ABSTRACT
ABSTRACT: Glucagonomas are rare types of pancreatic neuroendocrine tumors. They may present with a clinical entity called glucagonoma syndrome, which includes necrolytic migratory erythema as a skin component. Here we present a 26-year-old woman experiencing ongoing skin lesions, excessive weight loss, and nausea. She was diagnosed with metastatic glucagonoma. Her 68Ga-DOTATATE PET/CT showed increased uptake at the primary pancreatic lesion and hepatic metastases. She received 2 cycles of peptide receptor radionuclide therapy and had a partial response with a near-complete regression of her skin lesions.
Subject(s)
Glucagonoma , Necrolytic Migratory Erythema , Pancreatic Neoplasms , Adult , Erythema , Female , Glucagonoma/complications , Glucagonoma/diagnostic imaging , Glucagonoma/radiotherapy , Humans , Necrolytic Migratory Erythema/diagnostic imaging , Pancreatic Neoplasms/complications , Pancreatic Neoplasms/diagnostic imaging , Pancreatic Neoplasms/radiotherapy , Positron Emission Tomography Computed Tomography , Radioisotopes , Receptors, PeptideABSTRACT
A 56-year-old man presented with a history of 2 prior resections of a recurrent pancreatic glucagonoma in the past 4 years. Workup revealed new liver and abdominal nodal metastases with a rising serum glucagon level. He was started on peptide receptor radionuclide therapy with Lu DOTATATE, and his disease stabilized, while his glucagon levels decreased and also stabilized. After 4 induction and 2 maintenance cycles, he remains progression free for 23 months.
Subject(s)
Glucagonoma/radiotherapy , Neuroendocrine Tumors/radiotherapy , Octreotide/analogs & derivatives , Organometallic Compounds/therapeutic use , Pancreatic Neoplasms/radiotherapy , Radiopharmaceuticals/therapeutic use , Humans , Male , Middle Aged , Octreotide/therapeutic useABSTRACT
This is a rare case of a patient with type 1 multiple endocrine neoplasia (MEN-1) syndrome. The case is further unusual in that the glucagonoma debuted with two synchronic pancreatic masses at the time of diagnosis and with pulmonary metastases as the primary site of metastasis and not the more usual site of the liver (AU)