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Presented by Theresa Chan, M.D. and prepared by Jeffrey Seibel, M.D. Ph.D.
Case 6: 43 year-old female with a large mass in the base of the tongue.
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1. Question
Week 89: Case 6
43 year-old female with a large mass in the base of the tongue.images/050602case6a.jpg
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images/050602case6e.jpgCorrect
Answer: Adenoid cystic carcinoma
Histology: The mass consists of an infiltrative tumor with various histologic patterns, including cribriform, tubular and solid architecture. Within the cribriform areas, pseudocysts with hyaline-like material can be seen. Prominent perineural invasion is present.
Discussion: Adenoid cystic carcinoma is the most common malignant neoplasm in minor salivary glands, and the third most common malignancy in the parotid gland. The tumor is characterized by cells with hyperchromatic angulated nuclei and clear cytoplasm forming ducts. It can grow in three histologic patterns: cribriform, tubular and solid. Usually all three patterns are seen within the same tumor. Adenoid cystic carcinoma has a marked propensity for perineural invasion and the spread along nerves accounts for the high recurrence rate. Although these tumors are generally slow growing, they are highly malignant neoplasms, and most commonly metastasize to the lung. Lymph node metastases are rare. Immunohistochemistry will show that the tumor cells in the ducts react like intercalated duct cells, and will be positive for cytokeratin, CEA and S100. Cells lining the pseudocysts will react like myoepithelial cells, and therefore are positive for S100, actin, and variably for keratin.
Adenoid cystic carcinomas can be differentiated from pleomorphic adenomas by their infiltrative nature, the presence of perineural invasion and a lack of mesenchymal areas and foci of squamous metaplasia. Adenoid cystic carcinomas can be differentiated from mucoepidermoid carcinomas by the lack of the four cell types characteristically seen in a mucoepidermoid carcinoma: mucin-producing, squamous, intermediate and clear cells.
The prognosis of adenoid cystic carcinoma is influenced by the pattern of growth of the tumor. Studies have shown that the solid architectural growth pattern is associated with the worst prognosis, followed by cribriform and then tubular architecture. The fifteen year survival is reported to be 5% in tumors with predominantly solid growth pattern, 26% with predominantly cribriform growth pattern, and 39% with predominantly tubular pattern.
Incorrect
Answer: Adenoid cystic carcinoma
Histology: The mass consists of an infiltrative tumor with various histologic patterns, including cribriform, tubular and solid architecture. Within the cribriform areas, pseudocysts with hyaline-like material can be seen. Prominent perineural invasion is present.
Discussion: Adenoid cystic carcinoma is the most common malignant neoplasm in minor salivary glands, and the third most common malignancy in the parotid gland. The tumor is characterized by cells with hyperchromatic angulated nuclei and clear cytoplasm forming ducts. It can grow in three histologic patterns: cribriform, tubular and solid. Usually all three patterns are seen within the same tumor. Adenoid cystic carcinoma has a marked propensity for perineural invasion and the spread along nerves accounts for the high recurrence rate. Although these tumors are generally slow growing, they are highly malignant neoplasms, and most commonly metastasize to the lung. Lymph node metastases are rare. Immunohistochemistry will show that the tumor cells in the ducts react like intercalated duct cells, and will be positive for cytokeratin, CEA and S100. Cells lining the pseudocysts will react like myoepithelial cells, and therefore are positive for S100, actin, and variably for keratin.
Adenoid cystic carcinomas can be differentiated from pleomorphic adenomas by their infiltrative nature, the presence of perineural invasion and a lack of mesenchymal areas and foci of squamous metaplasia. Adenoid cystic carcinomas can be differentiated from mucoepidermoid carcinomas by the lack of the four cell types characteristically seen in a mucoepidermoid carcinoma: mucin-producing, squamous, intermediate and clear cells.
The prognosis of adenoid cystic carcinoma is influenced by the pattern of growth of the tumor. Studies have shown that the solid architectural growth pattern is associated with the worst prognosis, followed by cribriform and then tubular architecture. The fifteen year survival is reported to be 5% in tumors with predominantly solid growth pattern, 26% with predominantly cribriform growth pattern, and 39% with predominantly tubular pattern.
