Am J Otolaryngol 12:20-2.5,
1991
Maxillary
Ameloblastoma: Case Report
FRANKJ. SCACCIA,MD, MELVIN STRAUSS, MD, JAMESARNOLD, MD, AND ANTHONY 7. MANIGLIA, MD
Ameloblastoma of the maxilla is an unusual epithelial tumor of odontogenic
origin. Although it is considered benign, it can behave in a slowly growing infiltrative fashion, with multiple recurrences and eventual intracranial, or even distant, spread. Information on this tumor consists, to a large extent, of case reports presented in the oral surgery literature. This study is a retrospective review of our combined institutional experience with nasomaxillary tumors from 1960 to 1988. Among these cases were four patients with maxillary ameloblastoma, two males and two females, ranging in age from 16 to 66 years at presentation. Disease extent varied from ameloblastoma localized to a cyst in the maxillary sinus to extensive maxillary and ethmoid involvement. Follow-up ranged from 2 to 13 years, and disease course varied from apparent disease control to persistent intracranial tumor, despite attempts at extirpation. An analysis of this experience and the related literature is presented, along with recommendations for radical therapy, when appropriate, to best ensure control. AM J OTOLARYNGOL12:20-25. Copyright 0 1991 by W.B. Saunders Company Key words: ameloblastoma, maxilla, tumor.
proximately 50% of these maxillary tumors are found in the molar region, 30% in the area of the antrum, and the rest at other sites, including less than 2% in the anterior maxilla.g The significance of maxillary involvement is that the potential for serious consequences is much greater than for the patient with mandibular ameloblastoma. This is partially attributable to the fact that the thick compact bone found in the mandible confines the growth. In addition, because of the proximity of the maxilla to the nasal cavity, paranasal sinuses, orbit, and base of skull, maxillary tumors have a much greater tendency to extend into these structures.” Because maxillary ameloblastomas are rare, a standard treatment has yet to be established. Various modes of therapy have been used, including curettage, cryotherapy, cautery, simple excision, radical excision, radiotherapy, and chemotherapy.g While many investigators feel the only acceptable mode of treatment is radical en bloc resection, there are others who believe a more conservative surgical approach is often adequate.l’,ll A review of the otolaryngology literature reveals a paucity of material concerning this question. This report presents our diagnostic and therapeutic experience with four cases of maxillary ameloblastoma.
Ameloblastomas are rare epithelial tumors of the jaws, comprising approximately 1% of all tumors and cysts of odontogenic 0rigin.l Although the first complete description of these lesions is credited to Falkson (1879), it was not until 1933 that Churchill coined the term “ameloblastoma.“2 Since then, these tumors have been the subject of much controversy and discussion. Few generalizations can be made about ameloblastomas. They manifest no apparent tendencies according to sex or race,3 and tumors may appear at any age, with reports in the literature ranging from 21 months to 80 years.4 However, a study of 222 patients revealed that 70% of the tumors were diagnosed between the ages of 10 and 35 years, with an average of 30.1 years.’ The possible etiology of these lesions also varies; sites of origin include dental lamina remnants, the basal layer of oral mucous membrane, and the enamel organ.” It is generally agreed that only 20% of ameloblastomas occur in the maxilla, although some reports indicate an incidence as low as 1?L7*’ Ap-
Received May 22, 1990, from the Departments of Otolaryngology-Head and Neck Surgery and Pathology, Case Western Reserve University School of Medicine, Cleveland, OH. Accepted for publication September 7, 1990. Address correspondence and reprint requests to Melvin Strauss, MD, Department of Otolaryngology-Head and Neck Surgery, Case Western Reserve University School of Medicine, 207i Abington Rd, Cleveland, OH 44106. Copyright 0 1991 by W.B. Saunders Company 0196-0709/91/1201-0001$5.00/0
MATERIALS AND METHODS A retrospective bined 20
institutional
review was performed of the comexperience of this unusual nasomax-
SCACCIA ET AL
21 with removal of a cystic mass. Since the mass had produced bony destruction in the lateral nasal wall, the nasal cavity was explored, with subsequent removal of necrotic bone, The cyst did not penetrate the posterior sinus or communicate with the dentition. Postoperatively, the patient did well. Pathologic examination revealed a simple cyst whose wall was focally lined by areas of simple epithelium without ameloblastic differentiation, as well as areas with mural nests of ameloblastic epithelium (Figs 2, 3, and 4). It was felt that the ameloblastoma originated in a dentigerous cyst. On follow-up at 2 years, there was no evidence of tumor recurrence.
