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Intraosseous tumors of the maxilla
Original INTRAOSSEOUS H. BLUMEKTHAL,
M.D.,
Articles
TUMORS
OF THE
ASD H. BRUNNER,
MAXILLA
M.D.,
CHICAGO, ILL.
NTRAOSSEOUS tumors of the maxilla, except dentogenous cysts, are not common. Because these tumors may extend into the maxillary sinus, they are interesting for both the dentist and the rhinologist. We had the opportunity to perform an operation on two cases of this type. The rarity of the cases and the microscopic findings of the tumors warrant this report.
I
CASE REPORTS CASE I.-I. MI., white, female, aged 45. When 2 years of age, her first tooth appeared. Her mother told her that all her deciduous teeth were decayed when they came in except a few in front. The patient also said that all her permanent teeth except six mandibular teeth were decayed when they appeared. She claimed to have had much dental care all her life. Nine years ago she had all her teeth extracted except the six mandibular teeth in front At the time all her teeth were which have always remained in good condition. extracted, she said that most of them were only small pegs or roots which were remaining from the caries she had had. About three years ago, she began to have sudden attacks of sharp pain in the right cheek. It was first noticed on arising in the morning. Pain radiated to the right eye and to the right occipital region. The right side of the face became swollen and “black and blue.” Pain subsided in three to four hours and the swelling decreased in seven to eight hours. These attacks occur approximately once a week and are getting progressively worse. On admission, on Dec. 3, 1945, the pupils were found to be normal. She complained of a general urticaria. Several of the nails were longitudinally striated. A few showed loss of substance near t,he roots; one was yellowed and slightly irregular at the lunula. In the maxilla there were no teeth. There was a swelling of the right upper alveolar ridge. The swelling was hard in consistency, covered by a pale gingiva, and extended toward the hard palate. X-ray examination on Nov. 20, 1945, revealed bone destruction involving the right upper alveolar ridge and the floor of the right antrum. There was some cloudiness over the lateral border of the antrum. X-ray examination on Dec. 3, 1945, showed an osteoporosis of the alveolar ridge of the maxilla; the sinuses were normal. Blood examination, including Kahn’s test, did not reveal any pathology. The tentative diagnosis of a localized ostcit.is fibrosa was made. On December 4, a sublabial incision was made I cm. from College
From of
the Department Medicine.
of
Laryngolopy.
Rhinology 815
and
Otology,
IJniversity
of
Illinois.
H. Blumenthal
and H. Brunner
the alveolar edge and the mucosa elevated from the bone. A rongeur was used to remove four to five pieces of porous bone. The diagnosis was adamantinoma. On Jan. 12, 1946, a radical operation was performed. The bone of the canine fossa was extremely thin and, to a great extent, absent, exposing the mucosa of the maxillary sinus; the bone of the piriform aperture had a normal appearance. The maxillary sinus was empty. The mucosa was pale; at the site of the alveolar ridge it was thickened. The lateral wall of the inferior nasal meatus was extremely thin and was removed, creating a large communication with the nasal cavity. The inferior turbinate was apparently normal. The normal gingiva and the thickened paranasal mucosa were elevated from the alveolar ridge, exposing a tumor about 5 cm. long, which occupied the marrow of the alveolar process and extended approximately from the area of the second incisor to t,he posterior end of the alveolar process. The tumor had a red color, contained a small amount of blood vessels, and had the consistency of granulation tissue. It had distended the external corticalis of the alveolar process outward, and had perforated it at several sites so that the external surface of the tumor was covered only by fragments of thin bone (Fig. 1). It was not difficult to elevate the tumor from the internal cortex of the alveolar process which was about 3 cm. in thickness and was considerably sclerotic. Resection of the alveolar process was performed, bringing into view the entire tumor which extended between the mucosa and the bone of the hard palate toward the midline and toward the canalis pterygo-palatinus, where the tumor was firmly adherent to the blood vessels and nerves. The tumor was easily elevated from the bone of the hard palate. The latter, being soft in consistency, was removed up to the canalis pterygo-palatinus. The gingiva was sutured to the mucosa of the cheek and the maxillary sinus was drained through the nose. After the operation, the mucosa of t,he hard palate became necrotic due to the obliteration of the pterygoid artery. Nevertheless, the final defect of the hard palate was not conspicuous and did not bother the patient. On May 6, 1946, the patient complained of paresthesias in the left arm and leg. Neurological examination did not reveal any pathology. The defect in the mouth was small and there was no recurrence of the tumor. It was not possible to get further information about the patient. Microscopic Examination.-Gingiva consists of an inflamed connective tissue containing many blood vessels and a small amount of pigment. The cells within the connective tissue are lymphocytes, plasma cells, eosinophiles, and a great number of giant cells which are frequently situated around a capillary. The squamous epithelium was normal. The stroma of the tumor consisted of connective tissue which in some places makes up the greater part of the bulk of the tumor; in other places, it forms only narrow septa between the tumor cells. Small hemorrhages and small foci of inflammation are noticeable. Frequently, the connective tissue shows hyaline degeneration; the accumulation of hyaline masses may have caused a compression and degeneration of the tumor cells. In the stroma,
Intraosseous
Tumors
of
Maxilla.
