Sandeep Subhash Hedgire MBBS, Presenter: Nothing to Disclose

Anna Larson, Abstract Co-Author: Nothing to Disclose

Houman Sotoudeh MD, Abstract Co-Author: Nothing to Disclose

Shaunagh McDermott FFRRCSI, Abstract Co-Author: Nothing to Disclose

Elizabeth Anne Thiele MD, PhD, Abstract Co-Author: Nothing to Disclose

Mukesh Gobind Harisinghani MD, Abstract Co-Author: Nothing to Disclose

To evaluate the incidence of focal pancreatic lesions in tuberous sclerosis complex (TSC) patients.  

In this HIPPA compliant study, 709 cross sectional imaging studies, including contrast enhanced CT and MRI scans of 219 patients (92 males, 129 females) with tuberous sclerosis complex (TSC), were retrospectively analyzed to calculate the incidence of pancreatic lesions. The age range for this cohort was 0.1 to 73.3 years. These scans were performed over a period of 17.5 years and were primarily done for the evaluation of renal lesions in TSC. All studies were read by two radiologists and the following findings were recorded as consensus: presence or absence of focal lesions, characteristics of the lesion (cystic/solid), size on axial sections, enhancement post contrast, location and pancreatic duct status. When sequential scans were available, the lesions were assessed for change. Histopathology in patients who underwent resection was recorded. The imaging findings were correlated with clinical parameters and the TSC genotype.

Pancreatic lesions were seen in 8 patients (3.65%), five males and three females. The lesion had a cystic component in six patients. In 50 % of patients the lesion was present on the first scan and in the remaining 50 % it was seen on sequential imaging. The newly diagnosed lesions were seen at the following ages 7,18,35,56 years. Contrast enhancement was seen in 4 out of 8 lesions. Three pts underwent surgical resection and the histopathological examination revealed well differentiated pancreatic endocrine neoplasm. Two of these patients had a TSC2 germline mutation and the genetic status for the third remains unknown. Two pts with pancreatic neuroendocrine tumor were males (age 56, 19) and one was female (age of 21). Five percent of patients with a TSC2 mutation and 2.4 % of patients with a TSC1 mutation were found to have a pancreatic lesion. All patients experienced change in the largest diameter of the lesion. Increase in the size was noted in 4 patients, decrease in 3, and both increase and decrease in one pt.

Pancreatic neuroendocrine tumors and cysts are the two most commonly detected focal pancreatic lesions in TSC. In our cohort these were more frequent in patients with a TSC2 mutation.  

Pancreatic neuroendocrine tumors can be added to the spectrum of lesions associated with tuberous sclerosis complex.

Hedgire, S, Larson, A, Sotoudeh, H, McDermott, S, Thiele, E, Harisinghani, M, Focal Pancreatic Lesions in Tuberous Sclerosis Complex.  Radiological Society of North America 2011 Scientific Assembly and Annual Meeting, November 26 - December 2, 2011 ,Chicago IL. http://archive.rsna.org/2011/11006705.html