Figure 1. Coronal CT section demonstrating expansion and focal destruction of right maxillary sinus with abnormal soft tissue density.
illary tumor from 1980 to 1988 at Case Western Reserve School of Medicine-University Hospitals (Cleveland, OH), Cleveland Veterans Medical Center [Cleveland, OH), and the Milton S. Hershey Medical Center of Pennsylvania State University (Hershey, PA). Case NO. i. A 16-year-old black girl presented with a 2-month history of postnasal discharge. The patient had a previous surgery of unknown extent with a diagnosis of ameloblastoma at another institution 2 years prior to admission. On physical examination, there was evidence of fullness in the right lateral nasal wall. Sinus films demonstrated opacification of the right maxillary sinus with loss of the lateral and inferior walls. A computed tomography (CT) scan of the sinuses revealed diffuse expansion of the right maxillary sinus with abnormal soft tissue density and destruction of the inferolateral border [Fig 1). Treatment involved a right Caldwell-Luc approach
Case NO. 2. A 66-year-old white man was admitted with a 2-year history of left nasal obstruction. He reportedly underwent an earlier bilateral polypectomy at another institution. No follow-up was conducted until 18 months later when he presented to our clinic. A review of the pathology reports from his previous surgery demonstrated ameloblastoma. Physical examination was remarkable for a pink, moderately vascular, left intranasal mass. A biopsy of this lesion was positive for ameloblastoma. A CT scan revealed a mass completely filling the left maxillary sinus with erosion through the left lateral nasal wall to the nasal septum (Fig 5). In addition, there was opacification of the left ethmoid and sphenoid sinuses, with no intracranial or orbital extension noted. The patient was treated via a degloving approach with a left medial maxillectomy, ethmoidectomy, and sphenoidotomy. The area of the fovea ethmoidalis and sphenoid contained no tumor. The final pathologic report was ameloblastoma. The patient is doing well at 3 years postoperatively, with no evidence of recurrence.
Case NO. 3. A 53-year-old white man was initially diagnosed with ameloblastoma of the right maxillary alveolus 9 years previously. He presented with a recurrence involving the right maxillary sinuses for which he underwent a right maxilloethmoidectomy with preservation of the orbit. He subsequently had local excisions and cryotherapy of apparent small local recurrences in the infratemporal fossa. Eight years after presentation and 17 years after onset, a biopsy of the right infratemporal fossa was once again positive for tumor. A retromaxillary excision was then performed of the sphenoid, pterygoid plates, and infratemporal fossa. He did well for an additional 2 years, when he began developing right retro-orbital pain. A CT scan and mag-
Figure 2. Histologic section of cuboidal lining of dentigerous cyst. (Hematoxylin-eosin stain: magnification X372.)
MAXILLARY
netic resonance imaging demonstrated a right middle fossa tumor [Fig 6). Because it was felt that this was an extension of his ameloblastoma in close proximity to the cavernous sinus and carotid artery, with displacement of the temporal lobe, a two-stage resection was performed. The first stage consisted of creating a right middle fossa cranial neodura so that the dura of the right anterior temporal lobe could be resected with the tumor. This was followed 6 weeks later by a right lateral craniofacial resection and temporal flap reconstruction. It was noted during surgery that although the tumor was removed from the area of the orbit, optic nerve, and temporal lobe with good margins, there was an area adjacent to the cavernous sinus that was not totally eradicated. Ten months later, examination revealed a 1.6-cm mass in the posterior medial aspect of the infratemporal fossa. A CT scan was performed that confirmed this recurrence, with probable extension into the orbital apex, cavernous sinus, petrous apex, and middle cranial fossa. The patient subsequently underwent a sixth operative procedure with a revision maxillectomy and laser excision of the skull base tumor. Following
AMELOBLASTOMA
this, he received a course of postoperative radiotherapy. Currently, he is alive and functioning well, but with persistent disease 22 years after initial diagnosis.
Case No. 4. A 36-year-old white woman initially presented with sinusitis 2 years previously and then subsequently underwent a Caldwell-Luc and nasal polypectomy that revealed ameloblastoma. She was then referred for further evaluation and treatment. A CT scan was performed that demonstrated the presence of tumor in the left maxillary and ethmoid sinuses with possible extension into the infratemporal fossa. The physical examination was remarkable only for a healed left Caldwell-Luc incision and polypoid tissue emanating from the left middle meatus. The patient underwent a left maxilloethmoidectomy, sparing the orbit. Tumor was demonstrated in the nasal polypoid material and in the posterior inferior aspect of the antrum near the maxillary tubercle. The tumor was peeled from the fovea ethmoidalis of the anterior ethmoid. Two months later, because of the possible pres-
Figure 4. Histologic section of ameloblastoma focus within wall of cystic ameloblastoma. (Hematoxylineosin stain; magnification X60.)
23
SCACCIA ET AL
RESULTS A two-institution, retrospective review from 1980 to 1988 revealed a total of 120 neoplasms of the nasal fossa and paranasal sinuses. Four of these cases were maxillary ameloblastomas. These patients ranged in age from 16 to 66 years. The sex distribution was equal; two males and two females. Tumor location on presentation and recurrence included maxillary, ethmoid, and sphenoid sinuses; nasal cavity; infratemporal fossa; middle cranial fossa; and orbital apex. Three of these patients show no evidence of disease and one is alive with disease, with a range of follow-up of 2 to 13 years. Follicular and plexiform histologic variants and ameloblastoma in the wall of a dentigerous cyst were found. These findings are summarized in Table 1.
DISCUSSION
Figure 5. Coronal CT section demonstrating a mass filling the left maxillary sinus with destruction of the lateral nasal wall. Other views demonstrated opacification of the left posterior ethmoid and sphenoid sinuses. ence of residual disease at the fovea, a frontal craniotomy with craniofacial resection of the fovea and cranialization of the frontal sinuses was performed. No gross tumor was observed during the surgery, and the final pathology report was also negative for residual tumor. On follow-up 3 years later, there was no evidence of tumor recurrence.
Figure 6. Coronal fossa tumor.
