Gangliocytic paraganglioma derived from the neural crest is a peculiar neuroendocrine tumor. 88% of the reported lesions are located in the second part of the duodenum.1 Gangliocytic paraganglioma is characterized by benign behavior and a favorable outcome. Local excision is used to treat the disease and radical surgery and lymph node dissection can be avoided if gangliocytic paraganglioma is confirmed. Here we report a 67-year-old man with gangliocytic paraganglioma of the duodenum. Local resection was performed. There were no signs of tumor recurrence at follow-up after 15 months.
A 67-year-old Chinese man came to Beijing Friendship Hospital on January 5, 2009 presenting with recurrent melena and iron deficiency anemia for about two weeks. Gastroscopy revealed a 3.0 cm round lesion with a nodose surface and erosion and edema in the region of the descending part of the duodenum (Figure 1). Tumor cells were not found on histological biopsy. Gastroscopy and barium meal confirmed the presence of the tumor (Figure 2). Abdominal computed tomography (CT) revealed a 2.8 cm low-density mass, which was unenhanced during a contrast-enhanced scan (Figure 3). CT also revealed a local stenotic intestinal tract in the descending part of the duodenum. However, no peripheral lymph node metastasis was found.
A complete gross excision of the mass was carried out in this patient on January 13, 2009. Gross inspection revealed that the 3 cm lesion, which was located in the wall of the descending part of the duodenum, was submucosal, nodular and whitish-gray. The tumor had an ulcerated tip. The patient made an uneventful recovery. Histology revealed the size of the lesion was 3 cm× 1 cm× 1 cm and it was composed of epithelioid cell nests, areas of spindle cells and scattered ganglion cells. A positive cellular reaction for chromogranin, CD56, synaptophysin and S-100 was demonstrated by immunohistochemistry (Figures 4, 5). The tumor was revealed to be a gangliocytic paraganglioma, an unusual lesion previously described in association with Von Recklingshausen’s neurofibromatosis.
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Figure 1. Photograph of gastroscopy. Gastroscopy revealed a round lesion in the region of the descending part of the duodenum (arrow).
Figure 2. Barium meal. A filling defect was located in the region of the descending part of the duodenum (arrow).
Figure 3. Computed tomography. A low-density lesion was observed with weak, uneven enhancement during the contrast-enhanced scan (arrow).
Figure 4. Hematoxylin and eosin (HE) staining. The tumor was composed of epithelioid cell nests, areas of spindle cells, and scattered ganglion cells (HE, original magnification ×40).
Figure 5. Immunohistochemical staining. A positivity for chromogranin (A), CD56 (B), synaptophysin (C), and S-100 (D) (Immunohistochemical staining, original magnification ×40).
Gangliocytic paraganglioma of the duodenum was first reported by Dahl et al2 in 1957. Kepes and Zacharias3 described the characteristics of light microscopy and electron microscopy findings. Gangliocytic paraganglioma of the duodenum is extremely rare. Less than 40 cases were reported from 1957 to 2010, determined by searching PUBMED with the key words: “Gangliocytic paraganglioma” and “Duodenum”. Only two cases have been reported in China. Upper gastrointestinal bleeding is the main symptom of gangliocytic paraganglioma of the duodenum; however, the symptoms can appear as abdominal pain or abdominal discomfort. Tumors can appear as polyps or lumps, and biopsy results are usually negative because the tumors are submucosal. The reported duodenal paragangliomas have been submucosal, benign, and non-functional. They are typically composed of, as in our case, epithelioid cell nests, areas of spindle cells and scattered ganglion cells.4 The sessile polypoid tumor in our case consisted of epithelial cells, neuroid spindle cells and gangliocytic elements. Neurosecretory granules were detected by electron microscopy in the epithelial cells. The neuroid spindle cells exhibited the ultrastructural feature of Schwann cells. The tumor was immunocytochemically characterized by S-100 reactivity of the spindle cells and numerous PP-reactive epithelial cells.
Gangliocytic paragangliomas should be removed by local excision. The majority of the reported duodenal gangliocytic paraganglioma were of benign nature; therefore, radical surgery or lymph node dissection could be avoided with disease confirmation. However, thorough investigation for lymph node metastasis and postoperative follow-up are needed. The patient remains with no evidence of recurrence or metastasis for 15 months.
1. Cohen T, Zweig SJ, Tallis A, Tuazon R, Reich M. Paraganglioneuroma of the duodenum. Report of a case with radiographic findings, angiographic findings and a review of the literature. Am J Gastroenterol 1981; 75: 197-203.
2. Dahl EV, Waugh JM, Dahlin DC. Gastrointestinal ganglioneuromas; brief review with report of a duodenal ganglioneuroma. Am J Pathol 1957; 33: 953-965.
3. Kepes JJ, Zacharias DL. Gangliocytic paragangliomas of the duodenum. A report of two cases with light and electron microscopic examination. Cancer 1971; 27: 61-67.
4. Cooney T, Sweeney EC. Paraganglioneuroma of the duodenum: an evolutionary hybrid. J Clin Pathol 1978; 31: 233-244.