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Odontogenic ghost cell carcinoma
Odontogenic ghost cell carcinoma Joseph E. Grodjesk, D.D.S., * Herbert B. Dolinsky, D.D.S.,** L.C. Schneider, B.D.S., Ph.D.,*** Elaine H. Dolinsky, M.A., R.N., Ph.D.,**** John L. Doyle, D.D.S., M.Sc.D.,***** Jersey City, Hackensack, Newark, and Piscataway, N.J. CHRIST MEDICINE COLLEGE
HOSPITAL, FAIRLEIGH AND DENTISTRY OF NURSING
S. DlCKtNSON OF NEW JERSEY,
JR. COLLEGE NEW JERSEY
OF DENTAL MEDICINE, DENTAL SCHOOL, AND
UNIVERSITY RUTGERS
and
OF UNIVERSITY
An unusual malignant odontogenic tumor containing large numbers of ghost cells is described. A 46-year-old white man had an aggressive lesion of the right maxilla, multiple pulmonary nodules developed, and he died 1 year after initial diagnosis. Histologic features included columnar odontogenic epithelium, malignant epithelium, microcysts, masses of ghost cells with focal calcification, and small areas of “dentinoid.” The relationship is explored between this tumor and calcifying odontogenic cysts, particularly the “dentinogenic ghost cell tumor” variant. (ORAL SURG. ORAL MED. ORAL PATHOL. 1987;63:576-81)
*Chief, Department of Dentistry, Christ Hospital, Jersey, N.J., and Bayonne Hospital, Bayonne, N.J. **Clinical Associate Professor, Department of Pathology, Fairleigh S. Dickinson Jr. College of Dental Medicine, Hackensack, N.J. ***Professor, Department of Pathology, New Jersey Dental School, Newark, N.J. ****Assistant Professor, College of Nursing, Rutgers University, Piscataway, N.J. *****Professor and Chairman, Department of Pathology, Fairleigh S. Dickinson Jr. College of Dental Medicine, Hackensack, N.J.
Fig. 576
T
he odontogenic ghost cell carcinoma is a malignant counterpart of the keratinizing and calcifying odontogenic cyst (KCOC), a benign lesion originally described by Gorlin and coworkers’ and Gold* and recently redefined by Praetorius and associates.3 The present case is only the sixth to be reported. The first case, illustrated by one photomicrograph, was reported in the World Health Organization’s publication on odontogenic tumors in 197 1.4 The second report, which did not appear until 14 years later, cited still
1. Intraoral photograph of tumor showing palatal expansion.
Volume
63
Odontogenic ghost cell carcinoma
Number 5
2. Panoramicradiographshowingdestructionof right maxilla with obliteration of maxillary sinus (arrows).
Fig.
3. Photomicrographsof biopsy specimen.A, Low-power view showinga cystic lumen lined with anaplasticepithelial cells (arrow) and massesof keratinized ghost cells (KGC). (Hematoxylin and eosin stain.Original magnification,x25.) B, Groupsof ghostcellslying in a backgroundof anaplasticepithelium. (Original magnification, X40.) C, Longitudinal sectionthrough a tube of odontogenicepitheliumin which the central cellsresemblestellatereticulum (SR) surroundedby cells resemblinginner enamelepithelium that is being transformedinto anaplasticepitheilum. (Original magnification, X40.) Fig.
577
570
Grodjesk
et al.
