Vol. 98 No.

1 July 2004

ORAL AND MAXILLOFACIAL PATHOLOGY

Editor: Alan R. Gould

Hybrid odontogenic tumor of calcifying odontogenic cyst and

ameloblastic fibroma
Jung Hoon Yoon, DDS, PhD,a Hyung Jun Kim, DDS, PhD,b Jong In Yook, DDS, PhD,c
In Ho Cha, DDS, PhD,b Gary L. Ellis, DDS,d and Jin Kim, DDS, PhD,c Gwangju and Seoul,
Korea, and Salt Lake City, Utah
CHOSUN UNIVERSITY, YONSEI UNIVERSITY COLLEGE OF DENTISTRY, AND ARUP LABORATORIES

Odontogenic tumors composed of 2 distinct types of lesions are unusual. We report an odontogenic tumor that
was composed of calcifying odontogenic cyst and ameloblastic fibroma that occurred in the right posterior maxilla of
a 22-year-old Korean woman. The tumor had a cystic component with an ameloblastic epithelial lining and
conglomerates of so-called ghost cells, and there were deposits of dentinoid material adjacent to the cyst. These are
features characteristic of calcifying odontogenic cyst. Enamel organ-like epithelial islands were observed within a dental
papilla-like stroma of the cyst wall. Additionally, a solid portion of the tumor had characteristic features of ameloblastic
fibroma, i.e., a myxoid cellular stroma with numerous elongated islands of ameloblastic epithelium. Ghost cell masses
were found in the area of ameloblastic fibroma as well. The distribution of the ghost cells suggests that this is a hybrid
lesion rather than a collision tumor. (Oral Surg Oral Med Oral Pathol Oral Radiol Endod 2004;98:80-4)

Calcifying odontogenic cyst (COC), which was first

identified as an entity by Gorlin et al in 1962,1 has been
classified as an odontogenic tumor by the World Health
Organization.2 A variety of histopathologic features as
well as biologic behaviors have been described, and
several classifications have been proposed.3-5 Some
COCs appear to represent nonneoplastic cysts whereas
others have no cystic features and are regarded as
neoplastic.3,6 The characteristic microscopic features of
COC are a cystic epithelial lining with a well defined
basal layer of columnar cells and an overlying layer that
resembles stellate reticulum and masses of ghost cells
in the epithelial lining or in the cyst wall.2

The additional characteristic finding is that COCs are

frequently associated with odontogenic tumors,3,6-10
a finding which is a rare event in other types of
odontogenic cysts or tumors. The most common of these
is odontoma,8 but, rarely, ameloblastoma, adenomatoid
odontogenic tumor, odontoameloblastoma, ameloblastic
fibroma, ameloblastic fibro-odontoma, and odontogenic
myxofibroma have been identified.3,6-14
In this article, a hybrid odontogenic tumor composed
of COC and ameloblastic fibroma of the right posterior
maxilla that occurred in a 22-year-old Korean woman is
described.

CASE REPORT
a

Department of Oral Pathology, College of Dentistry, Chosun

University.
b
Department of Oral & Maxillofacial Surgery, Oral Cancer Research
Institute, Yonsei University College of Dentistry.
c
Department of Oral Pathology, Oral Cancer Research Institute, Brain
Korea 21 Project for Medical Sciences, Yonsei University College of
Dentistry.
d
Centers of Excellence, ARUP Laboratories, Salt Lake City, Utah.
Received for publication Apr 7, 2003; returned for revision Jun 30,
2003; accepted for publication Jan 7, 2004.
1079-2104/$ - see front matter
2004 Elsevier Inc. All rights reserved.
doi:10.1016/j.tripleo.2004.01.003

80

A 22-year-old Korean woman visited the Department of

Oral & Maxillofacial Surgery at Yonsei University Dental
Hospital and complained of discomfort when chewing and
mobility of the right maxillary teeth for the last 3 months. The
patients medical history was noncontributory. There was
slight swelling of the right side of her face, but skin color was
normal. The maxillary right first and second molars were
displaced buccally, and there was a vestibular swelling and an
exudate from the gingival sulcus. Computerized tomograms
demonstrated a circumscribed, 3 cm in diameter, partially
cystic, intraosseous soft tissue lesion with multiple calcified
clusters (Fig 1). The presumptive clinical diagnosis was COC

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Yoon et al 81

Fig 1. Computerized tomogram scan revealed a well defined intrabony cystic soft tissue lesion containing multiple radiopaque
clusters.

