Juvenile pleomorphic adenoma of the cheek: a case report and review of literature
© Dalati and Hussein; licensee BioMed Central Ltd. 2009
Received: 15 May 2009
Accepted: 22 September 2009
Published: 22 September 2009
Pleomorphic adenoma, also called benign mixed tumor, is the most common tumor of the salivary glands. About 90% of these tumors occur in the parotid gland and 10% in the minor salivary glands. The most common sites of pleomorphic adenoma of the minor salivary glands are the palates followed by lips and cheeks. Other rare sites include the throat, floor of the mouth, tongue, tonsil, pharynx, retromolar area and nasal cavity. In children, intraoral pleomorphic adenomas of the cheek are extremely rare with only three cases reported to date. Here we report a case of pleomorphic adenoma of minor salivary glands of the cheek in a 17-year-old girl. The mass was removed by wide local excision with adequate margins, and after a follow-up period of three years there were no recurrences. To conclude, pleomorphic adenoma should be considered in the differential diagnosis of cheek masses in youngsters. Wide local excision is to be recommended as the treatment of choice. A close follow-up is necessary postoperatively.
Pleomorphic adenomas are benign salivary gland tumors that represent about 3- 10% of the neoplasm of the head and neck region . They are the most common tumors (50%) of the major and minor salivary glands . The palate is considered as the most common intraoral site (42.8-68.8%), followed by the upper lip (10.1%) and cheek (5.5%) [3–5]. Other rare sites include the throat (2.5%), retromolar region (0.7%), floor of the mouth and the alveolar mucosa . Pleomorphic adenoma usually presents as a mobile slowly growing, painless firm swelling that does not cause ulceration of the overlying mucosa .
Pleomorphic adenoma consists of cells with epithelial and mesenchymal differentiation (mixed tumor). The highly variable morphology of this neoplasm is the result of interplay between these elements. Now it is widely accepted that both epithelial and mesenchymal (myxoid, hyaline, chondroid, osseous) elements often arise from same cell clone, which may be a myoepithelial or ductal reserve cell. There is no difference in behavior of this neoplasm based on proportion of various elements . Lee et al examined formalin-fixed, paraffin-embedded tissues from 13 pleomorphic adenomas of female patients. They used the polymerase chain reaction (HUMRA assay). HUMARA, the human androgen receptor gene, is located on the X chromosome and contains a segment of polymorphic CAG tandem repeats in exon 1. Several methylation-sensitive HhaI restriction sites are located 5' to these CAG repeats. It is an ideal tool to study clonality of female tissues by examining the methylation pattern. A monoclonal pattern was seen in the stromal, epithelial elements in the majority of cases. These findings suggest that the stromal and epithelial cells in pleomorphic adenomas of salivary gland arise from the same clone in most cases . Variants of pleomorphic adenoma include pleomorphic adenoma with lipomatous change , myxoliopmatous pleomorphic adenoma, pleomorphic adenoma with squamous differentiation and benign metastasizing mixed tumor .
The mucosa of the cheek is a uncommon site of occurrence for intraoral pleomorphic adenoma  and most of these cases have been reported in adults [11, 12]. In children, only three cases were reported to date [13–15]. Here we report a case of pleomorphic adenoma in 17 years old girl. The relevant studies were discussed.
Pleomorphic adenoma occurs more frequently in women than in men and is most common from the fourth to sixth decades with a mean age of 43-46 years [3, 4, 16]. Salivary gland tumors are rare in children and when they do arise, they preferentially affect major salivary glands, but minor salivary gland tumors have also been reported . Here we report a rare case of juvenile pleomorphic adenoma of the cheek in 17 years old girl. To the best of our knowledge, this is the fourth case to be reported in literature [13–15].
