Basic and Applied Pathology 2008; 1: 203205 doi:10.1111/j.1755-9294.2009.00044.

CASE REPORT

Ameloblastic brosarcoma: A case mimicking adenoid cystic carcinoma

Kyungeun Kim1 , Sang Yoon Kim2 and Kyung-Ja Cho1
Departments of 1 Pathology and 2 Otorhinolaryngology, University of Ulsan College of Medicine, Asan Medical Center, Seoul, Korea

Received 13 December 2008 Accepted 16 December 2008 Correspondence Kyung-Ja Cho, M.D., Department of Pathology, University of Ulsan College of Medicine, Asan Medical Center, 388-1 Pungnap-dong, Songpa-gu, Seoul 138-736, Korea. Tel: 02-3010-4545; Fax: 02-472-7898. Email: kjc@amc.seoul.kr

INTRODUCTION
Ameloblastic brosarcoma (AFS) is a rare odontogenic tumor comprising a benign epithelial component and a malignant ectomesenchymal component. AFS is regarded as the malignant counterpart of the ameloblastic broma, and along with ameloblastic brodentinosarcomas and ameloblastic bro-odontosarcomas, belongs to the group of odontogenic sarcomas according to the 2005 WHO classication.1 Although the name harbors the term brosarcoma, the mesenchymal component of AFS appears to be less committed than the cells of soft tissue brosarcomas.2,3 We describe a case of AFS with an unusual mesenchymal component.

CASE REPORT
A 63-year-old man was referred to the Asan Medical Center for the further evaluation of a painful and tender swelling in the left cheek that had persisted for 3 months. A biopsy had been performed at a clinic and the pathologic report suggested an adenoid cystic carcinoma. The patient had a history of surgery for a presumed ameloblastoma of his left maxilla 11 years pervious. An oral examination of the patient revealed neither ulceration nor protrusion. Neck computed tomography and magnetic resonance imaging revealed a heterogeneously enhancing mass in the left maxillary sinus, and destruction and remodeling of the anterior and posterior walls (Fig. 1). The mass appeared to extend to the left buccal and left retroantral spaces. Bone scan and fusion whole body positron emission tomography showed no evidence of distant metastasis. The patient subsequently underwent a left total maxillectomy. The excised specimen contained a relatively well-dened multinodular solid mass measuring 4.5 3.0 2.8 cm. It showed expansile growth with creamy white translucent cut surface and was abutting upon the hard palate (Fig. 2). Microscopically, the tumor was composed of three distinct components. The major component was nests of small ovoid cells forming cylindromatous microcystic spaces lled with hyaline or basophilic myxoid materials, resembling an adenoid cystic carcinoma (Fig. 3). The second component consisted of hypercellular short spindle cells arranged in a fascicular pattern, featuring a rather typical malignant odontogenic ectomesenchyme. Mitoses

were frequently observed in this component, at a rate of 9/50 high power eld (HPF) (Fig. 4). The third component was ameloblastic epithelium multifocally scattered between the spindle or cribriform components (Fig. 5). This component, which was not observed in the preoperative biopsy, consisted of geographic nests of uniform cuboidal or columnar cells with peripheral palisading and reverse polarization patterns, indicating the ameloblastic epithelium. The three components of the tumor were further characterized immunohistochemically with regard to cytokeratin (CK), smooth muscle actin (SMA), CD34 and ki-67 expression. The tumor cells of the cribriform area, which mimicked an adenoid cystic carcinoma, were unexpectedly negative for CK, and only focally positive for SMA (Fig. 6). The spindle cell component was negative for both CK and SMA. The epithelial component was positive for CK and negative for SMA. CD34 immunostaining was tried according to a previous report that the brosarcomatous component of AFS could express this immature mesenchymal marker,2 but the result in this tumor was negative. The ki-67 labeling index of the spindle cell component was increased up to 20%, indicating a moderately high proliferative activity. The possibility that the present tumor represented a composite or collision of an adenoid cystic carcinoma and an odontogenic tumor was excluded on the basis of the immunohistochemical ndings. The major cribriform component was considered a peculiar variation of the odontogenic ectomesenchyme, possibly with focal myobroblastic differentiation. The patient underwent 6660GY of adjuvant radiotherapy after the operation. To date, a 36-month follow-up has shown no evidence of local recurrence or distant metastasis.

DISCUSSION
Ameloblastic brosarcoma (AFS) is a very rare tumor, and not a great deal of its morphologic diversity is known. A characteristic feature of AFS is a biphasic pattern comprising various amounts of the epithelial and mesenchymal components. In the present case, the mesenchymal component displayed a peculiar cribriform growth pattern with a myxohyaline matrix, leading to a preoperative diagnosis of adenoid cystic carcinoma (ACC), which is not rare in the maxillary sinus. The diagnosis of AFS could be made after the examination of a total tumor, which proved to contain
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Figure 1 Magnetic resonance image showing a relatively well-dened heterogeneously enhancing soft tissue mass in the left maxillary sinus. Note destruction and remodeling of the maxillary sinus wall and alveolar bone.

Figure 3 A predominant cribriform proliferation of small ovoid cells in the myxohyaline stroma. This feature mimics an adenoid cystic carcinoma.

Figure 2 Gross photograph of the total maxillectomy specimen showing a solid multinodular mass abutting upon the hard palate (arrow).