CT section
demonstrating
a right middle
The most frequent site of involvement of ameloblastomas is within the molar-ramus region of the mandible. The third molar region is also the most commonly affected site in the maxilla. Such tumors may extend into the nose, orbit, maxillary sinus, or base of the skull. Rarely, ameloblastomas arise from a dentigerous cyst.l’ In such cases, both the lining epithelium seen in a dentigerous cyst and ameloblastic epithelium must be present side by side. The term “unicystic ameloblastoma” has been proposed for such lesions. They are reported to have slower progressive growth that may permit enucleation with complete removal rather than partial or complete jaw resection.13 Grossly, ameloblastomas are relatively wellcircumscribed, cystic, or solid lesions. Necrotic foci and cystic degeneration are common. Microscopically, islands of epithelium are embedded within a fibrous stroma in follicular or plexiform patterns. The cells at the periphery of the islands are composed of columnar epithelium whose nuclei are polarized away from the basal membrane. The center of the islands are composed of a loose stellate reticulin network that may contain squamous metaplastic foci. Stromal vascularity may at times be marked. Occasionally, a hyalinized zone may be noted in proximity to the ameloblastic layer. Our experience with ameloblastomas clearly demonstrates the tendency of these lesions to behave in a clinically malignant fashion with direct extension beyond the site of origin in the maxilla. Although all four patients are presently alive, case no. 3 has persistent intracranial tumor. Postoperative radiotherapy was applied in an attempt to palliate this inoperable condition. A review of the literature demonstrates that
24
MAXILLARY
TABLE 1. PATIENT No./ AGE (Y)/ RACE/SEX
PRESENTING SYMPTOMS
Summary
of Patients
HISTOLOGIC FINDINGS
With Maxillary
SITES op INVASION
AMELOBLASTOMA
Ameloblastoma
TREATMENT*
DISEASE DURATION
1/16/B/F
Postnasal discharge
Dentieerous cyst origin
Maxilla: nasal cavity
Caldwell-Luc; nasal cavity debridement (0)
Alive, diseasefree 2 yr
2/66iWfM
Nasal obstruction
Plexiform
Maxilla; nasal cavity; ethmoid; sphenoid
Medial maxillectomy; ethmoidectomy; sphenoidotomy
Alive, diseasefree 3 yr
3/53rwiM
Mass in alveolus
Plexiform
Maxilla; ethmoid
Maxillothmoidectomy (01
Infratemporal fossa
Local excision; cryotherapy (2.8) Retromaxillary excision of sphenoid, pterygoid plates, and infratemporal fossa (8)
Infratemporal fossa
4/36NviF
Postnasal drip; sinus pain
Plexiform
Middle fossa
Cranial neodura construction; craniofacial resection; temporal muscle flap (10)
Infratemporal fossa; orbital apex; middle fossa
Revision maxillectomy; laser excision; radiation (11)
Alive, with disease 13 yr
Ethmoid; maxilla; infratemporal fossa; fovea of anterior ethmoid
Maxilloethmoidectomy (0); craniofacial resection; cranialization frontal sinuses (2 mo)
Alive, diseasefree 3 yr
* Numbers in parentheses indicate number of years after presentation that treatment was undertaken.
many reports support the concept of radical while others still propose more therapy, 4~g~11**4-1g conservative approaches to control ameloblastoma.3~6~‘0~20~2*Sehdev et al characterize the controversy with their statement, “Vitriolic arguments for and against conservative and radical excision abound with no criteria for preference of either.“17 Although the issue of treatment modalities has not been settled, substantial evidence in the literature attests to the aggressiveness of these tumors. Komisarlg presented a case of maxillary ameloblastoma that failed curettage and presented 6 months later with a large recurrence in the maxillary sinus that extended into the pterygomaxillary space, infratemporal fossa, orbit, and nasal cavity. The patient was treated with a hemimaxillectomy and was free of disease 1 year postoperatively. Daramola et al” presented the case of an ameloblastoma that originated in the right side of the maxilla with later involvement of the orbit, frontal sinus, and nasal cavity, with multiple pulmonary metastases. Another tumor that filled the right maxillary sinus and later invaded the orbit and middle cranial cavities is described by Weiss et a1.15 Tsaknis and Nelson4 demonstrated a 50% recurrence rate for the 19 patients in their study
who were treated by local excision or curettage. Small and Waldron’ had similar results, with recurrence in 46% of their cases that underwent local removal or curettage. In this study, all four patients had minor procedures performed initially and all had persistent or recurrent disease. Two patients, then, had extensive surgery early on and have no evidence of recurrence. One patient had less radical surgery due to the apparent extent of disease, the fact that it was a variant of ameloblastoma in a dentigerous cyst, and the age of the patient. One patient presented 9 years after initial diagnosis and, despite radical surgery, his tumor persisted and extended intracranially, and has persisted there despite major extirpative efforts. Recurrence of tumor was almost universally cited as a poor prognostic factor. In their 13 patients who developed recurrences, Sehdev et al found that only 36% could be salvaged by partial l7 They also observed that or total maxillectomy. all of the patients in their study with ameloblastoma who received external irradiation or curettage as their initial treatment developed recurrences. Robinson5 and Small and Waldron,’ in their classic reports, described ameloblastomas as basi-
25
SCACCIA ET AL
