Cabeza y cuello. Caso 6. Hospital Clínic de Barcelona

Created by SEAP-IAP Sociedad Española de Anatomía Patológica on May 11, 2013 11:21:32 AM, Last modified by SEAP-IAP Sociedad Española de Anatomía Patológica on May 18, 2013 8:53:49 PM

Clinical information

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Llucia Alos, Hospital Clínic, Universidad de Barcelona Resumen de historia clínica: A 76 year-old woman complained of a painless, slowly growing tumour in the right parotid gland. Surgical resection of the lesion was performed.


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Hospital Clínic de Barcelona
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Gross observations

In the partial surgical resection of the parotid gland a tumoration measuring 1.6x1x1 cm. was seen.

Microscopic observations

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Histologically the tumour had a multicystic appearance, at a low-power magnification. It was composed of a mainly intraductal proliferation of epithelial cells forming a cystic and cribriform pattern with both basophilic and colloid-like eosinophilic material secretion. In the larger ducts the epithelial proliferation showed a mainly solid pattern and formed papillae and micropapillae. The cells in the surface had apocrine features, with large, eosinophilic microvacuolated cytoplasm. Focally, the ductal epithelium was preserved. There was hemorrhage and surrounding stroma reaction. The cribriform pattern predominated in the smaller ducts. The cells were cuboidal to oval, the cytoplasmic membranes were indistinct, and nuclei had bland chromatin. No mitotic figures were seen. Immunohistochemically the tumour proliferation showed strong nuclear and cytoplasmic S100 protein positivity. Myoepithelial markers, such as actin or p63 highlighted cell rimming around the ductal cystic spaces. The estrogen, progesterone and androgen receptors were negative, as was Her-2/neu immunostain.

Diagnosis information

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Salivary gland low-grade cribriform cystadenocarcinoma


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Discussion In the latest WHO classification, the term “cystadenocarcinoma” includes those neoplasms histologically characterized by prominent cystic and, frequently, papillary growth, but lacking features that characterize cystic variants of several common salivary gland carcinomas (1). Low-grade cribriform cystadenocarcinoma (LGCA) has received special attention and it is described in a separate chapter because of its distinctive characteristics. It histologically resembles the spectrum of the intraductal breast proliferations, from atypical intraductal hyperplasia to papillary or cribriform low-grade in situ carcinoma. This tumour type was previously described as “low-grade salivary duct carcinoma” (2,3), and the term “low-grade intraductal carcinoma of salivary gland” has recently been proposed (4). The latter describes perfectly the characteristic intraductal growth of this neoplasm. Actually the tumour is composed of a low-grade intraductal proliferation of cells having micropapillary or cribriform pattern of growth. Apocrine cells in the intraluminal surface, with cytoplasmic apocrine-type microvacuoles ( PAS-positive, diastase resistant) are frequently seen. Indeed, some cases with marked apocrine differentiation have been recorded (4). Rarely, focal high-grade transformation has been reported (2,3). In spite of being a tumour without a myoepithelial component, it usually expresses S100 protein with both nuclear and cytoplasmic positivity. Some cases positive for estrogen, progesterone and androgen receptors have been reported (4,5). Most LGCA arise in the parotid gland, but rare cases have been reported to arise in an intraparotid lymph node. Usually it presents in elderly patients, with no sex predilection (1-4). It is a tumour with an excellent prognosis after complete surgical resection. In the case herein, the patient was alive without disease 6 years after follow-up. The differential diagnoses include high-grade salivary duct carcinoma, other types of salivary gland cystadenocarcinomas, the papillary-cystic variant of acinic-cell carcinoma and the recently described mammary analogue secretory carcinoma of salivary glands. High-grade salivary duct carcinoma is an aggressive tumour composed of a cytologically high-grade epithelial proliferation with marked atypia, necrosis and mitotic figures. It has an intraductal component, but it is usually infiltrative. Histologically it resembles a ductal carcinoma of the breast with high-grade intraductal component with comedonecrosis. This tumour is usually positive for androgen receptors and Her-2/neu (6). Salivary gland cystadenocarcinomas are low-grade tumors that usually display cystic and papillary growth pattern, but lack the intraductal growth which characterizes LGSCA (7). In acinic cell carcinomas often one cell type and pattern predominate. However, the identification of other cell-types, including acinar differentiated, clear, non-specific glandular, intercalated duct-like and vacuolated cells, is useful in recognizing this tumour type. The mammary analogue secretory carcinoma of salivary glands has recently been described as a neoplasm resembling secretory carcinoma of the breast. It displays histological features resembling both salivary acinic cell carcinoma and low-grade cystadenocarcinoma. It shares with secretory carcinoma of the breast the characteristic translocation t(12;15) (p13;q25) ETV6-NTRK (8). References 1-Brandwein-Gensler MS, Gnepp DR. Low-grade cribriform cystadenonocarcinoma. In: Barnes L, Eveson JW, Reichart P, Sidransky D, eds. WHO Classification of Tumours. Pathology and genetics of head and neck Tumours. Lyon: IARC Press, 2005:235. 2- Delgado R, Klimstra D, Albores-Savedra J. Low Grade Salivary Duct Carcinoma. A distinctive variant with a low grade histology and predominant intraductal growth pattern. Cancer 1996; 78:958-67. 3- Brandwein-Gensler MS, Hille J, Wang BY, et al. Low-grade salivary duct carcinoma. Description of 16 cases. Am J Surg Pathol 2004; 28:1040-4. 4-Weinreb I, Tabanda-Lichauco R, Van der Kwast T, Perez-Ordoñez B. Low-grade Intraductal Carcinoma of Salivary Glands. Report of 3 cases with marked apocrine differentiation. Am J Surg Pathol 2006; 30:1014-1020. 5- KusafukaK, Itoh H, Sugiyama C, Nakajima T. Low-grade salivary duct carcinoma of the parotid gland: report of a case with immunohistochemical analysis. Med Mol Morphol 2010; 43:178-184. 6- Skalova A, Starek I, Vanecek T, et al. (2003). Expression of HER-2/neu gene and protein in salivary duct carcinoma of parotid gland as revealed by fluorescence in situ hybridization and immunohistochemistry. Histopathology 2003; 42: 1-9. 7- Foss R, Ellis GL, Auclair PL. Salivary gland cystadenocarcinomas: a clinicopathological study of 57 cases. Am J Surg Pathol 1996; 20:1440-1447. 8-Skalova A, Vanecek T, Sima R et al. Mammary analogue secretory carcinoma of salivary glands, containing the ETV6-NTRK3 fusion gene: a hitherto undescribed salivary gland tumor entity. Am J Surg Pathol 2010; 34:599-608.


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