Contrast-enhanced and colour-doppler ultrasonography of pancreatic and parathyroid involvement in multiple endocrine neoplasia type 1

Clinical History

Constipation and diffuse abdominal pain in a patient with multiple small stones in the renal parenchyma, a pancreatic mass and enlarged parathyroid glands on ultrasonographic examination.

Imaging Findings

The patient presented with constipation and diffuse abdominal pain. Abdominal ultrasonography showed the presence of multiple small stones in the renal parenchyma: nephrocalcinosis.

On axial ultrasonographic scanning along the pancreatic gland, a hypoechoic mass was identified at the pancreatic body with upstream dilation of the main pancreatic duct (Fig. 1a). After IV injection of ultrasound contrast agent (SonoVue, Bracco, Italy), in the earliest contrastographic phases, the pancreatic lesion became hyperechoic (Fig. 1b), with an echogenicity near that of the aorta. The tumour showed high vascularisation: an endocrine tumour. The results were confirmed on spiral CT (Fig. 1c). The pancreatic lesions became hyperdense in the arterial contrastographic phase (Fig. 1d).

Laboratory tests revealed the presence of hypercalcaemia with high values of parathyroid hormone (1270pg/ml; normal, 12-72pg/ml); moreover a level of chromogranin A of 1300ng/ml (normal, 19-98ng/ml) was demonstrated.

Neck ultrasonography highlighted an increase in volume of the parathyroid glands (Fig. 2a), which were seen to be highly vascularised on colour-doppler imaging (Fig. 2b) because of hyperplastic tissue: parathyroid adenomas.

A diagnosis of multiple endocrine neoplasia (MEN) type 1 was made. Pituitary gland involvement was excluded by means of laboratory tests and MR imaging. The patient was surgically treated, with a final diagnosis of pancreatic endocrine tumour.

Discussion

Multiple endocrine neoplasia (MEN) type 1 is quite a rare syndrome, occurring at a frequency of around 3-20 per 100000. It results from an inherited disorder that affects the endocrine glands. A genetic carrier is a person who has the MEN1 gene mutation, which is trasmitted directly to a child in 50 percent of cases.

MEN1 syndrome, also called endocrine adenomatosis or Wermer's syndrome, is characterised by hyperplasia and/or neoplasia of sometimes more than one group of endocrine glands such as the parathyroid, pancreatic and pituitary glands. However, MEN1 is the only MEN syndrome with frequent pancreatic involvement. Ultrasonographic diagnosis of pancreatic and parathyroid involvement is possible.

Differential Diagnosis List

Final Diagnosis

Endocrine pancreatic tumour and parathyroid adenomas in MEN1 syndrome

URL:	https://www.eurorad.org/case/2081
DOI:	10.1594/EURORAD/CASE.2081
ISSN:	1563-4086