817
there are cellular strands and tubules which may form a loose network. The strands consist of numerous nuclei which do not show a. specific arrangement. Mitoses are rare. Not infrequently there are within the cellular strands small cavities filled with a threadlike basophile ma.ss or a homogenous acidophilic substance (Fig. 2). The walls of the tubules likewise consist of many nuclei without specific arrangement, or of high cuboida.1 cells forming one layer and resembling glandular epithelium. There are no enameloblasts. The strands as well as the tubules show a fine basilar membrane. The lumen of the tubules is either narrow or large and irregular. In the latter case, the epithelium of the wall is flat, probably due to the pressure from within. The pressure may increase to such a degree that the wall of the tubules is broken up and the cells are scattered within a homogenous mass. The lumen of the tubules is most frequently empty. In several tubules, there is a threadlike mass or a plug of cellular or hyaline connective tissue or, occasionally, a blood vessel.
m
Fig. L-Situation of hard palate (P). 8, Nasal QI the tumor has destroyed
tumor (T) within the alveolar process of the maxilla and the septum ; M, maxillary sinus ; m, mucosa ; g, gingiva. From x to the external corticalis of the alveolar process.
However, on no place is the lumen filled with stellate reticulum, significant of adamantinoma. The tumor extends close to the epithelium of the mucosa and has invaded a muscle of the cheek, the muscle fibers being scattered between the strands and tubules. Where the tumor encroaches bone (Fig. 3), the connective tissue, cellular or hyaline, increases considerably. Though the bone is crumbled, osteoclasts are rare, but there is considerable formation of new bone, originating from the connective Gssue of the tumor and laying down a layer of osteoid substance upon the fragments of the original bone. Comment.-The case presented shows an intraosseous tumor of the maxilla which has grown by expansion. The tumor has destroyed the alveolar process of the maxilla and the bone of the canine fossa to a great extent, has invaded the muscles of the cheek, but did not enter the maxillary sinus. The principal clinical findings consisted of intense neuralgic pain and a diffuse swelling of
818
H. Bbunaenthnl
and H. Brunner
the alveolar process. Based on this finding, the tentative diagnosis of a localized osteitis fibrosa was made, particularly because the x-ray film revealed an osteoporosis. At operation, it became evident that the swelling of the alveolar process was caused by an intraosseous tumor. The pain was probably caused by an involvement of the terminal branches of the trigeminus; the temporary swelling of the face (which we never have seen) may have been caused by pressure exerted by the tumor on the veins within the canalis pterygo-palatinus.
Fig.
2.--A,
Tumor
in low
magnification; e, columnar epithelium; shown in higher magniflcation in
B.
2,
this
part
of
the
tumor
is
The tumor was classified as adamantinoma based upon the examination of the biopsy. However, the examination of the entire tumor renders this diagnosis doubtful. Although Ewing,l as well as Thorna,* emphasizes that adamantinomas fail to exhibit a uniform structure and may present a structure similar to adenocarcinoma of mixed tumor type, yet they always contain some tissue elements which are related to the embryonic enamel organ. The case presented failed to show tissue of this type. The stellate reticulum of the
Intraosseous
Tumors of Maxilla
819
reenamel organ and enameloblasts is absent. The microscopic examination vealed an adenocarcinoma of mixed tumor type which, to a great extent, showed a cylindromatous structure and was embedded within the marrow of the alveolar process. There were two types of cystlike cavities. The first type was caused by smadl cavities which appeared within the solid cellular strands and contained either a threadlike substance or a homogenous acidophilic mass. The other type was caused by the cellular strands which formed tubules and included connective tissue and occasionally capillaries.
Fig.
3.-Tumor
(T)
invading
the
bone. The ( t) causing
tumor enters osteogenesis
the (a).