Oral May,
Surg. 1987
in dentigerous cysts or odontomas, but also in other odoatogenic tumors.8-12Thus, it has come to be thought that their presence is not always pathognomonic of KCOC. This focal occurrence of ghost cells has been alluded to in regard to malignant tumors, as well.“,” Some authorities have included odontogenic ghost cell carcinomas as an entity in comprehensive classifications of odontogenic tumors.14 The present case offers the best support to date for this position. CASEREPORT
Fig. 4. Radiographicappearanceof patient at time of hospital admission.Waters projection showingopacification of antrum with extensioninto oral and nasalcavities and into ethmoid sinus.
another case from the Japanese literature.5 In 1986, Ellis and Shmookler6 reported three cases of a ghost cell tumor with malignant clinical and histologic features. The term ghost cell refers to a distinctive type of intracytoplasmic keratinization that results in preservation of both the outline of the cell and a clear area corresponding to the previous location of the nucleus. These ghost cells may occur in small groups or in large masses and are capable of inducing various types of stromal reactions. The epithelium from which they are derived has a close resemblance to that of the dental anlagen, having a basal layer of columnar cells and an overlying layer of cells resembling the stellate reticulum of the enamel organ. Aside from its occurrence in lesions of the toothbearing area, this type of ghost cell keratinization has been described only in craniopharyngioma,’ a tumor of the pituitary gland that presumably develops from epithelial remnants of Rathke’s pouch. In benign lesions, ghost cells have also been described as incidental findings, sometimes focal in nature, usually
On Nov. 14,1979, a 4dyear-old white man sought treatment for a large mass in the right maxilla. The mass extended extraorally to the infraorbital rim and across the nasal midline. Intraorally (Fig. I), there was buccal and palatal expansion of the right maxilla involving the tuberosity and the soft palate. The mass had been present for 4 weeks and had been accompanied by frequent, spontaneous bleeding. There was no significant medical history. A panoramic radiograph showed destruction of the right maxilla with obliteration of the maxillary sinus (Fig. 2). Occlusal and periapical radiographs showed an ill-defined area of moderate density in which partially resorbed tooth fragments were embedded. The tooth fragments were removed, and an incisional biopsy was performed. The biopsy specimen is illustrated in Fig. 3. There were multiple fragments of cellular and vascular connective tissue containing numerous epithelial nests; epithelium was also found on some aspects of the connective tissue surface. Mostly, the epithelial cells were small, pleomorphic, and hyperchromatic; there were frequent mitoses. Elsewhere, there were columnar cells of varying heights, some with polarized nuclei resembling internal enamel epithelium. Rows of tall columnar cells surrounding pale-staining, polygonal cells could be seen in some areas. Dramatic transitions from innocuous-appearing internal enamel epithelium to obviously malignant odontogenic epithelium were observed. Masses of ghost cells intermingling with small, basaloid cells constituted the major proportion of the lesion. These were undergoing calcification in focal areas. Likewise, the stroma contained areas of dystrophic calcification. Areas of necrosis with an acute and chronic inflammatory cell infiltrate were also present. A few small deposits of “dentinoid” in close association with ghost cells stained positively for collagen with the trichrome stains. No tubular dentin was identified. The pathologic diagnosis was carcinoma of the maxilla with “ghost cell” keratinization. The patient was admitted to the hospital. Facial bone radiographs, including tomographs, showed
Volume 63 Number
Odontogenic
ghost cell carcinoma
579
5
5. Photomicrographsof surgical specimen.A, Low-power view showinga proliferating epithelial tumor with the formation of microcystswith peripheralpalisadingof columnarcellsand aggregatesof ghost cells (arrows) in a moderately cellular stroma ISI. (Hematoxylin and eosinstain. Original magnification, ~25.) B, Medium-power view showinganaplasticepithelium (bottom) and ghost cells (top). (Original magnification, X40.) Fig.
