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Neuroendocrine tumor of head of pancreas

Case contributed by Fabio Macori
Diagnosis certain

Presentation

A patient with a history of a high hepatic marker.

Patient Data

Age: 50 years
Gender: Male

Ultrasound images show the presence of a large round shaped hypoechoic formation, with well-defined margins, between the head and the uncinate process of pancreas

CT scan of the abdomen confirmed the presence of the rounded lesion which shows a fleeting increase in its density after intravenous administration of contrast medium iodine.

Another smaller rounded lesion is demonstrated in the tail of the pancreas

In all sequences, the tumor is very clearly visible, relatively hypointense compared to the normal pancreatic parenchyma, while it is weakly hyperintense on T2-weighted sequences. After injection of MDC, the tumor, with dynamic behavior and linked to its typical rich vascularization, shows a marked and persistent increase in signal intensity, appearing hyperintense compared to the pancreatic parenchyma in the pancreatic arterial phase

Nuclear medicine

PET-CT shows a high signal of the lesion

Neuroendocrine tumor of pancreas (cromogranine, CDS6, positive synaptosin) according to WHO 2010, well differentiated G1, with

Focal positivity for cytokeratin 19.

The percentage of cyclic cells evaluated with Ki 67 was < 3% (WHO 2017 sec.), Gl. The lesion appears to be completely excised.

UICC 2017: pT2

Case Discussion

The neuroendocrine tumors of the pancreatic pancreas, pNET, represent a significant challenge for the radiologist, who is equally committed to evaluating local extension and remote localization in malignant, generalized forms that are not syndromic and therefore frequently at an advanced stage of diagnosis, or to researching very small lesions in patients with the typical clinical syndrome.

In particular, these tumors are often associated with congenital/hereditary syndromes (e. g. MEN 1, VHL, etc.) with a more frequent multifocal presentation.
The distinction between neuroendocrine cancer and other forms of solid pancreatic cancer, such as adenocarcinoma, is crucial from a diagnostic point of view for different prognosis and is based on its histological peculiarities.

The presence of numerous blood vessels in the context of stroma collagen determines the typical hypervascular aspect of imaging, most frequent in small tumors.

However, other factors play a role in defining the characteristics of these tumors, first of all the following dimensions: the tumors of more significant size are those that often undergo bleeding and necrosis, with consequent structural inhomogeneity and calcium deposits, and in which signs of local extension are associated with vascular infiltration, lymphadenopathy and metastasis at a distance.

The case presented has a double challenge considering the small rounded-shape mass inside the tail of the pancreas suspected to be a cystadenoma (not yet confirmed)

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