2016 Volume 145 Pages 96-97
Papillary cystadenocarcinoma is a salivary gland tumor that was classified as a subtype of adenocarcinoma by the World Health Organization (WHO) in 1991. It is histologically characterized by papillary growth with a cyst-like structure. The incidence is extremely low, with this tumor accounting for less than 0.2% of all salivary gland tumors. We report our experience of two patients with papillary cystadenocarcinoma, the tumor originating from a small salivary gland in the first case and from the sublingual gland in the second, with a review of the literature.
Patient 1 was a 73-year-old woman who presented with a cyst-like tumor at the base of the tongue. Biopsy led to the diagnosis of papillary cystadenocarcinoma, and tumor resection was performed by the suprahyoid approach under general anesthesia. Histopathology of the resected specimen revealed the diagnosis of papillary cystadenocarcinoma of low-grade malignancy originating from a small salivary gland. Until now, 4 years since treatment by surgery, the patient has shown no evidence of relapse. Patient 2 was a 67-year-old woman who was diagnosed as having papillary cystadenocarcinoma and underwent tumorectomy by the suprahyoid approach and manipulation from the oral cavity. The pathological diagnosis was papillary cystadenocarcinoma of high-grade malignancy originating from the sublingual gland. Because the resection line allowed for only a close margin, postoperative radiation therapy (60 Gy/30 Fr) was administered. Thereafter, the patient developed repeated recurrences, and despite salvage surgery, she died 4 years and 3 months after the initial surgery.
Although papillary cystadenocarcinoma shows slow growth and benign tumor-like features, in some cases, the tumor is classified as being of high-grade malignancy relapses occur, therefore, treatment should be selected with due consideration given to the possibility of relapse and metastasis.
