Autopsy and Case Reports
Autopsy and Case Reports
Clinical Case Report and Review

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

Satya Dutta; Biswajit Dey; Jaya Mishra; Vandana Raphael; Jonali Das; Donboklang Lynser

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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.


Papillary cystadenocarcinoma, Salivary gland neoplasm, Parotid


1 Seifert G, Sobin LH. Histological classification of salivary gland tumours. 2nd ed. Berlin: Springer-Verlag; 1991.

2 Auclair PL. Cystadenocarcinoma. In: Barnes L, Eveson JW, Reichart P, Sidransky D, editors. Pathology and genetics of head and neck tumours. Lyon: IARC; 2005. (World Health Organization Classification of Tumours; pp. 232-33).

3 El-Naggar AK, Chan JKC, Grandis JR, Takata T, Grandis J, Slootweg P. WHO classification of head and neck tumours. 4th ed. Lyon: IARC; 2017.

4 Ellis GL, Auclair PL. Tumour of the salivary glands. Washington, DC: Armed Forces Institute of Pathology; 2008. (Atlas of tumour pathology series; 4, pp. 281-9).

5 Shrestha P, Namba M, Yang L, Liu B, Oosumi H, Mori M. Papillary cystadenocarcinoma of salivary-glands - an immunohistochemical study. Int J Oncol. 1994;4(3):587-97. PMid:21566964.

6 Foss RD, Ellis GL, Auclair PL. Salivary gland cystadenocarcinomas. A clinicopathologic study of 57 cases. Am J Surg Pathol. 1996;20(12):1440-7. PMid:8944036.

7 Klijanienko J, Vielh P. Salivary carcinomas with papillae: cytology and histology analysis of polymorphous low grade adenocarcinoma and papillary cystadenocarcinoma. Diagn Cytopathol. 1998;19(4):244-9.<244::AID-DC3>3.0.CO;2-A. PMid:9784985.

8 Kapila K, Verma K. Cytomorphologic features of papillary cystadenocarcinoma of the parotid. Diagn Cytopathol. 2002;26(6):392-7. PMid:12112832.

9 Simionescu C, Florescu M, Margaritescu C, Marinescu M. Histopathologic and immunohistochemical study in one case of cystadenoma of parotid gland becoming malignant. Rom J Morphol Embryol. 1999;45:159-164.

10 Gallego L, Junquera L, Fresno MF, Vicente JC. Papillary cystadenoma and cystadenocarcinoma of salivary glands: two unusual entities. Med Oral Patol Oral Cir Bucal. 2008;13(7):E460-3. PMid:18587312.

11 Agarwal S, Das P, Singh MK, Sharma A, Karak AK. Papillary cystadenocarcinomas of salivary glands with oncocytic epithelial lining: report of 2 cases. Int J Surg Pathol. 2008;16(3):341-4. PMid:18573791.

12 Aloudah NM, Raddaoui E, Aldhahri S, Al-Abbadi MA. Low-grade papillary cystadenocarcinoma of the parotid gland: presentation of a case with cytological, histopathological, and immunohistochemical features and pertinent literature review. Diagn Cytopathol. 2009;37(2):128-31. PMid:19021236.

13 Telugu RB, Job AJ, Manipadam MT. Papillary cystadenocarcinoma of the parotid gland: a rare case report. J Clin Diagn Res. 2016;10(6):ED01-03. PMid:27504297.

14 Joshi U, Chufal SS, Thapliyal N, Khetan H. Cytomorphological features of papillary cystadenocarcinoma of parotid gland: a case report with review of literature. Cytojournal. 2016;13:12. PMid:27298629.

15 Jindal S, Handa U. Cytological diagnosis of papillary cystadenocarcinoma of the parotid gland. Ann Clin Cytol Pathol. 2015;1(2):1009.

16 Khatib Y, Dande M, Patel RD, Kane SV. Cytomorphological findings and histological correlation of papillary cystadenocarcinoma of the parotid: not always a low-grade tumor. Indian J Pathol Microbiol. 2016;59(3):368-71. PMid:27510680.

17 Saraireh OA, Eid S. Parotid duct bleeding, presentation of malignant parotid gland papillary cystadenocarcinoma: rare case report. J Otolaryngol ENT Res. 2018;10(4):233-5.

18 Wang KY, Kim DH, Xu Y, Rad MG, Lincoln CM. High-grade Features of papillary cystadenocarcinoma of the parotid gland. Oman Med J. 2019;34(2):164-8. PMid:30918612.

19 Guo B, Cao YA, Qin X, Ma C. The rare entity of cystadenocarcinoma (CAC) in parotid gland: a single-center experience. J Craniomaxillofac Surg. 2019;47(5):826-31. PMid:30857928.

20 Chowdhury Z, Mishra J, Shunyu NB. Papillary cystadenocarcinoma of salivary gland: report of a diagnostic pitfall and literature review. Ann Int Med Den Res. 2020;6(4):PT12-5.

21 Koç M, Yanilmaz M, Yildirim H, Gök U, Cobanoglu B. MRI findings of papillary cystadenocarcinoma of the submandibular gland. Diagn Interv Radiol. 2010;16(1):20-3. PMid:20039236.

22 Zhu S, Schuerch C, Hunt J. Review and updates of immunohistochemistry in selected salivary gland and head and neck tumors. Arch Pathol Lab Med. 2015;139(1):55-66. PMid:25549144.

23 Kobayashi I, Kiyoshima T, Ozeki S, et al. Immunohistochemical and ultrastructural study of a papillary cystadenocarcinoma arising from the sublingual gland. J Oral Pathol Med. 1999;28(6):282-6. PMid:10426203.

24 Pollett A, Perez-Ordonez B, Jordan RC, Davidson MJ. High-grade papillary cystadenocarcinoma of the tongue. Histopathology. 1997;31(2):185-8. PMid:9279572.

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