opacification of the right maxillary antrum extending into the nasal cavity with erosion of the medial wall (Fig. 4). The inferolateral wall of the antrum appeared to be eroded with extension of the mass into the oral cavity. In the basal view of the facial bone survey, apparent extension into the ethmoid sinuses was seen. The orbital floor was intact. A 99mTc scan showed focal satellite accumulation of tracer material in the right maxilla; the remaining skeleton was unremarkable. The mediastinum, heart, and lungs had a normal appearance at this time. A right maxillectomy was performed by means of the Weber-Ferguson approach; the external carotid
artery was ligated and a split-thickness skin graft was used to line the defect. The histologic appearance of the surgical specimen, shown in Fig. 5, was essentially similar to that of the biopsy specimen. The specimen was composed largely of islands and of columns of epithelium in a stroma of vascular fibrous connective tissue containing scattered inflammatory cell infiltrates. Epithelial cells were mostly polygonal with large, centrally located vesicular nuclei; the cytoplasm was generally eosinophilic but there were occasional foci of clear cells. Some of the epithelial cells were hyperchromatic, and there were occasional mitoses. Palisading of
580
Grodjesk
et al.
Oral Surg. May, 1987
visual obstruction of the right eye, which was closed. Two months later, he was again hospitalized with a large, cauliflower-like mass that infiltrated the right eye and deformed the nose. It was not possible to see inside the mouth. A chest radiograph showed multiple, well-circumscribed nodular densities in both lungs, suggestive of metastatic disease (Fig. 6). The patient was transferred to a cancer hospital, where he died the following day. No autopsy was performed. DISCUSSION
8. Chestradiographshowingnodular densitiesrepresentingmetastatictumor (arrows).
Fig.
the cuboidal and low columnar cells around the periphery of the islands and nests was a prominent feature in some areas of the tumor. There were also focal areas of necrosis. Ghost cells were present singly, in small groups, and in large masses. In some parts of the specimen, clefts occurred within the epithelial islands; cells in these areas exhibited acantholysis. Many of the central cells within these islands were ghost cells, while the peripheral cells were cuboidal or low columnar, an arrangement suggestive of microcyst formation. Ghost cells, giant cells, and occasional osteoid trabeculae were present in the connective tissue stroma. The pathologic diagnosis was epithelial odontogenic tumor, cytologically malignant. A course of cobalt 60 teletherapy was begun on Feb. 6, 1980. The neck region received a total of 5,000 rads; the right maxilla received 6,200 rads. In July 1980, the patient sought treatment for trismus and pain in the area of the right temporomandibular joint. He was readmitted a month later with a recurrent tumor, spontaneous bleeding both intraorally and from the nose, and weight loss. There was
The capacity of the lining of follicular cysts to undergo malignant transformation into squamous cell carcinoma has been appreciated for many years and is one of the reasons these cysts should be examined pathologically. The existence of malignant counterparts of benign odontogenic tumors is a matter of more recent recognition and has led to attempts at classification of these lesions. The World Health Organization (WHO) sponsored a classification of odontogenic tumors that listed three types of odontogenic carcinomas: malignant ameloblastoma, primary intraosseous carcinoma, and carcinomas arising from odontogenic epithelium, including those arising from odontogenic cysts.4 A modification of this classification was offered by Elzay,15 who subdivided malignant tumors arising from ameloblastomas into two groups; those that were well differentiated he called malignant ameloblastoma and those that were poorly differentiated he called ameloblastic carcinoma. He believed that malignant tumors arising from cysts