Desmoplastic ameloblastoma of the mandible: A case report and review of the literature

Desmoplastic ameloblastoma of the mandible: A case report and review of the literature

CASE REPORTS J Oral Maxillofac Surg 60:194-198, 2002 Desmoplastic Ameloblastoma of the Mandible: A Case Report and Review of the Literature Michael L...

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CASE REPORTS J Oral Maxillofac Surg 60:194-198, 2002

Desmoplastic Ameloblastoma of the Mandible: A Case Report and Review of the Literature Michael L. Beckley, DDS,* Vince Farhood, DDS,† Lisa K. Helfend, PhD, MD,‡ and Ali Alijanian, DDS§ Clinical examination revealed a firm mass in the left mental region. No lymphadenopathy or tenderness to palpation was noted. Intraoral examination revealed a painless, nonmobile, bony-hard mass on the facial aspect of the left mandibular alveolar process. The mass was approximately 2 cm in diameter and was located in the buccal vestibule adjacent to the roots of the canine and first premolar tooth. The surface epithelium appeared normal (Fig 1). An panoramic radiograph showed a diffuse osseous lesion with regions of mixed radiolucency and radiopacity. The mass had displaced the roots of the canine and first premolar (Fig 2). All teeth in the involved region were vital to pulp testing, and there was no evidence of infection. An incisional biopsy to remove the facial aspect of the lesion was performed with the patient under local anesthesia. Microscopic examination of the surgical specimen showed proliferating nests, islands, and sheets of loosely arranged spindle cells with peripherally pallisaded columnar to cuboidal hyperchromatic cells (Figs 3, 4). Some nests demonstrated central cystic degeneration. The surrounding marrow spaces ranged from loosely myxoid to densely collagenous. There was no evidence of granular cells or squamous metaplasia. A diagnosis of DA was made. The patient was treated with marginal resection of the anterior mandible and preservation of the inferior border via an intraoral approach. He also received an immediate reconstruction with a corticocancellous graft obtained from the ilium (Fig 5). A minor wound dehiscence developed, and a portion of the bone graft was lost. After local wound

The desmoplastic variant of ameloblastoma was first described by Eversole et al1 in 1984. Since then, 80 cases have been reported in the literature.1-22 The histologic pattern of desmoplastic ameloblastoma (DA) is characterized by extensive stromal collagenization, or desmoplasia.13 Immunohistochemical studies suggest that the desmoplasia originates from de novo synthesis of extracellular matrix proteins. DAs do not present with radiographic or clinical features that are typical of other variants of ameloblastoma. The radiographic presentation is similar to that of many benign fibro-osseous lesions, so this newly recognized variant should be considered in the differential diagnosis of patients who have tumors that resemble fibro-osseous tumors of the mandible and maxilla. The purpose of this article is to present the case of a patient with DA and a comprehensive review of the literature and to discuss treatment options and outcomes.

Report of a Case A 31-year-old Hispanic man was referred by his general dentist to Highland Hospital in May 1999 with a chief complaint of left mandibular swelling. The patient stated that the mass had been present for several years but that it had increased in size over the past few months. He denied pain, sensory deficit, or history of trauma, and his past medical history was unremarkable. Received from Alameda County Medical Center, Oakland, CA. *Chief Resident, Division of Oral and Maxillofacial Surgery. †Assistant Program Director, Division of Oral and Maxillofacial Surgery. ‡Attending, Department of Pathology and Laboratory Medicine. §Former Resident, Division of Oral and Maxillofacial Surgery. Address correspondence and reprint requests to Dr Beckley: Division of Oral and Maxillofacial Surgery, Alameda Country Medical Center, Highland Campus, 1411 E 31st St, Oakland, CA 94602; e-mail: [email protected] © 2002 American Association of Oral and Maxillofacial Surgeons

0278-2391/02/6002-0012$35.00/0

FIGURE 1. View of lesion in the left anterior mandible.

doi:10.1053/joms.2002.29822

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FIGURE 2. Panoramic radiograph showing a mixed radiopaque/ radiolucent lesion in the left anterior mandible.

care and minor debridement, the dehiscence resolved. He has experienced no recurrence after 19 months of follow-up.

