiologically the lesions were reported to be either unilocular or multilocular with smooth or scalloped margins. Based on their histopathological features they assumed the cysts to be of odontogenic origin and a histologic variant of the botryoid odontogenic cyst. The term glandular odontogenic cyst (GOC) was suggested. Shear3 favoured the term muco-epidermoid cyst, which was advocated by Sadeghi and co-workers.4 However, the latter term had already been used by Hodson5 to describe simple radicular, residual and dentigerous cysts showing mucous metaplasia of the epithelial linings. In 1992 the World Health Organisation accepted GOC as a distinct pathological entity and classied it as a developmental odontogenic cyst.3 Patron, Colmeri and Larrauri6 reported three cases in 1991, which did not recur after surgical removal. One case was associated with a squamous odontogenic tumour-like proliferation in its wall. They included thirteen previously reported cases in their study and found predilections for males (9/13) and the mandible (10/13) and an age range of 19 to 85 years with a mean age of 50 years. Radiologically they described the lesions as well dened, uni- or multilocular without specic diagnostic characteristics. The occurrence during the fth to seventh decade, location in the mandibular premolar region, multilocularity, tendency to recur and histological similarity of the epithelial lining led them to support the suggestion that GOC's are histologic variants of the botryoid odontogenic cyst. In 1994 Takeda7 reported a GOC in the mandibular third molar region that presented as a

*Correspondence to: C Noke, Department of Oral Radiology, Box D16, P.O. Medunsa, 0204, South Africa; E-mail: [email protected] Received 14 November 2001; revised 13 April 2002; accepted 19 August 2002

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lateral periodontal cyst with a unilocular appearance. He supported the unsubstantiated hypothesis that a lateral periodontal cyst may develop in a GOC. Hussain, Edmondson and Browne8 described four new cases of GOC in the mandible, with a predilection for females and a mean age of 44 years. The clinical and the radiological features were described as nonspecic. Semba et al9 added one new case in 1994, reviewed the clinical features of GOC and compared it with botryoid odontogenic cysts. He suggested that the GOC and the latter are histological variants in a separate group of non-keratizing odontogenic cysts, but share the same epithelial origin, namely the dental lamina, its remnants or reduced enamel epithelium. There was no sign of recurrence 2 years after surgical removal of their case. Agreement has been reached on the aggressive, somewhat neoplastic nature of GOC's and their tendency to recur.2,6,10,11 Toida and co-workers (1994)12 in their review of the literature found a predilection for the mandible (14/18), notably the anterior region (13/18) and an equal gender distribution. The age range was reported to be 14 to 85 years (mean age of 49 years) and the majority of patients were older than 40 years. Radiologically the lesion lacked specic features making distinction from ameloblastoma and odontogenic keratocyst dicult. A more aggressive surgical removal rather than simple curettage was suggested and cases should be carefully followed up. Economopoulou and Patrikiou13 added one case to the literature in 1995 and reviewed 19 cases in total. They found that GOC's occurred over a wide age range, with a predilection for men and the anterior mandible. The cysts may reach large dimensions, are often associated with expansion and radiological

ndings were reported to be non-specic.14 The most recent literature research revealed a total of 47 reported cases, a male to female ratio of 19 : 28 and mean age 46.7 years (range 14 75 years) in males and 50.0 years (range 21 72 years) in females, resulting in a mean age of 48.3 years for both genders.15 Our study was aimed at analysing the clinical, radiological and histopathological features of seven new cases of GOC's in a rural African population and to compare our ndings with those reported in the literature. The aggressive nature of GOC's makes distinction from other cystic lesions of the jawbones important. Diagnosis prior to surgical intervention is essential in this regard. Report of new cases Nine cases of GOC were diagnosed over the past 10 years in the Department of Oral Pathology at Medunsa, which serves mainly a rural Black population. Two of these were previously published as case reports.11 None of our cases recurred, however follow-up is poor due to the remoteness of the region. Clinical and radiological data are reected in Table 1. Radiographic examination was performed with panoramic, occlusal, Waters and peri-apical radiographs and measurements were made in horizontal and vertical dimensions on standardized panoramic lms. All GOC's showed cortical expansion (Figure 1) and those with a diameter of more than 6 cm, perforation. Maxillary GOC's had a well-circumscribed unilocular radiological appearance without exception (Figure 2). Both multilocular GOC's in our sample occurred in the

