PMC5312508_01
Axial CT-scan of the abdomen after IV contrast medium administration shows an oval shape (white arrow), well circumscribed mass in the third part of the duodenum, with maximum diameter of 3 cm.
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Initial work-up consisted of a computed tomography (CT)-scan imaging which revealed an intraluminal mass measuring 3 cm in diameter, and situated grossly at the junction of the third to the fourth portion of the duodenum (Fig. 1).
After catheterization of the common bile duct (white arrow), a longitudinal duodenal incision was performed to visualize the tumor (yellow arrow).
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Subsequently, a longitudinal duodenal incision was performed to allow direct visualization of the ampulla and the adjacent tumor (Fig. 2).
Circumscribed tumor with yellow to white cut surface.
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Macroscopically, after formalin fixation, a fairly circumscribed tumor with a yellow to white cut surface was seen (Fig. 3).
A circumscribed, but not encapsulated, tumor is noticed on scanning magnification (Haematoxylin and Eosin stain: 4x).
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Pathology review of the specimen revealed a fairly circumscribed tumor located in the duodenal submucosa and muscularis propria (Fig. 4), composed mainly of epithelioid cells with a variable admixture of spindle Schwann like cells and ganglion like cells (Fig. 5).
a) Staining for synaptophysin in epithelioid (endocrine) cells. b) Focal positivity for CKAE1/AE3 in epithelioid cells. c) S-100 highlights the spindle (Schwann like) component. d) NF positivity in both spindle and ganglion like cells.
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The epithelioid element of endocrine origin was diffusely positive for synaptophysin (Fig. 6a) and immunoreactive, albeit focally, for CKAE1/AE3 (Fig. 6b). In contrast, spindle cells were positive for S-100 (Fig. 6c) whereas both spindle and ganglions like cells were positive for neurofilament staining (Fig. 6d).