constituted the majority of cases. The histologic features required for the diagnosis of KCOC4 are an epithelial lining showing a welldefined basal layer of columnar cells; an overlying layer, often many cells thick, that may resemble stellate reticulum of the enamel organ; and masses of ghost epithelial cells, either in the cyst lining or in the fibrous wall, or in both. The epithelial ghost cells sometimes become calcified. A peculiar collagenous matrix often found in association with the basal layer of the epithelium traditionally has been referred to as “dentinoid,” although its true nature has never been established. More recently, it has been suggested3 that some lesions previously included as examples of KCOC were actually neoplastic rather than cystic. The term dentinogenic ghost cell tumor was proposed for this small minority of cases. The neoplastic variant shares many features with the cystic type but also exhibits proliferation of strands and islands of odontogenic epithelium in a solid growth pattern, with
Volume Number
63 5
cyst formation as a secondary phenomenon. Varying numbers of ghost cells are present in the epithelium, and dentinoid may be found in contact with the odontogenic epithelium. In the current case, the tumor contained odontogenie epithelium, evidence of cystic or microcystic structures, ghost cells with some calcification, dentinoid, and osteoid. It was cytologically malignant, recurred, and metastasized to the lungs with a fatal outcome. We agree that the most appropriate designation for this lesion is odontogenic ghost cell carcinoma, as previously suggested.14 It cannot be determined from reconstruction of this case whether the tumor originated from a previously benign lesion and, if so, whether that precursor was a cyst or a neoplasm. Origin from a previously benign lesion is probable, we believe, because of the foci of epithelium with a benign appearance within the tumor. ADDENDUM
The probability exists that our patient is identical to the patient in Case 3 in an article from the AFIP published during preparation of our manuscript.6 The contributor of that case was not identified, but the clinical description and photomicrographs appear identical with the important exception that their patient is described as being alive and free of disease 6 years after surgery. A notation on the hospital pathology report in our case indicates that an AFIP consultation was requested in January 1980. REFERENCES 1. Gorlin RJ, Pindborg JJ, Clausen FP, Vickers RA. The calcifying odontogenic cyst. A possible analogue of the cutaneous calcifying epithelioma of Malherbe. ORAL SURG ORAL MED ORAL PATHOL 1962: 15: 1235-43.
Odontogenic ghost cell carcinoma
581
2. Gold L. The keratinizing and calcifying odontogenic cyst. ORAL SURG ORAL MED ORAL PATHOL 1963;16:1414-24. F, Hjorting-Hansen E, Gorlin RJ, Vickers RA. 3. Praetorius Calcifying odontogenic cyst. Range, variations, and neoplastic potential. Acta Odontol Stand 1981;39:227-40. 4. Pindborg JJ, Kramer IR, Torloni H. Histological typing of odontogenic tumors, jaw cysts, and allied lesions. Geneva: World Health Organization, 197 I. 5. Ikemura K, Horie A, Tashiro H, Nandate M. Simultaneous occurrence of a calcifying odontogenic cyst and its malignant transformation. Cancer 1985;56:2861-4. 6. Ellis GL, Shmookler BM. Aggressive (malignant?) epithelial odontogenic ghost cell tumor. ORAL SURG ORAL MED ORAL PATHOL 1986;61:471-8. I. Regezi JA, Courtney RM, Kerry DA. Keratinization in odontogenic tumors. ORAL SURG ORAL MED ORAL PATHOL 1915;39:447-55. 8. Farman AG, Smith SN, Nortje CJ, Grotepass FW. Calcifying odontogenic cyst with ameloblastic fibro-odontome: one lesion or two? J Oral Pathol 1978;7:19-27. 9. Levy BA. Ghost cells and odontomas. ORAL SURG ORAL MED ORAL PATHOL 1973;36:851-5. 10. Osborne TP, Park JK, Levy BA, Tewes MD. Odontoma containing ghost cells in the maxillary sinus. ORAL SURG ORAL MED ORAL PATHOL 1974;38:819-23. 11. Sedan0 HO, Pindborg JJ. Ghost ceil epithelium in odontomas. J Oral Pathol 1975;4:27-30. 