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Duodenal Gangliocytic Paraganglioma: A Unique Cause of Abdominal Pain

Reis, Daniela MD1; Damião, Filipe MD1; Noronha Ferreira, Carlos MD1; Cruz, Rafael MD2; Vitorino, Emília MD2; Carrilho Ribeiro, Luís PhD1; Tato Marinho, Rui PhD1

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doi: 10.14309/crj.0000000000000272
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CASE REPORT

A 56-year-old man presented with a history of epigastric pain and early postprandial satiety for 1 year. Esophagogastroduodenoscopy showed an 11-mm polypoid mass with central ulceration in the second portion of the duodenum, near the major ampulla (Figure 1). Multiple biopsy specimens were performed, but a histologic examination revealed only nonspecific inflammatory changes. Thoracoabdominal computed tomography did not identify any regional lymphadenopathy or distant lesions. Endoscopic ultrasound showed a normal major duodenal papilla without abnormal findings.

Figure 1.
Figure 1.:
Endoscopic view of the polypoid mass with central ulceration near the ampulla of Vater.

The lesion was removed by endoscopic mucosal resection in one piece with hot snare polypectomy after submucosal injection of a solution of normal saline with methylene blue (Figure 2). The mucosal defect was closed with 3 through-the-scope clips. No adverse events were registered. His histologic examination revealed a tumor located in the duodenal submucosa, and negative margins were confirmed. The tumor was composed mainly of spindle cells arranged in a nest pattern, epithelioid cells, and ganglion cells (Figure 3). By immunohistochemical staining, both ganglion and epithelioid cell types showed reactivity with neuron-specific enolase and synaptophysin and were negative for DOG-1 and cytokeratins. Spindle cells were positive for S-100 protein (Figure 4). These findings were consistent with duodenal gangliocytic paraganglioma (DGP).

Figure 2.
Figure 2.:
Endoscopic appearance of the scar after mucosal resection of the mass.
Figure 3.
Figure 3.:
Immunohistochemical staining showing ganglion cell (arrow).
Figure 4.
Figure 4.:
Immunohistochemical staining showing spindle cells positive for S-100 protein.

DGP is a rare tumor arising next to the ampulla of Vater in 90% of the cases.1,2 The most common clinical presentation includes gastrointestinal bleeding and anemia, but abdominal pain and obstructive jaundice can also occur.3 This type of neoplasm generally follows a benign course but is often confused with other gastrointestinal neoplasms such as gastrointestinal stromal tumors.2 Because of both different natural history and treatment of these 2 entities, accurate diagnosis is mandatory and only possible after histologic examination.1,2 Nevertheless, endoscopic biopsy alone may not help because of the submucosal location of the tumor.1

Typical histologic pattern of DGP is characterized by ganglion cells, epithelial cells originating from ventral primordium of the pancreas, spindle cells arranged in a nest pattern, and immunoreactivity to neuroendocrine markers.1,4 Positive staining for S-100 protein and synaptophysin and negative staining for discovered on gastrointestinal stromal tumours 1 help to differentiate DGP from gastrointestinal stromal tumor.1 Endoscopic mucosal resection is usually a safe and effective procedure, and it is generally easy to perform because of tumor protrusion into the duodenum.1,2,4 Surgery is indicated if endoscopic management is not possible.2 Although regional lymph node involvement can occur in up to 7% of patients requiring an aggressive surgical approach, tumor recurrence is rare.1,2 Endoscopic and imaging surveillance strategy after resection should be individualized and discussed in multidisciplinary meetings.2,4

DISCLOSURES

Author contributions: D. Reis wrote the manuscript and reviewed the literature. C. Noronha Ferreira revised the manuscript for intellectual content and is the article guarantor. E. Vitorino provided the pathology images. F. Damião, R. Cruz, L. Carrilho Ribeiro, and R. Tato Marinho approved the final version.

Financial disclosure: None to report.

Informed consent was obtained for this case report.

REFERENCES

1. Loftus TJ, Kresak JL, Gonzalo DH, Sarosi GA Jr, Behrns KE. Duodenal gangliocytic paraganglioma: A case report and literature review. Int J Surg Case Rep. 2015;8:5–8.
2. Papaconstantinou D, Machairas N, Damaskou V, Zavras N, Kontopoulou C, Machairas A. Duodenal gangliocytic paraganglioma, successfully treated by local surgical excision-a case report. Int J Surg Case Rep. 2017;32:5–8.
3. Rowsell C, Coburn N, Chetty R. Gangliocytic paraganglioma: A rare case with metastases of all 3 elements to liver and lymph nodes. Ann Diagn Pathol. 2011;15(6):467–71.
4. Yang JI, Choi JS, Lee GH, et al. A case of ampullary gangliocytic paraganglioma. Korean J Intern Med. 2014;29:375–8.
© 2019 The Author(s). Published by Wolters Kluwer Health, Inc. on behalf of The American College of Gastroenterology.