Table 1 Clinical and radiological features of eight cases Case no. 1* 2* 3 4 5 6 7 8 9 Gender M F M M M F F F F Age (years) 14 27 50 15 17 58 11 59 59 Site R Max 12 13 L&R Mand 36 45 L&R Mand 34 43 R Max 22 23 L Max 12 17 L&R Mand 37 48 L&R Mand 33 46 RMand 47ramus Lmand 36 42 Size (cm) 3.262 663 762.5 363 4.564 16.564 5.763 462.7 964.5 Radiological features Unilocular, well circumscribed, smooth contour, tooth displacement Unilocular, well circumscribed, irregular borders, tooth displacement Unilocular, well circumscribed, smooth borders, tooth displacement Unilocular, well circumscribed, smooth borders, tooth displacement Unilocular, well circumscribed, smooth borders, tooth displacement Multilocular, variable circumscription, irregular borders, partially sclerotic with perforations, tooth displacement Unilocular, well circumscribed, irregular borders, tooth displacement Unilocular, well circumscribed, smooth sclerotic borders Multilocular, scalloped borders with perforation and tooth displacement Clinical Expansion Expansion Perforation Expansion Perforation Expansion Expansion Expansion Perforation Expansion Expansion Expansion Perforation

*Appeared as case reports11

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Figure 1

Case 3: buccal and lingual expansion with tooth displacement

Figure 2

Case 4: unilocular well-circumscribed cyst in the maxilla between teeth 12 and 13, with smooth borders and tooth displacement

mandible and perforated through the cortex and into the alveolar mucosa (Figures 3 and 4). All mandibular cases were limited to the body and symphysis except one case that extended into the ramus (Figure 5).

All cases fullled the histopathological criteria for a diagnosis of GOC advocated by Gardner and coworkers in 1988.2 The multilocular cases exhibited daughter cyst formation. Early invasion was characterDentomaxillofacial Radiology

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Figure 3

Case 6: multilocular mandibular lesion with partially sclerotic margins showing foci of perforation (arrows)

Figure 4

Case 9: multilocular mandibular lesion showing mild tooth displacement and perforation into the alveolar mucosa (arrow)

ized by the formation of adenoid structures which penetrated the connective tissue wall. These features were not seen in the unilocular types. Discussion Our series of nine cases of GOC, of which two had previously been reported11 was diagnosed over a period of 10 years, conrming its low prevalence. The male to female ratio in our sample was found to be equal. Our mean age (35 years) was a decade younger than generally reported14,15 mainly due to the signicantly younger average age of 24 years at presentation of our
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male patients. The younger age may represent a racial dierence in the manifestation of GOC. This tendency is, however, in agreement with the literature where males are generally reported to be aected at a younger age. Twice as many cases occurred in the mandible than maxilla, corresponding with the ndings of other studies.6,12,14,16 In our sample ve of the six mandibular cases involved the symphysis area, the site of prevalence reported in other series. One of our cases occurred in the molar area of the mandible and extended into the ramus. All our maxillary GOC's were present in the globulomaxillary area. Two of these were pear shaped and associated with divergence of the roots of the lateral incisor and canine teeth and one

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Figure 5

Case 8: unilocular well-circumscribed lesion with smooth sclerotic borders involving the mandibular ramus

