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Pathology – Pancreatic Endocrine Tumors
Pancreatic endocrine tumors are epithelial tumors of the pancreas that exhibit endocrine differentiation. Tumors can be active or inactive based on the presence of an abnormal hormone secretion syndrome.
Epidemiology: Rare tumors account for around 2% of all pancreatic tumors. • Peak incidence occurs between 30 and 60 years
• Unknown in infrequent occurrences. • Approximately 15% are linked to multiple endocrine neoplasia (MEN)-1. • Early genetic events include losses at chromosomes 1 and 11q and gains at 9q. • Increased changes can lead to malignant behaviors. Tumors that produce excess hormones may exhibit symptoms such as hypoglycemia (insulin-producing tumors), recurrent duodenal ulcers (gastrin-producing tumors), and necrolytic migratory erythema (glucagon-producing tumors). • Non-functioning tumors are detected by imaging or when they become large enough to cause symptoms or metastases. Macroscopy reveals well-defined tumors in the pancreas.
Cytopathology: • Fine needle aspiration (FNA) smears are cellular, with a monotonous population of cells present singly, in loose clusters, or in pseudorosettes. • The nuclei typically exhibit a distinct granular chromatin pattern ('salt-and-pepper').
Histopathology: • Tumor cells have granular cytoplasm and form solid nests, trabeculae, glands, or rosettes. • Immunostaining confirms the endocrine origin of the cells through reactivity for markers CD56, chromogranin, and synaptophysin.
Prognosis: • Often difficult to anticipate with confidence.
Long-term follow-up is necessary for all potentially malignant tumors, as metastases can form years after the main lesion is removed.
Clinicopathological classification of pancreatic endocrine tumors.
Well-differentiated endocrine tumor. Benign pancreatic tumors are non-angioinvasive, <2cm in size, with ≤ 2 mitoses />0hpf and ≤ 2% Ki-67 positive cells. Uncertain behavior is associated with the pancreas and one or more of the following features: ≥ 2cm size, 2-10 mitoses/10hpf, > 2% Ki-67 positive cells/10hpf, angioinvasion, or perineural invasion.
Well-differentiated endocrine carcinoma. Histologically low-grade, but with significant local invasion and/or metastasis.
poorly differentiated endocrine carcinoma. Histologically high-grade, having more than 10 mitoses/10 hpf
Pancreatic endocrine tumors are epithelial tumors of the pancreas that exhibit endocrine differentiation. Tumors can be active or inactive based on the presence of an abnormal hormone secretion syndrome.
Epidemiology: Rare tumors account for around 2% of all pancreatic tumors. • Peak incidence occurs between 30 and 60 years
• Unknown in infrequent occurrences. • Approximately 15% are linked to multiple endocrine neoplasia (MEN)-1. • Early genetic events include losses at chromosomes 1 and 11q and gains at 9q. • Increased changes can lead to malignant behaviors. Tumors that produce excess hormones may exhibit symptoms such as hypoglycemia (insulin-producing tumors), recurrent duodenal ulcers (gastrin-producing tumors), and necrolytic migratory erythema (glucagon-producing tumors). • Non-functioning tumors are detected by imaging or when they become large enough to cause symptoms or metastases. Macroscopy reveals well-defined tumors in the pancreas.
Cytopathology: • Fine needle aspiration (FNA) smears are cellular, with a monotonous population of cells present singly, in loose clusters, or in pseudorosettes. • The nuclei typically exhibit a distinct granular chromatin pattern ('salt-and-pepper').
Histopathology: • Tumor cells have granular cytoplasm and form solid nests, trabeculae, glands, or rosettes. • Immunostaining confirms the endocrine origin of the cells through reactivity for markers CD56, chromogranin, and synaptophysin.
Prognosis: • Often difficult to anticipate with confidence.
Long-term follow-up is necessary for all potentially malignant tumors, as metastases can form years after the main lesion is removed.
Clinicopathological classification of pancreatic endocrine tumors.
Well-differentiated endocrine tumor. Benign pancreatic tumors are non-angioinvasive, <2cm in size, with ≤ 2 mitoses />0hpf and ≤ 2% Ki-67 positive cells. Uncertain behavior is associated with the pancreas and one or more of the following features: ≥ 2cm size, 2-10 mitoses/10hpf, > 2% Ki-67 positive cells/10hpf, angioinvasion, or perineural invasion.
Well-differentiated endocrine carcinoma. Histologically low-grade, but with significant local invasion and/or metastasis.
poorly differentiated endocrine carcinoma. Histologically high-grade, having more than 10 mitoses/10 hpf
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