Volume 2, Issue 4 , Pages 250-253, December 2007
Peripheral dentinogenic ghost cell tumour in child: A case report
Article Outline
- Summary
- 1. Introduction
- 2. Case report
- 3. Discussion
- Conflict of interest
- Acknowledgements
- References
- Copyright
Summary
This case reports on a rare odontogenic tumour in a child presented with a gingival mass in the incisive papilla. Radiological examination did not show osseous alteration. A surgical excision was performed. A diagnosis of the peripheral dentinogenic ghost cell tumour was made. The patient is currently undergoing follow-up procedures and no recurrences have been reported. Peripheral cases of this tumour are few and tend to be quite rare, especially in children. This article is the first reported case of peripheral dentinogenic ghost cell tumour in children.
Keywords: Dentinogenic ghost cell tumour, Ghost cells, Gingival, Jaws, Odontogenic tumour
1. Introduction
The dentinogenic ghost cell tumour (DGCT) is a locally invasive tumour characterized by ameloblastoma-like islands of epithelial cells in a mature connective tissue stroma. Aberrant keratinization is found in the form of ghost cells in association with varying amounts of dysplastic dentin. In the past, DGCT was considered a solid variant of the calcifying odontogenic cyst. The peripheral DGCT is the peripheral counterpart of the central DGCT and exhibits a similar histological appearance [1].
Peripheral DGCT is rare and presents a predilection toward the anterior part of the jaws [1], [2], [3], [4], [5], [6], [7], [8], [9]. It is most commonly occurs in patients from the 2nd to the 9th decade of life, and is somewhat more common in men than in women [1], [2], [3], [4], [5], [6]. The peripheral DGCT is usually asymptomatic and slow growing and may appear as a sessile, sometimes pedunculated, exophytic nodule of the gingival or alveolar mucosa. Some cases have been reported in edentulous areas. The size is generally between 0.5 and 1
cm. Radiographs reveal saucerization of the underlying bone in about 20% of the cases. Teeth in the affected area may be displaced [1]. Surgery is considered as the most appropriate treatment for the peripheral DGCT and no recurrences have been reported in the literature [1], [2], [3], [4], [5], [6], [7], [8], [9], [10], [11].
The purpose of this article is to present a case of peripheral DGCT in a child. Only a small series of isolated cases have been reported in the English literature [2], [3], [4], [5], [6], [7], [8], [9], [10], [11]. This case is the first reported case of peripheral DGCT in children.
2. Case report
A 7-year-old white female patient was referred to the Oral Medicine Clinic, School of Dentistry, Universidade Federal de Minas Gerais (Belo Horizonte, Brazil) for evaluation of a nodule considered as isolated, exophytic, sessile, firm, painless, of 0.8mm in size, with similar coloring of the normal mucosa and well-defined, located in the incisive papilla (Fig. 1A). Both central permanent maxillary incisors showed vital pulps. The patient's physical status was good and investigation of her medical history proved to be irrelevant. At the time of the doctor's visit, the patient was not prescribed with any sort of medication. There were no significant radiographic findings in the periapical view (Fig. 1B). Preoperative clinical diagnosis was of a fibrous hyperplasia, palatal polyp, and gingival cyst. An informed consent was obtained from the parents, and a conservative excision surgery, maintaining a secure margin, was performed. The specimen was set in a 10% formalin buffer and sent for microscopic evaluation. Microscopic examination demonstrated an odontogenic epithelium proliferation with an overlying mucosal epithelium. The odontogenic epithelium proliferation was composed of ameloblastoma-like sheets. Minor cysts and dysplastic dentine were also present. These features were associated with abundant ghost cells (Fig. 1C–F). Thus, a diagnosis of peripheral DGCT was established. Postoperative healing was uneventful. Eleven months of follow-up revealed no recurrence (Fig. 1G).
Fig. 1.
(A) Clinical intra-oral view showing exophytic, sessile, with an average size of 0.8
mm, with similar coloring of the normal mucosa and well-defined nodule located in the incisive papilla. (B) Periapical radiographic view did not demonstrate any osseous involvement. (C) Odontogenic epithelium proliferation is covered with stratified squamous epithelium and well-circumscribed by connective tissue (Haematoxilin and eosin-HE, original magnification 25×). (D) Minor cyst and dysplastic dentine were observed (HE, original magnification 100×). (E) Odontogenic epithelium sheet presents cell ameloblastoma-like and ghost cells (HE, original magnification 100×). (F) Ghost cells are large, polygonal cells with pale eosinophilic, homogenous cytoplasm and shadowy nuclear outlines (HE, original magnification 400×). (G) Clinical intra-oral view after 11 months of follow-up showing the incisive papilla with normal aspects.
