PMC5478562_01
An endoscopic view of the duodenal tumor. An upward directed view showing a submucosal tumor with a smooth surface and a long stalk (A). An overhead view after spraying with indigo carmine shows the major papilla at the anal side of the stalk root (B). Forceps boring biopsies of the tumor, which were performed 22 times (C) [Hematoxylin and Eosin (H&E) staining, x12.5], demonstrated a very small number of ganglion-like cells infiltration (D) [H&E staining, x200; corresponding to the white square in (C)].
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Duodenoscopy demonstrated a pedunculated tumor with a smooth surface, at the duodenal minor papilla (Fig. 1A and B). The histological examination of multiple boring biopsies (Fig. 1C and D) of the tumor showed the infiltration of ganglion-like cells, which were positive for chromogranin-A and synaptophysin.
Endoscopic ultrasonography (EUS) showing a homogeneously low-echoic duodenal polyp of 17 mm in size, and the intact muscular propria of the duodenum (arrows).
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Endoscopic ultrasonography demonstrated that a tumor of 17 mm in size located within the submucosa with a homogeneously low echoic interior (Fig. 2).
Cross-section images. A high vascular tumor protruding into the duodenal lumen, as depicted by enhanced computed tomography (CT) (A: coronal view, B: horizontal view) and by enhanced magnetic resonance imaging (MRI) (C). Diffusion-weighted MRI showing a high-intensity signal at the tumor (D). White arrows indicate the tumor.
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Enhanced computed tomography (CT) and magnetic resonance image (MRI) (Fig. 3A-C) demonstrated the marginal enhancement of the tumor, but no metastasis. MRI showed a high-intensity signal within the tumor (Fig. 3D). Magnetic resonance cholangiopancreatography was normal, but the Santorini's duct was not visible. 18F-fluolodeoxyglucose positron emission tomography (FDG-PET) indicated no abnormal uptake in the duodenum, but the tumor was positive at the pulmonary apex area (SUV max: 3.70) (Fig. 4), which suggested malignancy.
18F-fluolodeoxyglucose positron emission tomography (FDG-PET) showing the abnormal uptake of FDG at the apex area of the left lung.
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MRI showed a high-intensity signal within the tumor (Fig. 3D). Magnetic resonance cholangiopancreatography was normal, but the Santorini's duct was not visible. 18F-fluolodeoxyglucose positron emission tomography (FDG-PET) indicated no abnormal uptake in the duodenum, but the tumor was positive at the pulmonary apex area (SUV max: 3.70) (Fig. 4), which suggested malignancy.
The histology of the resected duodenal tumor. A macroscopic view of the thin-sliced polyp demonstrating a whitish-yellow, well-demarcated tumor with an erosive area probably corresponding to the biopsy site (white arrow) (A). A loupe view of the polyp (B) [Hematoxylin and Eosin (H&E) staining]. A magnified view of the tumor histology showing three components: endocrine cells (C), ganglion cells (arrowheads) (D) and spindle cells (E) (H&E staining, x200). Santorini's duct running alongside the endocrine tumor component (arrowhead) (F) (H&E staining, x40).
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The macroscopic examination of the surgical specimen showed a whitish-yellow, well-demarcated tumor with erosion, probably corresponding to the biopsy site (Fig. 5A). The histological examination of the duodenal tumor showed a GP of 18 mm in size, which consisted of three neoplastic components: endocrine cells, spindle cells and ganglion-like cells (Fig. 5B-F).