Une cause rare d’hémorragie digestive haute : Paragangliome gangliocytique duodénal métastatique


La tunisie chirurgicale - 2011 ; Vol 21


 Le paragangliome gangliocytique est une tumeur rare qui se développe presque exclusivement au niveau du deuxiè- me duodénum. Sa dénomination dérive de son aspect histologique très particulier associant, à des proportions variables, 3 types de cellules : les cellules de Schwann, les cellules ganglionnaires et des cellules épithélioïdes. Il est habituellement de bon pronostic mais des métastases ganglionnaires régionales peuvent survenir. Nous en rapportons un cas révélé, chez une femme de 56 ans, par une hémorragie digestive haute, et traité par une duodénopancréatectomie céphalique avec un curage ganglionnaire. Les métastases ganglionnaires étaient également formées par les 3 types de cellules. Uniquement huit cas de métastases ganglionnaires accompagnant un paragangliome gangliocytique duodénal ont été publiés. Nous avons revu tous ces cas et on se propose de dégager les aspects diagnostiques et thé- rapeutiques de cette entité rare.

Mots Clés

hématémèse, duodénum, paragangliome, métastases ganglionnaires, duodénopancréatectomie céphalique.

Introduction :

Gangliocytic paraganglioma is a rare tumor encountered almost exclusively in the second portion of the duodenum. The name is derived from its unique histologic features of carcinoid tumor, paraganglioma and ganglioneuroma. The lesion is composed of 3 types of cells : spindle cells, ganglion-like cells, and epithelioid cells. These sessile or polypoid tumors most often present with abdominal pain, gastrointestinal hemorrhage, or obstruction or are discovered incidentally. It is generally considered benign and may be cured by simple excision of the tumor mass. In rare instances, metastatic spread to regional lymph nodes has been reported. We report a case of duodenal gangliocytic paraganglioma with regional lymph node metastasis that was treated by pancreaticoduodenectomy and lymph node dissection.


A 56-year-old woman with a history of type II diabete and drug-controlled coronary disease was referred to our department because of hematemesis and melena with anemia (hemoglobin at 6.6 g/dl). The duodenoscopy revealed a 4 cm submucosal tumor occupying the third of the circumference of the second duodenum with ulce ration in its center, there was no evidence of active hemorrhage. Standard forceps biopsy specimens were negative for malignancy. Abdominal computed tomography (CT) showed a 7.5 cm tissular mass of the second duodenum, smoothly outlined with thin peripheral calcification and enhancing homogeneously after injection of contrast (figure 1a). There was also a 10 mm pre-duodeno-pancreatic lymphadenopathy (figure 1b). There was not dilation of biliary ducts. In the segment III of liver, there was an hypodense lesion not enhanced after injection of contrast at the arterial phase and disappearing at the late phase (figure 1c-d). The presumed diagnosis was a duodenal stromal tumor with probable liver metastasis. At laparotomy, there was actually an 8 cm tumor of the second duodenum, adherent to the pancreatic head but still mobile. There were multiples periduodeno-pancreatic lymph nodes. There was no vascular invasion. The main bile duct was thin. The lesion of segment III of liver corresponded, in fact, to a hemangioma. A pancreaticoduodenectomy with lymph node dissection was then performed. A pancreatic fistula associated with intra-abdominal collection next to the pancreaticojejunal anastomosis occurred on the eleventh postoperative day. It was successfully managed conservatively, using somatostatin analogue, antibiotics and percutaneous drainage. Grossly, the surgical specimen consisted of the duodenum, distal stomach, head of the pancreas, main bile duct and gallbladder. There was a smooth, firm polypoid lesion in the second part of the duodenum reaching the genu superus, whose surface was covered by duodenal mucosa with large ulceration (figure 2). There was no gross evidence of capsule formation. Twelve regional lymph nodes were identified : ten retro-duodeno-pancreatic which the largest measuring 3 cm and two pre-duodeno-pancreatic measuring 1.5 cm. Microscopically, the tumor was present in the submucosa and invaded the lamina propria and muscularis propria (figure 3a). It was well circumscribed without capsule formation. It was composed of 3 cell types : epithelioid cells, spindle-shaped Schwann’s cells and ganglion cells (figure 3b). Mitotic activity and tumor necrosis were not observed. Seven of the twelve lymph nodes were metastatic (five retro-duodeno-pancreatic and two pre-duodeno-pancreatic) and were formed by the 3 types of cell, like primitive tumor (figure 3c). Surgical margins were healthy. In immunohistochemical study, the epithelioid and ganglion cells were positive for synaptophysin and chromogranin (figure 3d), the spindle cells were positive for S100 and all the cells were negative for c-Kit. Stromal tumor has been eliminated and diagnosis retained was a gangliocytic paraganglioma with lymph node metastasis. No further therapy was given. The patient was in good health without recurrence or metastatic disease 12 months after surgery.


Gangliocytic paraganglioma is a rare submucosal tumor of the gastrointestinal tract most commonly located in the second portion of the duodenum. Slight male predominance is reported. The age at presentation vary between 15 and 80 years (1). The tumor usually presents with gastrointestinal bleeding due to mucosal ulceration as experienced in the current case. Obstructive jaundice is less common. The histopathological diagnosis using biopsy specimens is difficult because of its submucosal location as illustrated in this case. Endoscopic ultrasonography (EUS) is not useful for diagnostic because of the absence of a pathognomonic pattern (2, 3). However, it provides accurate information about the local and regional staging of this tumor (4). Radiological findings on CT or magnetic resonance imaging (MRI) of a circumscribed solid homogenous enhancing lesion confined to the duodenum without biliary obstruction are not specific (5). Histologically, gangliocytic paraganglioma have a characteristic triphasic pattern with varying proportions of epithelioid cells, ganglion cells, and spindle cells (5). Using immunohistochemical studies, epithelioid cells are positive for low-molecular weight keratin, chromogranin, pancreatic polypeptide, neuron-specific enolase, serotonin and somatostatin. Ganglion cells are reactive to neuron-specific enolase, neurofilaments and synaptophysin. Spindle cells are always strongly positive for S-100 (1, 6, 7). Therefore, epithelial and ganglion cells have neuroendocrine features (7). These tumors are very often benign. However, there are reports of metastasis and tumor recurrence that suggest malignancy. There are 8 cases of lymph-node metastasis (8-15) and one case of metachrone distant bone metastasis (16) in the literature. Metastatic disease in 3 reports of lymph-node metastasis consisted purely of epithelioid cells (8-10). However, in the other reports, there were two (11) or all three (12 - 15) types of cells in the metastatic site (table 1). The current case was the ninth case of lymph node metastasis arising from duodenal gangliocytic paraganglioma. It was composed by 3 mixed types of cells. Thus, all 3 components of gangliocytic paraganglioma have malignant potential and not only the epithelioid one. A radical resection, represented by the pancreaticoduodenectomy with lymph node dissection, is the most appropriate treatment of these metastatic tumors (12). Local resection, surgically or more recently endoscopically, is not useful for these tumors but it’s reserved for small tumor without lymph-node metastasis (4, 17-20). Due to the rarity of the disease, no clear adjuvant treatment strategy has been determined in cases that demonstrate regional or distant metastasis. For this reason, no further treatment has been proposed for our patient.



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