Figure 4 Spindle cell mesenchymal component showing nuclear hyperchromatism, mitoses and increased cellularity.

unequivocal ameloblastic epithelium. The ACC-like cells proved to be of mesenchymal nature, and on review, appeared to oat in rows of often one layer, with indistinct microcystic luminal margins, compared to the well-formed ducts or tubules lined by inner epithelial and outer myoepithelial cells of ACC. It was unusual that the mesenchymal components in our case were dual. The major ACC-like component showed focal smooth muscle actin immunopositivity and CD34 negativity, suggesting a differentiation towards the myobroblastic lineage. The other spin204

dle cell component was smooth muscle actin-negative and showed higher mitotic gures and ki-67 labeling index (20%) than the major component (12%), being more representative of brosarcoma. Assessment of proliferative activity by immunohistochemistry or DNA ploidy has been helpful in differential diagnosis between AFS and ameloblastic broma.4,5 Recently, a case of AFS manifesting as a wide range of cellular compositions, including low grade spindle cell sarcoma and highly pleomorphic anaplastic features, has been reported.3 The sarcomatous component showed positivity for c-kit and p53. Another case

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ameloblastic broodontoma.58 Our patient also had a history of curettage at the same site (the maxillary sinus) for a presumed ameloblastoma 11 years previously. The former tumor might have been ameloblastic broma rather than ameloblastoma, since AFS arising from an ameloblastoma has not yet been documented. De novo AFS are known to occur in younger patients (mean 22 years old) than secondary AFS (mean 33 years old), but any histological differences between the two categories have not been documented.9 It was interesting to note that our case and the forenamed case3 both developed a decade after the rst operations and had heterogeneous malignant features. Further observation on the sarcomatous progression of ameloblastic broma is needed to understand the pathogenesis of this rare neoplasm. The treatment of choice for AFS is surgical resection with wide surgical margins. Local recurrence rates of AFS are reported to be 37% in areas of the gingiva, oor of the mouth and neck,9 but only one case has been documented to have distantly metastasized.10 In our case, as the majority malignant component displayed low grade features, the prognosis is expected to be better than other malignancies of the maxillary sinus, including ACC. It is important to recognize variable features of ectomesenchymal components of AFS to provide a proper diagnosis and treatment.
Figure 5 Scattered islands and nests of the ameloblastic epithelial components between mesenchymal components.

REFERENCES
1. Carlos R, Altini M, Takeda Y. Odontogenic sarcomas. In:Barnes L, Eveson JW, Reichart P, Sidransky P, eds. World Health Organization Classication of tumours Pathology and Genetics of Head and Neck Tumours. Lyon: IARC Press; 2005; 2945. 2. Lee OJ, Kim HJ, Lee BK, Cho KJ. CD34 expressing ameloblastic brosarcoma arising in the maxilla: a new nding. J Oral Pathol Med 2005; 34: 31820. 3. Williams MD, Hanna EY, El-Naggar AK. Anaplastic ameloblastic brosarcoma arising from recurrent ameloblastic broma: restricted molecular abnormalities of certain genes to the malignant transformation. Oral Maxillofac Pathol 2007; 104: 725. 4. Sano K, Yoshida S, Ninomiya H et al. Assessment of growth potential by MIB-1 immunohistochemistry in ameloblastic broma and related lesions of the jaws compared with ameloblastic brosarcomas. J Oral Pathol Med 1998; 27: 5963. 5. Muller S, Parker DC, Kapadia SB, et al. Ameloblastic brosarcoma of the jaw. A clinicopathologic and DNA analysis of ve cases and review of the literature with discussion of its relationship to ameloblastic broma. Oral Surg Oral Med Oral Pathol Oral Radiol Endod 1995; 79: 46977. 6. Howell RM, Burkes EJ Jr. Malignant transformation of ameloblastic bro-odontoma to ameloblastic brosarcoma. Oral Surg Oral Med Oral Pathol 1977; 43: 391401. 7. Takeda Y, Kaneko R, Suzuki A. Ameloblastic brosarcoma in the maxilla, malignant transformation of ameloblastic broma. Virchows Arch A Pathol Anat Histopathol 1984; 404: 25363. 8. Kobayashi K, Murakami R, Fujii T, Hirano A. Malignant transformation of ameloblastic broma to ameloblastic brosarcoma: case report and review of the literature. J Craniomaxillofac Surg 2005; 33: 3525. 9. Bregni RC, Taylor AM, Garcia AM. Ameloblastic brosarcoma of the mandible: report of two cases and review of the literature. J Oral Pathol Med 2001; 30: 31620. 10. Chomette G, Auriol M, Guilbert F, Delcourt A. Ameloblastic brosarcoma of the jaw- report of three cases. Clinico-pathologic, histoenzymological and ultrastructural study. Pathol Res Pract 1983; 178: 407.

Figure 6 Immunohistochemical stain showing smooth muscle actin positivity of the cribriform component.

report of AFS described CD34 expression in the sarcomatous component.2 Although the term AFS is currently uniformly applied to ameloblastic neoplasms with a malignant connective tissue component, it appears that differentiation levels or directions of the ectomesenchymal component of AFS are variable. Pathological subclassication of AFS may be required after accumulation of larger series. Approximately a third of ameloblastic brosarcomas are reported to arise from a preexisting benign ameloblastic broma or
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