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Presented by Risa Mann, M.D. and prepared by Greg Seidel, M.D.
Case 3: 42-year-old female with non-specific abdominal symptoms and an MRI mass in the body and tail of the pancreas.
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Week 105: Case 3
42-year-old female with non-specific abdominal symptoms and an MRI mass in the body and tail of the pancreas./images/0923023a.jpg
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/images/0923023e.jpgCorrect
Answer: Solid-pseudopapillary tumor (SPT) of the pancreas
Histology: The tumor is a relatively well-circumscribed cellular epithelial tumor, which at low power shows varying growth pattern. There are focal areas of hemorrhage. The most characteristic feature of this tumor is the papillary and cystic growth pattern. The cellular proliferation is relatively bland with the cells showing round to oval nuclei with few mitoses and relatively inconspicuous nucleoli. There are areas in which the tumor displays pseudo-papillae. In some areas, there are fibrovascular cores that show areas of perivascular myxoid degenerative changes.
The immunostains performed on this tumor demonstrate that the tumor cells show strong positivity for CD10 and Beta catenin. The tumor cells are negative for chromogranin and weakly positive for synaptophysin.
Discussion: The differential diagnosis in this case rests primarily between that of the so-called solid-pseudopapillary tumor of the pancreas and an islet cell tumor. The presence of a tumor in the tail or body of the pancreas in a young woman is a common presentation for the solid-pseudopapillary tumor of the pancreas. The tumor is usually well–circumscribed and surrounded by a capsule. The most characteristic feature of this tumor is the presence of a pseudo-papillae. Foamy histiocytes and hyaline bodies may be seen in association with the tumor. The thick fibrovascular cords often demonstrate areas of myxoid degenerative changes. From a structural standpoint, studies have shown that the tumor cells may show evidence of acinar and ductal differentiation. Occasionally neuroendocrine differentiation may also be seen. The tumor cells are positive for keratin and may show positivity for trypsin and chymotrypsin and amylase. Focal positivity has also been observed with NSE and various islet cell markers. It is also positive for CD10 and * catenin.
Strong staining for the latter two markers favors the diagnosis of solid-pseudopapillary tumor* and would be uncommon in an islet cell tumor and is helpful in establishing the above diagnosis. The ultrastructural and immunohistochemical studies suggest that the tumor cells in the solid pseudopapillary tumor have the capability to show acinar, ductal and in some instances, neuroendocrine differentiation.
The overall prognosis of this tumor is excellent with surgical resection; however, local occurrence and metastases have been reported.
Incorrect
Answer: Solid-pseudopapillary tumor (SPT) of the pancreas
Histology: The tumor is a relatively well-circumscribed cellular epithelial tumor, which at low power shows varying growth pattern. There are focal areas of hemorrhage. The most characteristic feature of this tumor is the papillary and cystic growth pattern. The cellular proliferation is relatively bland with the cells showing round to oval nuclei with few mitoses and relatively inconspicuous nucleoli. There are areas in which the tumor displays pseudo-papillae. In some areas, there are fibrovascular cores that show areas of perivascular myxoid degenerative changes.
The immunostains performed on this tumor demonstrate that the tumor cells show strong positivity for CD10 and Beta catenin. The tumor cells are negative for chromogranin and weakly positive for synaptophysin.
Discussion: The differential diagnosis in this case rests primarily between that of the so-called solid-pseudopapillary tumor of the pancreas and an islet cell tumor. The presence of a tumor in the tail or body of the pancreas in a young woman is a common presentation for the solid-pseudopapillary tumor of the pancreas. The tumor is usually well–circumscribed and surrounded by a capsule. The most characteristic feature of this tumor is the presence of a pseudo-papillae. Foamy histiocytes and hyaline bodies may be seen in association with the tumor. The thick fibrovascular cords often demonstrate areas of myxoid degenerative changes. From a structural standpoint, studies have shown that the tumor cells may show evidence of acinar and ductal differentiation. Occasionally neuroendocrine differentiation may also be seen. The tumor cells are positive for keratin and may show positivity for trypsin and chymotrypsin and amylase. Focal positivity has also been observed with NSE and various islet cell markers. It is also positive for CD10 and * catenin.
Strong staining for the latter two markers favors the diagnosis of solid-pseudopapillary tumor* and would be uncommon in an islet cell tumor and is helpful in establishing the above diagnosis. The ultrastructural and immunohistochemical studies suggest that the tumor cells in the solid pseudopapillary tumor have the capability to show acinar, ductal and in some instances, neuroendocrine differentiation.
The overall prognosis of this tumor is excellent with surgical resection; however, local occurrence and metastases have been reported.