small
narrow
spaces
of the
bone
It is not possible to trace the origin of the tumor. Since neither the gingiva nor the mucosa of the anterior part of the hard palate contain glands, it is likely that the tumor has derived from the mucosa of the posterior rJart of the hard palate where glands are to be found and where adenocarcinoma of mixed tumor type is not uncommon, To sum up, we feel that the tumor is an adenocarcinoma which is situated beneath the mucosa of the hard palate and within the marrow of the alveolar ridge, distending the outer cortex of the alveolar ridge but not extending into
820
H. Blumenthal
and H. Brunner
In the the maxillary sinus. These tumors are likely to cause recurrences. case described there was no recurrence five months after the operation. This period of time is, of course, too short to determine the final outcome. Ringertz3 reported two cases of mixed tumor which were located at the same site as our case. In both cases, the tumor had an almost adenoma-like structure. In both cases, recurrences occurred. In one case, the recurrence had the structure of a squamous-cell carcinoma, in the second case, the recurrence had the same structure as the primary tumor, but there was evidence of invasion of the neighboring tissue. CASE 11.-G. R., white, female, aged 16. Three weeks ago, her left cheek and left upper jaw became swollen and slightly tender. A similar episode occurred in the fall of 1945. During the latter period, an incision was made in the region of the left upper premolar to evacuate pus. At examination on July 9, 1946, the general condition of the patient was good. Her temperature was 99.8” F. The left cheek and the left alveolar process of the maxilla were moderately swollen and tender. There was no discharge into the nose or the pharynx. Transillumination revealed a dark antrum on the left side. X-ray examination (Fig. 4) showed a densely opaque mass within the left maxillary sinus containing several malformed dental structures. For the most part, these deformed dental elements had the appearance of molar teeth which had not erupted. A tentative diagnosis of odontoma was made. X-ray examination of the teeth showed several cavities and several fillings. The lower third molar on the right side was impacted and unerupted. The lower third molar on the left side and the upper third molar on the right side were unerupted. From the area of the second left upper premolar as far posteriorly through the area of the third molar, an opaque mass was evident The mass showing a faint outline of one or more teeth, apparently embedded. is suggestive of an odontoma (Dr. W. Rousar). After five days of penicillin therapy, the swelling of the cheek and the tenderness over the left antral region subsided. On July 20, an incision was made in the mouth extending from the left upper premolar area to 1 cm. from the’ left piriform aperture. The fossa canina was exposed. After removal of the anterior wall of the maxillary sinus, the tumor presented itself closely adjacent to the anterior wall. The other attachments of the tumor could be pried loose from the rest of the antral walls and the palate bone with dental elevators. However, the. tumor mass was too large to be delivered in one piece through the anterior opening. Therefore a drill had to be applied cutting the tumor in half and then removing each piece by means of dental extraction forceps. The second premolar which was loose in the alveolar process was extracted. The antrum was drained through the nose. The postoperative course was uneventful except for a small antral fistula in the region ‘of the second premolar tooth. However, this fistula healed spontaneously within three weeks following surgery. An x-ray film, taken on Aug. 16, 1946, revealed an irregularly shaped mass, having the density and configuration of maldeveloped dental structure,
A.
Fig.
4.--4
an11 R,
X-ravs
showing
the
tumor
within
the
maxillary
sinu
822
H. Blument,hal
and H. Brunner
and measuring ,about 2 cm. in the superior half of the antrum. Apparently this mass was part of the original tumor mass which had broken off and blended in with the posterior antral wall. Inasmuch as the patient was symptom-free, it was decided to watch the future outcome. Another x-ray film from Dec. 16, 1946, showed the remnant of the odontoma somewhat smaller than before. Macroscopic Pinding of the Removed Part of the Tumor.-(Fig. 5.) The removed tumor is oval in shape; the size is about 3.5 by 4 cm., the weight about 20 grams. The consistency is that of a hard, osseous tumor. The surface is irregular and resembles the shell of a walnut. The tumor is covered by a firm, fibrous capsule. It is grown together with the removed premolar. On cross section, the tumor consists only of hard tissue which has a striated appearance. There are a few small cavities, but there is no soft tissue within the tumor. Microscopic Examination.-We are indebted to Dr. J. Weinmann for his kind assistance in interpreting the microscopic findings. The tumor consists of dental tissues, enamel, dentine, cementum, and a small amount of soft tissue covering the tumor (Fig. 6). There is a great number of pulplike cavities which occasionally contain frank pus. Many cavities are empty. It cannot be stated as to whether the tissue which probably had filled these cavities was lost during the preparation of the specimen or became necrotic. It is certain that these cavities do not contain a tissue comparable to a normal pulp. ‘The dentine is on some places regular, on others, irregular. The calcification of t,he dentine is highly irregular and often incomplete. Globules of calcified dentine surround wide areas of predentine or are simply distributed in uncalcified matrix. There is a great amount of enamel. Much of it is immature. The acid insoluble matrix is clearly defined with quite regular rods and inter-rod substance. Empty spaces in the specimen have possibly been filled with mature acid-soluble enamel. Frequently, the enamel is covered by a thin basophilic cuticular layer. At several sites, cub&d cells are found, derived from the epithelial organ. Where these cells form alveolus-like structures, they include cells resembling the stellate reticulum of the enamel organ (Figs. 7 and 8). The cementum is either cellular or acellular. If it is cellular, the lacunae usually do not contain cells indicating a necrosis of the tissue. In addition, there is a great amount of amorphous calcification. The dentine as well as the cementum, shows Howship’s lacunae on several sites. Giant cells are absent. The soft tissue covering the surface of the tumor (Fig. 6) is frequently separated from the tumor by a basophilic line of insertion. The soft tissue consists of the following : (a) connective tissue, and (b) epithelial tissue (Fig. 9). The connective tissue is rich in cells. These cells are, to a great extent, plasma cells, polymorphonuclear leucocytes, and lymphocytes, but there is also a certain amount of connective tissue cells. There is a moderate amount of blood vessels. Within the connective tissue there are cuboid epithelial cells which form strands or glandular-like structures, or pieces of epithelium. -Formation of dentine or enamel does not take place within the connective tissue.
Intraosseous
Fig.
Fig.
B.-Section
5.-Odontoma
through
removed
by
surgery.
Tumors
Inset
of Maxilla
shows
tooth
C, the odontoma. A, rudimentary teeth: masses ; S, soft tissue ; a, enamel organ.
823
and
adherent
cementurn
tmnor.
and
amorphous
824
H. Blumenthal
Fig.
7.-a,
Fig.
enamel
8.-a,
epithelium;
enamel
and H. Brunner
b, stratum
; b, enamel
intermedium;
epithelium:
c, stellate
C, stellate
reticulum.
reticulum.