12. Vuletin JC, Solomon MP, Pertschuk LP. Periapical odontogenie tumor with ghost-cell keratinization. A histologic fluorescent microscopic, and ultrastructural study. ORAL SURG ORAL MED ORAL PATHOL 1978;45:406-15. 13. Anneroth G, Hansen LS. Variations in kertinizing odontogenic cysts and tumors. ORAL SURG ORAL MED ORAL PATHOL l982;54:530-46. 14. Pindborg JJ, Rick GM. Odontogenic tumors: classification and diagnostic problems. Orlando, Florida: Continuing Education Program, American Academy of Oral Pathology, 1983. 15. Elzay RP. Primary intraosseous carcinoma of the jaws. Review and update of odontogenic carcinoma. ORAL SURG ORAL MED ORAL PATHOL 1982;54:299-303. Reprint requests to: Dr. Joseph E. Grodjesk 2784 Kennedy Boulevard Jersey City, NJ 07306
HOSPITAL, FAIRLEIGH AND DENTISTRY OF NURSING
S. DlCKtNSON OF NEW JERSEY,
JR. COLLEGE NEW JERSEY
OF DENTAL MEDICINE, DENTAL SCHOOL, AND
UNIVERSITY RUTGERS
and
OF UNIVERSITY
An unusual malignant odontogenic tumor containing large numbers of ghost cells is described. A 46-year-old white man had an aggressive lesion of the right maxilla, multiple pulmonary nodules developed, and he died 1 year after initial diagnosis. Histologic features included columnar odontogenic epithelium, malignant epithelium, microcysts, masses of ghost cells with focal calcification, and small areas of “dentinoid.” The relationship is explored between this tumor and calcifying odontogenic cysts, particularly the “dentinogenic ghost cell tumor” variant. (ORAL SURG. ORAL MED. ORAL PATHOL. 1987;63:576-81)
*Chief, Department of Dentistry, Christ Hospital, Jersey, N.J., and Bayonne Hospital, Bayonne, N.J. **Clinical Associate Professor, Department of Pathology, Fairleigh S. Dickinson Jr. College of Dental Medicine, Hackensack, N.J. ***Professor, Department of Pathology, New Jersey Dental School, Newark, N.J. ****Assistant Professor, College of Nursing, Rutgers University, Piscataway, N.J. *****Professor and Chairman, Department of Pathology, Fairleigh S. Dickinson Jr. College of Dental Medicine, Hackensack, N.J.
Fig. 576
T
he odontogenic ghost cell carcinoma is a malignant counterpart of the keratinizing and calcifying odontogenic cyst (KCOC), a benign lesion originally described by Gorlin and coworkers’ and Gold* and recently redefined by Praetorius and associates.3 The present case is only the sixth to be reported. The first case, illustrated by one photomicrograph, was reported in the World Health Organization’s publication on odontogenic tumors in 197 1.4 The second report, which did not appear until 14 years later, cited still
1. Intraoral photograph of tumor showing palatal expansion.
Volume
63
Odontogenic ghost cell carcinoma
Number 5
2. Panoramicradiographshowingdestructionof right maxilla with obliteration of maxillary sinus (arrows).
Fig.
3. Photomicrographsof biopsy specimen.A, Low-power view showinga cystic lumen lined with anaplasticepithelial cells (arrow) and massesof keratinized ghost cells (KGC). (Hematoxylin and eosin stain.Original magnification,x25.) B, Groupsof ghostcellslying in a backgroundof anaplasticepithelium. (Original magnification, X40.) C, Longitudinal sectionthrough a tube of odontogenicepitheliumin which the central cellsresemblestellatereticulum (SR) surroundedby cells resemblinginner enamelepithelium that is being transformedinto anaplasticepitheilum. (Original magnification, X40.) Fig.
577
570
Grodjesk
et al.
Oral May,
Surg. 1987
in dentigerous cysts or odontomas, but also in other odoatogenic tumors.8-12Thus, it has come to be thought that their presence is not always pathognomonic of KCOC. This focal occurrence of ghost cells has been alluded to in regard to malignant tumors, as well.“,” Some authorities have included odontogenic ghost cell carcinomas as an entity in comprehensive classifications of odontogenic tumors.14 The present case offers the best support to date for this position. CASEREPORT
Fig. 4. Radiographicappearanceof patient at time of hospital admission.Waters projection showingopacification of antrum with extensioninto oral and nasalcavities and into ethmoid sinus.
