Calcifying odontogenic cyst with odontoma of the maxilla A Case report MIT. Yacoubi", M. Mokni*, M. Soui-Mhiri***, H. Khochtali", S. Korbi* INTRODUCTION The calcifying odontogenic cyst (COC) was first recognized by Gorin, in 1962" It is generally regarded as a benign lesion of odontogenic origin, which can present either a cyst or many classifica- tions of this lesion were formulated, Praetorius * classified the COC into 2 ‘main types: cystic and neoplastic (solid), and proposed the term “dentinogenic ghost cell tumor’ for the neoplastic type. We report a case of COC with odon- toma, rarely described in the literature ‘and we review the clinical and histomor- phological features of COC and we will discuss the terminology of the lesion and its histogenesis, ‘A 31-year-old man was hospitalized in the department of Head and Neck surgery for a swelling. in his right upper maxilla. 5 years. ago, the patient had ‘mass involving the right cheek, diag- nosed clinically as a radicular cyst, to which surgery had not been indicated; otherwise, he would lose sight * Department of Pathology, F Hached Hospital, Sousse, Tunisia. “Department of Head and Neck Surgery, Sahloul Hospital, Sousse. Tuni “Department of Radiology, Sousse. Tunisia Sahloul DENTAL NEWS Yolame X, Number 12003 ABSTRACT Calcifying odontogenic cyst of the maxilla is most commonly found in the second decade of life and occurs usually in bone with destructive behavior, its association ‘with odontoma is rarely described. We report a new case occurring in 3 -year-old man, located to the right maxilla. In CT imaging the lesion was extensive measuring 7 cm in great diameter involv. ing the bone and the adjacent sinus with regular margins and intracystic calcif- cations, We discuss the criteria of diagnosis, histogenesis and associated lesions to this entity. odontogénique calcné du maxi atteint fréquemment Faduite Jeune et se développe dans os avec un pouvoir destructit. Son association & un ‘odontome est rarement décrite. ‘rapportons un nouveau cas, survenant chez un homme agé de 31 ans et recat u niveau du bord droit du maxillaire supérieur. A examen tomodensitométrique, la lésion mesure 7 cm de plus grand diametre, détruit fos et atteint le sinus adjacent avec des bords réguliers et renferme des ‘calcifications. cot ee itons les critéres du diagnostic, Vhistogénése et les Iésions associées 4 = Correspondence address ‘Mohamed Tahar Yacoubi, MD Department of Pathology (Professor Sadok Korb) F. Hached Hospital Sousse 4000, Republic of Tunisia Tel: 00 2169 202 683 Fax : 00216 3226702 E Mail : taharyacoubi@yahoo.fr Hospital, Caleifying odontogenic cyst with odontoma of the maxilla | 19 |2 months ago, he consulted because the lesion increased in size. Loco fegional examination showed a swelling of the right maxillary gingiva prolonged to. nasogenial line, measuring 5 cm in great diameter. The lesion was adjacent to maxillary bone with the “Ping-Pong: sign. The tumefaction was situated at 0,5 cm near the margin of piriform or fice; the facing skin was normal. Endo oral examination showed a swelling of the right upper vestibule, with neithe lesion of the mucosae nor a mobility of tooth, The palate was normal Fig. 1: a- Unilocular radiolucent area of the right maxilla with calcification. - Lytic lesion with hydric density pro- jected on the maxillary sinus and extension to the right nasal fossa. Conventional radiography findings: Panoramic radiograph showed a well defined unilocular radiolucent area of the anterior region of the right maxilla with resorption of the root of the lateral incisor and distal displacement of the canine (Figure 1a). Blondeau incidence radiograph objecti vates a lytic lesion of hydric density located in the maxilary sinus with exten: sion to the right nasal fossa without of the orbit floor (Figure 1b). CT imaging: Multiple sagittal incidence has been performed showing a cystic lesion with calcified margin located in the right hemi-maxilla with cortical rupture (Figures 2a and 2b) Pathological findings: Surgical excision of the lesion was per: formed The surgical specimen consisted of pieces of cyst lining measuring 7 and 3,5 cm in great dimension associated to bony pieces Histology: The specimen was fixed in formalin and processed in the routine Decalcification was performed for the solid part, Sections were initially stained with Hematoxylin and Eosin, subsequent sections were stained with van Gieson Microscopically, the specimen consisted of a fibrous connective tissue wall lined with stratified squamous