ABSTRACT

Duodenal gangliocytic paraganglioma is a rare tumorthat characteristically occurs in the second portion ofthe duodenum and typically presents with gastrointesti-nal bleeding. Duodenal gangliocytic paraganglioma ha-ve a good prognosis after surgical resection but metas-tatic spread to regional lymph nodes and recurrencemay rarely occur. A 41-year-old man underwent pan-creaticoduodenectomy for the proposed diagnosis ofcarcinoma of the ampulla vateri. Macroscopically, thetumor measuring 2,7 cm in its greatest diameter, loca-ted in the ampulla without infiltration of the pancreaswas detected. Histologically, the tumor composed ofepitheloid cells, ganglion cells with abundant cytoplasmand vesicular nuclei, and spindle cells arranged in bro-ad fascicles. Immunohistochemically, tumor cells werestrongly 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 gangliocyticparaganglioma and review the differential diagnosis ofthis infrequent tumor.

Key words: Paraganglioma, gangliocytic, gastrointesti-nal tract

ÖZET

Duodenal gangliyositik paragangliyom, karakteristikolarak duodenumun ikinci k›sm›nda görülen ve tipikolarak gastrointestinal kanama ile ortaya ç›kan nadirbir tümördür. Cerrahi rezeksiyon sonras› prognoz iyi-dir, ancak bölgesel lenf nodu metastaz› ve rekürrensnadiren görülebilir. Ampulla vateri karsinomu ön tan›-s› ile 41 yafl›ndaki erkek hastaya pankreatikoduodenek-tomi yap›ld›. Makroskopik olarak pankreas› infiltre et-meyen 2,7 cm uzun çapl› kitle saptand›. Histolojik ola-rak tümör, epiteloid hücreler, veziküler nükleuslu veeozinofilik sitoplazmal› gangliyon hücreleri ve fasikül-ler oluflturan i¤si hücrelerden meydana gelmekteydi.‹mmünhistokimyasal olarak tümör hücrelerinde S-100protein, sitokeratin AE1/ AE3 kokteyli, kromogranin Ave sinaptofizin ile boyanma gözlendi. Bölgesel lenf nodumetastaz› görülmedi. Bu çal›flmada nadir görülen birtümör olan duodenal gangliyositik paragangliyom olgu-su, ay›r›c› tan› özellikleri ile tart›fl›ld›.

Anahtar sözcükler: Paragangliyom, gangliyositik, gas-trointestinal sistem

INTRODUCTION

Gangliocytic paraganglioma (GP) is a raretumor occurring exclusively in the second porti-on of the duodenum (1,2). Although these tu-mors generally have a benign clinical course, li-mited number of cases metastasized to lymphnodes 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 beenreported (6). We report herein a case of duode-nal GP and discuss differential diagnosis of thisrare tumor from histopathological perspective.

CASE REPORT

A 41-year-old man was admitted to an ex-ternal hospital with complaints of dyspepsia andweakness. Laboratory findings showed iron de-ficiency anemia. Endoscopic examination andcomputed tomography (CT) revealed an ulcera-

Duodenal gangliocytic paraganglioma: A casereport

Duodenal gangliyositik paragangliyom: Olgu sunumu

Gülflah KAYGUSUZ1, Hatice GERMEN1, Onur B‹RSEN2, Kürflat KARADAYI2, Hilmi KOCAO⁄LU2,Esra ERDEN1

Ankara University, School of Medicine, Departments of Pathology1 and Surgery, Section of Surgical Oncology2, ANKARA

Corresponding Author: Gulsah Kaygusuz, M.D., Ankara Uni-versity, School of Medicine Department of Pathology MorfolojiBinasi, Sihhiye, 06100, Ankara, Turkey

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ted polypoid mass in the ampullary region (Fig.1). After endoscopic biopsy specimen diagno-sed as adenocarcinoma, the patient transferredto our hospital. The patient underwent pancre-aticoduodenectomy, regional lymphadenec-tomy, omentectomy, and cholesistectomy. Mac-

roscopically, a polypoid tumor, 2,7 cm in grea-test diameter located in ampulla vateri withoutinfiltration of the pancreas was detected. Surgi-cal specimen was fixed in neutral buffered for-malin and embedded in paraffin.

