Ameloblastoma containing mucus glands

Short communications & casereports

Ameloblastoma containing mucus glands Kenneth M. Hart&an, La Crosse, Wis. GUNDERSEN

CLINIC,

D.M.D.,’

and Bernard

LTD., AND LA CRQSSE LUTHERAN

Kalfayan,

M.D.,@*

HOSPITAL

A 53-year-old woman had a large, deforming mass involving the mandible and was treated by subtotal mandibulectomy. Histologically, the mass proved to be an acanthomatous ameloblastoma that contained mucus glands. Eleven years after the mandibulectomy, the tumor has not recurred or metastasized.

A

meloblastoma is an uncommon tumor and accounts for about 1 per cent of all cysts and tumors of the jawb0nes.l Approximately 80 per cent of these tumors arise in the mandible, and most of them occur in the molar-ramus area.*e4 The controversies regarding histogenesis, classification, malignant potential, and treatment of ameloblastomas have been discussed in several excellent articles in recent years.4-7 It is the purpose of this article to document a previously undescribed histologic type of ameloblastoma. CASE REPORT A 53.year-old white woman was seen in July, 1964, with an enlargement in the anterior mandibular area. She had first become aware of the mass 6 to 7 months previously and claimed that it had grown larger during the past 3 months. It was painless and caused no appreciable Examination revealed a large, deforming mass in the difficulty in chewing or swallowing. area of the mandibular symphysis, extending to the right with intact overlying skin (Fig. 1). Intraorally, the lesion extended from the right angle of the mandible to the midportion of the left body. The patient was wearing a full upper denture but had been unable to wear a lower denture. No areas of anesthesia or paresthesia were present, and there mere no palpable masses in the neck. X-ray studies revealed a large, multilocular, honeycombed neoplasm of the mandible which involved the entire right body, expanded the symphysis area markedly, and extended through the left body to the first molar area (Fig. 2). Review of the patient’s past medical history revealed that she had undergone a thyroidectomy for thyrotoxicosis in 1944 and excision of a lipoma of the neck in 1961. The results of

*Resident in Oral Surgery, Gundersen Clinic, Ltd., and La Crosse Lutheran Hospital. **Consultant Pathologist and Lecturer in Oral Pathology, Gundersen Clinic, Ltd., Crosse Lutheran Hospital.

and La

Ameloblastoma

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Fig. 1. Patient

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deforming

contaking

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physical examination and laboratory studies mere essentially unremarkable, with the exception of the mandibular tumor. On July 27, 1964, a subtotal mandibulectomy with bilateral supraomohyoid dissection and tracheostomy was performed. Reconstruction at that time was carried out by placement of a Hirschner wire. The patient’s recovery was uneventful, and she desired no further reconstruction of the mandible. She was followed regularly, and a Panorex film taken in July, 1974, showed no recurrence of the tumor. Her further medical course was unrelated to the mandibular lesion. In 1968 she underwent a cholecystectomy for relief of cholecystitis and cholelithiasis. In 1971 a left radical mastectomy was performed for removal of an infiltrating carcinoma of the breast. In 1973 the patient was found to have diabetes mellitus, which has since been controlled by diet. She was seen most recently in May, 1975, complaining of a cough which had been present since August, 1974. A bronchial biopsy revealed metastatic breast carcinoma in the wall of the right mainstem bronchus. At present she is being closely followed because of her metastatic disease. Pathology

The specimen consisted of a mandible which had been resected bilaterally at approximately the beginning of the ascending rami. In the anterior portion and extending across the midline was a 6 by 4 by 4 cm. lesion which distorted the mandible. Portions of mucus membrane and skeletal muscle from the floor of the mouth and both sublingual glands were included (Fig. 3). The mandible was sectioned into halves, revealing a large multicystic lesion. A slightly cloudy mucoid yellowish fluid was present within the cysts. The largest cyst occupied the symphysis area and measured 5 by 3.5 cm. Another cyst occupied most of the mandibular body on the right, and a few smaller cysts were present in the left body (Fig. 4). Microscopic examination. Histologic sections of decalcified mandible disclosed an ameloblastoma that formed large and small cysts and infiltrated bone. Bone destruction was accompanied by limited new bone formation. The tumor had broken through the cortex of the man-

