Papillary cystadenocarcinoma of the parotid gland: a rare sub-variant of salivary gland adenocarcinoma

Papillary cystadenocarcinoma of the salivary gland is a very rare malignant neoplasm accounting for only 2% of all salivary gland lesions. In 1991 it was first included as a separate entity in the World Health Organization (WHO) classification of salivary gland tumors and in 2017 WHO Classification, the tumor was clubbed as a sub-variant of adenocarcinoma, not otherwise specified. It most commonly occurs in the major salivary glands. Herein we report a case of salivary papillary cystadenocarcinoma in a 54-year-old female, who presented with rapid enlargement of the right parotid swelling. Based on radiology and fine-needle aspiration cytology, a working diagnosis of the malignant tumor involving the superficial lobe of the right parotid gland was made. In view of the malignant nature of the swelling, superficial parotidectomy was done. The histopathology and immunohistochemistry of the mass confirmed the diagnosis of papillary cystadenocarcinoma of the right parotid. With the revised 2017 WHO classification of salivary gland tumors, it is important to report all rare subtypes in order to understand their biology and behavior.

Cytomorphological findings and histological correlation of papillary cystadenocarcinoma of the parotid: Not always a low‑grade tumor.

Papillary cystadenocarcinoma (PCAC) is a rare salivary gland tumor characterized by a predominantly cystic growth that often exhibits intraluminal papillary growth without specific histologic features of other cystic salivary gland tumors. The preoperative cytological diagnosis can pose a diagnostic challenge as it has to be differentiated from other cystic papillary tumors such as mucoepidermoid carcinoma, papillary cystic variant of acinic cell carcinoma, and low‑grade cribriform CAC. It is considered to be a low‑grade malignant salivary gland tumor with an indolent biological behavior. We report a case of PCAC of the parotid in a 55‑year‑old male diagnosed on fine needle aspiration cytology. Although it showed mild atypia cytologically, on excision tumor showed vascular and perineural invasion with regional node metastasis indicating a wider morphologic spectrum than what is described. This prompted us to write a case report describing the cytological and histological features of this rare tumor and also discuss the diagnostic challenges.

A Case of Papillary Cystadenocarcinoma Arising from Submandibular Gland / 대한이비인후과학회지

Papillary cystadenocarcinoma originated from salivary gland is a lower grade neoplasm that rises most commonly in major salivary glands, although involvement of lip, palate, buccal mucosa and tongue has also been described. Papillary cystadenocarcinoma was first defined in 1991 by WHO. Until then, it was classified as an atypical type of adenocarcinoma. We experienced a case of papillary cystadenocarcinoma of the submandibular gland, so we report this case with a review of the literature.

Papillary Cystadenocarcinoma Arising from Minor Salivary Glands in the Retromolar Trigone / 대한이비인후과학회지

Papillary cystadenocarcinoma is a very rare malignant neoplasm of the salivary gland. We report a case of papillary cystadenocarcinoma arising from the minor salivary gland in the retromolar trigone of a 56-year-old female patient presenting intermittent hemorrhage from the oral cavity. We discuss the clinical and histopathological features of the papillary cystadenocarcinoma of the salivary gland with the review of the literature.