epithelium. The thelium had a well-defined basal cell layer that exhibited polarization of their nuclei away from the basement mem brane. Superficial to this was a loosely structured network of stellate reticulum (Figures 3a and 3b). Cell and focal Fig. 2: (@tb): CT imaging (axial inci- dence in bone window): Cystic lesion containing calcification ( arrow) located in the right maxilla with cortical rupture. Oral Pa Fig, 3a: (HE staining x 40): Cystic wall covered with stratified epithelium. Fig. 3b: (HE staining x 200). Fig. 3ctd: (HE Epithelium surface with ghost cells and calcifications. staining x 400:Fig. 3e: (« 100) Confirmation of the ghost cells by Von Gieson staining. Fig. 3f: (HE staining x 4 tooth structure in the wall of the cyst. masses of ghost cells underwent vary: ing degree of calcification (Figures 3c, 3 and 3e). The solid zone identified in {gross was interpreted as rudimentary tooth structure (Figure 39, The histolog ic appearance conformed to the neces sary criteria for a diagnosis of calcifying cyst (COO as defined by the WHO com mittee. The tooth structure associated to the COC was diagnosed as odontoma laa Discussion Since the first description of Calcifying odontogenic cyst (COO), other terms have been presented to designate this lesion : mixed odontogenic tumor, kera tinizing and calcifying cyst, atypical adamantinoma, calcifying ghost cell odontogenic tumor, variants of cholesteatoma and ‘cystic calcifying odontogenic tumor", COC has been histopathologically defined by the WHO {as anon neoplastic cystic lesion that is lined by enamel organlike epithelium, contains denucleated eosinophilic ghost cells and calcification in the epithelium DENTALNEWS, Youn Nunbe 2003 ‘and connective tissue wall, and is some. times associated with other features Although ghost cell formation can be seen in several odontogenic and non- ‘odontogenic lesions, it is characteristic of COC. This lesion is most commonly found in the 2nd decade of life, with an almost equal sex distribution and a sim: ilar incidence in the mandible and the maxilla *. Radiographically, the lesion appears as a well-defined radiolucency containing varying amounts of radiopaque materiel‘, as in our case. COC usually occurs within bone but may also occur in the soft tissues of the tooth-bearing area °. It sometimes resembles with ameloblastoma, and pre- Viously most cases were diagnosed as atypical ameloblastoma ‘Ghost epithelial cells’, often calcified like those seen in pilomatrixoma, are one of the most distinctive features of the calcifying ‘odontogenic cyst, although they may also occur in the ameloblastoma and in certain other odontogenic epithelial lesions*”. Various stains, ike van Gieson and fluorescence microscopy after stain ing with Rodamine B, may be useful in distinguishing ghost cells from other aci dophilic masses. The staining reaction of the ghost cells suggests that they are keratinizing, and they are entirely thioflavineT-negative *. Many investiga: tors have made efforts to clarify the nature of ghost cells by employing spe ial histochemical and immunohisto- chemical methods ®, transmission elec tron microscopy , and scanning electron microscopy, and various theories have been proposed without any general agreement. Gorlin et al.’ believed that ghost cells represent normal or abnor: mal keratinization. Levy "investigated ghost cells in odontomas and suggested that they represent squamous metapla- sia with subsequent calcification caused by ischemia, Sedano and Pindborg thought that the ghost cells represented different stages of normal and aberrant keratin formation and they were derived from the metaplastic transformation of ‘odontogenic epithelium. It is suggested that in COC, central liquefaction necrosis of odontogenic epithelium clusters may take place in the initiation phase of cys: tic development, and coagulative necro: sis occurs at the same time or later in Portions of the cystlining epithelium, with resultant ghost cell formation Calcification then occurs as a dystrophic phenomena’. According to the classifi cation of COC proposed by Hong’, our case can be classified as a ID COC type, it shows combined features of non prolif erative COC and odontoma, Praetorius et al.’ believed that the odontoma devel oped in the COC, other authors thought that the COC developed in a manner similar to that of dentigerous cysts, that is, the COC developed as a result of the ‘odontoma 1+ Gontn Ry, Pindborg JJ, Redman RS, Willamson 4, Hansen LS. 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