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Fig. 2. Po&eroanterior combed neoplasm.

view

of the mandible

Oral Surg. April, 1976

showing

an expansile,

multiloeular,

honey-

Fig. 3. Subtotal mandibulectomy specimen. A large mandibular mass projects anteriorly intraorally. Fig. 4. Sectioned halves of mandible showing a multicystic lesion involving prominently symphysis area and the right body, with small cysts in the left body.

and the

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Fig. 5. Ameloblastoma. Spongiosis and early cystic degeneration of the stellate epithelium. small numbers of leukocytes. (Hematoxylin and eosin stain. Magnification, x125.) Fig. 6. Follicular ameloblastoma invading bone. Limited osteoblastie activity and abundant dense collagenous stroma. (Hematoxylin and eosin stain. Magnification, x50.) Note

dible and lay adjacent to striated muscle and oral mucus membrane, although invasion of these structures had not occurred. The ameloblastic epithelium displayed the characteristic peripheral layer of tall columnar cells and the central zone of cells resembling stellate reticulum. The peripheral cells contained palisaded nuclei that were polarized away from a prominent basement membrane. Spongiosis and microcyst formation among the central cells were common phenomena, and the tumor exhibited both plexiform and follicular growth patterns (Fig. 5). In certain areas, especially in the solid portions of the tumor invading bone, the peripheral columnar cells were not as uniform in size or as orderly in arrangement; the basement membrane was either poorly developed or absent; and the central cells frequently lacked the orderly mosaic arrangement and appeared as closely compacted cells with dark-staining oval or spindle-shaped nuclei (Fig. 6). Nevertheless, histologic features of anaplasia and mitotic activity were not present. The abundant fibrous stroma of the tumor appeared densely collagenous (Fig. 6) or fibrillar (Fig. 7). In some areas, particularly in the walls of the large cysts, the stroma was loose, myxoid, and infiltrated with small numbers of neutrophilic granulocytes or a few lymphocytes and plasma cells (Fig. 5). A few microcysts were also present in the stroma.

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Fig. 7. Ameloblastoma. Fibrillar fihrous stroma with a spicule of bone, upper right. Focal squamous metaplasia and two epithelial microcysts. (Hematoxylin and eosin stain. Magnification, x125.) Fig. 8. Amelohlastoma invading bone. Note the small glands lined with clear cells in the central zone of a large sheet of ameloblastic epithelium. Swirls of squamous epithelium amid stellate reticulumlike cells. (Hematoxylin and eosin stain. Magnification, x50.)

Many islands of amelohlastic epithelium showed squamous metaplasia among the central stellate reticulum-like cells. Intercellular bridges, cytoplasmic keratinization, and a few sometimes appeared as abortive squamous “pearls” were present. The squamous epithelium sharply circumscribed small islands (Fig. ‘i) and sporadically formed larger sheets or swirls that alternated with the surrounding central cells. Cystic degeneration was occasionally observed within the squamous epithelium. In the solid portions of the tumor and among the central stellate or squamous cells, there were many glands lined with clear cells (Fig. 8). Their cytoplasm was distended and appeared empty or coarsely vacuolated. The oval or rounded nuclei were situated at the base of the cells and often appeared compressed against the basement membrane (Fig. 9). The mucus cells usually formed glands and rarely were dispersed as single cells or small nests among the central cells of the ameloblastoma. The mucus cells stained positively with PAS, Alcian blue at pH 2.5, and Mayer’s mucicarmine stain (Figs. 10 and 11). The positive PAS reaction was not altered by diastase digestion. Mucus cells and glands were not seen in the ameloblastic epithelium

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Fig. 9. Mucus glands in central stain. Magnification, x312.)

lining the large cysts or among granular cells were identified.

