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Presented by Dr. Elizabeth Thompson and prepared by Austin McCuiston.
This is a pancreatic mass in a 9 year old boy.
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1. Question
This is a pancreatic mass in a 9 year old boy.
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Answer: Solid-pseudopapillary neoplasm
Histology: The tumor is comprised of poorly cohesive monomorphic cells separated by hyalinized to myxoid stromal bands with thin walled blood vessels. The neoplastic cells have variably eosinophillic to foamy or clear cytoplasm. The nuclei show fine chromatin with grooves or indentations. Mixed growth patterns demonstrating variably solid, pseudopapillary and cystic areas are seen. Hyaline globules, clusters of foamy macrophages and cholesterol clefts are present. While well-demarcated from the normal pancreas, a subtle infiltrative pattern can be appreciated at the periphery. The tumor cells labeled for CD10 with aberrant nuclear labeling for beta-catenin.
Discussion: Solid-pseudopapillary neoplasms (SPN) are low grade malignancies arising in the pancreas, predominantly in young women, although this patient was a young boy. The histogenesis of SPN is unclear. The differential is that of solid cellular neoplasms of the pancreas: acinar cell carcinoma, pancreatoblastoma, and neuroendocrine tumors. Acinar cell carcinomas would be bcl10, trypsin and chymotrypsin positive with prominent nucleoli. Pancreatoblastomas can show immunohistochemical evidence of multiple lines of differentiations (acinar, ductal and/or neuroendocrine) with distinct squamoid nests that show nuclear labeling for beta-catenin; squamoid nests are required for the diagnosis and the combination of acinar differentiation plus squamoid nests is diagnostic. Neuroendocrine tumors would be keratin, synaptophysin and chromogranin positive.
Incorrect
Answer: Solid-pseudopapillary neoplasm
Histology: The tumor is comprised of poorly cohesive monomorphic cells separated by hyalinized to myxoid stromal bands with thin walled blood vessels. The neoplastic cells have variably eosinophillic to foamy or clear cytoplasm. The nuclei show fine chromatin with grooves or indentations. Mixed growth patterns demonstrating variably solid, pseudopapillary and cystic areas are seen. Hyaline globules, clusters of foamy macrophages and cholesterol clefts are present. While well-demarcated from the normal pancreas, a subtle infiltrative pattern can be appreciated at the periphery. The tumor cells labeled for CD10 with aberrant nuclear labeling for beta-catenin.
Discussion: Solid-pseudopapillary neoplasms (SPN) are low grade malignancies arising in the pancreas, predominantly in young women, although this patient was a young boy. The histogenesis of SPN is unclear. The differential is that of solid cellular neoplasms of the pancreas: acinar cell carcinoma, pancreatoblastoma, and neuroendocrine tumors. Acinar cell carcinomas would be bcl10, trypsin and chymotrypsin positive with prominent nucleoli. Pancreatoblastomas can show immunohistochemical evidence of multiple lines of differentiations (acinar, ductal and/or neuroendocrine) with distinct squamoid nests that show nuclear labeling for beta-catenin; squamoid nests are required for the diagnosis and the combination of acinar differentiation plus squamoid nests is diagnostic. Neuroendocrine tumors would be keratin, synaptophysin and chromogranin positive.