3. Discussion
Peripheral DGCT is an exceedingly rare epithelial odontogenic tumour [2], [3], [4], [5], [6], [7], [8], [9], [10], [11]. The clinical and histological features of the current case provide clinical support for the diagnosis of peripheral DGCT. Vuletin et al. [9] described a case of a peripheral odontogenic tumour with ghost-cell keratinization in a 3-year-old girl. However, because the stroma of the tumour was composed of cellular myxoid fibroblastic tissue, the case was excluded [9]. Therefore, to our knowledge, the present case shows the first clinical documentation of the peripheral DGCT in children. Hirshberg et al. [3], Gunhan et al. [4], Hong et al. [11], Raubenheimer et al. [5], Castro et al. [6], Piattelli et al. [10], Lombardi et al. [7], and Wong et al. [8] presented cases of peripheral DGCT in patients from 37 to 83 years of age [3], [4], [5], [6], [7], [8], [10], [11]. Bone erosion is only found in approximately 20% of the cases [1], [6], [10], but in this case there were no significant radiographic findings in the periapical view.
The present case represents a solid peripheral variant. Although peripheral DGCT most commonly appears as a localized gingival swelling, the clinical features are not sufficiently distinctive to separate them from other gingival masses such as fibroma, fibrous hyperplasia, palatal polyps, and peripheral odontogenic tumours [1], [6], [7], [12]. Subsequently, histological criteria are considered fundamental in the diagnosis of peripheral DGCT. Microscopic features, however, do not differ in the central and peripheral variants [1]. Abnormal expression and mutation of β-catenin was observed in calcifying odontogenic cyst and in DGCT [13], [14]. Stabilization of β-catenin and subsequent constitutive activation of TCF/LEF-dependent transcription may be caused by β-catenin mutation during odontogenesis. This may disrupt the proper differentiation process coordinated by Wnt signaling pathway, resulting in formation of DGCT [14].
Peripheral DGCT is easily excised and the lack of recurrence of any of the peripheral tumours, as reported in the literature and confirmed in the present case, suggests a favorable course for this peripheral variant, as well as the other odontogenic tumours [1], [2], [3], [4], [5], [6], [7], [8], [9], [10], [11].
In summary, the present case reported the clinical and histological findings of peripheral DGCT in a female child, emphasizing the need to consider this entity as a possible diagnosis by the clinician when focal gingival growth is identified. The peripheral DGCT is well described in the literature, but this is the first report of its kind in children.
Conflict of interest
We disclosed any financial and personal relationships with other people or organizations that could inappropriately influence in the manuscript entitled “Peripheral dentinogenic ghost cell tumour in child: A case report”.
Acknowledgements
This study was supported by grants from CAPES, FAPEMIG (CDS 895/05), and CNPq (484974/2006-8). RA MESQUITA and RS GOMEZ are research fellows of CNPq.
References
- In: Barnes L, Eveson JW, Reichart P, Sidransky D editor. World Health Organization Classification of Tumours. Pathology and Genetics of Head and Neck Tumours. Lyon: IARC Press; 2005;
- . Odontogenic ghost cell tumor. Semin. Diagn. Pathol. 1999;16:288–292
- . Dentinogenic ghost cell tumor. Int. J. Oral Maxillofac. Surg. 1987;16:620–625
- . Epithelium odontogenic ghost-cell tumour: report of a peripheral. Ann. Dent. 1991;50:8–11
- . Peripheral dentinogenic ghost cell tumor. J. Oral Pathol. Med. 1992;21:93–95
- . Peripheral dentinogenic ghost-cell tumor: a case report. Quintessence Int. 1997;28:45–47
- . Epithelial odontogenic ghost cell tumour of the mandibular gingiva. Oral Oncol. 1999;35:439–442
- . Peripheral dentinogenic ghost cell tumor presenting as a gingival mass. Br. J. Oral Maxillofac. Surg. 2004;42:173–175
- . Peripheral odontogenic tumor with ghost-cell keratinization: a histologic, fluorescent microscopic, and ultrastructural study. Oral Surg. 1978;45:406–415
- . Immunohistochemical analysis of a dentinogenic ghost cell tumour. Oral Oncol. 1998;34:502–507
- . Calcifying odontogenic cyst: a review of ninety-two cases with reevaluation of their nature as cysts or neoplasms, the nature of ghost cells, and subclassification. Oral Surg. Oral Med. Oral Pathol. 1991;72:56–64
- . Three cases of palatal polyps in infants. Int. J. Paediatr. Dent. 1999;9:213–217
- Beta-catenin mutations are frequent in calcifying odontogenic cysts, but rare in ameloblastomas. Am. J. Pathol. 2003;163:1707–1712
- . Investigation of the beta-catenin gene in a case of dentinogenic ghost cell tumor. Oral Surg. Oral Med. Oral Pathol. Oral Radiol. Endod. 2007;103:97–101
PII: S1871-4048(07)00064-0
doi:10.1016/j.pedex.2007.07.004
© 2007 Elsevier Ireland Ltd. All rights reserved.
Volume 2, Issue 4 , Pages 250-253, December 2007