Ameloblnstoma containing mucus glawis

zone of ameloblastic

the basal

columnar

epithelium.

cells

(Hematoxylin

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and eosin

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DISCUSSION

In 1957 Hodson discussed ameloblastoma with special reference to certain “mucoepidermoid variations” and credited McGregor with having been the first to report epithelial mucin in an ameloblastoma in 1935. Hodson reported studying “three cases of classical adamantinoma where considerable amounts of epithelial mucin were present and one case where only a small amount was present.” He did not discuss the cases in detail, but he documented discrete, PAS-positive, mucusfilled cells that were “always associated with squamous or epidermoid epithelium.” He further stated that “no metaplastic mucus cells occurred in the basal and columnar cells,” and our findings confirm this observation. Some of Hodson’s illustrations show mucus cells among squamous cells lining microcysts. No true mucus glands were described or illustrated by him, and it appears that the case reported here represents the first example of an ameloblastoma containing mucus glands. Hodson and others9 have also demonstrated the presence of mutinous material in the granular cells of granular-cell ameloblastomas, but no granular cells were seen in this case. The staining characteristics of the mucus cells in the present case (namely, positive reactions with PAS after diastase digestion, Alcian blue at pH 2.5, and Mayer’s mucicarmine stains) are those of epithelial mucins which are rich in acidic and vicinal hydroxyl groups. lo In the cases reported by Hodson, toluidine blue, Alcian blue, and mucicarmine stains gave variable results. It is of interest that prolonged decalcification of the mandible prior to sectioning had not affected the staining properties of the mucus in our case. The coexistence of mucus glands and squamous epithelium in this tumor would seem to support Hodson’s implication of a close relationship between the two cell types. Such a relationship between mucus cells and squamous epithelium

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Fig. 20. Mucus glands in ameloblastoma. Left: Dark-staining PAS-positive glands. (PAS stain with hematoxylin counter stain. Magnification, x312.) Right: The blue cytoplasm of mucus cells with Alcian blue stain at pH 2.5 appears light gray in the black and whitr photomicrograph. The glands lie amid squamous and stellate ~11s. (,Ucinn Irlw stain with K(~rnechtrot counterstain. Magnification, x125.)

is a feature of mucoepidermoid carcinomas and papillomatosis (inverted papillomas) of the nasal cavities and paranasal sinuses,ll to name the best known but by no means the only examples. In our case, however, we did not see evidence to suggest that the mucus glands originated from the squamous cells. On the contrary, the glands were more often surrounded by the central modified reticulumlike cells of the ameloblastoma, rather than by squamous epithelium, and 110 squamous cells were identified in the wall of the mucus glands. The incidence of squamous epithelium in ameloblastomas is difficult to assess from the literature in view of the marked divergence of the estimates, namely, 8 to 15 per cenL2s 4 Most authors ascribe the origin of squamous epithelium to a process of metaplasia, presumablv from the central stellate reticulum-like cells. A similar origin for mucus cells cannot be discounted, but it is more likely that mucus glands arise directly from the primitive oral epithelium or its derivatives from which ameloblastomas arise. For a review of the origin of ameloblastomas and the multipotential properties of the oral epithelium and the epithelial lining of follicular cysts, the reader is referred to the papers of Baden, Gorlin,” and Stanley and Diehl.ls Failure of the tumor to recur during 11 years of follow-up indicates the excellent prognosis of ameloblastomas following adequate excision and supports Hodson’s contention that the presence of mucus cells in an ameloblastoma does

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Pig. 11. Mucus glands in ameloblastoma. The carminophilic cytoplasm of mucus glands appears dark gray in the black and white photomicrograph. The glands lie among central stellate cells. (Mayer’s mucicarmine stain with hematoxylin counterstain. Magnification, x312.)

not seemto alter its clinical behavior. The term “mutinous” or “mucoacanthomatous” ameloblastoma seems to us more appropriate than Hodson’s designation “mucoepidermoid variations.” True mucoepidermoid tumors do occur in the jawbones,14and this literature has been recently reviewed by Silverglade15 and Tothl” and their co-workers. These are tumors derived from heterotopic salivary gland tissue or oral epithelium entrapped in bone and are similar histologically and clinically to the mucoepidermoid tumors of salivary glands. Finally, the tumor described here bears no histologic resemblance to the rare ameloblastic adenomatoid tumor (adenomatoid odontogenic tumor) in which the ductlike structures are lined with palisaded nonmucinous columnar cells. REFERENCES