extended distally to the second molar tooth. No maxillary GOC has been reported to occur in another location. The sizes of our lesions ranged between 2 and 16.5 cm in horizontal dimensions, the latter being the largest GOC reported thus far. The two multilocular GOC's were the largest lesions in our series, measuring 16.5 and 9 cm in horizontal dimension respectively. This may indicate that multilocularity is a size dependant phenomenon, developing only in the larger lesions. All our GOC's which measured in excess of 6 cm showed bone expansion with perforation, a feature supporting their aggressive expansile behaviour.3 Most authors conclude that there is no radiological feature distinctive for GOC.14 16 All maxillary GOC's in our series were well circumscribed unilocular with regular borders, ndings that correspond with those of a previous paper.15 In our sample unilocular GOC's with irregular borders were common in the mandible (two out of four lesions). Two of our mandibular lesions showed sclerotic borders and one case scalloped between the roots of mandibular canine and premolar teeth. The two largest GOC were multilocular and resembled ameloblastoma radiologically. Radiological features which may be helpful in distinguishing multilocular GOC's from ameloblastomas include irregular loculations and a partially sclerotic border with foci of perforation. One GOC was, however, reported to be associated with an ameloblastoma2 and representative histological sampling of large multicystic lesions is required to exclude the possibility of this manifestation. The question whether a GOC in association with an ameloblastoma represents a collision growth of two initially distinct lesions or a metaplastic phenomenon within an ameloblastoma (or

GOC) remains speculative. The epithelial lining of a GOC may possess the ability to induce an ameloblastomatous proliferation in the connective tissue wall, similar to the phenomenon described in calcifying odontogenic cysts.17 The proliferative capacity of the lining of GOC's could explain the histogenesis of the squamous odontogenic tumour-like proliferation reported in the wall of a GOC.6 The presence of the epithelial plaques in a small number of GOC's is in our opinion not sucient to conrm an association between this aggressive cyst type and the more innocuous lateral periodontal and botryoid odontogenic cysts. Both our multilocular GOC's showed proliferations that inltrated the connective tissue wall. This should not be confused with the plaques in the latter two cyst types, which are in fact localized thickenings consisting of mitotically inactive clear cells.18 The GOC's in our sample were not associated with impacted teeth but rather tended to displace erupted teeth. This nding is indicative that GOC's generally develop after all permanent teeth have erupted. No signicant resorption of the roots of involved teeth were observed in our study. Taking the above mentioned radiological appearances into account, the provisional diagnoses for GOC on a radiograph would include odontogenic keratocyst, unicystic and multicystic ameloblastoma, lateral periodontal cyst, botryoid odontogenic cyst, simple bone cyst and central mucoepidermoid tumour. Features which may increase a suspicion of GOC include a sclerotic border with ne perforations or a pear shaped unilocular cyst with smooth margins in the globulo-maxillary region of the maxilla. Histologically the central muco-epidermoid carcinoma is considered the most important dierential diagnosis.18 Care should
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furthermore be taken not to interpret mucous cell metaplasia, which occurs commonly in a variety of odontogenic cysts and even ameloblastomas19 as foci of GOC transformation. In order to prevent this from occurring, microscopic criteria for the diagnosis of GOC should stringently be applied. These include a supercial layer of cuboidal or columnar epithelial cells occasionally with cilia and a glandular or pseudoglandular structures and intraepithelial crypts frequently containing mucin. The remaining of the cyst may be lined by thin non keratinised stratied squamous epithelium.3 Our study showed that the multicystic type exhibits neoplastic features with inltration of the surrounding tissue and daughter cyst formation. The distinction between low-grade central mucoepidermoid carcinoma and GOC is dicult, if not impossible. Both are reported to be
References
1. Padayachee A, Van Wyk CW. Two cystic lesions with features of both the botryoid odontogenic cyst and the central mucoepidermoid tumor: sialo-odontogenic cyst? J Oral Pathol 1987; 16: 499 504. 2. Gardner DG, Kessler HP, Morency R, Schaner DL. The glandular odontogenic cyst: an apparent entity. J Oral Pathol 1988; 17: 359 366. 3. Shear M. Cysts of the oral regions. 3rd edn, Cambridge: University Press, 1992: pp.72 74. 4. Sadeghi EM, Weldon LL, Kwon PH, Sampson E. Mucoepidermoid odontogenic cyst. Int J Oral Maxillofac Surg 1991; 20: 142 143. 5. Hodson JJ. Muco-epidermoid odontogenic cysts of the jaws with special reference to those in the mandible. Proc R Soc Med 1956; 49: 637 641. 6. Patron M, Colmero C, Larrauri J. Glandular odontogenic cyst: Clinicopathologic analysis of three cases. Oral Surg Oral Med Oral Pathol 1991; 72: 71 74. 7. Takeda Y. Glandular odontogenic cyst mimicking a lateral periodontal cyst: a case report. Int J Oral Maxillofac Surg 1994; 23: 96 97. 8. Hussain K, Edmondson HD, Browne RM. Glandular odontogenic cysts. Diagnosis and treatment. Oral Surg Oral Med Oral Pathol 1995; 79: 593 602. 9. Semba I, Kitano M, Mimura T, Miyawaki A. Case Report. Glandular odontogenic cyst: analysis of cytokeratin expression and clinicopathological features. J Oral Pathol Med 1994; 23: 377 382. 10. Ficarra G, Chou L, Panzoni E. Glandular odontogenic cyst (sialo-odontogenic cyst). Int J Oral Maxillofac Surg 1990; 19: 331 333. 11. Van Heerden WFP, Raubenheimer EJ, Turner MJ. Glandular odontogenic cyst. Head and Neck 1992; 14: 316 320. 12. Toida M, Nakashima E, Okumura Y, Tatematsu N. Glandular odontogenic cyst: A case report and literature review. J Oral Maxillofac Surg 1994; 52: 1312 1316.

