Nodular Lesion in the Buccal Mucosa

Home , Chondromyxoid fibroma, Nodule (medicine)

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Nodular lesion in the buccal mucosa

Article in Journal of the American Dental Association (1939) · March 2015 Impact Factor: 2.01 · DOI: 10.1016/j.adaj.2014.11.020 · Source: PubMed

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Ana Carolina Amorim Pellicioli Marco Antonio T Martins University of Campinas Universidade Federal do Rio Grande d…

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DIAGNOSTIC CHALLENGE Nodular lesion in the buccal mucosa

THE CHALLENGE Bruna Jalfim Maraschin, MSc; Ana 55 Carolina Amorim Pellicioli, MSc; Lélia -year-old woman showing symptoms of a nodular lesion 5 Batista de Souza, PhD; Pantelis involving the left buccal mucosa with a history of approximately ’ Varvaki Rados, PhD; Marco Antonio years sought treatment at our dental clinic. The patient s medical Trevizani Martins, PhD; Manoela A history revealed diabetes mellitus, hypertension, and arthrosis Domingues Martins, PhD treated with metformin, enalapril, hydrochlorothiazide, and ibuprofen. The patient reported no alcohol use or tobacco consumption. The extraoral examination revealed no abnormalities. The intraoral examination revealed a single, well-circumscribed, submucosal, nodular lesion covered with normal epithelium, measuring approximately 1.0 centimeter in diameter (Figure 1A). On palpation, the lesion was asymptomatic, had a hard consistency, and appeared to be attached firmly to subjacent tissue. The panoramic radiograph revealed no bone alterations. On the basis of these findings, the clinical diagnosis was benign mesenchymal neoplasm. Excisional biopsy was performed. During the surgical procedure, the specimen was grayish, had fibrous consistency, and measured 1.0 cm in diameter (Figure 1B). The lesion appeared to be inserted into the adjacent tissues. No capsule was identified. The specimen was submitted to histopathologic analysis, which revealed a well-defined circumscribed lesion with no well-defined capsule (Figure 2A). Ovoid, polygonal, and spindle cells were arranged in lobes, surrounded by dense connective tissue (Figure 2A). These cells were inter- spersed with myxoid matrix and chondroid matrix (Figures 2A and 2B). No local invasion, mitosis, necrosis, or minor salivary glands were observed. Some areas had foci with cellular atypia, exhibiting cells with nuclear hyperchromatism (Figure 2C) as well as the presence of binucleated and multinucleated cells. An immunohistochemical profile was performed including vimentin, S100 protein, glial fibrillary acidic protein (GFAP), CD57, desmin, smooth muscle actin, and pancytokeratin AE1 and AE3. The panel produced the following results: focally positive for vimentin (Figure 3A), S100 protein (Figure 3B), GFAP (Figure 3C), CD57 (Figure 3D), desmin (Figure 3E), and smooth muscle actin (SMA) (Figure 3F), and negative for AE1 and AE3.

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Figure 1. A. Clinical aspect reveled a nodular lesion covered with normal epithelium situated in buccal mucosa. B. The macroscopic appearance of the specimen was grayish and white, had an irregular surface, and measured 1.0 centimeter in diameter.

Figure 2. A. Low-power photomicrograph showing no well-defined continuous capsule. Matrix composed of myxoid and chondroid zones associated with tumor cells can be seen (hematoxylin and eosin stain, x40 original magnification). B. High-power photomicrograph demonstrating ovoid, polygonal, and spindle cells related to chondroid areas (hematoxylin and eosin stain, x200 original magnification). C. Focal area of chondroid tissue exhibiting cellular atypia with nuclear hyperchromatism (hematoxylin and eosin stain, x400 original magnification).

Figure 3. A. Tumor cells showing focal positive in cytoplasm for vimentin in chondroid and connective tissue area (streptavidin-biotin complex [sABC] method, x100 original magnification). B. S100 protein positive in cytoplasm distributed in some chondroid and connective cells (sABC method, x100 original magnification). C. Focally positive cytoplasm reaction for glial fibrillary acidic protein in chondroid and connective tissue area (sABC method, x200 original magnification). D. Cytoplasm tumor cells were positive for CD57 in chondroid and connective tissue area (sABC method, x100 original magnification). E. Focal positive cytoplasm cells for desmin in chondroid and connective tissue area (sABC method, x200 original magnification). F. Cytoplasm tumor spindle cells were focally positive for smooth muscle actin in connective tissue area (sABC method, x200 original magnification).