Fig 2. Photomicrograph of incisional biopsy specimen showing primitive dental papillaelike mesenchymal tissues admixed with
proliferating odontogenic epithelium, resembling an ameloblastic fibroma (H-E, 3100).
or ameloblastic fibro-odontoma. Incisional biopsy was
performed.
The biopsy specimen was composed of cellular, dental
papillaelike mesenchymal tissues admixed with numerous

elongated, irregularly shaped nests of odontogenic epithelium

(Fig 2). The diagnosis was ameloblastic fibroma. However, the
radiographic finding of multiple calcified clusters within the
lesion raised a question as to whether the biopsy was

82 Yoon et al

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July 2004

Fig 3. A, A well defined cystic lesion lined by an ameloblastoma-like odontogenic epithelium and large eosinophilic ghost cells with
deposits of dentinoid material (H-E, 340). B, The proliferating epithelial strands from the cyst lining and the associated
condensations of cells within the stroma resemble primary ectomesenchymal induction of the dental lamina (H-E, 3200). C, Ghostcell clusters are present in islands of ameloblastic epithelium. These islands are situated in the myxoid stroma of the ameloblastic
fibroma component of the tumor (H-E, 3200).

representative of the entire lesion. The lesion was excised under

general anesthesia. Postoperative course was uneventful, and
no recurrence was observed at a 1-year follow-up.
Grossly, the excised lesion was primarily cystic, but there
was a solid portion. Microscopically, the cystic portion had
conglomerates of ghost cells within an ameloblastoma-like
odontogenic epithelium. There were deposits of dentinoid
material adjacent to the epithelial lining (Fig 3, A). Enamel

organ-like epithelial islands were observed within the primitive

dental papillaelike mesenchymal tissue of the cyst wall, which
in focal areas was associated with a condensation of the stromal
cells (Fig 3, B). The solid portion of the lesion had a cellular
myxoid stroma with numerous islands of ameloblastic
epithelium, characteristic of ameloblastic fibroma. Ghost cell
masses were also found within ameloblastic epithelium in the
area of ameloblastic fibroma (Fig 3, C).

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Yoon et al 83

Fig 3. (continued).

DISCUSSION
Praetorius et al3 classified the COC into 2 main
entities, a cyst and a neoplasm. The former presented
primarily as a unilocular lesion. Grossly, the lesion
described in this report was primarily cystic, but there
was a solid portion as well. The cystic portion was
typical of COC, and the solid portion was characteristic
of ameloblastic fibroma. Interestingly, a few islands of
ameloblastic epithelium in the ameloblastic fibroma
portion manifested ghost cell features.
The biologic mechanism causing such a unique combination is not readily apparent. The possible pathogenic
mechanisms would seem to be either a collision of
2 separate lesions or a transformation of one lesion
to another. The collision of 2 separate tumors seems
unlikely in this case because both the COC and ameloblastic fibroma components exhibited ghost-cell
changes. It is more likely that this was a single neoplastic
process manifesting 2 distinct types of odontogenic
lesions. COCs have been associated with other odontogenic tumors, most frequently odontoma.3,4,6 It is not
fully understood whether those COCs secondarily
developed features of other odontogenic tumors3,7,10,15
or that the COC features were secondary phenomena in
pre-existing odontogenic tumors.16,17 However, several
investigators have suggested that proliferating odontogenic epithelial islands in COC might induce the adjacent
mesenchymal tissue to develop features of other odontogenic tumors.3,7,9,10,15
In the current case, the proliferation of strands
of odontogenic epithelium from the cyst lining and
the associated condensation of cells within the stroma

resembled primary ectomesenchymal induction of the

dental lamina. It can be speculated that the ameloblastic
fibroma in the lesion may have been induced by the epithelium of the COC. Finally, the lesion may represent
divergent differentiation that the tumors may take,
depending upon the initial inductive stimulus and the
degree of odontogenesis prior to application of the stimulus.9,10,15 Prognosis in this case will probably be as expected for ameloblastic fibroma.
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Reprint requests:
Jin Kim, DDS, PhD
Department of Oral Pathology
Oral Cancer Research Institute
Brain Korea 21 Project for Medical Sciences
Yonsei University College of Dentistry
Seodaemun-gu Shinchon-dong 134
Seoul 120-752, Korea
jink@yumc.yonsei.ac.kr