Tntraosseous
Tumors
of
825
Maxilla
case presented shows a compound odontoma which is made Comment.--The up of a large number of more or less rudimentary teeth and a great amount This type of tumor is not frequent, of cementum and amorphous calcifications. particularly in the maxilla. In the presented case, the tumor had grown into the maxillary sinus. A Luc-Caldwell operation was performed and a great part of the tumor was removed. There are two concepts concerning the genesis of these tumors: Malasse.? refers the majority of odontomas to proliferation of
Fig.
9.-Section
through
the
soft
tissue
(S
in Fig.
F).
n, cpithelial
cords
; b, mesenchymal
~11s.
the ‘ ’ debris epitheliaux paradentaires. ’ ’ Perthes, Schloessmann, and Thoma trace the origin to misplacement of a dental follicle during the early period of growth. Both concepts do not agree with the findings of the presented case. The concept of Malassez explains the proliferation of the epithelial parts of the tumor, but fails to offer an explanation of the proliferation of the mesodermal parts. The tumor of the presented case consists, to a great extent,
826
H. Blumenthal
and H. Brunner
of dentine and cementum and both are mesodermal in origin. For this reason, we agree with Ewing who would limit the domain of the “debris paradentaires” to the adamantinomas and the cystic tumors lined by epithelium, while the various odontomas may be referred to the enamel organ and the pulp tissue. The concept of Perthes, Schloessmann, and Thoma is based upon the hypothesis indicating that during the development of the teeth a soft tooth germ is misplaced above a more or less mature tooth, due to lack of space, and is split by the latter. The dispersed small enamel organs develop into tooth germs and give rise to all kinds and shapes of small teeth which may be united by connective tissue, cementum, or bone. This concept concerns particularly the favored site of odontomas, namely, the confined quarters of the angle of the lower jaw. It is in agreement with this concept that, in general, bdontomas do not exhibit a boundless proliferation, but are apparently subject to the law of physiologic growth. When the developmental power of the dispersed pieces of the broken-up tooth germ is exhausted and the tumor is built up by hard tissue, the growth of the tumor ceases (Schloessmann) . This concept does not agree with the findings in the presented case for two reasons: The tumor was not located in the angle of the lower jaw, but in the maxilla where the spatial conditions scarcely interfere with the development of the teeth. Furthermore, the tumor of the presented case did not exhibit a boundless growth, but it did grow beyond the limits of physiologic growth. This is proved by the fact that it almost filled the maxillary sinus and that, despite its size, it contained soft tissue forming a capsule, which undoubtedly can be considered as a potential matrix of an additional tumor growth. In the case presented the first two molars were merged in the tumor. This and the microscopic findings suggest that the tumor arose from the original dental follicle of one or both molars. If, in agreement with Ewing,3 we apply the term ‘(teratoma” to a group of tumors derived from more than one germ layer of distinct regional stamp and reproducing the organs of these regions, and if we, furthermore, consider that these tumors are usually associated with a defect in the formation of the parts from which they spring, it seems logical to classify the presented odontoma as a teratoma which originated a true benign tumor. Interesting is the finding of cementum and of an infection within the tumor. According to Schloessmann, cementum appears in odontomas which became manifest at a time in which, under normal circumstances, the formation of the tooth root, merged in the tumor, has come to an end. For this reason, odontomas containing cementum are found almost exclusively in patients between 20 and 24 years of age. In this age, the formation of the root and of cementum has come to an end at the first two molars, and is in full swing at the third molar. Odontomas which become manifest prior to the normal term of cementum formation do not contain cementum (Schloessmann). In the case presented there was a great amount of cementum, particularly an acellular type. Yet the patient was probably not even 16 when the tumor was first noted. In this age the molars have not reached maturity. This again indicates that the odontoma of the case presented is subject to the law of tumor growth, but not to the law of physiologic growth.
Intraosseous
Tumors
of Maxilla
827
The tumor has offered a remarkable resistance to infection. There are several “pulp channels” which are filled with frank pus and there is some resorpt,ion of dentine which however does not exceed microscopic limits; furthermore, there is a great amount of necrotic calcified tissue, the necrosis of which eventually may be caused by the infection; but,, in spite of the long-standing infeetion, the tumor does not show gross changes caused by the infection. This is probably due to the thick connective tissue which covers the tumor and which, in contradistinction to the tumor, is severely inflamed. I’urtherrrore, the tumor contains only a very small amount of blood vessels while the connective tissue capsule is rich in blood vessels. E’or this reason, the chief pathway for the infection to travel into the tumor is absent. REFERENCES
Neoplastic Diseases, ed. 4, Philadelphia, 1940, W. B. Saunders Company. H.: Oral Pathology, ed. 2, St. Louis, 1944? The C. V. Mosby Company. N.: Pathology of Malignant Tumors Arming in the Nasal and Paranasal Cavities and Maxilla, Acta Oto-laryng. Supp. 27: pp. l-405, 1938. 4. Schloessmann: Zur Pathologie der Odontome, Beitr. z. path. Anat. u. g. allg. Path. 44: 311,1908. 5. Perthes, G.: Verletzungen und Ilrkrankungen der Kiefer, Handb. d. prakt. Chir. I, p, 779, 1913.
1. Ewing, 2. Thoma, 3. Ringer@
J.: K.
