J. Cranio-Max.-Fac.Surg. i8 (1990) 247 J. Cranio-Max.-Fac.Surg. 18 (1990) 247-250 © GeorgThiemeVerlagStuttgart • New York
Summary
Ameloblastic Carcinoma of the Maxilla Metastatic to the Mandible
A case of maxillary ameloblastic carcinoma metastatic to the mandible is presented. Of 33 cases of ameloblastic carcinoma reported in the English literature 10 have occurred in the maxilla. Of these, none produced mandibular metastases. The authors review the literature, describing clinical presentation, histological appearance, and treatment of this rare lesion.
Case Report Linda Lee, Walter George Maxymiw, Robert Edgar Wood Dept. of Dentistry(Head: W. G. Maxymiw,D. D. S.), PrincessMargaret Hospital, Toronto, Canada Submitted 29.1.90; accepted 23.2. 90
Introduction Malignant ameloblastomas have been classified by the W H O (Table 1) as turnouts where primary and metastatic lesions retain their benign appearance (Pindborg et al., 1971). In this classification, intraosseous carcinomas include only squamous cell carcinomas, which may have areas that resemble the malignant ameloblastoma (Pindborg et al., 1971). In 1982 a classification of primary intraosseous carcinoma was proposed (Table2), which subclassified metastatic ameloblastomas into those which retained a well differentiated appearance (malignant ameloblastoma) and those that were poorly differentiated (ameloblastic carcinoma) (Elzay, 1982). In this latter group, the tumours exhibit features of both ameloblastoma and squamous cell carcinoma (Elzay, 1982). A further attempt at subclassification (Table 3) categorized malignant ameloblastoma as representing tumours that, in spite of cytologically innocuous appearances, metastasize as well differentiated lesions (Slootweg and Miiller, 1984). It further expanded the definition of ameloblastic carcinomas, as lesions that combine features of an ameloblastoma with less differentiated areas. Utilizing these criteria we present the first documented case of a maxillary ameloblastic carcinoma metastatic to the mandible. Case Report Mr. G.S., a 56-year-old white male, presented to his family dentist (10/88) with a complaint of pain in the left maxilla. This was attributed to his left maxillary third molar, and this tooth was removed. Pain persisted and a panoramic radiograph revealed a cystic area in the left posterior maxilla. A biopsy was done (17. 1.89) and the pathological report indicated an ameloblastic carcinoma (Fig. 1). Sections of the biopsy and resection revealed irregular nests and islands of malignant epithelium in a fibrovascular stroma. Peripheral palisading of columnar cells was prominent as were mitotic figures, pleomorphic, hyperchromatic nuclei and keratin pearl formation. Evidence of cystic lining showing severe dysplasia was present in the initial biopsy specimen.
Key words Ameloblastic carcinoma - Maxilla - Mandible - Metastatic
CT scans followed and the patient underwent a left subtotal maxillectomy (3.2.89). At surgery, frozen tissue sections showed all margins free of tumour. Following this he was pain free but had limited movements of his jaw. A surgical obturator was fitted and he functioned well (Maxymiw et al., 1989). Follow-up physical evaluation prior to radiation therapy revealed a patient in good general health. He had no palpable lymph nodes and except for his maxillectomy
Table 1
WHO
Odontogenic Carcinoma A. Malignant ameloblastoma B. Primary intraosseous carcinoma C.Qther carcinoma arising from odontogenic epithelium including those arising from odontogenic cysts
Table 2
Elzay (1982)
Primary Intraosseous Carcinoma Type 1 Arising ex odontogenic cyst Type 2 Arising ex ameloblastoma A. well differentiated - malignant amelobtastoma B. poorly differentiated - ameloblastic carcinoma Type 3 Arising de novo Non-keratinizing B. keratinizing
Table 3
Type 1 Type 2
Type3
Slootweg and M011er(1984) Primary intraosseous carcinoma ex odontogenic cyst A. Malignant ameloblastoma B. Ameloblastic carcinoma, arising de novo, ex ameloblastoma or ex odontogenic cyst Primary intraosseous carcinoma arising de novo A. Non-keratinizing B. Keratinizing
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L. Lee et aL
Fig.2 Periapical dental radiograph showing the region of the metastatic lesion (arrows).
Fig. 1 Histological section (HE x 30) of the original maxillary lesion shows irregular nests and islands of malignant epithelium in a fibrovascular stroma. The lining epithelium exhibits cytologic and morphologic alterations consisting of increased and abnormal mitoses, pleomorphic and hyperchromatic nuclei and increased nuclear/cytoplasmic ratios. There is promiment palisading of peripheral columnar cells.
