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Primary Intraosseous Carcinoma of the Jaws
Narcisse Zwetyenga, MD;
Jacques Pinsolle, MD;
Janine Rivel, MD;
Claire Majoufre-Lefebvre, MD;
Alain Faucher, MD;
Vincent Pinsolle, MD
Arch Otolaryngol Head Neck Surg. 2001;127:794-797.
ABSTRACT
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Objectives To present 8 new cases of primary intraosseous carcinoma of the jaws
and to review the literature for an analysis of treatment modalities and patient
outcomes.
Data Sources A MEDLINE search from 1970 to 1999. The articles chosen and the study
of the references of every one that produced additional articles provided
database information for 28 patients. Eight new patients from our institutions
were added.
Study Selection Our criteria of inclusion included the absence of ulceration of the
oral mucosa, a negative result in the search for a distant primary tumor,
and convincing histological documentation.
Data Extraction The variables of the analysis included age, sex, site of the tumor,
condition of the oral mucosa, tumor size, neck status, treatment modalities,
recurrences, and survival.
Data Synthesis Twenty-eight patients were identified in the literature, for a total
of 36 patients. There were 28 males (78%) and 8 females (22%) ranging in age
from 4 to 76 years (mean, 54 years). The tumor site was the mandible in 33
patients (92%) and the maxilla in 3 (8%). Of the 34 patients treated, 19 (56%)
had recurrences. Overall 2- and 4-year survival was 60.5% and 39.9%, respectively.
Patients who underwent radical surgery and postoperative radiotherapy (n =
11) had a 2- and 3-year survival probability of 61.3% and 40.9%, respectively,
whereas in the remaining patients (n = 25), the rates were 59.7% and 31.3%,
respectively (P = .60).
Conclusions Strict diagnostic criteria must be applied. The prognosis associated
with primary intraosseous carcinoma of the jaws is poor and suggests the need
for aggressive treatment.
INTRODUCTION
PRIMARY INTRAOSSEOUS carcinoma (PIOC) of the jaws is a rare tumor presumably
developing from residues of the odontogenic epithelium.1-3
It is one of the odontogenic carcinomas arising in the jaws, which are divided
into 4 types: (1) malignant ameloblastoma, (2) PIOC, (3) malignant variants
of other odontogenic epithelial tumors, and (4) malignant changes in odontogenic
cysts.2, 4 Only a few cases have
been reported in the literature. Some reports3
include an exhaustive review of the published cases to define the diagnostic
criteria. However, information about treatment modalities, patient outcomes,
and comparisons with other oral cavity primary sites of squamous cell carcinoma
are rarely available. The tumor usually presents as a painful swelling in
the jaw area,3, 5 but may be asymptomatic
and found on a routine panoramic radiograph.6
The diagnostic criteria are now well-defined: no ulceration of oral mucosa
should be observed (Figure 1), and
another primary site should be excluded.3 The
tumor seems to arise from any residual odontogenic epithelium, such as remnants
of the dental lamina or of the Hertwig root sheath.7
Microscopically, most PIOCs show the same histological features as squamous
cell carcinoma of any squamous epithelium (eg, the oral mucosa). The tumor
is composed of sheets; islands; and strands of squamous cells with marked
cellular pleomorphism, nuclear hyperchromatism, and mitotic activity. In a
few cases, however, the tumor has a distinctly odontogenic pattern, with basal-type
cells arranged in alveoli or in a plexiform pattern with palisading of the
peripheral cells (Figure 2). The
standard treatment includes surgery and/or radiation therapy,5, 8-9
and the prognosis is severe.3, 5, 8, 10
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Figure 1. In the parasagitally sectioned
surgical specimen, a strictly intraosseous tumor is visible with a pseudocapsula.
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Figure 2. Photomicrograph of a well-differentiated
cell carcinoma with peripheral bone resorption; islands of epithelium display
peripheral palisading and individual cell keratinization. Abnormal keratinization
with large pearl formation and cellular pleomorphism is noted (hematoxylin-eosin-saffron,
original magnification x100).
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We reviewed information about cases of intraosseous carcinoma from published
reports and performed an analysis of the data including 8 new cases from our
institutions. The aims of the study were to confirm the diagnostic criteria,
to assess the difficulty of staging such tumors and of determining patient
outcomes, and to try to define a consistent method of treatment.
MATERIALS AND METHODS
Two sources were used to identify well-documented case reports of PIOC.
A MEDLINE search from 1970 to 1999 was performed first. The choice was based
on the reports of treatment methods and survival data for individual patients.
The articles chosen and the study of the references of every one that produced
additional articles amounted to 27,5-6,8-32
215, 8-10,12, 14-17,19-22,24, 26, 28-32
of which presented 28 identified cases of 63 reported cases. Thirty-five cases
from different reports were rejected owing to a lack of a search for another
primary site,10-11,13, 18, 23
different histological features,20, 23, 27, 32
the presence of an ulceration of the oral mucosa,11
or a tumor arising from an odontogenic cyst.23, 28
Individual patient data, and not results of series, were entered into the
database.
