Abstract
Background: Neuroendocrine tumors are rare, but when coexisting with Multiple Endocrine Neoplasia Type 1 Syndrome in the same patient, the literature shows 80 to 100% association. Represents an autosomal dominant disorder due to mutations in tumor suppressor gene MEN1 and it's defined as the development of two or more tumors from the main three endocrine sites: the parathyroid glands, the pancreatic islet and the Pituitary gland. We present a case of a patient with a compatible family history and classic imaging findings for MEN1 Syndrome. Case report: Male, 28 years old patient genetically diagnosed with the MEN 1 Syndrome who presented to our image service for abdominal tumors' screening. Submitted through multiples imaging exams such as CT, MRI, PET/CT to follow up the diagnosis, in which was found a pancreatic mass measuring over 3 cm, with another 3 little pancreatic lesions as well. The patient received indication to surgery, with further anatomopathological and immunohistochemistry studies confirming the well- differentiated neuroendocrine tumor. Conclusion: Due to the wide range of malignant potential, it's recommended annual screening, as minimum, with biochemical and image techniques. Surgery is the only treatment capable of tumor's eradication, which consists on a pancreatectomy (lesions sized more than 1-2 cm).
Highlights
Multiple Endocrine Neoplasia Type 1 Syndrome (MEN1)J.C.A.M, 28 years, male, genetically diagnosed with Multiple Endocrine Neoplasia Type 1 Syndrome (MEN 1), attends to the image service for abdominal tumors’ screening
We present a 28 years old male patient with family history and genetic diagnose of MEN 1 syndrome who was diagnosed with a NF-Pancreatic Neuroendocrine Tumors (PNETs)
MEN 1 is defined as the development of two or more tumors from the main three endocrine sites: the parathyroid glands, the pancreas/duodenum/foregut area and the Pituitary glands [1]
Summary
J.C.A.M, 28 years, male, genetically diagnosed with Multiple Endocrine Neoplasia Type 1 Syndrome (MEN 1), attends to the image service for abdominal tumors’ screening. He has a sister, mother and five uncles genetically diagnosed with the syndrome. Magnetic Ressonance Image (MRI) showed little cystic areas in the lesion, restriction to diffusion in the same spot and in other three milimeterical spots in the pancreatic body (Fig 1 – 5). Positron Emission Tomography (PET/CT) with DOTATOC - 68Ga showed mass’s increased radiotracer caption (SUVmax – Standardized Uptake Value – 59.4) and in three other focal areas (SUVmax 15.1). PET/CT with DOTATOC/Ga68 exhibits increased radiotracer caption, greater in Pancreas’s head (SUVmax 59,4) and in three other focal areas
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