another case from the Japanese literature.5 In 1986, Ellis and Shmookler6 reported three cases of a ghost cell tumor with malignant clinical and histologic features. The term ghost cell refers to a distinctive type of intracytoplasmic keratinization that results in preservation of both the outline of the cell and a clear area corresponding to the previous location of the nucleus. These ghost cells may occur in small groups or in large masses and are capable of inducing various types of stromal reactions. The epithelium from which they are derived has a close resemblance to that of the dental anlagen, having a basal layer of columnar cells and an overlying layer of cells resembling the stellate reticulum of the enamel organ. Aside from its occurrence in lesions of the toothbearing area, this type of ghost cell keratinization has been described only in craniopharyngioma,’ a tumor of the pituitary gland that presumably develops from epithelial remnants of Rathke’s pouch. In benign lesions, ghost cells have also been described as incidental findings, sometimes focal in nature, usually
On Nov. 14,1979, a 4dyear-old white man sought treatment for a large mass in the right maxilla. The mass extended extraorally to the infraorbital rim and across the nasal midline. Intraorally (Fig. I), there was buccal and palatal expansion of the right maxilla involving the tuberosity and the soft palate. The mass had been present for 4 weeks and had been accompanied by frequent, spontaneous bleeding. There was no significant medical history. A panoramic radiograph showed destruction of the right maxilla with obliteration of the maxillary sinus (Fig. 2). Occlusal and periapical radiographs showed an ill-defined area of moderate density in which partially resorbed tooth fragments were embedded. The tooth fragments were removed, and an incisional biopsy was performed. The biopsy specimen is illustrated in Fig. 3. There were multiple fragments of cellular and vascular connective tissue containing numerous epithelial nests; epithelium was also found on some aspects of the connective tissue surface. Mostly, the epithelial cells were small, pleomorphic, and hyperchromatic; there were frequent mitoses. Elsewhere, there were columnar cells of varying heights, some with polarized nuclei resembling internal enamel epithelium. Rows of tall columnar cells surrounding pale-staining, polygonal cells could be seen in some areas. Dramatic transitions from innocuous-appearing internal enamel epithelium to obviously malignant odontogenic epithelium were observed. Masses of ghost cells intermingling with small, basaloid cells constituted the major proportion of the lesion. These were undergoing calcification in focal areas. Likewise, the stroma contained areas of dystrophic calcification. Areas of necrosis with an acute and chronic inflammatory cell infiltrate were also present. A few small deposits of “dentinoid” in close association with ghost cells stained positively for collagen with the trichrome stains. No tubular dentin was identified. The pathologic diagnosis was carcinoma of the maxilla with “ghost cell” keratinization. The patient was admitted to the hospital. Facial bone radiographs, including tomographs, showed
Volume 63 Number
Odontogenic
ghost cell carcinoma
579
5
5. Photomicrographsof surgical specimen.A, Low-power view showinga proliferating epithelial tumor with the formation of microcystswith peripheralpalisadingof columnarcellsand aggregatesof ghost cells (arrows) in a moderately cellular stroma ISI. (Hematoxylin and eosinstain. Original magnification, ~25.) B, Medium-power view showinganaplasticepithelium (bottom) and ghost cells (top). (Original magnification, X40.) Fig.
opacification of the right maxillary antrum extending into the nasal cavity with erosion of the medial wall (Fig. 4). The inferolateral wall of the antrum appeared to be eroded with extension of the mass into the oral cavity. In the basal view of the facial bone survey, apparent extension into the ethmoid sinuses was seen. The orbital floor was intact. A 99mTc scan showed focal satellite accumulation of tracer material in the right maxilla; the remaining skeleton was unremarkable. The mediastinum, heart, and lungs had a normal appearance at this time. A right maxillectomy was performed by means of the Weber-Ferguson approach; the external carotid
artery was ligated and a split-thickness skin graft was used to line the defect. The histologic appearance of the surgical specimen, shown in Fig. 5, was essentially similar to that of the biopsy specimen. The specimen was composed largely of islands and of columns of epithelium in a stroma of vascular fibrous connective tissue containing scattered inflammatory cell infiltrates. Epithelial cells were mostly polygonal with large, centrally located vesicular nuclei; the cytoplasm was generally eosinophilic but there were occasional foci of clear cells. Some of the epithelial cells were hyperchromatic, and there were occasional mitoses. Palisading of
580
Grodjesk
et al.