Meanwhile, we reviewed in our files 15 cases with the suspected diagnosis
of PIOC. Careful assessment of the clinical criteria eliminated 4 cases. Of
the 11 remaining cases, 3 were eliminated after a new histological examination
of the specimens. Therefore, 8 new cases were identified and added to the
28 previous cases, so a total of 36 cases were included in the statistical
analysis.
Our criteria of inclusion were the absence of ulceration of the oral
mucosa, a negative result in the search for a distant primary tumor, and convincing
histological documentation.2-3
The analytical variables included age, sex, site of the tumor, condition of
the oral mucosa, tumor size, neck status, treatment modalities, recurrences,
and survival. All patient data were recorded on a computer file (MEDLOG).
Survival and recurrence data were analyzed with Statistical Product and Service
Solutions software (SPSS Inc, Chicago, Ill). The survival probabilities were
calculated with the actuarial method. The Wilcoxon (Gehan) test was used for
comparison between factors.
RESULTS
The tumors of the 8 cases from our institutions were all located in
the mandible, with only 1 anterior location. The first symptom was swelling
in the mandibular area in 7 cases, which was painful in 3. One tumor was found
on a routine panoramic radiograph. The oral mucosa was normal, and the search
for a distant primary tumor was negative in all patients. All patients underwent
radical surgery (hemimandibulectomy) at the tumor site. Details about the
size of tumors, neck treatment, and follow-up are shown in Table 1.
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Details of the Patients From Our Institutions*
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Twenty-eight patients with the confirmed diagnosis of PIOC were identified
in the literature, for a total of 36 patients. There were 28 males (78%) and
8 females (22%) ranging in age from 4 to 76 years (mean, 54 years). Twenty-four
patients (67%) were aged 50 years or older. The primary tumor site was the
mandible in 33 patients (92%) and the maxilla in 3 (8%) (Figure 3). Twenty-eight patients (78%) had swelling in the jaw,
which was painful in 15 (54%) of them. Twelve patients had lower lip anesthesia,
complete in 5 (14%) and incomplete in 7 (19%). Dental mobility was reported
in 10 patients (28%). Eighteen patients (50%) had undergone teeth removal
during the year previous to the diagnosis. The mean delay between presenting
symptoms and diagnosis was 5.9 months (range, 2-18 months). Ten patients (28%)
had clinically homolateral positive neck nodes at the time of the diagnosis,
7 in the submandibular triangle and 3 in the lateral neck. Follow-up information
was available for 35 patients (mean follow-up, 29 months). As for radiographic
features, osteolytic bone changes were noted in all patients. The margins
were irregular in 22 patients (63%) and regular in 10 (29%); no precision
was available for 3 patients (9%) (percentages total >100 because of rounding).
Five patients (14%) had a pathological fracture of the mandible.
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Figure 3. Anatomical distribution of the
36 lesions.
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Data about treatment modalities were available for 34 patients and are
as follows:
Surgery at the tumor site consisted of hemimandibulectomy in 23 patients,
marginal mandibular resection in 5, curettage in 2, and maxillectomy in 3
(for tumors of the maxilla). In the 33 patients treated by surgery, 23 underwent
a neck dissection (22 primary and 1 delayed dissection). In 10 (43%) of them,
there was histological evidence of node metastasis in the neck specimen. Ten
patients underwent mandibular reconstruction. Of the 34 patients treated,
19 (56%) had recurrences; 12 experienced a local recurrence in a mean delay
of 7.8 months (range, 1-36 months), 2 had a neck recurrence, and 5 had distant
metastases (2 bone and 3 lung metastases). The 2-year probability of nonrecurrence
was 51.0%. None of these recurrences could eventually be controlled. The estimated
overall 2-year survival was 60.5%. Patients who underwent radical surgery
and postoperative radiotherapy (n = 11) had a 2- and 3-year survival probability
of 61.3% and 40.9%, respectively, whereas in the remaining patients (n = 25),
the rates were 59.7% and 31.3%, respectively (P =
.60). The median survival was 40.6 months in the former and 29.6 months in
the latter. The comparison of estimated survival between patients who underwent
a neck dissection and the other patients was not statistically significant
(P = .81). The study of prognostic factors was not
significant.