tally benign lesions. However, this is based on their morphologic characteristics and not their propensity for local invasion. Of the 1,000 cases from the world literature that Small and Waldron reviewed, they felt that only three cases could meet the verifiable criteria for malignancy, concluding that the incidence of malignant ameloblastoma was extremely rare. Despite its categorization as a benign tumor, a few researchers have emphasized the metastatic potential of this tumor. Madiedo et al reviewed the literature and found 28 cases of ameloblastomas with histologically confirmed metastasis.23 However, in only two cases was the maxilla the primary site. The most common sites of metastasis are the lung and the regional lymph nodes. However, metastatic deposits have also been found in the pleura, vertebra, skull, liver, parotid, and even the small intestine.23 SUMMARY
1. Maxillary ameloblastoma is an unusual neoplasm, with few studies reporting more than isolated cases. 2. In this study, four cases of maxillary ameloblastoma are added to the literature. 3. Although limited, our experience indicates that for complete extirpation of maxillary ameloblastoma, radical surgery, including skull base resection, is indicated in operable cases and offers the best possibility of cure. The possible exception to this is that of tumor confined within the walls of a well-defined dentigerous cyst. 4. Maxillary ameloblastomas are slow-growing infiltrative tumors; regular, long-term follow-up is necessary to recognize and treat recurrences. Acknowledgment. The authors wish to thank Jerald Goldberg, DDS, Director, Division of Oral Surgery, Case Western Reserve University School of Dentistry; Kelly Sorensen, MD, Department of Pathology, Case Western Reserve University School of Medicine; Mark Klingensmith, MD, Department of Otolaryngology, Geisinger Clinic; Robert Page, MD, Division of Neurosurgery, MS. Hershey Medical Center; and John Price, MD, Baltimore, MD, for their roles in the care of these patients.
References 1. Small IA, Waldron CA: Ameloblastoma of the jaws: Oral Surg 1955;8:281-297 2. Shaw HJ, Katsikas DK: Ameloblastoma of the maxilla. J Laryngol Otoi 1973;87:873-884 3. Sandler KA, Novo RM, Rudner BE: A study of ameloblastoma: Age, sex, and location statistics. NY State Dent J 1983; 49:682-684 4. Tsaknis PJ, Nelson JF: The maxillary ameloblastoma: An analysis of 24 cases. Oral Surg 1980; 38:336-342 5. Robinson HBG: Ameloblastoma. A survey of 379 cases from the literature: Arch Path01 1937; 23:831-843 6. Cheney ML, Wolf C, Cox RA. et al: Ameloblastoma presenting as chronic sinusitis. La State Med Sot J 1986; 2:11-14 7. Seabaugh JL, Templer JW, Havey A: Ameloblastoma presenting as a nasopharyngeal tumor. Otolaryngol Head Neck Surg 1986; 94:265-267 8. Adekeye EO, Lavey KM: Recurrent ameloblastoma of the maxilla-facial region. J Maxillofac Surg 1986;14:153-157 9. Batsakis JG. McClatchey KD: Ameloblastoma of the maxilla and peripheral ameloblastomas. Ann Oto Rhino1 Laryngol 1983; 92:532-533 10. Porter J, Miller R, Sartigos GT: Ameloblastoma of the maxilla. J Oral Surg 1977; 44:34-38 11.Weissman BW, Wetli C: Ameloblastoma of the maxilla. South Med J 1977; 70251-253 12.Kane JP: Odontogenic Tumors. A Statistical and Morphological Study of 88 Cases. Thesis, Georgetown University, Washington, DC, 1951 13. Robinson L, Martinez MG: Unicystic ameloblastoma. A prognostically distinct entity. Cancer i977; 40:2278-2285 14.Bredenkamu TK, Zimmerman M. Mickel R: Maxillarv ameloblastoma. krch Otolaryngol Head Neck Surg 1989; 115:99-104 15. Weiss JS, Bressler SB, Jacobs EF, Jr, et al: Maxillary ameloblastoma with orbital invasion. A clinical pathological study. Ophthalmology 1985; 92:71o-713 16. BiGrklund A, Elner A, Snorradothr M: Ameloblastoma of the maxilla. A report of three cases. J Laryngol Otol 1979: 93:1105-1113 17. Sehdev MK, Huvos AG, Srong EW, et al: Ameloblastoma of the maxilla and mandible. Cancer 1974; 33:324-333 18. Goodsell JP, Yamashita D-D, Moody R: Ameloblastoma of the anterior maxilla. J Surg Oncol 1977; 9:407-416 19. Komisar A: Plexiform ameloblastoma of the maxilla with extension to the skull base. Head Neck Surg 1984; 6:172175 20. Crawley WA, Levin LS: Treatment of ameloblastoma. A controversy. Cancer 1978; 42:357-363 21. Van Wyk CW, Thompson OC, Wyma G: A unicystic ameloblastoma mimicking a globulo-maxillary cyst: A case report. Br J Oral Surg 1986; 24:422-425 22.Daramola JO, Abioye AA, Ajagbe HA, et al: Maxillary malignant ameloblastoma with intraorbital extension: A report of a case.J Oral Surg 1980;38:203-206 23. Madiedo G, Hongyung C, Kleinman JG: Ameloblastoma of the maxilla with distant metastasis and hypercalcemia. Am J Clin Path01 1981; 75:585-591
1991
Maxillary
Ameloblastoma: Case Report
FRANKJ. SCACCIA,MD, MELVIN STRAUSS, MD, JAMESARNOLD, MD, AND ANTHONY 7. MANIGLIA, MD
Ameloblastoma of the maxilla is an unusual epithelial tumor of odontogenic
origin. Although it is considered benign, it can behave in a slowly growing infiltrative fashion, with multiple recurrences and eventual intracranial, or even distant, spread. Information on this tumor consists, to a large extent, of case reports presented in the oral surgery literature. This study is a retrospective review of our combined institutional experience with nasomaxillary tumors from 1960 to 1988. Among these cases were four patients with maxillary ameloblastoma, two males and two females, ranging in age from 16 to 66 years at presentation. Disease extent varied from ameloblastoma localized to a cyst in the maxillary sinus to extensive maxillary and ethmoid involvement. Follow-up ranged from 2 to 13 years, and disease course varied from apparent disease control to persistent intracranial tumor, despite attempts at extirpation. An analysis of this experience and the related literature is presented, along with recommendations for radical therapy, when appropriate, to best ensure control. AM J OTOLARYNGOL12:20-25. Copyright 0 1991 by W.B. Saunders Company Key words: ameloblastoma, maxilla, tumor.
