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A 36-year-old man with a history of ulcerative colitis was referred to the visceral surgery department of our institution for further evaluation. Ultrasonography showed a well-circumscribed heterogeneous solid-appearing lesion of the pancreatic isthmus. Contrast-enhanced computed tomography (CT) confirmed the presence of an 18-mm rounded low-attenuation area in the inferior edge of the pancreatic isthmus (Figure 1A). Contrast-enhanced magnetic resonance imaging was thus performed. During the arterial phase, the mass was hypointense and showed a slight progressive enhancement, almost imperceptible on the portal phase (Figure 1B). No tumoral cells were obtained from endoscopic ultrasound-guided fine-needle aspiration. Pancreatic hormone secretion was normal. To investigate the possibility of a nonfunctioning atypical neuroendocrine tumor (NET), the patient underwent both somatostatin receptor scintigraphy (SRS) single photon emission CT (SPECT)/CT and 6-L-18F-fluorodihydroxyphenylalanine (FDOPA) positron emission tomography (PET)/CT. Although SRS was strictly normal (Figure 1C), PET/CT showed an increased focal uptake exclusively localized to the pancreatic nodule (Figure 1, D and E). The lesion was particularly evident thanks to carbidopa premedication reducing the physiological pancreatic FDOPA uptake (1). Hence, the diagnosis of pancreatic NET was done, and the patient was referred to surgery (Figure 1F). Unexpectedly, pathological examination revealed a solid pseudopapillary tumor (SPT) with low mitotic activity, strongly and diffusely positive for β-catenin, CD10, and vimentin, and negative for synaptophysin and chromogranin (Figure 1, G–I).

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Special features > Special Features - Image in Endocrinology
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