Discussion The DA is a relatively newly recognized variant of ameloblastoma. Since its initial description in 1984, 80 cases have been reported in the literature.1-22 Its incidence ranges from 0.9% to 12.1% of all ameloblastomas.4 Despite these reports, relatively little is known about the origin or natural history of this entity. A literature search on DA was conducted using a Medline database that revealed 80 previously reported cases. The mean age at initial presentation was 41.2 years (range, 18 to 70 years), and men and women are affected equally. When lesions were divided into those that were less than 3.0 cm and those that were 3.0 cm or larger in maximum diameter, the majority were larger than 3.0 cm at initial presentation and were almost equally distributed between the maxilla and mandible. Radiographic borders in the majority of cases were described as poorly defined. Tumors were further classified according to whether

FIGURE 3. Photomicrograph showing scattered islands of odontogenic epithelium in a follicular pattern within a densely collagenous fibrous stroma (hematoxylin-eosin stain, medium power).

FIGURE 4. Photomicrograph showing peripheral palisading of tumor cells in a pattern similar to that seen in ameloblasts of the enamel organ (hematoxylin-eosin stain, high power).

there was no loculation, uniloculation, or multiloculation, and radiographic appearance was described as either mixed radiolucent/radiopaque or radiolucent. A summary of the information obtained is shown in Tables 1 and 2. The majority of DAs appear to have been treated in a manner similar to that of the multicystic variants of ameloblastoma, but the treatments were not uniform. The only recurrences noted were in patients who underwent enucleation.4,7,14 Although the number of reported cases continues to increase, information and trends in the epidemiology, behavior, and prognosis of DAs should be periodically reviewed. Unfortunately, the information available for each reviewed case was not uniform, so the potential exists for an interpretative error. For example, descriptive adjectives such as “soap bubble,” multilocular, and variations in radiolucency and radiopacity were not consistently applied. Furthermore, when information is pooled from a multitude of languages, clinical and histopathologic descriptors can be lost or misinterpreted in the process. Despite these limitations, some interesting characteristics of this tumor and its differences from other types of ameloblastomas are revealed. The mean age at the time of presentation for DA is similar to the

FIGURE 5. Panoramic radiograph showing immediate reconstruction of the surgical defect.

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Table 1. GENERAL DATA ON REPORTED CASES OF DESMOPLASTIC AMELOBLASTOMA

Gender

Race

Sakashita et al5 Fukushima et al6 Keszler et al7 (series of 14 cases)

50 47 45 to 50 56 42 50 68 37 18 37 64 60 70 19 to 62 (mean, 37.8)

F F F M F M F F M M M F M NA

White Black Malaysian NA NA NA Chinese Chinese Chinese Chinese Chinese Japanese NA NA

⬎3.0 ⬎3.0 ⬎3.0 ⬎3.0 ⬍3.0 ⬎3.0 ⬍3.0 ⬍3.0 ⬎3.0 ⬍3.0 ⬎3.0 ⬎3.0 ⬎3.0 NA

Thompson et al8 Raubenheimer et al9 Ng and Siar11 (series of 17 cases)

31 NA 21 to 60 (mean, 36.6)

F

⬎3.0 NA Range, 1.8 to 8.5

Ashman et al12 Philipsen et al13

53 21 53 55 21 to 68 (mean, 45.5)

M M M M NA

Black African 7 Chinese 6 Malay 2 Indian 1 Sikh 1 Kadazan Black Chinese Chinese Chinese NA

Higuchi et al15 (series of 5 cases, 2 cases of hybrid lesions)

38 to 70 (mean, 45)

1 F, 4M

NA

3 ⬎3.0 2 ⬍2.0

Tanimoto et al16 Yoshimura and Saito17 Kaffe et al18 Gratz and Makek19

24 36 40 59 33 36 27 32 35 41 33 31

Reference Eversole et al1

Kawai et al2 Ludvikoya et al3 Lam et al4

Waldron and el-Mofty14 (series of 14 cases; radiographs available only for 6 cases)

Furuichi et al20 Morita et al21

Louis et al22 Beckley et al (current case report)