9113 SOUTH COMMERCIAL AVENGE 307 NORTH MICHIGAN BOULEVARD
M.D.,
Articles
TUMORS
OF THE
ASD H. BRUNNER,
MAXILLA
M.D.,
CHICAGO, ILL.
NTRAOSSEOUS tumors of the maxilla, except dentogenous cysts, are not common. Because these tumors may extend into the maxillary sinus, they are interesting for both the dentist and the rhinologist. We had the opportunity to perform an operation on two cases of this type. The rarity of the cases and the microscopic findings of the tumors warrant this report.
I
CASE REPORTS CASE I.-I. MI., white, female, aged 45. When 2 years of age, her first tooth appeared. Her mother told her that all her deciduous teeth were decayed when they came in except a few in front. The patient also said that all her permanent teeth except six mandibular teeth were decayed when they appeared. She claimed to have had much dental care all her life. Nine years ago she had all her teeth extracted except the six mandibular teeth in front At the time all her teeth were which have always remained in good condition. extracted, she said that most of them were only small pegs or roots which were remaining from the caries she had had. About three years ago, she began to have sudden attacks of sharp pain in the right cheek. It was first noticed on arising in the morning. Pain radiated to the right eye and to the right occipital region. The right side of the face became swollen and “black and blue.” Pain subsided in three to four hours and the swelling decreased in seven to eight hours. These attacks occur approximately once a week and are getting progressively worse. On admission, on Dec. 3, 1945, the pupils were found to be normal. She complained of a general urticaria. Several of the nails were longitudinally striated. A few showed loss of substance near t,he roots; one was yellowed and slightly irregular at the lunula. In the maxilla there were no teeth. There was a swelling of the right upper alveolar ridge. The swelling was hard in consistency, covered by a pale gingiva, and extended toward the hard palate. X-ray examination on Nov. 20, 1945, revealed bone destruction involving the right upper alveolar ridge and the floor of the right antrum. There was some cloudiness over the lateral border of the antrum. X-ray examination on Dec. 3, 1945, showed an osteoporosis of the alveolar ridge of the maxilla; the sinuses were normal. Blood examination, including Kahn’s test, did not reveal any pathology. The tentative diagnosis of a localized ostcit.is fibrosa was made. On December 4, a sublabial incision was made I cm. from College
From of
the Department Medicine.
of
Laryngolopy.
Rhinology 815
and
Otology,
IJniversity
of
Illinois.
H. Blumenthal
and H. Brunner
the alveolar edge and the mucosa elevated from the bone. A rongeur was used to remove four to five pieces of porous bone. The diagnosis was adamantinoma. On Jan. 12, 1946, a radical operation was performed. The bone of the canine fossa was extremely thin and, to a great extent, absent, exposing the mucosa of the maxillary sinus; the bone of the piriform aperture had a normal appearance. The maxillary sinus was empty. The mucosa was pale; at the site of the alveolar ridge it was thickened. The lateral wall of the inferior nasal meatus was extremely thin and was removed, creating a large communication with the nasal cavity. The inferior turbinate was apparently normal. The normal gingiva and the thickened paranasal mucosa were elevated from the alveolar ridge, exposing a tumor about 5 cm. long, which occupied the marrow of the alveolar process and extended approximately from the area of the second incisor to t,he posterior end of the alveolar process. The tumor had a red color, contained a small amount of blood vessels, and had the consistency of granulation tissue. It had distended the external corticalis of the alveolar process outward, and had perforated it at several sites so that the external surface of the tumor was covered only by fragments of thin bone (Fig. 1). It was not difficult to elevate the tumor from the internal cortex of the alveolar process which was about 3 cm. in thickness and was considerably sclerotic. Resection of the alveolar process was performed, bringing into view the entire tumor which extended between the mucosa and the bone of the hard palate toward the midline and toward the canalis pterygo-palatinus, where the tumor was firmly adherent to the blood vessels and nerves. The tumor was easily elevated from the bone of the hard palate. The latter, being soft in consistency, was removed up to the canalis pterygo-palatinus. The gingiva was sutured to the mucosa of the cheek and the maxillary sinus was drained through the nose. After the operation, the mucosa of t,he hard palate became necrotic due to the obliteration of the pterygoid artery. Nevertheless, the final defect of the hard palate was not conspicuous and did not bother the patient. On May 6, 1946, the patient complained of paresthesias in the left arm and leg. Neurological examination did not reveal any pathology. The defect in the mouth was small and there was no recurrence of the tumor. It was not possible to get further information about the patient. Microscopic Examination.-Gingiva consists of an inflamed connective tissue containing many blood vessels and a small amount of pigment. The cells within the connective tissue are lymphocytes, plasma cells, eosinophiles, and a great number of giant cells which are frequently situated around a capillary. The squamous epithelium was normal. The stroma of the tumor consisted of connective tissue which in some places makes up the greater part of the bulk of the tumor; in other places, it forms only narrow septa between the tumor cells. Small hemorrhages and small foci of inflammation are noticeable. Frequently, the connective tissue shows hyaline degeneration; the accumulation of hyaline masses may have caused a compression and degeneration of the tumor cells. In the stroma,
Intraosseous
Tumors
of
Maxilla.
