Multidisciplinary approach including receptor radionuclide therapy with 90Y-DOTATOC ([90Y-DOTA0, Tyr3]-octreotide) and 177Lu-DOTATATE ([177Lu-DOTA0, Tyr3]-octreotate) in ectopic cushing syndrome from a metastatic gastrinoma: a promising proposal.

OBJECTIVE: To present a case of a young woman with Cushing syndrome caused by ectopic production of adrenocorticotropic hormone from a metastatic pancreatic gastrin-secreting endocrine carcinoma, who had a good response to combination peptide receptor radionuclide therapy. METHODS: We review the history, physical examination, laboratory investigations, and radiographic findings in this unusual patient. Moreover, the multimodal interventions are described and discussed. RESULTS: In a 38-year-old woman with typical signs of cortisol excess, laboratory studies revealed diabetes mellitus, hypokalemia, and high levels of adrenocorticotropic hormone, plasma cortisol, and urinary cortisol. Abdominal computed tomography showed a 4-cm pancreatic mass and multiple metastatic lesions in the liver, and ectopic Cushing syndrome was diagnosed. Treatment consisted of surgical debulking of the tumor, ketoconazole, somatostatin analogues, chemoembolization of the liver metastatic lesions, and peptide receptor radionuclide therapy with the radiolabeled somatostatin analogues 90Y-DOTATOC ([90Y-DOTA0, Tyr3]-octreotide) and 177Lu-DOTATATE ([177Lu-DOTA0, Tyr3]-octreotate). The 5 1/2-year follow-up showed positive results, which included complete regression of all clinical and hormonal evidence of the tumor and substantial decrease in the size and number of hepatic metastatic lesions. The patient achieved and still maintains an optimal quality of life. CONCLUSION: To the best of our knowledge, this is the first report of a multidisciplinary approach including peptide receptor radionuclide therapy with 90Y-DOTATOC and 177Lu-DOTATATE, which proved to be effective in improving clinical outcome in a case of metastatic endocrine carcinoma of the pancreas in conjunction with ectopic Cushing syndrome. In this unusual case, the patient has one of the longest durations of survival in this setting described in the literature.

Contrast-enhanced ultrasonography of small solid pseudopapillary tumors of the pancreas: enhancement pattern and pathologic correlation of 2 cases.

OBJECTIVE: The purpose of this series is to describe the value of contrast-enhanced ultrasonography (CEUS) in the characterization of small pseudopapillary tumors (SPTs) of the pancreas. METHODS: Two cases of SPTs found on conventional ultrasonography, both located at the pancreatic body, were studied with spiral computed tomography, magnetic resonance imaging, and CEUS. A final diagnosis was obtained with histologic analysis after distal pancreatectomy. Immunohistochemical analysis of the tumors with the CD34 antigen was performed. RESULTS: At CEUS, the 2 SPTs had a slight peripheral rim enhancement in the early dynamic phases. The presence of this enhancement pattern at CEUS, not clear at spiral computed tomography or at magnetic resonance imaging, suggested the diagnosis of SPTs with a peripheral pseudocapsule deriving from the compression of the pancreatic parenchyma. The presence of a perilesional pseudocapsule was proved at immunohistochemical analysis of the tumors with the CD34 antigen. CONCLUSIONS: Contrast-enhanced ultrasonography can improve the characterization of SPTs by showing the typical rim enhancement of these pancreatic tumors.

Contrast agents for hepatic magnetic resonance imaging.

The current availability of liver-specific contrast media (LSCM) allows the possibility to obtain an accurate diagnosis when studying focal liver lesions (FLL). It is necessary to have an in-depth knowledge of the biologic and histologic characteristics of FLL and the enhancement mechanism of LSCM to gain significant accuracy in the differential diagnosis of FLL. It is possible to subdivide FLL into three main groups according to the kinetics of contrast enhancement: hypervascular FLL, hypovascular FLL, and FLL with delayed enhancement. Dynamic contrast-enhanced magnetic resonance imaging is an important tool in the identification and characterization of FLL. LSCM with a first phase of extracellular distribution give both dynamic (morphologic) and late phase (functional) information useful for lesion characterization. With LSCM it is possible to differentiate with high accuracy benign from malignant lesions and hepatocellular from nonhepatocellular lesions. To understand contrast behavior after injection of LSCM, it is necessary to correlate contrast enhancement with the biologic and histologic findings of FLL.