unilocular or multilocular and may inltrate and destroy bone. Microscopically, the lining of the cystic spaces of both exhibit squamous-, cylindrical- and cuboidal epithelium and mucus producing cells arranged in papillary folds. Within the epithelial lining of both mucus containing crypts (or gland-like structures) are found.20,21 The only feature which has not been reported in low-grade central mucoepidermoid carcinoma and which may justify the existence of GOC as a separate entity is occasional presence of epithelial plaques, similar to those seen in lateral periodontal cysts. In conclusion, in view of the histogenetic relationship that had been proposed between GOC and central mucoepidermoid carcinoma of the jaw,1,10,22 the possibility that both entities represent a spectrum of one disease, should be investigated.

13. Economopoulou P, Patrikiou A. Glandular odontogenic cyst of the maxilla: a report of a case. J Oral Maxillofac Surg 1995; 53: 834 837. desjo 14. Magnusson B, Go ransson L, O B, Gro ndahl K, Hirsch JM. Glandular odontogenic cyst. Report of seven cases. Dentomaxillofac Radiol 1971; 26: 26 31. 15. Koppang HS, Johannessen S, Haugen LIC, Haanaes HR, Solheim T, Donath K. Glandular odontogenic cyst (sialoodontogenic cyst): a report of two cases and literature review of 45 previously reported cases. J Oral Pathol Med 1998; 27: 455 462. 16. Ramer M, Montazem A, Lane SL, Lumerman H. Glandular odontogenic cyst. Report of a case and review of the literature. Oral Surg Oral Med Oral Pathol 1997; 84: 54 57. 17. Praetorius F. Calcifying odontogenic cyst. Range, variations and neoplastic potential. Acta Odontol Scand 1981; 39: 237 240. 18. Waldron CA. Odontogenic cysts and tumors. In: Neville BW, Damm DD, Allen CM, Bouquot JE (eds). Oral and Maxillofacial Pathology. Philadelphia: WB Saunders 1995: pp. 493 530. 19. Raubenheimer EJ, van Heerden WFP, Noke CEE. Infrequent clinicopathological ndings in 108 ameloblastomas. J Oral Pathol Med 1995; 24: 227 232. 20. Auclair PL, Ellis GL. Mucoepidermoid carcinoma. In: Surgical Pathology of the Salivary Glands. Philadelphia: WB Saunders 1991: pp. 291 295. 21. Kramer IRH, Pindborg JJ, Shear M. Histological typing of odontogenic tumours. 2nd edn. Berlin: Springer-Verlag, 1992; p. 28. 22. Waldron CA, Koh ML. Central mucoepidermoid carcinoma of the jaws: a report of four cases with analysis of the literature and discussion of the relationship to mucoepidermoid, sialodontogenic, and glandular odontogenic cysts. J Oral Maxillofac Surg 1990; 48: 871 877.

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