Tissue sections were stained with routinehematoxylin and eosin (HE). Histological exa-mination revealed a tumor extending into thesubmucosa and muscularis propria with focalmucosal ulceration. The tumor was composedof epitheloid cells, arranged in trabecular or pse-udoglandular pattern, spindle cells, and gangli-on cells with abundant cytoplasm and vesicularnuclei (Fig. 2a-e). There was no significant mi-totic activity, necrosis or infiltration of the pan-creas. No metastasis was found in the regionallymph nodes and omentum .

Immunohistochemical analyses were per-formed on tissue sections using Ventana Auto-mated Immunostainer. The antibodies used inc-luded: cytokeratin AE1/ AE3 cocktail (1:100 di-

Figure 1. Endoscopically, an ulcerated polypoid tumor in theperiampullary region.

Figure 2. a. Duodenal submucosal location of the tumor. (HE x100). b. Tumor has a triphasic morphology. (HE x 200). c. Epitheloidtumor 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).

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lution, Neomarkers), chromogranin A (monoc-lonal, 1:1000 dilution, Neomarkers), synaptoph-ysin (27G12, 1:200 dilution, Novocastra), S-100(4C4.9, 1:200 dilution, Neomarkers), cytokera-tin 7 (OV-TL12/30, 1:150 dilution, Neomar-kers), cytokeratin 20 (Ks20.8, 1:100 dilution,Neomarkers), and CEA (monoclonal, 1:1000 di-lution, Neomarkers). Immunohistochemically,the epitheloid cells were positive for cytokeratinAE1/ AE3 cocktail, chromogranin A, andsynaptophysin (Fig. 3b-d). S-100 protein labe-led the sustentacular cells and the spindle cellcomponent (Fig. 3a). Ganglion cells were posi-tive for synaptophysin (Fig. 3b). The tumor cells

were negative for cytokeratin 7, cytokeratin 20,and CEA.

DISCUSSION

Gangliocytic paraganglioma is an extre-mely rare benign neuroendocrine tumor of thegastrointestinal tract. Most GPs are characteris-tically located in the second portion of the du-odenum with a predilection for the ampulla va-teri, as in this case. Duodenal GP has been re-ported in patients 15 to 84 years of age with amale predominance. The patients with GP clini-cally present with abdominal pain, gastrointesti-

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

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Duodenal gangliocytic paraganglioma: A case report

nal bleeding, or they can be asymptomatic. CTfinding of duodenal GP is that of a polypoid orsessile solid mass (7), as in our case.

Histologically, GP is an uncapsulated be-nign triphasic tumor, composed of epitheloidcells, ganglion cells and spindle cells in variab-le proportions. The general pattern is characteri-zed by the features of carcinoid, paraganglioma,and ganglioneuroma. The tumors show no nec-rosis or conspicuous mitotic activity. Immuno-histochemically, the epitheloid cells are positivefor NSE, synaptophysin, chromogranin A andsometimes positive for cytokeratins, similar toour case. The ganglion cells express NSE andsynaptophysin whereas spindle cells positivelystain for S-100 protein. Although there are manyhypothesis about the histogenesis of GPs, thereis no clear explanation yet.

It may be difficult to make a differentialdiagnosis, if endoscopic biopsy specimens donot contain all three histologic components. Thespindle cell tumors, epithelial tumors or gangli-oneuroma may be recognized according to thepresence of three related different elements, asin the current case. The histological differentialdiagnosis of duodenal GP includes well-diffe-rentiated neuroendocrine carcinoma, ganglione-uroma, paraganglioma, and spindle cell malig-nancies (nerve sheath, smooth muscle, and gas-trointestinal stromal tumors) (8). The lack of im-munohistochemical S-100 protein positivespindle cells and ganglion cells favor the diag-nosis of well-differentiated neuroendocrine car-cinoma. Beside immunohistochemical profile ofthe tumor, CD117 and CD34 negativity, and thepresence of ganglion cells exclude the diagnosis

of gastrointestinal stromal tumor. Immunohis-tochemical expression of smooth muscle mar-kers is not a diagnostic feature of duodenal GP.Typical duodenal submucosal location withoutinfiltration of the pancreas, without any lymphnode metastasis of the tumor, triphasic morpho-logy, immunohistochemical positivity forsynaptophysin, chromogranin A, S-100 proteinand cytokeratins of the tumor cells are the mostimportant diagnostic features of GP.

Although GPs are accepted as a benign tu-mors, careful assessment is necessary for recur-rences or metastases.

REFERENCES

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