proximately 50% of these maxillary tumors are found in the molar region, 30% in the area of the antrum, and the rest at other sites, including less than 2% in the anterior maxilla.g The significance of maxillary involvement is that the potential for serious consequences is much greater than for the patient with mandibular ameloblastoma. This is partially attributable to the fact that the thick compact bone found in the mandible confines the growth. In addition, because of the proximity of the maxilla to the nasal cavity, paranasal sinuses, orbit, and base of skull, maxillary tumors have a much greater tendency to extend into these structures.” Because maxillary ameloblastomas are rare, a standard treatment has yet to be established. Various modes of therapy have been used, including curettage, cryotherapy, cautery, simple excision, radical excision, radiotherapy, and chemotherapy.g While many investigators feel the only acceptable mode of treatment is radical en bloc resection, there are others who believe a more conservative surgical approach is often adequate.l’,ll A review of the otolaryngology literature reveals a paucity of material concerning this question. This report presents our diagnostic and therapeutic experience with four cases of maxillary ameloblastoma.
Ameloblastomas are rare epithelial tumors of the jaws, comprising approximately 1% of all tumors and cysts of odontogenic 0rigin.l Although the first complete description of these lesions is credited to Falkson (1879), it was not until 1933 that Churchill coined the term “ameloblastoma.“2 Since then, these tumors have been the subject of much controversy and discussion. Few generalizations can be made about ameloblastomas. They manifest no apparent tendencies according to sex or race,3 and tumors may appear at any age, with reports in the literature ranging from 21 months to 80 years.4 However, a study of 222 patients revealed that 70% of the tumors were diagnosed between the ages of 10 and 35 years, with an average of 30.1 years.’ The possible etiology of these lesions also varies; sites of origin include dental lamina remnants, the basal layer of oral mucous membrane, and the enamel organ.” It is generally agreed that only 20% of ameloblastomas occur in the maxilla, although some reports indicate an incidence as low as 1?L7*’ Ap-
Received May 22, 1990, from the Departments of Otolaryngology-Head and Neck Surgery and Pathology, Case Western Reserve University School of Medicine, Cleveland, OH. Accepted for publication September 7, 1990. Address correspondence and reprint requests to Melvin Strauss, MD, Department of Otolaryngology-Head and Neck Surgery, Case Western Reserve University School of Medicine, 207i Abington Rd, Cleveland, OH 44106. Copyright 0 1991 by W.B. Saunders Company 0196-0709/91/1201-0001$5.00/0
MATERIALS AND METHODS A retrospective bined 20
institutional
review was performed of the comexperience of this unusual nasomax-
SCACCIA ET AL
21 with removal of a cystic mass. Since the mass had produced bony destruction in the lateral nasal wall, the nasal cavity was explored, with subsequent removal of necrotic bone, The cyst did not penetrate the posterior sinus or communicate with the dentition. Postoperatively, the patient did well. Pathologic examination revealed a simple cyst whose wall was focally lined by areas of simple epithelium without ameloblastic differentiation, as well as areas with mural nests of ameloblastic epithelium (Figs 2, 3, and 4). It was felt that the ameloblastoma originated in a dentigerous cyst. On follow-up at 2 years, there was no evidence of tumor recurrence.
Figure 1. Coronal CT section demonstrating expansion and focal destruction of right maxillary sinus with abnormal soft tissue density.