The clinicopathologic features of the case reported here concurs with previous studies. Yamamoto et el reported a 9-year-old Japanese girl with pleomorphic adenoma of the cheek mucosa. Cohen and Kronenberg reported two more cases of juvenile pleomorphic adenomas of the cheek (girls, age <18 years) [13–15]. No recurrence was reported in these cases [13–15]. Dhanuthai et al reported the first case of palatal pleomorphic adenoma in a 13 year-old child . In children, Jorge et al reported five cases of intraoral pleomorphic adenoma in patients under 18 years of age in two Brazilian institutions. Four patients were females and one was male; two cases affected the palate, two the upper lip and one the tongue. The cases were treated by local excision and long-follow up showed no recurrences were observed. Jorge et al concluded that intraoral pleomorphic adenoma seems to have similar biological characteristics as in adults, with low recurrence rates after surgical resection . The surgical treatment for the pleomorphic adenoma in both juvenile and adult patients is principally the same and includes complete wide surgical excision with good safety margins. Inadequate resection or rupture of the capsule or tumors spillage during excision can lead to local recurrence as these tumors often have microscopic interruptions in the capsule . In adults, Van Heerden and his colleagues examined the clinicopathologic features of the oral salivary gland neoplasms. Seventy cases were diagnosed during 8-year period. Pleomorphic adenoma was the most common entity that accounted for 48% of all tumors (not in the cheek), but none of these lesions affected the cheek mucosa. Polymorphous low-grade adenocarcinoma comprised 15.7% of the tumors .
Differential diagnosis of juvenile pleomorphic adenoma of the cheek
Salient histological features
Carcinoma ex pleomorphic adenoma
Adenoid cystic carcinoma
- Cribriform and pseudoglandular patterns of basaloid cells with hyalinized stroma
- Frequent perineural invasion
- Minimal nuclear pleomorphism
- No squamous differentiation
- No high grade dysplasia or squamous carcinoma in situ, no extensive necrosis
Polymorphous low grade adenocarcinoma
- Uniform plump columnar cells with bland nuclei arranged in variable growth patterns (tubular, cribriform, papillary, solid, fascicular, microcystic, single file, pseudoadenoid cystic, strand-like, mixed)
- Perineural invasion common around small nerves
- Infiltrative borders
Basal cell adenoma
- Elongated and branching canaliculi separated by hyaline stroma
Acinic cell carcinoma
- Variable cell types (serous, clear, vacuolated, intercalated duct cells) arranged in solid, microcystic, papillary cystic and follicular patterns
- few mitotic figures
Clear cell carcinoma-hyalinizing type
-Trabeculae, cords, islands or nests of monomorphic clear cells surrounded by hyalinized bands with foci of myxohyaline stroma
- Invasive, cystic growth pattern, 75% had
- Conspicuous papillary component
- Composed of small cuboidal cells, large cuboidal cells, tall columnar cells or mixture
- Cystic spaces with hemorrhage and granulation tissue are frequent
- intraductal neoplasm with micropapillary, cribriform, solid, comedo or clinging patterns, with preservation of myoepithelial cells surrounding intraductal tumor
- Cords, sheets, clusters of mucous, squamous, intermediate and clear cells; low to high grade
Epithelial myoepithelial carcinoma
- Both epithelial and myoepithelial components seen
- Most tumor cells have myoepithelial features with clear cytoplasm or naked nuclei
- Focally, there are ducts or tubules with an outer rim of myoepithelial cells and inner, dark ductal cells with scant eosinophilic cytoplasm and round, bland nuclei
To conclude, juvenile pleomorphic adenoma of the cheek is a rare neoplasm and therefore its diagnosis requires a high index of suspicion. Complete wide surgical excision is the treatment of choice. Recurrence after many years of surgical excision as well as malignant transformation should be a concern and therefore long- term follow- up is necessary.
Written informed consent was obtained from the patient for publication of this case report and accompanying images. A copy of the written consent is available for review by the Editor-in-Chief of this journal.