1. Small, I. A., and Waldron, C. A.: Ameloblastoma of Jaw, ORAL SURG. 8: 281-297, 1955. 2. Gorlin, R. J., and Goldman, H. M.: Thoma’s Oral Pathology, ed. 6, St. Louis, 1970, The C. V. Mosby Company. 3. Shafer, TV. G., Hine, M. K., and Levy, B. M.: A Textbook of Oral Pathology, ed. 3, Philadelphia, 1974, W. B. Saunders Company. 4. Baden, E. : Odontogenic Tumors, In Sommers, S. C. (editor): Pathology Annual 1971, New York, 1971, Appleton-Century-Crofts, p. 475. 5. Smith, J. F.: The Controversial Ameloblastoma, ORAL SURG.26: 45-75, 1968. 6. Mehlisch, D. R., Dahlin, D. C., and Masson, J. K. : Ameloblastoma: A Clinicopathologic Report, J. Oral Surg. 30: 9-22, 1972. 7. Sehdev, M. K., Huvos, A. G., Strong, E. W., Gerold, F. P., and Willis, G. W.: Ameloblastoma of Maxilla and Mandible, Cancer 33: 324-333, 1974. 8. Hodson, 5. J.: Observations on the Origin and Nature of the Adamantinoma TVith Special Reference to Certain Muco-epidermoid Variations, Br. J. Plast. Surg. 10: 38-59, 1957. 9. Smith, J. F., Sollee, N., Drake, J., and Blankenship, J.: Granular Cell Ameloblastoma With Remarkable Mucin Production, ORAL SURG. 21: 499-505, 1966. 10. Mowry, R. W.: The Special Value of Methods That Color Both Acidic and Vicinal Hydroxyl Groups in the Histochemical Study of Mu&m, With Revised Directions for the Colloidal Iron Stain, the Use of Alcian Blue GSx and Their Combinations With the Periodic Acid-Schiff Reaction, Ann. N. Y. Acad. Sci. 106: 402-423: 1963. 11. Snyder, R. N., and Perzin, K. H.: Papillomatosis of Nasal Cavity and Paranasal Sinuses (Inverted Papilloma, Squamous Papilloma)! Cancer 30: 668-690, 1972. 12. Gorlin, R. J. : Potentialities of Oral Epithelmm Manifest by Mandibular Dentigerous Cysts, ORAL SURG. 10: 271-284, 1957.

33. Stanley, H. R., and Diehl, I). I,.: Amelol~lastoma Potential of Folliculnr Cysts,
Dentin dysplasiaType I Clinical,

morphologic,

and genetic

studies of a case

Richard K. Wesley, D.D.S., M.S.D.,* George P. Wysocki, D.D.S., Ph.D.,“* Sheldon ill. Mints, D.D.S., X.S.,*** md Jack Jackson, D.D.S.,*“*” Detroit, Mich., and Tbldon, Owtnrio, Callada UNIVERSITY

OF DETROIT

SCHOOL

OF DENTISTRY

Dentin dysplasia Type I is a rare hereditary disturbance in dentin formation characterized by teeth with short blunted roots, complete pulpal obliteration, periapical a.bscesses or cysts without an obvious causative factor, and spontaneous exfoliation. This report describes the clinical, radiographic, histologic, ultrastructural, and genetic features of dentin dysplasia Type I in a 17.year-old boy and discusses the features which separate it from dentin dysplasia Type II and dentinogenesis imperfecta.

D

entin dysplasia is one of the rarest hereditary disturbances of dentin formation. It is characterized by the presence of normal enamel and atypical dentin deposition which results in pulpal obliteration. In addition, the teeth exhibit defective roots and a predisposition to periapical pathosis in the form of radicular cysts or periapical abscess without an obvious cause.‘, ’ *Assistant Professor, Department of Pathology, University of Detroit School of Dentistry. **Associate Professor, Department, of Pathology, University of Western Ontario. **“Practicing Oral Surgeon, Dearborn, Mich.; Clinical Associate Professor, Department of Pathology, University of Detroit School of Dentistry. ****Practicing Oral Surgeon, Dearborn, Mieh.; Clinical Assistant Professor, Department of Pathology, University of Detroit School of Dentistry.

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