illary tumor from 1980 to 1988 at Case Western Reserve School of Medicine-University Hospitals (Cleveland, OH), Cleveland Veterans Medical Center [Cleveland, OH), and the Milton S. Hershey Medical Center of Pennsylvania State University (Hershey, PA). Case NO. i. A 16-year-old black girl presented with a 2-month history of postnasal discharge. The patient had a previous surgery of unknown extent with a diagnosis of ameloblastoma at another institution 2 years prior to admission. On physical examination, there was evidence of fullness in the right lateral nasal wall. Sinus films demonstrated opacification of the right maxillary sinus with loss of the lateral and inferior walls. A computed tomography (CT) scan of the sinuses revealed diffuse expansion of the right maxillary sinus with abnormal soft tissue density and destruction of the inferolateral border [Fig 1). Treatment involved a right Caldwell-Luc approach
Case NO. 2. A 66-year-old white man was admitted with a 2-year history of left nasal obstruction. He reportedly underwent an earlier bilateral polypectomy at another institution. No follow-up was conducted until 18 months later when he presented to our clinic. A review of the pathology reports from his previous surgery demonstrated ameloblastoma. Physical examination was remarkable for a pink, moderately vascular, left intranasal mass. A biopsy of this lesion was positive for ameloblastoma. A CT scan revealed a mass completely filling the left maxillary sinus with erosion through the left lateral nasal wall to the nasal septum (Fig 5). In addition, there was opacification of the left ethmoid and sphenoid sinuses, with no intracranial or orbital extension noted. The patient was treated via a degloving approach with a left medial maxillectomy, ethmoidectomy, and sphenoidotomy. The area of the fovea ethmoidalis and sphenoid contained no tumor. The final pathologic report was ameloblastoma. The patient is doing well at 3 years postoperatively, with no evidence of recurrence.
Case NO. 3. A 53-year-old white man was initially diagnosed with ameloblastoma of the right maxillary alveolus 9 years previously. He presented with a recurrence involving the right maxillary sinuses for which he underwent a right maxilloethmoidectomy with preservation of the orbit. He subsequently had local excisions and cryotherapy of apparent small local recurrences in the infratemporal fossa. Eight years after presentation and 17 years after onset, a biopsy of the right infratemporal fossa was once again positive for tumor. A retromaxillary excision was then performed of the sphenoid, pterygoid plates, and infratemporal fossa. He did well for an additional 2 years, when he began developing right retro-orbital pain. A CT scan and mag-
Figure 2. Histologic section of cuboidal lining of dentigerous cyst. (Hematoxylin-eosin stain: magnification X372.)
MAXILLARY
netic resonance imaging demonstrated a right middle fossa tumor [Fig 6). Because it was felt that this was an extension of his ameloblastoma in close proximity to the cavernous sinus and carotid artery, with displacement of the temporal lobe, a two-stage resection was performed. The first stage consisted of creating a right middle fossa cranial neodura so that the dura of the right anterior temporal lobe could be resected with the tumor. This was followed 6 weeks later by a right lateral craniofacial resection and temporal flap reconstruction. It was noted during surgery that although the tumor was removed from the area of the orbit, optic nerve, and temporal lobe with good margins, there was an area adjacent to the cavernous sinus that was not totally eradicated. Ten months later, examination revealed a 1.6-cm mass in the posterior medial aspect of the infratemporal fossa. A CT scan was performed that confirmed this recurrence, with probable extension into the orbital apex, cavernous sinus, petrous apex, and middle cranial fossa. The patient subsequently underwent a sixth operative procedure with a revision maxillectomy and laser excision of the skull base tumor. Following
AMELOBLASTOMA
this, he received a course of postoperative radiotherapy. Currently, he is alive and functioning well, but with persistent disease 22 years after initial diagnosis.
Case No. 4. A 36-year-old white woman initially presented with sinusitis 2 years previously and then subsequently underwent a Caldwell-Luc and nasal polypectomy that revealed ameloblastoma. She was then referred for further evaluation and treatment. A CT scan was performed that demonstrated the presence of tumor in the left maxillary and ethmoid sinuses with possible extension into the infratemporal fossa. The physical examination was remarkable only for a healed left Caldwell-Luc incision and polypoid tissue emanating from the left middle meatus. The patient underwent a left maxilloethmoidectomy, sparing the orbit. Tumor was demonstrated in the nasal polypoid material and in the posterior inferior aspect of the antrum near the maxillary tubercle. The tumor was peeled from the fovea ethmoidalis of the anterior ethmoid. Two months later, because of the possible pres-
Figure 4. Histologic section of ameloblastoma focus within wall of cystic ameloblastoma. (Hematoxylineosin stain; magnification X60.)
23
SCACCIA ET AL
RESULTS A two-institution, retrospective review from 1980 to 1988 revealed a total of 120 neoplasms of the nasal fossa and paranasal sinuses. Four of these cases were maxillary ameloblastomas. These patients ranged in age from 16 to 66 years. The sex distribution was equal; two males and two females. Tumor location on presentation and recurrence included maxillary, ethmoid, and sphenoid sinuses; nasal cavity; infratemporal fossa; middle cranial fossa; and orbital apex. Three of these patients show no evidence of disease and one is alive with disease, with a range of follow-up of 2 to 13 years. Follicular and plexiform histologic variants and ameloblastoma in the wall of a dentigerous cyst were found. These findings are summarized in Table 1.
DISCUSSION
Figure 5. Coronal CT section demonstrating a mass filling the left maxillary sinus with destruction of the lateral nasal wall. Other views demonstrated opacification of the left posterior ethmoid and sphenoid sinuses. ence of residual disease at the fovea, a frontal craniotomy with craniofacial resection of the fovea and cranialization of the frontal sinuses was performed. No gross tumor was observed during the surgery, and the final pathology report was also negative for residual tumor. On follow-up 3 years later, there was no evidence of tumor recurrence.
Figure 6. Coronal fossa tumor.