817
there are cellular strands and tubules which may form a loose network. The strands consist of numerous nuclei which do not show a. specific arrangement. Mitoses are rare. Not infrequently there are within the cellular strands small cavities filled with a threadlike basophile ma.ss or a homogenous acidophilic substance (Fig. 2). The walls of the tubules likewise consist of many nuclei without specific arrangement, or of high cuboida.1 cells forming one layer and resembling glandular epithelium. There are no enameloblasts. The strands as well as the tubules show a fine basilar membrane. The lumen of the tubules is either narrow or large and irregular. In the latter case, the epithelium of the wall is flat, probably due to the pressure from within. The pressure may increase to such a degree that the wall of the tubules is broken up and the cells are scattered within a homogenous mass. The lumen of the tubules is most frequently empty. In several tubules, there is a threadlike mass or a plug of cellular or hyaline connective tissue or, occasionally, a blood vessel.
m
Fig. L-Situation of hard palate (P). 8, Nasal QI the tumor has destroyed
tumor (T) within the alveolar process of the maxilla and the septum ; M, maxillary sinus ; m, mucosa ; g, gingiva. From x to the external corticalis of the alveolar process.
However, on no place is the lumen filled with stellate reticulum, significant of adamantinoma. The tumor extends close to the epithelium of the mucosa and has invaded a muscle of the cheek, the muscle fibers being scattered between the strands and tubules. Where the tumor encroaches bone (Fig. 3), the connective tissue, cellular or hyaline, increases considerably. Though the bone is crumbled, osteoclasts are rare, but there is considerable formation of new bone, originating from the connective Gssue of the tumor and laying down a layer of osteoid substance upon the fragments of the original bone. Comment.-The case presented shows an intraosseous tumor of the maxilla which has grown by expansion. The tumor has destroyed the alveolar process of the maxilla and the bone of the canine fossa to a great extent, has invaded the muscles of the cheek, but did not enter the maxillary sinus. The principal clinical findings consisted of intense neuralgic pain and a diffuse swelling of
818
H. Bbunaenthnl
and H. Brunner
the alveolar process. Based on this finding, the tentative diagnosis of a localized osteitis fibrosa was made, particularly because the x-ray film revealed an osteoporosis. At operation, it became evident that the swelling of the alveolar process was caused by an intraosseous tumor. The pain was probably caused by an involvement of the terminal branches of the trigeminus; the temporary swelling of the face (which we never have seen) may have been caused by pressure exerted by the tumor on the veins within the canalis pterygo-palatinus.
Fig.
2.--A,
Tumor
in low
magnification; e, columnar epithelium; shown in higher magniflcation in
B.
2,
this
part
of
the
tumor
is
The tumor was classified as adamantinoma based upon the examination of the biopsy. However, the examination of the entire tumor renders this diagnosis doubtful. Although Ewing,l as well as Thorna,* emphasizes that adamantinomas fail to exhibit a uniform structure and may present a structure similar to adenocarcinoma of mixed tumor type, yet they always contain some tissue elements which are related to the embryonic enamel organ. The case presented failed to show tissue of this type. The stellate reticulum of the
Intraosseous
Tumors of Maxilla
819
reenamel organ and enameloblasts is absent. The microscopic examination vealed an adenocarcinoma of mixed tumor type which, to a great extent, showed a cylindromatous structure and was embedded within the marrow of the alveolar process. There were two types of cystlike cavities. The first type was caused by smadl cavities which appeared within the solid cellular strands and contained either a threadlike substance or a homogenous acidophilic mass. The other type was caused by the cellular strands which formed tubules and included connective tissue and occasionally capillaries.
Fig.
3.-Tumor
(T)
invading
the
bone. The ( t) causing
tumor enters osteogenesis
the (a).
