3P’s of Wermer/MEN1 Syndrome on 68Ga-DOTANOC PET/CT Scan

Abstract

Wermer/MEN1 syndrome is composed of the triad of pancreatic, pituitary, and parathyroid (3P’s) tumors. We describe the 68Ga-DOTANOC PET/CT findings of a 43-year-old man, which revealed the classic 3P’s of MEN1 syndrome with somatostatin receptor expression. The patient further underwent surgery for parathyroid lesions, which were suggestive of adenomas.

Unusual Variant of Breast Cancer Presenting With Pituitary Symptoms Detected on 68Ga-DOTATATE PET/CT

“…1 Although physiological expression is seen in normal pituitary, pituitary adenomas also show intense uptake, hence DOTA PET/CT is a problem solving tool to further characterize sellar-suprasellar lesions on MR, in known extracranial primaries. [2][3][4] Intense DOTA uptake in sellar lesion is thus diagnostic for adenoma, but in our case, the extremely rare histology of neuroendocrine differentiation in conventional breast adenocarcinoma led to SSTR expression in not just pituitary but also other sites, which was diagnostic for metastases. Neuroendocrine differentiation in invasive breast cancer has been an underdiagnosed entity, 5 because immunohistochemistry markers for neuroendocrine tumor are not routinely performed for breast lesions.…”

“…Intense DOTANOC uptake is directly suggestive of high SSTR expression 1. Although physiological expression is seen in normal pituitary, pituitary adenomas also show intense uptake, hence DOTA PET/CT is a problem solving tool to further characterize sellar-suprasellar lesions on MR, in known extracranial primaries [2][3][4]. Intense DOTA uptake in sellar lesion is thus diagnostic for adenoma, but in our case, the extremely rare histology of neuroendocrine differentiation in conventional breast adenocarcinoma led to SSTR expression in not just pituitary but also other sites, which was diagnostic for metastases.…”

68Ga-DOTANOC PET/CT in Multiple Endocrine Neoplasia 1 With Associated Adrenocortical Carcinoma

“…The tumor cells also expressed immunoreactivity to cytokeratin and vimentin, and Ki67 index was approximately 2%. Multiple endocrine neoplasia 1 is an autosomal dominant endocrine syndrome with classic triad of parathyroid hyperplasia, pancreatic neuroendocrine tumors, and pituitary adenomas 1,2 . Adrenocortical carcinomas are rarely associated with MEN1 syndrome 3,4 .…”

“…Multiple endocrine neoplasia 1 is an autosomal dominant endocrine syndrome with classic triad of parathyroid hyperplasia, pancreatic neuroendocrine tumors, and pituitary adenomas. 1,2 Adrenocortical carcinomas are rarely associated with MEN1 syndrome. 3,4 In 60% to 85% of adults with adrenocortical carcinoma, patients present with a large mass or symptoms related to mass effect.…”