12 F, 5M

F F M M F M M F F M M M

Japanese Japanese NA NA NA NA NA NA NA NA Black Hispanic

⬎3.0 ⬎3.0 ⬎3.0 ⬎3.0 “Most” ⬍3.0

⬎3.0 ⬎3.0 ⬎3.0 ⬎3.0 ⬎3.0 ⬍3.0 NA NA NA NA ⬎3.0 ⬍3.0

Tumor Location

Tumor Borders

Mandible: A, PM, M Mandible: A Maxilla: PM, M Mandible: M Mandible: PM Mandible: PM, M Maxilla Mandible: A Maxilla: A Mandible: A Maxilla: A, PM, M Maxilla: A, PM Maxilla: A, PM, M 83% Mandible 17% Maxilla

Poorly defined Poorly defined Poorly defined Well defined Poorly defined Poorly defined Poorly defined Poorly defined Poorly defined Poorly defined Poorly defined Poorly defined Poorly defined 29% Well defined 71% Poorly defined

Unilocular No loculation No loculation Unilocular No loculation No loculation Multilocular Multilocular Multilocular No loculation No loculation Multilocular No loculation 33% Unilocular 42% Multilocular

Lucent Mixed RL/RO Mixed RL/RO Lucent Lucent Lucent Lucent Lucent Lucent Lucent Lucent Lucent Mixed RL/RO 75% RL 25% Mixed RL/RO

Maxilla: A, PM, M NA 10 Mandible 7 Maxilla

Poorly defined Not stated 7 Poorly defined *Others not stated

No loculation Not stated 7 No loculation 2 Multilocular *Others not stated

Mixed RL/RO Not stated 7 Mixed RL/RO 3 RL *Others not stated

Mandible: A, PM Maxilla: A, PM, M Maxilla: A, PM, M Mandible: A, PM, M 2 Mandible: A 3 Mandible: PM 2 Mandible M 6 Maxilla: A 1 Maxilla: PM 2 Mandible: A 1 Mandible: A, PM 1 Mandible: M, R 1 Maxilla: A, PM Mandible: A, PM, M Maxilla: A, PM, M Maxilla: A, PM, M Mandible: PM, M Maxilla: A, PM, M Maxilla: A Maxilla: M Mandible: A Maxilla: PM Maxilla: PM Maxilla: A, PM, M Mandible: A, PM

Well defined Poorly defined Poorly defined Mixed 5 Poorly defined 1 Well defined

No loculation Multilocular Multilocular Multilocular 1 Unilocular 5 No loculation

Mixed RL/RO Lucent Lucent Mixed RL/RO 4 Mixed RL/RO

6 Poorly defined

6 Multilocular

6 Mixed RL/RO

4 Enucleation and curettage 1 Sectional mandibulectomy

Poorly defined Poorly defined Poorly defined Poorly defined Poorly defined Well defined Poorly defined Poorly defined Poorly defined Poorly defined Poorly defined Poorly defined

No loculation Multilocular No loculation No loculation No loculation No loculation No loculation No loculation No loculation No loculation No loculation No loculation

Mixed Mixed Mixed RL Mixed RL Mixed Mixed Mixed Mixed Mixed Mixed

Marginal resection Subtotal maxillectomy Hemimaxillectomy Not stated Subtotal maxillectomy Enucleation Enucleation Marginal resection Marginal resection Marginal resection Subtotal maxillectomy Marginal resection

Abbreviations: A, anterior; M, molar; NA, data not available; PM, premolar; RL, radiolucent; RO, radiopaque.

Tumor Locularity

Tumor Radiographic Appearance

RL/RO RL/RO RL/RO RL/RO RL/RO RL/RO RL/RO RL/RO RL/RO RL/RO

Treatment Not stated En bloc resection Hemimaxillectomy Block resection Excision Partial mandibulectomy Enucleation Segmental mandibulectomy Maxillectomy Marginal mandibulectomy Maxillectomy Partial maxillectomy Partial maxillectomy 36% Resection 50% Enucleation 14% Curettage Partial maxillectomy Not stated 1 Marginal resection 1 Hemimaxillectomy 2 Partial maxillectomy 2 Excision *Others not stated Marginal resection Maxillectomy Maxillectomy Marginal mandibulectomy Not stated