small
narrow
spaces
of the
bone
It is not possible to trace the origin of the tumor. Since neither the gingiva nor the mucosa of the anterior part of the hard palate contain glands, it is likely that the tumor has derived from the mucosa of the posterior rJart of the hard palate where glands are to be found and where adenocarcinoma of mixed tumor type is not uncommon, To sum up, we feel that the tumor is an adenocarcinoma which is situated beneath the mucosa of the hard palate and within the marrow of the alveolar ridge, distending the outer cortex of the alveolar ridge but not extending into
820
H. Blumenthal
and H. Brunner
In the the maxillary sinus. These tumors are likely to cause recurrences. case described there was no recurrence five months after the operation. This period of time is, of course, too short to determine the final outcome. Ringertz3 reported two cases of mixed tumor which were located at the same site as our case. In both cases, the tumor had an almost adenoma-like structure. In both cases, recurrences occurred. In one case, the recurrence had the structure of a squamous-cell carcinoma, in the second case, the recurrence had the same structure as the primary tumor, but there was evidence of invasion of the neighboring tissue. CASE 11.-G. R., white, female, aged 16. Three weeks ago, her left cheek and left upper jaw became swollen and slightly tender. A similar episode occurred in the fall of 1945. During the latter period, an incision was made in the region of the left upper premolar to evacuate pus. At examination on July 9, 1946, the general condition of the patient was good. Her temperature was 99.8” F. The left cheek and the left alveolar process of the maxilla were moderately swollen and tender. There was no discharge into the nose or the pharynx. Transillumination revealed a dark antrum on the left side. X-ray examination (Fig. 4) showed a densely opaque mass within the left maxillary sinus containing several malformed dental structures. For the most part, these deformed dental elements had the appearance of molar teeth which had not erupted. A tentative diagnosis of odontoma was made. X-ray examination of the teeth showed several cavities and several fillings. The lower third molar on the right side was impacted and unerupted. The lower third molar on the left side and the upper third molar on the right side were unerupted. From the area of the second left upper premolar as far posteriorly through the area of the third molar, an opaque mass was evident The mass showing a faint outline of one or more teeth, apparently embedded. is suggestive of an odontoma (Dr. W. Rousar). After five days of penicillin therapy, the swelling of the cheek and the tenderness over the left antral region subsided. On July 20, an incision was made in the mouth extending from the left upper premolar area to 1 cm. from the’ left piriform aperture. The fossa canina was exposed. After removal of the anterior wall of the maxillary sinus, the tumor presented itself closely adjacent to the anterior wall. The other attachments of the tumor could be pried loose from the rest of the antral walls and the palate bone with dental elevators. However, the. tumor mass was too large to be delivered in one piece through the anterior opening. Therefore a drill had to be applied cutting the tumor in half and then removing each piece by means of dental extraction forceps. The second premolar which was loose in the alveolar process was extracted. The antrum was drained through the nose. The postoperative course was uneventful except for a small antral fistula in the region ‘of the second premolar tooth. However, this fistula healed spontaneously within three weeks following surgery. An x-ray film, taken on Aug. 16, 1946, revealed an irregularly shaped mass, having the density and configuration of maldeveloped dental structure,
A.
Fig.
4.--4
an11 R,
X-ravs
showing
the
tumor
within
the
maxillary
sinu
822
H. Blument,hal
and H. Brunner
and measuring ,about 2 cm. in the superior half of the antrum. Apparently this mass was part of the original tumor mass which had broken off and blended in with the posterior antral wall. Inasmuch as the patient was symptom-free, it was decided to watch the future outcome. Another x-ray film from Dec. 16, 1946, showed the remnant of the odontoma somewhat smaller than before. Macroscopic Pinding of the Removed Part of the Tumor.-(Fig. 5.) The removed tumor is oval in shape; the size is about 3.5 by 4 cm., the weight about 20 grams. The consistency is that of a hard, osseous tumor. The surface is irregular and resembles the shell of a walnut. The tumor is covered by a firm, fibrous capsule. It is grown together with the removed premolar. On cross section, the tumor consists only of hard tissue which has a striated appearance. There are a few small cavities, but there is no soft tissue within the tumor. Microscopic Examination.-We are indebted to Dr. J. Weinmann for his kind assistance in interpreting the microscopic findings. The tumor consists of dental tissues, enamel, dentine, cementum, and a small amount of soft tissue covering the tumor (Fig. 6). There is a great number of pulplike cavities which occasionally contain frank pus. Many cavities are empty. It cannot be stated as to whether the tissue which probably had filled these cavities was lost during the preparation of the specimen or became necrotic. It is certain that these cavities do not contain a tissue comparable to a normal pulp. ‘The dentine is on some places regular, on others, irregular. The calcification of t,he dentine is highly irregular and often incomplete. Globules of calcified dentine surround wide areas of predentine or are simply distributed in uncalcified matrix. There is a great amount of enamel. Much of it is immature. The acid insoluble matrix is clearly defined with quite regular rods and inter-rod substance. Empty spaces in the specimen have possibly been filled with mature acid-soluble enamel. Frequently, the enamel is covered by a thin basophilic cuticular layer. At several sites, cub&d cells are found, derived from the epithelial organ. Where these cells form alveolus-like structures, they include cells resembling the stellate reticulum of the enamel organ (Figs. 7 and 8). The cementum is either cellular or acellular. If it is cellular, the lacunae usually do not contain cells indicating a necrosis of the tissue. In addition, there is a great amount of amorphous calcification. The dentine as well as the cementum, shows Howship’s lacunae on several sites. Giant cells are absent. The soft tissue covering the surface of the tumor (Fig. 6) is frequently separated from the tumor by a basophilic line of insertion. The soft tissue consists of the following : (a) connective tissue, and (b) epithelial tissue (Fig. 9). The connective tissue is rich in cells. These cells are, to a great extent, plasma cells, polymorphonuclear leucocytes, and lymphocytes, but there is also a certain amount of connective tissue cells. There is a moderate amount of blood vessels. Within the connective tissue there are cuboid epithelial cells which form strands or glandular-like structures, or pieces of epithelium. -Formation of dentine or enamel does not take place within the connective tissue.
Intraosseous
Fig.
Fig.
B.-Section
5.-Odontoma
through
removed
by
surgery.
Tumors
Inset
of Maxilla
shows
tooth
C, the odontoma. A, rudimentary teeth: masses ; S, soft tissue ; a, enamel organ.
823
and
adherent
cementurn
tmnor.
and
amorphous
824
H. Blumenthal
Fig.
7.-a,
Fig.
enamel
8.-a,
epithelium;
enamel
and H. Brunner
b, stratum
; b, enamel
intermedium;
epithelium:
c, stellate
C, stellate
reticulum.
reticulum.