CAN YOU MAKE THE DIAGNOSIS? A. extraskeletal chondroma C. soft-tissue chondromyxoid ﬁbroma B. pleomorphic adenoma D. ectomesenchymal chondromyxoid tumor

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THE DIAGNOSIS

D. ectomesenchymal chondromyxoid tumor

Ectomesenchymal chondromyxoid tumor (ECT) is an chondroma as a cartilaginous choristoma owing to the uncommon, benign, mesenchymal, soft-tissue neoplasm presence of a tumorlike mass composed of “normal 1 15 of the oral mucosa. ECT was first described in 1995, with tissues” in an “abnormal location.” The histologic 52 cases reported in the English-language literature: 50 analysis reveals a well-circumscribed, lobulated mass cases were in the tongue; 1 was in the hard palate; and 1 composed of mature hyaline cartilage surrounded by 1 8 was in the buccal mucosa. - The cases described in the condensed collagenous tissue. Some cellular atypia oc- 16 literature reported that ECT most commonly affects casionally is observed. In the immunohistochemical adults in the third to sixth decades of life. Clinically, ECT analysis, the cells are positive for S100 protein and presents as a slow-growing, painless, firm, well- vimentin as well as negative for pancytokeratin (AE1 circumscribed, submucosal nodule, covered by normal and AE3), myoepithelial markers, and GFAP. The mucosa, that appears to involve only the oral cavity and treatment of choice is local excision, and the recurrence 17 usually is located on the anterior dorsum of the rate is 10%to15%. 2 9 10 tongue. , , Such tumors range in size from 0.3 to 2.0 Pleomorphic adenoma. Pleomorphic adenoma is the 9 10 cm. , The diagnosis of ECT is grounded on clinical most common salivary gland tumor, accounting for 18 characteristics, light microscopy, and approximately 60% of all salivary neoplasms. The 10 immunohistochemistry. mean age of presentation is 40 years, but the age of The microscopic features (low-power microscopic patients with this condition can range from the first to 19 analysis) reveal lobular sheets of cells surrounded by the ninth decades of life. Approximately 80%of 20 skeletal muscle fascicles and connective tissue. The pleomorphic adenomas arise in the parotid gland. lesion is not encapsulated, but loose connective tissue Clinically, these lesions are usually painless, slow- 21 may be found at the interface between the tumor and growing tumors with a hard consistency, measuring 2 20 normal tissue. Three different microscopic patterns are to 5 cm in diameter. There is a proliferation of generally seen: cellular, myxoid, and chondroid matrix. epithelial and myoepithelial cells consisting of glandlike Cellular areas are characterized by the proliferation of structures, ducts, cell nests, cords, spindle-shaped, and 9 oval, polygonal, and spindle cells. ECT may exhibit plasmacytoid cells, intermingled with a mesenchymal or focal areas of cellular atypia, including pleomorphism chondromyxoid component with frequent metaplastic 2 and mitotic figures. The immunopathologic character- changes. The arrangement of these tissues varies from istics indicate a tumor of mesenchymal, neurogenic predominantly cellular to predominantly myxochon- 11 22 origin with the expression of vimentin, S100 protein, droid. The tumor cells are positive for pancytokeratin and GFAP. ECT presents variable immunoreactivity for and are variably positive for S100 protein, a-SMA, CD57, desmin, and SMA, as we observed in the case GFAP, calponin, CD10, and muscle-specific actin. Cells 9 described in this article. Ki-67 protein labeling is low, in the chondroid areas are positive for both vimentin 2 20 indicating the benign nature of the tumor. The pres- and pancytokeratin. Treatment is complete surgical ence of biphasic myxoid and chondroid patterns and the excision. The recurrence rate as a carcinoma ex pleo- 23 positivity of tumor cells for vimentin, S100 protein, and morphic adenoma is nearly 3%. GFAP are important findings for making the diagnosis Soft-tissue chondromyxoid fibroma. Soft-tissue 12 of ECT. chondromyxoid fibroma is a rare, benign, cartilaginous The treatment for this tumor is conservative surgical tumor that mostly arises from the metaphysis of long 9 24 excision. The recurrence rate of ECT is 7%. In the bones and rarely is seen in the head and neck region. follow-up of the case described in this article, the patient Investigators have reported the extension of the tumor 25 remained free of disease after 12 months, but the to soft tissue. However, to the best of our knowledge, intraoral affected area exhibited paresthesia. investigators have described only 2 cases of a primary 25 26 tumor with no bone involvement. , In the histologic DIFFERENTIAL DIAGNOSIS analysis, soft-tissue chondromyxoid fibroma exhibits a Extraskeletal chondroma. Extraskeletal chondroma is a lobular pattern with stellate or spindle-shaped cells on a benign, soft-tissue, cartilaginous tumor usually located myxoid or chondroid background. The presence of 13 in the hands and feet, but it occasionally occurs in the mitosis is uncommon. Giant cells often are found at the head and neck region, in which the most reported sites periphery of the tumor. Immunohistochemically, the are the paranasal sinuses, nasal cavity, larynx, and cells are positive for S100 protein, and the periphery of 14 tongue. Investigators have described extraskeletal the chondroid area demonstrates positive staining for

