Gastrointestinal ImagingMen syndrome
dominantly inherited proliferative disorders characterized by endocrine functioning tumours located in different organs. MEN is the acronym for Multiple Endocrine Neoplasia syndrome. Two main types are distinguished.
MEN I (Wermers syndrome) is characterized by the combined occurrence of tumours in the pituitary glands and in the pancreatic islets associated with parathyroid hyperplasia. Islet cell tumours of the pancreas may include gastrinomas, insulinomas, carcinoids and more rarely glucagonomas, VIPomas and GRFomas. The islet cell tumours in MEN I syndrome are frequently small and multiple.
The most prominent and common symptoms of MEN II syndrome are related to hyperparathyroidism. MEN IIa (Sipples syndrome) is characterized by phaeochromocytoma, medullary thyroid carcinoma and hyperparathyroidism. MEN IIb is similar to MEN IIa but without involvement of the parathyroids and with the presence of multiple mucosal neuromas, ganglioneuromas throughout the gastrointestinal tract and a marfanoid habitus. There are mixed forms of these syndromes. MEN II syndromes are not associated with islet cell tumours. Most of these tumours have a high degree of malignancy. US, CT and MRI (Fig.1) contributes significantly to the radiological visualization of pancreatic islet cell tumours.
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MEN syndrome.
a. and b. MRI fat suppressed T1-weighted sequence, transverse section. Two hypointense round areas (arrowheads) are depicted in the head and body of the pancreas, respectively. The normal pancreas parenchyma has a high signal intensity due to the presence of aqueous protein in the acini of the pancreas.
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Men syndrome, Fig.1 (a) | | Men syndrome, Fig.1 (b) | |