Mucoepidermoid Carcinoma Arising in a Background of Sialadenoma Papilliferum: A Case Report
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Sialadenoma papilliferum is a rare tumor, primarily of minor salivary gland origin, first described by Abrams and Finck (Cancer 24:1057–63, 1969). It is both an exophytic and endophytic papillary lesion histologically resembling syringocystadenoma papilliferum of sweat gland. The tumor is considered benign although rare recurrent cases have been reported. Three cases of malignant transformation of sialadenoma papilliferum have been described in the literature. We report a high grade mucoepidermoid carcinoma arising in a background of sialadenoma papilliferum, at the base of the tongue, an unusual location for minor salivary gland neoplasms. Eleven months after excision and nodal dissection, there is no evidence of recurrence or metastasis.
KeywordsSialadenoma papilliferum Mucoepidermoid carcinoma Salivary gland
Sialadenoma papilliferum is a rare tumor of minor salivary gland origin, first described by Abrams and Finck in 1969 . It is both an exophytic and endophytic papillary lesion histologically resembling syringocystadenoma papilliferum of sweat gland. The tumor is considered benign although rare recurrent cases have been reported [2, 3]. Three cases of malignant transformation of sialadenoma papilliferum have been described in the literature [4, 5, 6]. We report a fourth case, a high grade mucoepidermoid carcinoma arising in a background of sialadenoma papilliferum, at the base of the tongue, an unusual location for minor salivary gland neoplasms.
An 82-year-old female with medical history of diabetes mellitus and hypertension presented with a 3-month history of dysphagia secondary to a 2-cm papillary, exophytic mass at the left base of the tongue. This tumor ultimately was interpreted as a high grade mucoepidermoid carcinoma arising in a background of a sialadenoma papilliferum. The patient subsequently underwent tumor excision and follow-up margin excision with level II, III and IV left neck lymph node dissection. No residual tumor was identified and four lymph nodes were negative for tumor. All specimens were negative for microvascular invasion. The patient was discharged. She was regularly followed-up and no recurrence of the tumor was documented eleven months after the surgery.
Sialadenoma papilliferum usually occurs in older males in sites that have minor salivary glands, such as palate, buccal mucosa, retromolar pads and lips . The base of tongue location, as in our case, has not been documented previously, although minor salivary glands are found in this location. The proliferative, dilated salivary gland ducts with papillary fronds protruding into the lumen, together with the hyperplastic, hyperkeratotic overlying squamous epithelium, led us to the observation that there is a component of sialadenoma papilliferum, continuous with a deeper high grade mucoepidermoid carcinoma.
The true origin and nature of sialadenoma papilliferum have been controversial. The authors who first described this lesion believed it arises from myoepithelial cells ; while others have proposed that it is a result of focal hyperplasia following blockage of a salivary gland duct . The presence of ciliated cells in the lining epithelium of the duct in our case is consistent with the possibly metaplastic, reactive nature of sialadenoma papilliferum . Most recently, it was reported that sialadenoma papilliferum is a neoplasm that originates from ductal surface cells .
Mucoepidermoid carcinoma sometimes is seen proliferating from a terminal salivary duct, supporting the concept that the tumor may originate from cells of the salivary gland excretory and intercalated ducts . Although mucoepidermoid carcinoma can be associated with a large excretory salivary duct, a papillary, proliferating ductal lesion, as in this case, is not a feature. Chen  reported a case of intraductal low grade mucoepidermoid carcinoma occurring in minor salivary gland. The lesion was lobulated and composed of cystic ducts filled with tumor, which differs from the surface papillary configuration of a sialadenoma papilliferum.
Considering the papillary architecture of the lesion, our differential diagnosis consists of (1) a sialadenoma papilliferum with a papillary squamous cell carcinoma, (2) an adenosquamous carcinoma with prominent papillary features, (3) papillary squamous cell carcinoma, and (4) a papillary cystadenocarcinoma. Papillary squamous cell carcinoma is a distinct variant of squamous cell carcinoma characterized by an exophytic, papillary growth, which can resemble sialadenoma papilliferum in its gross appearance . However, a glandular component with mucin-secreting cells is not present in this lesion. Adenosquamous carcinoma has distinctively separate components of adeno- and squamous differentiation with no papillary configuration . Cystadenocarcinoma is a rare malignant tumor characterized by predominantly cystic growth and conceptually it is the malignant counterpart of the benign cystadenoma. It does not include squamous elements . Distinctively squamous differentiation is not a feature of papillary cystadenocarcinoma. Cell types that comprise the lining epithelium of cysts in papillary cystadenocarcinoma include, most often, cuboidal and columnar cells. Other cells types such as mucous, clear and oncocytic cells occasionally are noted as well .
Four reported cases of malignant transformation of sialadenoma papilliferum (SP) (1978–2009)
Solomon et al. 
Squamous cell carcinoma
Shimoda et al. 
Junction between the soft and hard palate
Ponniah et al. 
Floor of mouth
Carcinoma in situ in the squamous component of benign SP
Liu et al. (current case)
Base of tongue
High-grade mucoepidermoid carcinoma
Our case demonstrates a mucoepidermoid carcinoma, apparently arising from a sialadenoma papilliferum. The superficial benign portion of the lesion retains the configuration of sialadenoma papilliferum. In comparison to the malignant transformation of a sialadenoma papilliferum reported by Shimoda et al. , in which the carcinoma component and the benign lesion are in close contact and share a transitional zone, the mucoepidermoid carcinoma in this case comprises the deeper portion of sialadenoma papilliferum while leaving the exophytic, papillary, surface structure intact. This case represents a rare example of malignant transformation of sialadenoma papilliferum, occurring in an unusual location for minor salivary gland tumors.
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