site, the rest of his examination was normal. A direct left anterior and right lateral wedge to the left maxilla with a total dose of 5,100 cGy was delivered over 20 fractions. This was completed on the 7.4.89. The patient did well in follow-up until 7.7. 89. At that time he complained of increasing pain in the left buccal mucosa, and the distal aspect of his maxillectomy site. Although the patient felt the obturator was responsible for this pain, clinical assessment did not bear this out. Repeat CT scans revealed a recurrent tumour extending through the pterygopalatine fossa to the floor of the middle cranial fossa, with epidural extension. The tumour was untreatable and the patient was given palliative care. Mr. G.S. was referred back to the department of dentistry on 29.9.89 with a complaint of swelling in the left mandible. On examination a 4 cm sessile mass was seen on the labial surface of the left mandible in the region of the left mandibular first and second premolar teeth. The patient complained of paraesthesia and a periapical radiograph of the area revealed findings consistent with a metastatic lesion (Fig. 2). A biopsy was done on 3.10.89 and revealed
Fig.3 The metastatic lesion of the left mandible is essentially the same histologically as that of the primary lesion. Keratin pearl formation is evident (arrow) in some areas. (HE x 20).
microscopic features similar to the primary lesion (Fig. 3). The patient was given a single dose of radiation (800 cGy) which provided partial relief. Discussion and Conclusions The case presented is that of a histologically malignant odontogenic tumour which, after aggressive therapy, invaded the middle cranial fossa and metastasized to the mandible. According to the WHO classification, this would fall into the category of malignant ameloblastoma (type A/C) on the basis of metastasis or "other carcinomas" on the basis of histology (Pindborg et al., 1971). In Elzay's (1982) modification it would fall into poorly differentiated ameloblastic carcinoma (type 2B). In Slootweg and Miiller's (1984) scheme, it would be ameloblastic carcinoma, arising de novo or ex ameloblastoma (type 2B). It is apparent that this nosological disorder may lead to confusion and errant reporting of rare and important cases. The features which distinguish a useful classification system include: accuracy, well-defined parameters and clinical sig-
Ameloblastic Carcinoma of the Maxilla Metastatic to the Mandible
J. Cranio-Max.-Fac. Surg. 18 (1990)
249
Table 4 Authors
Year
Sex
Age
Duration
Clinical course
Recurrence time
Treatment
Time to metastases
Site of metastases
Andersen and Bang
1986
M
73
not specified
not specified
4years
surgical resection
not specified
Corio et al
1987
M
15
not specified
not specified
not applicable
surgical resection
not applicable
Daramola et al
1980
M
20
not specified
not specified
1st. 2 years 2nd. 5 years
not applicable
Eda et al
1972
F
43
10 years
swelling
Grimes and Stephens Inoue et al Krempien et al
1948
F
46
10 years
1988 1979
F M
51 5.5
not specified not specified
ankylosis of TMJ not specified not specified
1st. 3 yrs 7 mo 2rid. 6 yrs 3 mo 3rd. 6 yrs 11 mo 4th. 7 yrs 7 mo 5th. 8 yrs not applicable
Madiedo et al
1981
M
49
not specified
not specified
1 yr 6 mo
1st. resection 2nd. chemoand radiotherapy resection resection resection resection radiation surgery and radiation resection surgery and chemotherapy resection
not present or not histologically proven not present or not histologically proven lung
McC/atcheyet al
1989
F
77
not specified
not specified
not applicable
resection
not applicable
Sawyeret al
1986
M
14
3 mo
swelling and pain
11 me
resection
not applicable
M
56
not specified
pain
6 mo
resection radiation
8 mo
Lee et al
nificance. The difficulty with this particular lesion relates to its rarity and speculative histogenesis. Analyzing each of the proposed classifications, it is evident that the WHO's is the least specific and may be confusing because it is based to a large degree on histogenesis (Pindborg et al., 1971). The question often raised with the "malignant ameloblastoma", is that the biological behaviour of metastasis is the basis for inclusion in this category. The behaviour of this lesion is not consistently predictable and, therefore, this diagnosis is one that can only be made "after the fact". Here the clinical significance would be minimal A review of the reported maxillary lesions for comparison, is presented in Table 4. Elzay (1982), Slootweg and Miiller (1984) have identified this pitfall and have tried to overcome it by using the term ameloblastic carcinoma to relate the histological features of malignancy. The degree of differentiation of epithelial neoplasms is usually considered to be significant in predicting the biological behaviour of metastasis. The main difference between Elzay's (1982), Slootweg and Miiller's (1984) schemes relates to the minor point of histogenesis. The latter recognized that carcinomatous transformation of an ameloblastoma regardless of histogenesis; whereas the former limits ameloblastic carcinomas arising only from malignant transformation of a preexisting ameloblastoma. As speculation of the histogenesis of such a rare lesion may prove to be purely an academic pursuit without clinical sig-
11 yrs 5 mo not applicable
10 years
lung, vertebrae lymph nodes
10 years
lung
15 years 6 years
lung lung lymph nodes diaphragm chest wall lung small intestine peritoneum not present or not histologically proven not present or not histologically proven mandible
3 years
nificance, we prefer to agree with Slootweg and Miiller (1984). Reviewing this classification the following interpretation is made: 1 - Primary intraosseous carcinoma ex odontogenic cyst This would be represented histologically by a cystic lining which shows areas of progressive dysplasia and obvious malignant change arising from this altered epithelium. The malignancy would be a squamous cell carcinoma without particular features to indicate an odontogenic origin. 2A - Malignant ameloblastoma This would be typified by an ameloblastoma with no histological evidence of malignancy, but one in which metastasis has occurred regardless of the degree of differentiation of the metastatic lesion. This could be identical to the primary or less differentiated. 2B -Ameloblastic carcinoma, arising de novo, ex ameloblastoma, or ex odontogenic cyst This would be represented by an ameloblastoma which has histological evidence of malignancy. Whether it had arisen from a preexisting cyst or ameloblastoma is not a prerequisite. This is favoured because it obviates the problem of sampling. Mso, metastasis is not a condition of its inclusion.