CT section
demonstrating
a right middle
The most frequent site of involvement of ameloblastomas is within the molar-ramus region of the mandible. The third molar region is also the most commonly affected site in the maxilla. Such tumors may extend into the nose, orbit, maxillary sinus, or base of the skull. Rarely, ameloblastomas arise from a dentigerous cyst.l’ In such cases, both the lining epithelium seen in a dentigerous cyst and ameloblastic epithelium must be present side by side. The term “unicystic ameloblastoma” has been proposed for such lesions. They are reported to have slower progressive growth that may permit enucleation with complete removal rather than partial or complete jaw resection.13 Grossly, ameloblastomas are relatively wellcircumscribed, cystic, or solid lesions. Necrotic foci and cystic degeneration are common. Microscopically, islands of epithelium are embedded within a fibrous stroma in follicular or plexiform patterns. The cells at the periphery of the islands are composed of columnar epithelium whose nuclei are polarized away from the basal membrane. The center of the islands are composed of a loose stellate reticulin network that may contain squamous metaplastic foci. Stromal vascularity may at times be marked. Occasionally, a hyalinized zone may be noted in proximity to the ameloblastic layer. Our experience with ameloblastomas clearly demonstrates the tendency of these lesions to behave in a clinically malignant fashion with direct extension beyond the site of origin in the maxilla. Although all four patients are presently alive, case no. 3 has persistent intracranial tumor. Postoperative radiotherapy was applied in an attempt to palliate this inoperable condition. A review of the literature demonstrates that
24
MAXILLARY
TABLE 1. PATIENT No./ AGE (Y)/ RACE/SEX
PRESENTING SYMPTOMS
Summary
of Patients
HISTOLOGIC FINDINGS
With Maxillary
SITES op INVASION
AMELOBLASTOMA
Ameloblastoma
TREATMENT*
DISEASE DURATION
1/16/B/F
Postnasal discharge
Dentieerous cyst origin
Maxilla: nasal cavity
Caldwell-Luc; nasal cavity debridement (0)
Alive, diseasefree 2 yr
2/66iWfM
Nasal obstruction
Plexiform
Maxilla; nasal cavity; ethmoid; sphenoid
Medial maxillectomy; ethmoidectomy; sphenoidotomy
Alive, diseasefree 3 yr
3/53rwiM
Mass in alveolus
Plexiform
Maxilla; ethmoid
Maxillothmoidectomy (01
Infratemporal fossa
Local excision; cryotherapy (2.8) Retromaxillary excision of sphenoid, pterygoid plates, and infratemporal fossa (8)
Infratemporal fossa
4/36NviF
Postnasal drip; sinus pain
Plexiform
Middle fossa
Cranial neodura construction; craniofacial resection; temporal muscle flap (10)
Infratemporal fossa; orbital apex; middle fossa
Revision maxillectomy; laser excision; radiation (11)
Alive, with disease 13 yr
Ethmoid; maxilla; infratemporal fossa; fovea of anterior ethmoid
Maxilloethmoidectomy (0); craniofacial resection; cranialization frontal sinuses (2 mo)
Alive, diseasefree 3 yr
* Numbers in parentheses indicate number of years after presentation that treatment was undertaken.
many reports support the concept of radical while others still propose more therapy, 4~g~11**4-1g conservative approaches to control ameloblastoma.3~6~‘0~20~2*Sehdev et al characterize the controversy with their statement, “Vitriolic arguments for and against conservative and radical excision abound with no criteria for preference of either.“17 Although the issue of treatment modalities has not been settled, substantial evidence in the literature attests to the aggressiveness of these tumors. Komisarlg presented a case of maxillary ameloblastoma that failed curettage and presented 6 months later with a large recurrence in the maxillary sinus that extended into the pterygomaxillary space, infratemporal fossa, orbit, and nasal cavity. The patient was treated with a hemimaxillectomy and was free of disease 1 year postoperatively. Daramola et al” presented the case of an ameloblastoma that originated in the right side of the maxilla with later involvement of the orbit, frontal sinus, and nasal cavity, with multiple pulmonary metastases. Another tumor that filled the right maxillary sinus and later invaded the orbit and middle cranial cavities is described by Weiss et a1.15 Tsaknis and Nelson4 demonstrated a 50% recurrence rate for the 19 patients in their study
who were treated by local excision or curettage. Small and Waldron’ had similar results, with recurrence in 46% of their cases that underwent local removal or curettage. In this study, all four patients had minor procedures performed initially and all had persistent or recurrent disease. Two patients, then, had extensive surgery early on and have no evidence of recurrence. One patient had less radical surgery due to the apparent extent of disease, the fact that it was a variant of ameloblastoma in a dentigerous cyst, and the age of the patient. One patient presented 9 years after initial diagnosis and, despite radical surgery, his tumor persisted and extended intracranially, and has persisted there despite major extirpative efforts. Recurrence of tumor was almost universally cited as a poor prognostic factor. In their 13 patients who developed recurrences, Sehdev et al found that only 36% could be salvaged by partial l7 They also observed that or total maxillectomy. all of the patients in their study with ameloblastoma who received external irradiation or curettage as their initial treatment developed recurrences. Robinson5 and Small and Waldron,’ in their classic reports, described ameloblastomas as basi-
