Submandibular gland cystadenocarcinoma with mucinous adenocarcinoma-like areas: a case report
© Zhang et al.; licensee BioMed Central Ltd. 2014
Received: 11 February 2014
Accepted: 23 March 2014
Published: 28 April 2014
Cystadenocarcinoma is primarily characterized by cystic structures of varying sizes, that are lined by epithelial cells. As a rare neoplasm, only four cases of cystadenocarcinoma of submandibular glands have been previously reported. Herein, we reported a unique case of submandibular gland cystadenocarcinoma in a 44-year-old man. By in large, this case had typical morphologic cystadenocarcinoma features. However, mucinous adenocarcinoma-like areas were additionally observed in the tumor tissues. This case was initially misdiagnosed as mucinous adenocarcinoma due to the low incidence of submandibular gland cystadenocarcinoma and the presentation of mucinous adenocarcinoma-like areas in the tumor tissues. Histopathology of additional tumor tissues revealed that mucinous adenocarcinoma-like areas accounted for only a small percentage of the tumor tissues, confirming the submandibular gland cystadenocarcinoma diagnosis.
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Letter to the editor
Immunohistochemical staining showed that the tumor cells were CK, CKH, CK8/18, CK7, CK19, and S-100 positive. Approximately 2% of the cells were Ki67 positive. The cells were CK5/6, CEA, Actin, P63, Calponin, SMA, P53, CDX2, CK20, Villin, C-erbB-2, TTF1, SP-A, and TG negative. Alcian blue and Periodic Acid Schiff staining demonstrated that both cysts and tumor cells were filled with mucus-like materials (Figure 2D). Based on these observations, this tumor was diagnosed as a submandibular gland cystadenocarcinoma.
In 2005, cystadenocarcinoma of salivary glands was reclassified by the World Health Organization (WHO) as a malignant tumor that rarely occurs in submandibular glands . Salivary gland cystadenocarcinomas account for approximately 2% of malignant salivary gland tumors. It is a rare tumor, compared with other tumor in the salivary gland [2, 3]. Most cystadenocarcinoma have been reported in the parotid gland and minor salivary glands. Since being first described in 2006, only four cases of submandibular gland cystadenocarcinoma have been reported. Among the four cases, all patients were male, aged 12, 54, 67, and 74 years old [4–7]. Similarly, the patient in our study was a 44-year-old man. The main symptom of a submandibular gland cystadenocarcinoma is swelling in the submandibular gland region. Some patients complain of pain or tenderness, but bleeding, ulceration, and dysfunction are rare. Risk factors for cystadenocarcinoma remain unclear. However, smoking, alcohol use, environmental factors, and genetic abnormalities may contribute to the carcinogenesis. The most effective treatment is surgical excision of the tumor and most patients recover.
Based on WHO descriptions, cystadenocarcinoma is primarily characterized by a cystic structure of varying sizes, lined by epithelial cells with or without papillary projections. In the first round of biopsy and histopathology examination, while these typical features were observed, we also identified a number of cysts lacking epithelial lining that were filled with abundant mucus-like materials. Scattered single tumor cells and cell clusters floated freely in the mucous lakes. These areas were shown to have conventional morphologic features of mucinous adenocarcinoma. Due to the low incidence of submandibular gland cystadenocarcinoma and presentation of mucinous adenocarcinoma-like areas, this tumor was initially misdiagnosed as a mucinous adenocarcinoma. Following consultation with senior physicians, additional tumor tissues were examined. Generally speaking, the majority of tumor tissues presented morphologic features of cystadenocarcinoma, but limited areas had cysts lacking epithelial cell lining caused by hemorrhaging and shedding of epithelial cells. The immunohistochemical staining of the markers mentioned above revealed no significant difference between these two types of tumors because both have glandular epithelium origins. Therefore, differential diagnosis primarily depends on examination of more tissues.
Written informed consent was obtained from the patient for publication of this case report.
- Barnes L, Eveson JW, Reichart P, Sidransky D: World Health Orga-nization Classification of Tumours. Pathology and Genetics of Head and Neck Tumours. 2005, Lyon: IARC press, 232-Google Scholar
- Jung MJ, Roh JL, Choi SH, Nam SY, Kim SY, Lee SW, Cho KJ: Basal cell adenocarcinoma of the salivary gland: a morphological and immunohistochemical comparison with basal cell adenoma with and without capsular invasion. Diagn Pathol. 2013, 8: 171-10.1186/1746-1596-8-171.PubMedPubMed CentralView ArticleGoogle Scholar
- Wang L, Liu Y, Lin XY, Zhang D, Li QC, Qiu XS, Wang EH: Low-grade cribriform cystadenocarcinoma of salivary glands: report of two cases and review of the literature. Diagn Pathol. 2013, 8: 28-10.1186/1746-1596-8-28.PubMedPubMed CentralView ArticleGoogle Scholar
- Harimaya A, Somekawa Y, Sasaki M, Ohuchi T: Cystadenocarcinoma (papillary cystadenocarcinoma) of the submandibular gland. J Laryngol Otol. 2006, 120: 1077-1080.PubMedView ArticleGoogle Scholar
- Czarnecki EJ, Spickler EM, Keohane M, Roennecke W: Cystic papillary adenocarcinoma of the submandibular gland in a child. Am J Neuroradiol. 1996, 17: 1038-1040.PubMedGoogle Scholar
- Mardi K, Sharma S, Gupta N: Papillary cystadenocarcinoma of submandibular salivary gland: a rare case report. J Cancer Res Ther. 2010, 6: 330-332. 10.4103/0973-1482.73346.PubMedView ArticleGoogle Scholar
- Koc M, Yanilmaz M, Yidirim H, Gok U, Cobanoglu B: MRI findings of papillary cystadenocarcinoma of the submandibular gland. Diagn Interv Radiol. 2010, 16: 20-23.PubMedGoogle Scholar
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