Tntraosseous
Tumors
of
825
Maxilla
case presented shows a compound odontoma which is made Comment.--The up of a large number of more or less rudimentary teeth and a great amount This type of tumor is not frequent, of cementum and amorphous calcifications. particularly in the maxilla. In the presented case, the tumor had grown into the maxillary sinus. A Luc-Caldwell operation was performed and a great part of the tumor was removed. There are two concepts concerning the genesis of these tumors: Malasse.? refers the majority of odontomas to proliferation of
Fig.
9.-Section
through
the
soft
tissue
(S
in Fig.
F).
n, cpithelial
cords
; b, mesenchymal
~11s.
the ‘ ’ debris epitheliaux paradentaires. ’ ’ Perthes, Schloessmann, and Thoma trace the origin to misplacement of a dental follicle during the early period of growth. Both concepts do not agree with the findings of the presented case. The concept of Malassez explains the proliferation of the epithelial parts of the tumor, but fails to offer an explanation of the proliferation of the mesodermal parts. The tumor of the presented case consists, to a great extent,
826
H. Blumenthal
and H. Brunner
of dentine and cementum and both are mesodermal in origin. For this reason, we agree with Ewing who would limit the domain of the “debris paradentaires” to the adamantinomas and the cystic tumors lined by epithelium, while the various odontomas may be referred to the enamel organ and the pulp tissue. The concept of Perthes, Schloessmann, and Thoma is based upon the hypothesis indicating that during the development of the teeth a soft tooth germ is misplaced above a more or less mature tooth, due to lack of space, and is split by the latter. The dispersed small enamel organs develop into tooth germs and give rise to all kinds and shapes of small teeth which may be united by connective tissue, cementum, or bone. This concept concerns particularly the favored site of odontomas, namely, the confined quarters of the angle of the lower jaw. It is in agreement with this concept that, in general, bdontomas do not exhibit a boundless proliferation, but are apparently subject to the law of physiologic growth. When the developmental power of the dispersed pieces of the broken-up tooth germ is exhausted and the tumor is built up by hard tissue, the growth of the tumor ceases (Schloessmann) . This concept does not agree with the findings in the presented case for two reasons: The tumor was not located in the angle of the lower jaw, but in the maxilla where the spatial conditions scarcely interfere with the development of the teeth. Furthermore, the tumor of the presented case did not exhibit a boundless growth, but it did grow beyond the limits of physiologic growth. This is proved by the fact that it almost filled the maxillary sinus and that, despite its size, it contained soft tissue forming a capsule, which undoubtedly can be considered as a potential matrix of an additional tumor growth. In the case presented the first two molars were merged in the tumor. This and the microscopic findings suggest that the tumor arose from the original dental follicle of one or both molars. If, in agreement with Ewing,3 we apply the term ‘(teratoma” to a group of tumors derived from more than one germ layer of distinct regional stamp and reproducing the organs of these regions, and if we, furthermore, consider that these tumors are usually associated with a defect in the formation of the parts from which they spring, it seems logical to classify the presented odontoma as a teratoma which originated a true benign tumor. Interesting is the finding of cementum and of an infection within the tumor. According to Schloessmann, cementum appears in odontomas which became manifest at a time in which, under normal circumstances, the formation of the tooth root, merged in the tumor, has come to an end. For this reason, odontomas containing cementum are found almost exclusively in patients between 20 and 24 years of age. In this age, the formation of the root and of cementum has come to an end at the first two molars, and is in full swing at the third molar. Odontomas which become manifest prior to the normal term of cementum formation do not contain cementum (Schloessmann). In the case presented there was a great amount of cementum, particularly an acellular type. Yet the patient was probably not even 16 when the tumor was first noted. In this age the molars have not reached maturity. This again indicates that the odontoma of the case presented is subject to the law of tumor growth, but not to the law of physiologic growth.
Intraosseous
Tumors
of Maxilla
827
The tumor has offered a remarkable resistance to infection. There are several “pulp channels” which are filled with frank pus and there is some resorpt,ion of dentine which however does not exceed microscopic limits; furthermore, there is a great amount of necrotic calcified tissue, the necrosis of which eventually may be caused by the infection; but,, in spite of the long-standing infeetion, the tumor does not show gross changes caused by the infection. This is probably due to the thick connective tissue which covers the tumor and which, in contradistinction to the tumor, is severely inflamed. I’urtherrrore, the tumor contains only a very small amount of blood vessels while the connective tissue capsule is rich in blood vessels. E’or this reason, the chief pathway for the infection to travel into the tumor is absent. REFERENCES
Neoplastic Diseases, ed. 4, Philadelphia, 1940, W. B. Saunders Company. H.: Oral Pathology, ed. 2, St. Louis, 1944? The C. V. Mosby Company. N.: Pathology of Malignant Tumors Arming in the Nasal and Paranasal Cavities and Maxilla, Acta Oto-laryng. Supp. 27: pp. l-405, 1938. 4. Schloessmann: Zur Pathologie der Odontome, Beitr. z. path. Anat. u. g. allg. Path. 44: 311,1908. 5. Perthes, G.: Verletzungen und Ilrkrankungen der Kiefer, Handb. d. prakt. Chir. I, p, 779, 1913.
1. Ewing, 2. Thoma, 3. Ringer@
J.: K.
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