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Presented by Dr. Elizabeth Thompson and prepared by Dr. Monica Butcher
This is a 25 year old female with a pancreatic mass.
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1. Question
This is a 25 year old female with a pancreatic mass.
Diagnosis:
Correct
Answer: A
Histologic description: H&E sections show a solid cellular neoplasm, well-demarcated, with subtle infiltration into adjacent pancreas without associated desmoplastic reaction. The cells are round to oval-shaped with fairly bland cytologic features and scattered nuclear grooves. In areas the cells appear discohesive and fall apart around blood vessels creating “pseudo-papillae.” There are focal areas of foamy macrophage accumulation and clusters of hyaline globules. Immunostains show that the tumor cells have patchy, focal labeling for cytokeratin AE1/3 and are positive for synaptophysin and negative for chromogranin. Beta-catenin shows an abnormal pattern of nuclear and cytoplasmic labeling. The morphology and immunoprofile are consistent with a solid-pseudopapillary neoplasm (SPN).
Differential diagnosis: The primary differential diagnosis is with other solid cellular neoplasms of the pancreas: well-differentiated neuroendocrine tumor (WDNET) and acinar cell carcinoma (ACC). Typically, the most morphologic similarities occur with WDNET, but these tumors show characteristic “salt and paper” chromatin with nested and trabecular architecture. Both synaptophysin and chromogranin are typically positive and cytokeratin is positive. The weak, focal cytokeratin labeling and synaptophysin positivity in SPN are a potential diagnostic pitfall, particularly in small biopsies. ACC have characteristic single, prominent nucleoli with granular, eosinophilic cytoplasm and morphology that ranges from acinar formations to sheet-like growth. This particular example of SPN is on the more cellular end of the spectrum, but degenerative changes are extremely common, which can help with the distinction between other solid cellular pancreatic neoplasms. All SPN are considered malignant, however, metastasis and recurrence are rare. Most patients are cured with complete surgical resection.
Incorrect
Answer: A
Histologic description: H&E sections show a solid cellular neoplasm, well-demarcated, with subtle infiltration into adjacent pancreas without associated desmoplastic reaction. The cells are round to oval-shaped with fairly bland cytologic features and scattered nuclear grooves. In areas the cells appear discohesive and fall apart around blood vessels creating “pseudo-papillae.” There are focal areas of foamy macrophage accumulation and clusters of hyaline globules. Immunostains show that the tumor cells have patchy, focal labeling for cytokeratin AE1/3 and are positive for synaptophysin and negative for chromogranin. Beta-catenin shows an abnormal pattern of nuclear and cytoplasmic labeling. The morphology and immunoprofile are consistent with a solid-pseudopapillary neoplasm (SPN).
Differential diagnosis: The primary differential diagnosis is with other solid cellular neoplasms of the pancreas: well-differentiated neuroendocrine tumor (WDNET) and acinar cell carcinoma (ACC). Typically, the most morphologic similarities occur with WDNET, but these tumors show characteristic “salt and paper” chromatin with nested and trabecular architecture. Both synaptophysin and chromogranin are typically positive and cytokeratin is positive. The weak, focal cytokeratin labeling and synaptophysin positivity in SPN are a potential diagnostic pitfall, particularly in small biopsies. ACC have characteristic single, prominent nucleoli with granular, eosinophilic cytoplasm and morphology that ranges from acinar formations to sheet-like growth. This particular example of SPN is on the more cellular end of the spectrum, but degenerative changes are extremely common, which can help with the distinction between other solid cellular pancreatic neoplasms. All SPN are considered malignant, however, metastasis and recurrence are rare. Most patients are cured with complete surgical resection.