COMMENT
Primary intraosseous carcinoma is a rare tumor whose diagnostic criteria
have been well-defined3, 5 and
must be fully applied to confirm any new case. To eliminate an oral cavity
cancer (particularly originating in the gum) with osseous involvement, no
ulceration of the oral mucosa should be observed. Moreover, excluding a metastatic
lesion of the bone for another primary site necessitates a careful physical
examination and a chest radiograph with a posttreatment observation period
of at least 6 months. In the event of an early death, an autopsy reporting
no evidence of another primary site is mandatory. Moreover, the pathologist
must differentiate the lesion from a malignant ameloblastoma or "other carcinomas
arising from the odontogenic epithelium including those arising from odontogenic
cysts."1(p21),2
More than two thirds of the patients are aged 50 years or older, but the tumor
may arise in young patients (there were 2 patients <20 years), unlike oral
cavity carcinomas, which are exceptional in the first 2 decades of life. The
male-female ratio is 3.5:1, but the classic oral mucosa carcinoma background
of alcohol or tobacco abuse is not present. The predominant location is the
posterior part of the mandible, which is similar to benign tumors and odontogenic
cysts.3, 11 However, in the anterior
cases, the origin might be epithelial remnants in the deeper structures during
the fusion of the facial processes,4-5
which is at variance with the World Health Organization's definition. Yet,
remnants of the dental lamina can be found in a median location. The tumor
usually presents as possibly painful swelling in the jaw area,3, 5
but may be asymptomatic and found on a routine panoramic radiograph.6 In most cases, the diagnostic delay is due to previous
teeth removal without a panoramic radiograph and to having attributed the
symptoms to operative complications. The radiographic features show great
variations in size and shape, but in many cases a large tumor is found. Histologically,
it may be difficult to decide whether the tumor should be classified as a
PIOC or as a malignant ameloblastoma when it presents an odontogenic pattern.
The diagnosis of malignant ameloblastoma can be made if there is histological
evidence of a previous tumor. If not, the diagnosis is differentiated squamous
carcinoma. A definite diagnosis of PIOC is possible only with reliable clinical
and radiographic data.2, 7
No conclusion can be drawn from this study regarding prognostic factors
owing to the few patients enrolled. However, the number was large enough and
the follow-up long enough to evaluate local and general results. Apart from
age and general status or delay in establishing the correct diagnosis, we
suspect that, as in oral cavity squamous carcinoma,33
the tumor extension (herein equivalent to a T4 tumor) and neck node involvement
are of the utmost importance. In fact, there is a problem concerning the staging
of such tumors. Since they are squamous carcinoma, they cannot be classified
as primary bone tumors even if their location is intraosseous. Also, they
cannot be classified according to the international classifications of head
and neck squamous carcinomas because every tumor would be classified T4 regardless
of its size since they are closely in contact with the bone marrow, with a
high risk of neck node involvement, thus accounting for their poor outcome.
The only possibility seems to be to report the tumor size on computed tomographic
scanning.
As for treatment, there is no consensus so far. Standard treatment includes
surgery and/or radiation therapy,5, 8-9
and the prognosis is severe.3, 5, 8, 10
All our patients but 1 were treated with radical surgery, neck dissection,
and postoperative radiotherapy. The only exception was a patient with an initially
false diagnosis of ameloblastoma. Of the 7 patients who underwent the complete
treatment sequence, only 1 experienced a local recurrence. Two patients died
of pulmonary metastases, and in both there was histological neck node involvement
with extracapsular spread. The mainstay of treatment remains surgery, owing
to the intraosseous location of the tumor, which proscribes definitive radiation
therapy. Postoperative radiotherapy seems to be useful. In fact, 2 arguments
favor combined treatment. First, because PIOC is clearly similar to locally
advanced squamous carcinoma of the oral mucosa, particularly T4 gum carcinoma,
the same aggressive treatment seems to be recommendable.34
Second, while the comparison of survival according to the treatment modalities
is not statistically significant, the trend is in favor of radical surgery
and postoperative radiotherapy. Therefore, for mandibular tumors, hemimandibulectomy
with neck dissection (functional or radical, according to the neck status)
can be suggested along with postoperative radiotherapy including the primary
site and bilateral neck areas.5, 8
This attitude is justified by the need to obtain safe margins in the primary
site and the high risk of neck node involvement with a tumor closely in contact
with the bone marrow. As for tumors in the maxilla, maxillectomy seems mandatory,
but neck dissection might be optional because of the different lymphatic drainage
mechanisms at this site.
CONCLUSIONS
Primary intraosseous carcinoma is a rare tumor that behaves like a locally
advanced carcinoma of the oral cavity. Strict diagnostic criteria must be
applied, with primary emphasis on the nonconnection of the tumor with the
oral mucosa and on the histological features. The prognosis associated with
PIOC of the jaws is poor and suggests the need for aggressive treatment. Surgery
and postoperative radiation therapy seem to provide the best results.
AUTHOR INFORMATION
Accepted for publication March 16, 2001.
Presented as a poster at the annual meeting of the American Head and
Neck Society, Fifth International Conference on Head and Neck Cancer, San
Francisco, Calif, July 29-August 2, 2000.
We thank Veronique Picot for performing the statistical analysis.
Corresponding author and reprints: Jacques Pinsolle, MD, Department
of Maxillofacial and Plastic Surgery, Centre Hospitalier Universitaire de
Bordeaux, 33076 Bordeaux CEDEX, France (e-mail: jacques.pinsolle{at}chu-bordeaux.fr).
From the Departments of Maxillofacial and Plastic Surgery (Drs Zwetyenga,
J. Pinsolle, and Majoufre-Lefebvre) and Pathology (Dr Rivel), Centre Hospitalier
Universitaire de Bordeaux, and the Department of Surgery, Institut Bergonié
(Drs Faucher and V. Pinsolle), Bordeaux, France.
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