DESMOPLASTIC AMELOBLASTOMA OF THE MANDIBLE

Age (yr)

Tumor Size (cm)

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Table 2. SUMMARY OF DATA ON REPORTED CASES OF DESMOPLASTIC AMELOBLASTOMA

No. Patient age (yr) Mean, 41.2 Range, 18 to 70 Gender (n ⫽ 52) Female Male Tumor size (cm) (n ⫽ 31) ⬍3.0 ⬎3.0 Tumor location (n ⫽ 66) Maxilla Mandible Tumor borders (n ⫽ 48) Poorly defined Well defined Tumor locularity (n ⫽ 50) No loculation Unilocular Multilocular Tumor radiographic appearance (n ⫽ 49) Mixed radiolucent/radiopaque Radiolucent

%

80

26 26

50 50

8 23

25.80 74.20

32 34

48 52

44 4

92 8

32 3 15

64 6 30

32 17

65 35

mean ages for the other variants of ameloblastoma that have been reported (between 35 and 45 years).23 There also is no gender predilection for DA; this is consistent with other variants of ameloblastoma. Regarding tumor size, 74.2% of the DAs were larger than 3.0 cm at the initial presentation. In a review of 3,677 ameloblastomas, Reichart et al24 reported that average tumor size at the initial presentation was 4.3 cm (median, 3.0 cm). The ratio of ameloblastoma of the mandible to that of the maxilla is 5:1 for nondesmoplastic ameloblastomas.24 In our review of DAs, the incidence was similar for the maxilla and the mandible (48% in the maxilla and 52% in the mandible). This differs significantly from a series of 17 patients with DA reported by Keszler et al7 in 1996 in which the mandible was involved in 83% of the DAs and the maxilla was involved in only 17%. Whether this is due to limited data or a specific patient population is uncertain. On examination of the radiographic borders, DAs were described as poorly defined in 92%. This contrasts with the well-defined, often sclerotic borders seen in other variants of ameloblastoma. Nondesmoplastic variants of ameloblastoma are traditionally described radiographically in terms of locularity. In the series by Reichart et al,24 51.1% of the tumors were described as unilocular, whereas 48.9% were described as multilocular or multicystic. When this criterion was applied to DAs, 64% were not loculated, 6% were unilocular, and 30% were multilocular. In a review of the case reports, it became evident that the

use of these descriptors was not uniform. With respect to the overall radiographic appearance of DA, 65% were described as mixed radiolucent/radiopaque lesions and 35% were described as radiolucent. Histologically, a DA is composed of small islands of tumor cells located within an extensively collagenous stroma (desmoplasia). Typical ameloblastic columnar cells may be scant, and peripheral pallisading may be absent. Most tumors contain cords of odontogenic epithelium, but these tumors tend to lack the more typical follicular pattern. Osteoplasia may also be present.13 Treatment modalities were broad ranging and varied. The majority of tumors were treated in a manner consistent with the treatment of multicystic ameloblastoma. Since 1984, 80 cases of DA have appeared in the literature worldwide, and this case brings the number to 81. Based on the available information, the clinician should keep in mind some salient points when diagnosing and treating patients with these neoplasms. First, the majority of DAs present with a radiographic presentation that resembles many fibro-osseous lesions. Second, the definitive diagnosis of ameloblastoma and other tumors is based on the histology and not the radiographic or clinical findings. Third, due to the clinical features of this tumor, it is likely that many of these lesions will be misdiagnosed and initially “treated” with enucleation and curettage. It has been shown that enucleation and curettage of this variant results in recurrence,14 so it is incumbent on the surgeon to try to establish a definitive diagnosis through biopsy and then to resect the tumor in a manner similar to that used for the multicystic variants of ameloblastoma. Acknowledgment The authors thank Dr Sabine Hill and Ms Eriko Ikihara for providing assistance with language translation.

