43,44) These glandular carcinoids often contain psammoma bodies but do not produce the somatostatinoma syndrome as reported in patents with pancreatic endocrine D cell tumors.
Neurofibromatosis, pheochromocytomas and somatostatinoma.
Somatostatinoma of the papilla of Vater with multiple gastrointestinal stromal tumors in a patient with von Recklinghausen's disease.
Association of GIST and somatostatinoma in a patient with type 1 neurofibromatosis: is there a common pathway?
Neuroendocrine tumors, produce excessive amounts of hormones and include carcinoid syndrome, Zollinger Ellison syndrome, VIPomas, insulinomas gastrinoma, glucaginoma and somatostatinoma
, among others.
These tumors are classified according to the predominant hormone of oversecretion: insulinoma, gastrinoma, vasoactive intestinal peptide-secreting tumors (VIPoma), glucagonoma, somatostatinoma, and growth-hormone-releasing factor-secreting tumors (GRFomas).
Similarly, VIPomas, somatostatinomas, and GRFomas may also arise outside of the pancreas.
Endocrinopathies including acromegaly, Cushing's syndrome, glucagonoma, pheochromocytoma, hyperthyroidism, somatostatinoma
, and aldosteronoma;
Although they secrete somatostatin, somatostatinomas
do not produce the somatostatinoma
syndrome in MEN 1 patients.
Pancreatic tumors classification * Primary tumors Exocrine Ductal adenocarcinoma Serous cystic tumor Mucinous cystic tumor Solid-pseudopapillarymucinous tumor Acinar cell carcinoma Anaplastic carcinoma Pancreatoblastoma Endocrine Insulinoma Gastrinoma Glucagonoma Vipoma Somatostatinoma
Nonhyperfunctiong tumor Nonepithelial tumor Lymphoma Teratoma Lymphangioma Lipoma Neural tumors Secondary tumors * Table data adapted from Solcia E, Capella C, Kloppel G.
Composite tumor of mucinous cystadenoma and somatostatinoma
of the kidney.
The 2 exceptions are the stroma with amyloid deposits identifying insulinoma and the glandular pattern with psammoma bodies identifying somatostatinoma