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SMA, muscle-specific actin (known as HHF-35), and 2. Palma Guzmán JM, de Andrade BA, Rizo VH, Romanach MJ, Leon JE, 27 CD34. The prognosis of soft-tissue chondromyxoid de Almeida OP. Ectomesenchymal chondromyxoid tumor: histopathologic fi and immunohistochemical study of two cases without a chondroid broma has not been reported in the literature, likely component. J Cutan Pathol. 2012;39(8):781-786. owing to its rarity. 3. Nigam S, Dhingra KK, Gulati A. Ectomesenchymal chondromyxoid In addition to the neoplasms described previously, tumor of the hard palate: a case report. JOralPatholMed. 2006;35(2):126-128. 4. Yoshioka Y, Ogawa I, Tsunematsu T, et al. Ectomesenchymal chon- other important differential diagnoses for ECT include dromyxoid tumor of the tongue: insights on histogenesis. Oral Surg Oral myoepithelioma, myxoid neurofibroma, and myxoid Med Oral Pathol Oral Radiol. 2013;115(2):233-240. 10 chondrosarcoma. Myoepithelioma and ECT have 5. Closmann JJ, Eliot CA, Foss RD. Ectomesenchymal chondromyxoid tumor: report of a case with description of histologic and immunohisto- some similar characteristics, but the clinical location and chemical findings. J Oral Maxillofac Surg. 2013;71(3):545-549. absence of salivary glands adjacent to ECT are impor- 6 10 . Pak MG, Kim KB, Shin N, et al. Ectomesenchymal chondromyxoid tant features that differentiate these 2 lesions. tumor in the anterior tongue: case report of a unique tumor. Korean J Furthermore, myoepithelioma is usually positive for a- Pathol. 2012;46(2):192-196. 7. Cardin MJ, Fiset PO, Zeitouni AG, Caglar D. Ectomesenchymal chon- SMA and does not present condromyxoid-exuberant dromyxoid tumour ofthe posterior tongue.HeadNeckPathol. 2014;8(3):329-333. 1,9 areas. Myxoid neurofibroma is a tumor with cells that 8. Tsai SY, Chang KC, Tsai HW, Jin YT. Ectomesenchymal chon- contain wavy nuclei. Atypia or chondroid areas are not dromyxoid tumor of tongue. Indian J Pathol Microbiol. 2012;55(4):519-520. 9. Allen CM. The ectomesenchymal chondromyxoid tumor: a review. found in myxoid neurofibromas; nevertheless, both le- 2008 14 5 390 395 1,10 Oral Dis. ; ( ): - . sions express vimentin, S100 protein, and GFAP. 10. Angiero F. Ectomesenchymal chondromyxoid tumour of the tongue: Extraskeletal myxoid chondrosarcoma typically exhibits a review of histological and immunohistochemical features. Anticancer Res. 2010 30 11 4685 4690 more areas of cellular atypia and more aggressive clin- ; ( ): - . 10 11. de Visscher JG, Kibbelaar RE, van der Waal I. Ectomesenchymal ical behavior than ECT. chondromyxoid tumor of the anterior tongue: report of two cases. Oral Oncol. 2003;39(1):83-86. 12. Gouvêa AF, Díaz KP, Léon JE, Vargas PA, de Almeida OP, CONCLUSIONS Lopes MA. Nodular lesion in the anterior hard palate. Oral Surg Oral Med In this article, we described the case of a patient with a Oral Pathol Oral Radiol. 2012;114(2):154-159. 13. De Riu G, Meloni SM, Gobbi R, Contini M, Tullio A. Soft-tissue chon- nodular lesion on the left buccal mucosa who had a droma of the masticatory space. Int J Oral Maxillofac Surg. 2007;36(2):174-176. histopathologic diagnosis of ECT. A dental clinician can 14. Falleti J, De Cecio R, Mentone A, et al. Extraskeletal chondroma of make the diagnosis of this type of rare oral neoplasm the masseter muscle: a case report with review of the literature. Int J Oral only through the combined analysis of clinical, histo- Maxillofac Surg. 2009;38(8):895-899. 