References 1. Eversole LR, Leider AS, Hansen LS: Ameloblastomas with pronounced desmoplasia. J Oral Maxillofac Surg 42:735, 1984 2. Kawai T, Kishino M, Hiranuma H, et al: A unique case of desmoplastic ameloblastoma of the mandible: Report of a case and brief review of the English language literature. Oral Surg Oral Med Oral Pathol Oral Radiol Endod 87:258, 1999 3. Ludvikoya M, Michal M, Zamecnik M, et al: Desmoplastic ameloblastoma. Cesk Patol 34:94, 1998 4. Lam KY, Chan AC, Wu PC, et al: Desmoplastic variant of ameloblastoma in Chinese patients. Br J Oral Maxillofac Surg 36:129, 1998 5. Sakashita H, Miyata M, Okabe K, et al: Desmoplastic ameloblastoma in the maxilla: A case report. J Oral Maxillofac Surg 56:783, 1998 6. Fukushima D, Kobayashi H, Takeda I, et al: A case of desmoplastic ameloblastoma of the maxilla. Bull Tokyo Dent Coll 38:223, 1997

198 7. Keszler A, Paparella ML, Dominguez FV: Desmoplastic and non-desmoplastic ameloblastoma: A comparative clinicopathological analysis. Oral Dis 2:228, 1996 8. Thompson IO, van Rensburg LJ, Phillips VM: Desmoplastic ameloblastoma: Correlative histopathology, radiology and CT-MR imaging. J Oral Pathol Med 25:405, 1996 9. Raubenheimer EJ, van Heerden WF, Noffke CE: Infrequent clinicopathological findings in 108 ameloblastomas. J Oral Pathol Med 24:227, 1995 10. Siar CH, Ng KH: Patterns of expression of intermediate filaments and S-100 protein in desmoplastic ameloblastoma. J Nihon Univ Sch Dent 35:104, 1993 11. Ng KH, Siar CH: Desmoplastic variant of ameloblastoma in Malaysians. Br J Oral Maxillofac Surg 31:299, 1993 12. Ashman SG, Corio RL, Eisele DW, et al: Desmoplastic ameloblastoma: A case report and literature review. Oral Surg Oral Med Oral Pathol 75:479, 1993 13. Philipsen HP, Ormiston IW, Reichart PA: The desmo- and osteoplastic ameloblastoma: Histologic variant or clinicopathologic entity? Case reports. Int J Oral Maxillofac Surg 21:352, 1992 14. Waldron CA, el-Mofty SK: A histopathologic study of 116 ameloblastomas with special reference to the desmoplastic variant. Oral Surg Oral Med Oral Pathol 63:441, 1987 15. Higuchi Y, Nakamura N, Ohishi M, et al: Unusual ameloblastoma with extensive stromal desmoplasia. J Cranio Maxillofac Surg 19:323, 1991

16. Tanimoto K, Takata T, Suei Y, et al: A case of desmoplastic variant of a mandibular ameloblastoma. J Oral Maxillofac Surg 49:94, 1991 17. Yoshimura Y, Saito H: Desmoplastic variant of ameloblastoma: Report of a case and review of the literature. J Oral Maxillofac Surg 48:1231, 1990 18. Kaffe I, Buchner A, Taicher S: Radiologic features of desmoplastic variant of ameloblastoma. Oral Surg Oral Med Oral Pathol 76:525, 1993 19. Gratz KW, Makek M: Desmoplastic ameloblastoma: A new morphological subtype of ameloblastoma. Dtsch Z Mund Kiefer Gesichtschir 12:445, 1988 20. Furuichi Y, Shimada J, Takeshima H, et al: A case of desmoplastic ameloblastoma coexisting with a primordial cyst observed in the anterior region of maxilla. Jpn J Oral Diag/Oral Med 9:149, 1996 21. Morita S, Arika T, Nakajima M, et al: A clinical, radiographic, and pathologic study of desmoplastic ameloblastoma. Jpn J Oral Maxillofac Surg 40:988, 1994 22. Louis PJ, Fugler RC, August M: Mixed radiolucent/radiopaque lesion of the maxilla. J Oral Maxillofac Surg 58:86, 2000 23. Regezi JA, Sciubba J: Oral Pathology: Clinical-Pathologic Correlations. Philadelphia, PA, Saunders, 1993 24. Reichart PA, Philipsen HP, Sonner S: Ameloblastoma: Biological profile of 3677 cases. Eur J Cancer B Oral Oncol 31B:86, 1995