Oral Surg. May, 1987
visual obstruction of the right eye, which was closed. Two months later, he was again hospitalized with a large, cauliflower-like mass that infiltrated the right eye and deformed the nose. It was not possible to see inside the mouth. A chest radiograph showed multiple, well-circumscribed nodular densities in both lungs, suggestive of metastatic disease (Fig. 6). The patient was transferred to a cancer hospital, where he died the following day. No autopsy was performed. DISCUSSION
8. Chestradiographshowingnodular densitiesrepresentingmetastatictumor (arrows).
Fig.
the cuboidal and low columnar cells around the periphery of the islands and nests was a prominent feature in some areas of the tumor. There were also focal areas of necrosis. Ghost cells were present singly, in small groups, and in large masses. In some parts of the specimen, clefts occurred within the epithelial islands; cells in these areas exhibited acantholysis. Many of the central cells within these islands were ghost cells, while the peripheral cells were cuboidal or low columnar, an arrangement suggestive of microcyst formation. Ghost cells, giant cells, and occasional osteoid trabeculae were present in the connective tissue stroma. The pathologic diagnosis was epithelial odontogenic tumor, cytologically malignant. A course of cobalt 60 teletherapy was begun on Feb. 6, 1980. The neck region received a total of 5,000 rads; the right maxilla received 6,200 rads. In July 1980, the patient sought treatment for trismus and pain in the area of the right temporomandibular joint. He was readmitted a month later with a recurrent tumor, spontaneous bleeding both intraorally and from the nose, and weight loss. There was
The capacity of the lining of follicular cysts to undergo malignant transformation into squamous cell carcinoma has been appreciated for many years and is one of the reasons these cysts should be examined pathologically. The existence of malignant counterparts of benign odontogenic tumors is a matter of more recent recognition and has led to attempts at classification of these lesions. The World Health Organization (WHO) sponsored a classification of odontogenic tumors that listed three types of odontogenic carcinomas: malignant ameloblastoma, primary intraosseous carcinoma, and carcinomas arising from odontogenic epithelium, including those arising from odontogenic cysts.4 A modification of this classification was offered by Elzay,15 who subdivided malignant tumors arising from ameloblastomas into two groups; those that were well differentiated he called malignant ameloblastoma and those that were poorly differentiated he called ameloblastic carcinoma. He believed that malignant tumors arising from cysts constituted the majority of cases. The histologic features required for the diagnosis of KCOC4 are an epithelial lining showing a welldefined basal layer of columnar cells; an overlying layer, often many cells thick, that may resemble stellate reticulum of the enamel organ; and masses of ghost epithelial cells, either in the cyst lining or in the fibrous wall, or in both. The epithelial ghost cells sometimes become calcified. A peculiar collagenous matrix often found in association with the basal layer of the epithelium traditionally has been referred to as “dentinoid,” although its true nature has never been established. More recently, it has been suggested3 that some lesions previously included as examples of KCOC were actually neoplastic rather than cystic. The term dentinogenic ghost cell tumor was proposed for this small minority of cases. The neoplastic variant shares many features with the cystic type but also exhibits proliferation of strands and islands of odontogenic epithelium in a solid growth pattern, with
Volume Number
63 5
cyst formation as a secondary phenomenon. Varying numbers of ghost cells are present in the epithelium, and dentinoid may be found in contact with the odontogenic epithelium. In the current case, the tumor contained odontogenie epithelium, evidence of cystic or microcystic structures, ghost cells with some calcification, dentinoid, and osteoid. It was cytologically malignant, recurred, and metastasized to the lungs with a fatal outcome. We agree that the most appropriate designation for this lesion is odontogenic ghost cell carcinoma, as previously suggested.14 It cannot be determined from reconstruction of this case whether the tumor originated from a previously benign lesion and, if so, whether that precursor was a cyst or a neoplasm. Origin from a previously benign lesion is probable, we believe, because of the foci of epithelium with a benign appearance within the tumor. ADDENDUM
The probability exists that our patient is identical to the patient in Case 3 in an article from the AFIP published during preparation of our manuscript.6 The contributor of that case was not identified, but the clinical description and photomicrographs appear identical with the important exception that their patient is described as being alive and free of disease 6 years after surgery. A notation on the hospital pathology report in our case indicates that an AFIP consultation was requested in January 1980. REFERENCES 1. Gorlin RJ, Pindborg JJ, Clausen FP, Vickers RA. The calcifying odontogenic cyst. A possible analogue of the cutaneous calcifying epithelioma of Malherbe. ORAL SURG ORAL MED ORAL PATHOL 1962: 15: 1235-43.
