Turkish Journal of Pathology

Türk Patoloji Dergisi

Turkish Journal of Pathology

Turkish Journal of Pathology

2007, Vol 23, Num, 2     (Pages: 107-110)

Duodenal gangliocytic paraganglioma: A case report

Gülşah KAYGUSUZ 1, Hatice GERMEN 1, Onur BİRSEN 2, Kürşat KARADAYI 2, Hilmi KOCAOĞLU 2, Esra ERDEN 1

1 Ankara University, School of Medicine, Department of Pathology, ANKARA
2 Ankara University, School of Medicine, Department of Surgery, Section of Surgical Oncology, ANKARA

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Summary

Duodenal gangliocytic paraganglioma is a rare tumor that characteristically occurs in the second portion of the duodenum and typically presents with gastrointestinal bleeding. Duodenal gangliocytic paraganglioma have a good prognosis after surgical resection but metastatic spread to regional lymph nodes and recurrence may rarely occur. A 41-year-old man underwent pancreaticoduodenectomy for the proposed diagnosis of carcinoma of the ampulla vateri. Macroscopically, the tumor measuring 2,7 cm in its greatest diameter, located in the ampulla without infiltration of the pancreas was detected. Histologically, the tumor composed of epitheloid cells, ganglion cells with abundant cytoplasm and vesicular nuclei, and spindle cells arranged in broad fascicles. Immunohistochemically, tumor cells were strongly positive for S-100 protein, cytokeratin AE1/AE3 cocktail, chromogranin A, and synaptophysin. There was no metastasis in the regional lymph nodes. We present here a rare case of duodenal gangliocytic paraganglioma and review the differential diagnosis of this infrequent tumor.

Introduction

Gangliocytic paraganglioma (GP) is a rare tumor occurring exclusively in the second portion of the duodenum[1,2]. Although these tumors generally have a benign clinical course, limited number of cases metastasized to lymph nodes or recurred locally have been reported[3,4]. Dahl et al[5] first described this tumor in 1957, and since more than 130 cases have been reported[6]. We report herein a case of duodenal GP and discuss differential diagnosis of this rare tumor from histopathological perspective.

Case Presentation

A 41-year-old man was admitted to an external hospital with complaints of dyspepsia and weakness. Laboratory findings showed iron deficiency anemia. Endoscopic examination and computed tomography (CT) revealed an ulcerated polypoid mass in the ampullary region (Fig. 1). After endoscopic biopsy specimen diagnosed as adenocarcinoma, the patient transferred to our hospital. The patient underwent pancreaticoduodenectomy, regional lymphadenectomy, omentectomy, and cholesistectomy. Macroscopically, a polypoid tumor, 2,7 cm in greatest diameter located in ampulla vateri without infiltration of the pancreas was detected. Surgical specimen was fixed in neutral buffered formalin and embedded in paraffin.

Tissue sections were stained with routine hematoxylin and eosin (HE). Histological examination revealed a tumor extending into the submucosa and muscularis propria with focal mucosal ulceration. The tumor was composed of epitheloid cells, arranged in trabecular or pseudoglandular pattern, spindle cells, and ganglion cells with abundant cytoplasm and vesicular nuclei (Fig. 2a-e). There was no significant mitotic activity, necrosis or infiltration of the pancreas. No metastasis was found in the regional lymph nodes and omentum.

Immunohistochemical analyses were performed on tissue sections using Ventana Automated Immunostainer. The antibodies used included: cytokeratin AE1/ AE3 cocktail (1:100 dilution, Neomarkers), chromogranin A (monoclonal, 1:1000 dilution, Neomarkers), synaptophysin (27G12, 1:200 dilution, Novocastra), S-100 (4C4.9, 1:200 dilution, Neomarkers), cytokeratin 7 (OV-TL12/30, 1:150 dilution, Neomarkers), cytokeratin 20 (Ks20.8, 1:100 dilution, Neomarkers), and CEA (monoclonal, 1:1000 dilution, Neomarkers). Immunohistochemically, the epitheloid cells were positive for cytokeratin AE1/ AE3 cocktail, chromogranin A, and synaptophysin (Fig. 3b-d). S-100 protein labeled the sustentacular cells and the spindle cell component (Fig. 3a). Ganglion cells were positive for synaptophysin (Fig. 3b). The tumor cells were negative for cytokeratin 7, cytokeratin 20, and CEA.

Figure 1: Endoscopically, an ulcerated polypoid tumor in the periampullary region.

Figure 2: a. Duodenal submucosal location of the tumor. (HE x100). b. Tumor has a triphasic morphology. (HE x 200). c. Epitheloid tumor cells arranged in pseudoglandular pattern (HE x400). d. Ganglionic tumor cells with abundant cytoplasm and vesicular nuclei (HE x400). e. Spindle tumor cells (HE x400).

Figure 3: a. Immunohistochemical S-100 protein expression of the sustentacular and spindle cells (x 200). b. Synaptophysin labelled epitheloid cells and ganglion cells (x 200). c. Cytokeratin AE1/ AE3 staining of the tumor cells (x 200). d. Chromogranin A expression of the epitheloid tumor cells (x 200).

Discussion

Gangliocytic paraganglioma is an extremely rare benign neuroendocrine tumor of the gastrointestinal tract. Most GPs are characteristically located in the second portion of the duodenum with a predilection for the ampulla vateri, as in this case. Duodenal GP has been reported in patients 15 to 84 years of age with a male predominance. The patients with GP clinically present with abdominal pain, gastrointestinal bleeding, or they can be asymptomatic. CT finding of duodenal GP is that of a polypoid or sessile solid mass[7], as in our case.

Histologically, GP is an uncapsulated benign triphasic tumor, composed of epitheloid cells, ganglion cells and spindle cells in variable proportions. The general pattern is characterized by the features of carcinoid, paraganglioma, and ganglioneuroma. The tumors show no necrosis or conspicuous mitotic activity. Immunohistochemically, the epitheloid cells are positive for NSE, synaptophysin, chromogranin A and sometimes positive for cytokeratins, similar to our case. The ganglion cells express NSE and synaptophysin whereas spindle cells positively stain for S-100 protein. Although there are many hypothesis about the histogenesis of GPs, there is no clear explanation yet.

It may be difficult to make a differential diagnosis, if endoscopic biopsy specimens do not contain all three histologic components. The spindle cell tumors, epithelial tumors or ganglioneuroma may be recognized according to the presence of three related different elements, as in the current case. The histological differential diagnosis of duodenal GP includes well-differentiated neuroendocrine carcinoma, ganglioneuroma, paraganglioma, and spindle cell malignancies (nerve sheath, smooth muscle, and gastrointestinal stromal tumors)[8]. The lack of immunohistochemical S-100 protein positive spindle cells and ganglion cells favor the diagnosis of well-differentiated neuroendocrine carcinoma. Beside immunohistochemical profile of the tumor, CD117 and CD34 negativity, and the presence of ganglion cells exclude the diagnosis of gastrointestinal stromal tumor. Immunohistochemical expression of smooth muscle markers is not a diagnostic feature of duodenal GP.

Typical duodenal submucosal location without infiltration of the pancreas, without any lymph node metastasis of the tumor, triphasic morphology, immunohistochemical positivity for synaptophysin, chromogranin A, S-100 protein and cytokeratins of the tumor cells are the most important diagnostic features of GP. Although GPs are accepted as a benign tumors, careful assessment is necessary for recurrences or metastases.

Keywords : Paraganglioma, gangliocytic, gastrointestinal tract