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J. Cranio-Max.-Fac. Surg. 18 (1990)
3 A - N o n - k e r a t i n i z i n g primary intraosseous carcinoma arising de novo This would be represented by a poorly differentiated squamous cell carcinoma with the exclusion of salivary gland neoplasms, metastases from elsewhere in the body and direct extension of surface neoplasms. 3B - Keratinizing primary intraosseous carcinoma arising de novo This would be the more differentiated counterpart to 3A above. A possible criticism of this last category may be in the case where there exists within the same lesion areas which appear to represent squamous cell carcinoma without the "odontogenic" features of peripheral palisading of columnar cells and characteristic areas of ameloblastic carcinoma. Whether this represents an ameloblastic carcinoma with areas of dedifferentiation or a primary intraosseous carcinoma with areas of peripheral palisading is a m o o t point since they both carry dismal prognoses. Acknowledgements The autors wish to thank Mr. J. Jackson for assistance in preparing the reference material, Mr. K. Oxley for preparing the illustrative material, Ms. J. Patterson for her secretarial assistance, and the order of the Eastern Star for financial assistance.
L. Lee et al.: Ameloblastic Carcinoma lungs and thoracic vertebrae. Bull. Tokyo Dent. Coll. 13 (1972) 91-101 Elzay, R.P.: Primary intraosseous carcinoma of the jaw. Oral Surg. 54 (1982) 299-303 Grimes, O.F., H.B. Stephens: Adamantinoma of the maxilla metastatic to the lung. Ann. Surg. 128 (1948) 999-1005 ]noue, N., M. Shimojyo, H. Iwai, H. Ohtsuki, R. Yasumizu, M. Shintaku, N. Taniguchi, M. Inada, T. Arika, S. Morita, H. Okano, S. Ikehara: Malignant ameloblastoma with pulmonary metastasis and hypercalcemia. Am.J. ~lin. Pathol. 90 (1988) 474-481 Krempien, B., W.E. Brandeis, R. Singer: Mestastasierendes Ameloblastom im Kindesalter. Virchows Arch. (A) 381 (1979) 211 Madiedo, G., H. Choi, J. G. Kleinman: Ameloblastoma of the maxilla with distant metastases and hypercalcemia. Am.J. Clin. Pathol. 75 (1981) 585-591 Maxymiw, W. G., R.E. Wood, J. D. Anderson: The immediate role of dentist in the maxillectomy patient. J. Otolaryngol. 18 (1989) 303 -305 McClatchey, K.D., M.J. Sullivan, D.R. Paugh: Peripheral ameloblastic carcinoma: A case report of a rare neoplasm. J. Otolaryng. 18 (1989) 109-111 Pindborg~ J.J., L R. Kramer, H. Torloni: Histological typing of odontogenic tumours, jaw cysts, and allied lesions. International Histological Classification of Turnouts. World Health Organization. Geneva (1971) Book 5, 24-28 Sawyer, D.R., A.L. Nwoku, A. Mosadomi, A.T. Kekere-Ekun: Odontogenic carcinoma with dentinoid. Int.J. Oral Maxillofac. Surg. 15 (1986) 105-107 Slootweg, P.J., H. M~iller: Malignant ameloblastoma or ameloblastic carcinoma. Oral Surg. 57 (1984) 168-176
References
Andersen, E., G. Bang: Ameloblastic carcinoma of the maxilla. J. Max.-Fac. Surg. 14 (1986) 338-340 Corio, R.L., L.I. Goldblatt, P.A. Edwards, K.S. Hartman: Ameloblastic carcinoma: a clinicopathologic study and assessment of eight cases. Oral Surg. 64 (1987) 570-576. Daramola, J. 0., A. A. Abioye, J. A. Ajagbe, P. U. Aghadiuno: Maxillary malignant ameloblastoma with intraorbital extension: Report of case. J. Oral Surg. 38 (1980) 203-206 Eda. S., H. Koike, T. Taehikawa, H. Yamane, M. Shimono, H. Iri, T. Yamamura, T. Shigematsu, Y. Komiya, S. Takahashi, T. Mutoo, Y. Yamazaki, Y. Umezawa: An autopsy case of the malignant ameloblastoma with metastases to the submaxillary lymph nodes,
W. G. Maxymiw, D.D.S. The PrincessMargaretHospital 500 SherbourneStreet, Toronto, Ontario Canada, M4X 1K9 Linda Lee Dept. of Dentistry PrincessMargaretHospital Toronto Canada