15. Norris O, Mehra P. Chondroma (cartilaginous choristoma) of the pathologic, and immunohistochemical characteristics. It tongue: report of a case. J Oral Maxillofac Surg. 2012;70(3):643-646. is important to perform an accurate diagnosis of this 16. Kim Y, Moses M, Zegarelli DJ, Yoon AJ. Cartilage choristoma (soft type of lesion, because it may be confused with other tissue condroma): a rare presentation of the lower lip. J Clin Pediatr Dent. 2009;33(3):253-254. lesions that have similar clinical features but a different 17. Han JY, Han HS, Kim YB, Kim JM, Chu YC. Extraskeletal chon- prognosis. n droma of the fallopian tube. J Korean Med Sci. 2002;17(2):276-278. http://dx.doi.org/10.1016/j.adaj.2014.11.020 18. Fonseca FP, Carvalho Mde V, de Almeida OP, et al. Clinicopathologic analysis of 493 cases of salivary gland tumors in a southern Brazilian pop- Copyright ª 2015 American Dental Association. All rights reserved. ulation. Oral Surg Oral Med Oral Pathol Oral Radiol. 2012;114(2):230-239. 19. de Oliveira FA, Duarte EC, Taveira CT, et al. Salivary gland tumor: a re- Dr. Jalfim Maraschin is a doctoral student in oral pathology, School of view of 599 cases in a Brazilian population. Head Neck Pathol. 2009;3(4):271-275. Dentistry, Federal University of Rio Grande do Sul, Porto Alegre, Brazil. 20. Barnes L, Eveson JW, Reichart P, Sidransky D, eds. World Health Dr. Amorim Pellicioli is a master’s degree student in oral pathology, Organization Classification of Tumours: Pathology and Genetics of Head School of Dentistry, Federal University Rio Grande do Sul, Porto Alegre, and Neck Tumours. Lyon, France: International Agency for Research on Brazil. Cancer (IARC) Press; 2005. Dr. Batista de Souza is a professor of oral pathology, Department of 21. Thangaswamy V, Sivakumar A, Sivakumar A, Sivaraj, Pugazhendi SK, Dentistry, Federal University of Rio Grande do Norte, Natal, Brazil. Thambiah L. Pleomorphic adenoma in an adolescent. J Pharm Bioallied Dr. Varvaki Rados is a professor of oral pathology, School of Dentistry, Sci. 2012;4(suppl 2):S435-S436. Federal University of Rio Grande do Sul, Porto Alegre, Brazil. 22. Wenig BM, Hitchcock CL, Ellis GL, Gnepp DR. Metastasizing mixed Dr. Trevizani Martins is a professor of oral medicine, School of Dentistry, tumor of salivary glands: a clinicopathologic and flow cytometric analysis. Federal University of Rio Grande do Sul, Porto Alegre, Brazil. Am J Surg Pathol. 1992;16(9):845-858. Dr. Domingues Martins is a professor of oral pathology, School of 23. Soames JV, Southam JC. Hyperplastic, neoplastic, and related dis- Dentistry, Federal University of Rio Grande do Sul, Rua Ramiro Barcelos, orders of oral mucosa. In: Soames JV, Southam JC, eds. Oral Pathology. 2492/503, CEP 90035-003, Porto Alegre, Rio Grande do Sul, Brazil, e-mail 4th ed. New Delhi: Oxford University Press; 2005:101-115. [email protected]. Address correspondence to Dr. Domingues 24. McClurg SW, Leon M, Teknos TN, Iwenofu OH. Chondromyxoid Martins. fibroma of the nasal septum: case report and review of literature. Head Neck. 2013;35(1):E1-E5. Disclosures. None of the authors reported any disclosures. 25. Kim HR, Lee SM, Ha DH, Kang H, Rho JY. Soft tissue chondromyxoid fibroma of the foot: sonographic findings. J Clin Ultrasound. The authors thank Artur Cunha Vasconcelos, PhD, for his important 2012;40(2):109-111. contribution with the immunohistochemical reactions described in this 26. Park JM, Woo YK, Kang MI, Kang CS, Hahn ST. Oncogenic osteomalacia article. associated with soft tissue chondromyxoid fibroma. Eur J Radiol. 2001;39(2):69-72. 27. Fletcher CD, Unni KK, Mertens F, eds. World Health Organization 1. Smith BC, Ellis GL, Meis-Kindblom JM, Williams SB. Ectomesen- Classification of Tumours: Pathology and Genetics of Tumours of Soft chymal chondromyxoid tumor of the anterior tongue: nineteen cases of a Tissue and Bone. Lyon, France: International Agency for Research on new clinicopathologic entity. Am J Surg Pathol. 1995;19(5):519-530. Cancer (IARC) Press; 2002.

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