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Presented by Fred Askin, M.D. and prepared by Jeffrey Seibel, M.D. Ph.D.
Case 1: The patient is a 65-year-old woman with abdominal fullness and pancreatic mass seen on CAT scan.
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1. Question
Week 84: Case 1
The patient is a 65-year-old woman with abdominal fullness and pancreatic mass seen on CAT scan./images/040102case1a.jpg
/images/040102case1b.jpg
/images/040102case1c.jpgCorrect
Answer: Serous microcystic adenoma
Histology: The lesion consists of small cysts containing proteinaceous fluid. The cysts are lined by a single layer of cuboidal or flattened epithelium with pale to clear cytoplasm.
Discussion: This lesion is a serous cystadenoma of the microcystic type. It is a benign neoplasm composed of numerous small cysts arranged around a central stellate scar. Other terms used for this lesion have been microcystic adenoma and glycogen-rich cystadenoma. Serous cystadenoma is an uncommon lesion and accounts for only 1-2 percent of all pancreatic tumors. This neoplasm occurs more often in women than men and the usual age at presentation is around 66 years. A series of serous cystadenomas at Johns Hopkins includes 111 patients (85 women, 28 men). The average age in our material was 64 years with a median of 64. The age of the patients ranged from 36 to 82. In the literature, approximately one half to two thirds of the tumors have occurred in the body or tail. The lesions are usually solitary and range in diameter from 1 to 25 cm. in greatest dimension (average 6 to 10 cm.).
The gross characteristic of these lesions is the presence of a dense, centrally located fibrous scar from which trabeculae radiate to the periphery. In larger tumors, this “scar” may be calcified. Microscopically, lesions consists of small cysts containing proteinaceous fluid. The cysts are lined by a single layer of cuboidal or flattened epithelium with pale to clear cytoplasm. Rarely, the cytoplasm may be eosinophilic. The tumor cells contain glycogen and stain with EMA and cytokeratin 7, but are uniformly negative for CEA. Rarely, malignant variants of serous cystadenoma have been described. We have one such case in our files. This lesion involved the pancreas and liver in a very infiltrative fashion, although the cytologic features of the cells which lined the cyst were quite bland.
There are, in fact, two types of serous cystadenoma of the pancreas. The common lesion, as seen here, is microcystic. There is also a rare macrocystic adenoma composed of a few relatively large cysts lined by epithelium showing evidence of ductal differentiation. This lesion has been described in adults and in children and lack the central stellate scars seen in the microcystic lesion.
Differential diagnosis of serous cystadenoma would include lymphangioma (the tumor cells would be CD31 positive and negative for cytokeratins and EMA), and metastatic renal cell carcinoma (characterized by small tubular structures and cells with variable and irregular nuclei, often with vimentin positive cytoplasm). The most important distinction would be between serous and mucinous cystic neoplasm. Mucinous cystic neoplasms need to be examined in their entirety because of the danger of focal carcinoma. This feature is not generally a problem in serous cystadenoma.
Incorrect
Answer: Serous microcystic adenoma
Histology: The lesion consists of small cysts containing proteinaceous fluid. The cysts are lined by a single layer of cuboidal or flattened epithelium with pale to clear cytoplasm.
Discussion: This lesion is a serous cystadenoma of the microcystic type. It is a benign neoplasm composed of numerous small cysts arranged around a central stellate scar. Other terms used for this lesion have been microcystic adenoma and glycogen-rich cystadenoma. Serous cystadenoma is an uncommon lesion and accounts for only 1-2 percent of all pancreatic tumors. This neoplasm occurs more often in women than men and the usual age at presentation is around 66 years. A series of serous cystadenomas at Johns Hopkins includes 111 patients (85 women, 28 men). The average age in our material was 64 years with a median of 64. The age of the patients ranged from 36 to 82. In the literature, approximately one half to two thirds of the tumors have occurred in the body or tail. The lesions are usually solitary and range in diameter from 1 to 25 cm. in greatest dimension (average 6 to 10 cm.).
The gross characteristic of these lesions is the presence of a dense, centrally located fibrous scar from which trabeculae radiate to the periphery. In larger tumors, this “scar” may be calcified. Microscopically, lesions consists of small cysts containing proteinaceous fluid. The cysts are lined by a single layer of cuboidal or flattened epithelium with pale to clear cytoplasm. Rarely, the cytoplasm may be eosinophilic. The tumor cells contain glycogen and stain with EMA and cytokeratin 7, but are uniformly negative for CEA. Rarely, malignant variants of serous cystadenoma have been described. We have one such case in our files. This lesion involved the pancreas and liver in a very infiltrative fashion, although the cytologic features of the cells which lined the cyst were quite bland.
There are, in fact, two types of serous cystadenoma of the pancreas. The common lesion, as seen here, is microcystic. There is also a rare macrocystic adenoma composed of a few relatively large cysts lined by epithelium showing evidence of ductal differentiation. This lesion has been described in adults and in children and lack the central stellate scars seen in the microcystic lesion.
Differential diagnosis of serous cystadenoma would include lymphangioma (the tumor cells would be CD31 positive and negative for cytokeratins and EMA), and metastatic renal cell carcinoma (characterized by small tubular structures and cells with variable and irregular nuclei, often with vimentin positive cytoplasm). The most important distinction would be between serous and mucinous cystic neoplasm. Mucinous cystic neoplasms need to be examined in their entirety because of the danger of focal carcinoma. This feature is not generally a problem in serous cystadenoma.