Odontogenic ghost cell carcinoma
581
2. Gold L. The keratinizing and calcifying odontogenic cyst. ORAL SURG ORAL MED ORAL PATHOL 1963;16:1414-24. F, Hjorting-Hansen E, Gorlin RJ, Vickers RA. 3. Praetorius Calcifying odontogenic cyst. Range, variations, and neoplastic potential. Acta Odontol Stand 1981;39:227-40. 4. Pindborg JJ, Kramer IR, Torloni H. Histological typing of odontogenic tumors, jaw cysts, and allied lesions. Geneva: World Health Organization, 197 I. 5. Ikemura K, Horie A, Tashiro H, Nandate M. Simultaneous occurrence of a calcifying odontogenic cyst and its malignant transformation. Cancer 1985;56:2861-4. 6. Ellis GL, Shmookler BM. Aggressive (malignant?) epithelial odontogenic ghost cell tumor. ORAL SURG ORAL MED ORAL PATHOL 1986;61:471-8. I. Regezi JA, Courtney RM, Kerry DA. Keratinization in odontogenic tumors. ORAL SURG ORAL MED ORAL PATHOL 1915;39:447-55. 8. Farman AG, Smith SN, Nortje CJ, Grotepass FW. Calcifying odontogenic cyst with ameloblastic fibro-odontome: one lesion or two? J Oral Pathol 1978;7:19-27. 9. Levy BA. Ghost cells and odontomas. ORAL SURG ORAL MED ORAL PATHOL 1973;36:851-5. 10. Osborne TP, Park JK, Levy BA, Tewes MD. Odontoma containing ghost cells in the maxillary sinus. ORAL SURG ORAL MED ORAL PATHOL 1974;38:819-23. 11. Sedan0 HO, Pindborg JJ. Ghost ceil epithelium in odontomas. J Oral Pathol 1975;4:27-30. 12. Vuletin JC, Solomon MP, Pertschuk LP. Periapical odontogenie tumor with ghost-cell keratinization. A histologic fluorescent microscopic, and ultrastructural study. ORAL SURG ORAL MED ORAL PATHOL 1978;45:406-15. 13. Anneroth G, Hansen LS. Variations in kertinizing odontogenic cysts and tumors. ORAL SURG ORAL MED ORAL PATHOL l982;54:530-46. 14. Pindborg JJ, Rick GM. Odontogenic tumors: classification and diagnostic problems. Orlando, Florida: Continuing Education Program, American Academy of Oral Pathology, 1983. 15. Elzay RP. Primary intraosseous carcinoma of the jaws. Review and update of odontogenic carcinoma. ORAL SURG ORAL MED ORAL PATHOL 1982;54:299-303. Reprint requests to: Dr. Joseph E. Grodjesk 2784 Kennedy Boulevard Jersey City, NJ 07306