25
SCACCIA ET AL
tally benign lesions. However, this is based on their morphologic characteristics and not their propensity for local invasion. Of the 1,000 cases from the world literature that Small and Waldron reviewed, they felt that only three cases could meet the verifiable criteria for malignancy, concluding that the incidence of malignant ameloblastoma was extremely rare. Despite its categorization as a benign tumor, a few researchers have emphasized the metastatic potential of this tumor. Madiedo et al reviewed the literature and found 28 cases of ameloblastomas with histologically confirmed metastasis.23 However, in only two cases was the maxilla the primary site. The most common sites of metastasis are the lung and the regional lymph nodes. However, metastatic deposits have also been found in the pleura, vertebra, skull, liver, parotid, and even the small intestine.23 SUMMARY
1. Maxillary ameloblastoma is an unusual neoplasm, with few studies reporting more than isolated cases. 2. In this study, four cases of maxillary ameloblastoma are added to the literature. 3. Although limited, our experience indicates that for complete extirpation of maxillary ameloblastoma, radical surgery, including skull base resection, is indicated in operable cases and offers the best possibility of cure. The possible exception to this is that of tumor confined within the walls of a well-defined dentigerous cyst. 4. Maxillary ameloblastomas are slow-growing infiltrative tumors; regular, long-term follow-up is necessary to recognize and treat recurrences. Acknowledgment. The authors wish to thank Jerald Goldberg, DDS, Director, Division of Oral Surgery, Case Western Reserve University School of Dentistry; Kelly Sorensen, MD, Department of Pathology, Case Western Reserve University School of Medicine; Mark Klingensmith, MD, Department of Otolaryngology, Geisinger Clinic; Robert Page, MD, Division of Neurosurgery, MS. Hershey Medical Center; and John Price, MD, Baltimore, MD, for their roles in the care of these patients.
References 1. Small IA, Waldron CA: Ameloblastoma of the jaws: Oral Surg 1955;8:281-297 2. Shaw HJ, Katsikas DK: Ameloblastoma of the maxilla. J Laryngol Otoi 1973;87:873-884 3. Sandler KA, Novo RM, Rudner BE: A study of ameloblastoma: Age, sex, and location statistics. NY State Dent J 1983; 49:682-684 4. Tsaknis PJ, Nelson JF: The maxillary ameloblastoma: An analysis of 24 cases. Oral Surg 1980; 38:336-342 5. Robinson HBG: Ameloblastoma. A survey of 379 cases from the literature: Arch Path01 1937; 23:831-843 6. Cheney ML, Wolf C, Cox RA. et al: Ameloblastoma presenting as chronic sinusitis. La State Med Sot J 1986; 2:11-14 7. Seabaugh JL, Templer JW, Havey A: Ameloblastoma presenting as a nasopharyngeal tumor. Otolaryngol Head Neck Surg 1986; 94:265-267 8. Adekeye EO, Lavey KM: Recurrent ameloblastoma of the maxilla-facial region. J Maxillofac Surg 1986;14:153-157 9. Batsakis JG. McClatchey KD: Ameloblastoma of the maxilla and peripheral ameloblastomas. Ann Oto Rhino1 Laryngol 1983; 92:532-533 10. Porter J, Miller R, Sartigos GT: Ameloblastoma of the maxilla. J Oral Surg 1977; 44:34-38 11.Weissman BW, Wetli C: Ameloblastoma of the maxilla. South Med J 1977; 70251-253 12.Kane JP: Odontogenic Tumors. A Statistical and Morphological Study of 88 Cases. Thesis, Georgetown University, Washington, DC, 1951 13. Robinson L, Martinez MG: Unicystic ameloblastoma. A prognostically distinct entity. Cancer i977; 40:2278-2285 14.Bredenkamu TK, Zimmerman M. Mickel R: Maxillarv ameloblastoma. krch Otolaryngol Head Neck Surg 1989; 115:99-104 15. Weiss JS, Bressler SB, Jacobs EF, Jr, et al: Maxillary ameloblastoma with orbital invasion. A clinical pathological study. Ophthalmology 1985; 92:71o-713 16. BiGrklund A, Elner A, Snorradothr M: Ameloblastoma of the maxilla. A report of three cases. J Laryngol Otol 1979: 93:1105-1113 17. Sehdev MK, Huvos AG, Srong EW, et al: Ameloblastoma of the maxilla and mandible. Cancer 1974; 33:324-333 18. Goodsell JP, Yamashita D-D, Moody R: Ameloblastoma of the anterior maxilla. J Surg Oncol 1977; 9:407-416 19. Komisar A: Plexiform ameloblastoma of the maxilla with extension to the skull base. Head Neck Surg 1984; 6:172175 20. Crawley WA, Levin LS: Treatment of ameloblastoma. A controversy. Cancer 1978; 42:357-363 21. Van Wyk CW, Thompson OC, Wyma G: A unicystic ameloblastoma mimicking a globulo-maxillary cyst: A case report. Br J Oral Surg 1986; 24:422-425 22.Daramola JO, Abioye AA, Ajagbe HA, et al: Maxillary malignant ameloblastoma with intraorbital extension: A report of a case.J Oral Surg 1980;38:203-206 23. Madiedo G, Hongyung C, Kleinman JG: Ameloblastoma of the maxilla with distant metastasis and hypercalcemia. Am J Clin Path01 1981; 75:585-591