- Garcia Berrocal JR, Ramirez Camacho R, Trinidad A, Salas C: Mixed tumor (pleomorphic adenoma) of head and neck. Typical and atypical patterns. An Otorrinolaringol Ibero Am. 2000, 27: 333-40.PubMed
- Traiger J, Rosen MB: Mixed Tumor of the Cheek; Report of a Case. Oral Surg Oral Med OralPathol. 1965, 19: 711-4. 10.1016/0030-4220(65)90339-7.View Article
- van Heerden WF, Raubenheimer EJ: Intraoral salivary gland neoplasms: a retrospective study of seventy cases in an African population. Oral Surg Oral Med Oral Pathol. 1991, 71: 579-82. 10.1016/0030-4220(91)90366-K.View ArticlePubMed
- Wang D, Li Y, He H, Liu L, Wu L, He Z: Intraoral minor salivary gland tumors in a Chinese population: a retrospective study on 737 cases. Oral Surg Oral Med Oral Pathol Oral Radiol Endod. 2007, 104: 94-100. 10.1016/j.tripleo.2006.07.012.View ArticlePubMed
- Toida M, Shimokawa K, Makita H, Kato K, Kobayashi A, Kusunoki Y, Hatakeyama D, Fujitsuka H, Yamashita T, Shibata T: Intraoral minor salivary gland tumors: a clinicopathological study of 82 cases. Int J Oral Maxillofac Surg. 2005, 34: 528-32. 10.1016/j.ijom.2004.10.010.View ArticlePubMed
- Kaminski M, Janicki K: A case of giant pleomorphic adenoma of the cheek with two malignant centers. Otolaryngol Pol. 2002, 56: 385-7.PubMed
- Lee PS, Sabbath-Solitare M, Redondo TC, Ongcapin EH: Molecular evidence that the stromal and epithelial cells in pleomorphic adenomas of salivary gland arise from the same origin: clonal analysis using human androgen receptor gene (HUMARA) assay. Hum Pathol. 2000, 31: 498-503. 10.1053/hp.2000.6716.View ArticlePubMed
- Kondo T: A case of lipomatous pleomorphic adenoma in the parotid gland. Diagn Pathol. 2009, 4: 16-10.1186/1746-1596-4-16.PubMed CentralView ArticlePubMed
- Ide F, Kusama K: Myxolipomatous pleomorphic adenoma: an unusual oral presentation. J Oral Pathol Med. 2004, 33: 53-5. 10.1111/j.1600-0714.2004.00110.x.View ArticlePubMed
- Bablani D, Bansal S, Shetty SJ, Desai R, Kulkarni SR, Prasad P, Karjodkar FR: Pleomorphic adenoma of the cheek: a case report and review. J Oral Maxillofac Surg. 2009, 67: 1539-42. 10.1016/j.joms.2008.07.026.View ArticlePubMed
- Vyas KC, Mathur SP: Pleomorphic salivary adenoma of cheek. A case report J Laryngol Otol. 1984, 98: 421-3.View ArticlePubMed
- Houston GD: Oral pathology. Pleomorphic adenoma. J Okla Dent Assoc. 2003, 94: 31-3.PubMed
- Yamamoto H, Fukumoto M, Yamaguchi F, Sakata K, Oikawa T: Pleomorphic adenoma of the buccal gland in a child. Int J Oral Maxillofac Surg. 1986, 15: 474-7.View ArticlePubMed
- Cohen MA: Pleomorphic adenoma of the cheek. Int J Oral Maxillofac Surg. 1986, 15: 777-9.View ArticlePubMed
- Kronenberg J, Horowitz A, Creter D: Pleomorphic adenoma arising in accessory salivary tissue with constriction of Stensen's duct. J Laryngol Otol. 1988, 102: 382-3. 10.1017/S0022215100105055.View ArticlePubMed
- Jorge J, Pires FR, Alves FA, Perez DE, Kowalski LP, Lopes MA, Almeila OP: Juvenile intraoral pleomorphic adenoma: report of five cases and review of the literature. Int J Oral Maxillofac Surg. 2002, 31: 273-5. 10.1054/ijom.2002.0206.View ArticlePubMed
- Dhanuthai K, Sappayatosok K, Kongin K: Pleomorphic adenoma of the palate in a child: a case report. Med Oral Patol Oral Cir Bucal. 2009, 14: E73-5.PubMed
- Baldus SE, Streppel M, Stennert E, Dienes HP: Pleomorphic adenoma (mixed tumor) of the external auditory canal. Differential diagnosis of the tumors of the ceruminal glands. Pathologe. 1999, 20: 125-9. 10.1007/s002920050331.View ArticlePubMed
- Cerulli G, Renzi G, Perugini M, Becelli R: Differential diagnosis between adenoid cystic carcinoma and pleomorphic adenoma of the minor salivary glands of palate. J Craniofac Surg. 2004, 15: 1056-60. 10.1097/00001665-200411000-00036.View ArticlePubMed
- Harada H: Histomorphological investigation regarding to malignant transformation of pleomorphic adenoma (so-called malignant mixed tumor) of the salivary gland origin: special reference to carcinosarcoma. Kurume Med J. 2000, 47: 307-23.View ArticlePubMed
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