Safe endoscopic resection of gangliocytic paraganglioma of the major duodenal papilla

P. Sánchez-Pobre, S. Sáenz-López, S. Rodríguez, F. Sánchez, I. Alemany¹, G. López¹, F. Colina¹, P. Martínez-Montiel,
J. C. Marín, G. Castellano and J. A. Solís Herruzo

Services of Digestive Diseases and ¹Pathology. Hospital Universitario 12 de Octubre. Madrid. Spain

 

ABSTRACT

A case of gangliocytic paraganglioma of the papilla of Vater in a 76-year-old man with a history of recurrent obstructive jaundice is presented. This is the first case of gangliocytic paraganglioma of the major papilla succesfully resected by endoscopic ampullectomy.

Key words: Duodenal gangliocytic paraganglioma. Major duodenal papilla tumors. Endoscopic resection.

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Sánchez-Pobre P, Sáenz-López S, Rodríguez S, Sánchez F, Alemany I, López G, Colina F, Martínez-Montiel P, Marín JC, Castellano G, Solís Herruzo JA. Safe endoscopic resection of gangliocytic paraganglioma of the major duodenal papilla. Rev Esp Enferm Dig 2004; 96: 660-664.

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Recibido: 30-09-03.
Aceptado: 02-10-03.

Correspondencia: P. Sánchez-Pobre Berajano. Servicio de Medicina de Aparato Digestivo. Hospital Universitario 12 de Octubre. Avda. de Córdoba, s/n. 28041 Madrid. Telf.: 913 908 199. e-mail: psanchez@meditex.es

 

INTRODUCTION

Gangliocytic paragangliomas (PG) are rare gastrointestinal tumors that are found primarily in the second part of the duodenum (1,2). Histologically, they are composed of three types of cells: epitheloid cells, ganglion cells, and spindle cells. The more common presenting symptoms of these sessile or polypoid tumors are abdominal pain, gastrointestinal bleeding, and biliary obstruction; otherwise they are discovered incidentally (2,3). These tumors are usually benign and can be cured by local excision. However, the finding of regional lymph node metastases (4-6) or recurrence after incomplete excision has been reported in some cases. The endoscopic resection of periampullary gangliocityc paraganglioma has been described only when the minor papilla was involved (7).

We describe a case of PG of the major papilla without lymph node metastases that presented with recurrent obstructive jaundice and cholangitis. Endoscopic resection by endoscopic ampullectomy was successfully performed, with no later evidence of recurrence.

CASE REPORT

A 76-year-old man with a history of hypertension, infero-posterior myocardial infarction, and drug-controlled coronary three-vessel disease was attended in our department. He underwent subtotal gastrectomy for a perforated duodenal ulcer 25 years earlier. He was referred to our department because of recurrent cholestasis, abdominal pain, and an episode of acute cholangitis. A computed tomography (CT) and a magnetic resonance imaging (MRI) of the abdomen did not identify any obstructive lesion in the bilio-pancreatic tree. The patient underwent an endoscopic retrograde cholangiopancreatography (ERCP), which revealed a sessile polypoid lesion measuring 1.5 cm located at the papilla that caused obstruction of the biliary tree. Its surface was covered by normal duodenal mucosa. An endoscopic sphincterectomy of the papilla was performed (Fig. 1), and biopsy specimens from the lesion showed histological features consistent with gangliocytic paraganglioma. An ecoendoscopy confirmed the submucosal nature of the tumor and a lack of lymphatic spread.

With the diagnosis of PG of the papilla without lymph node involvement, excision of the lesion was decided, in spite of the benign course of the lesion, in order to avoid the potential risk of local growth or lymph node metastasis. Due to the high surgical risk of the patient and the localized nature of the lesion, the tumor was resected endoscopically by ampullectomy.

A histopathological evaluation of the duodenal polypoid lesion showed a well circumscribed tumor with rich vascularization. Microscopically, three different cell types were recognized: spindle cells arranged in fascicles; polygonal cells with granular eosinophilic cytoplasm and stippled nuclei with some nucleoli arranged in nests, and epitheloid cells with a trabecular or organoid growth pattern (endocrine growth pattern). There were also a number of larger polygonal cells with an eosinophilic cytoplasm and prominent nucleoli (ganglion cells). Necrosis, mitotic figures, and vascular invasion were absent (Fig. 2).

For the last 12 months the patient has been well and free of symptoms, without new episodes of cholestasis, with normal laboratory tests, and without ecoendoscopic signs of biliary obstruction, local recurrence or lymph node metastasis

DISCUSSION

PGs are rare neuroendocrine submucosal tumors of the gastrointestinal tract that are almost exclusively located in the second portion of the duodenum. In this location, they have been described in the major (5, 8-12) and in the minor papilla (7). There is a slight male predominance. They are usually diagnosed in the sixth decade of life, although patient ages have ranged from 15 to 80 years. The most common presenting symptom is gastrointestinal bleeding, but other cases presented with abdominal pain and, occasionally, biliary obstruction (2,8). Some patients are asymptomatic and tumors are found incidentally (2,5).

Endoscopy reveals a sessile or polypoid submucosal lesion (2). Histologically, it is generally not encapsulated and has a very characteristic pattern. It is typically composed of a variable proportion of ganglion cells with varying degrees of differentiation, spindle cells, and epitheloid cells (3). Epithelial cells are most numerous. They have a clear cytoplasm and form nests or cords extending to the mucosa and submucosa. Ganglion cells are sparse, constantly associated with spindle cells. Spindle cells are associated with epithelial cells as substentacular cells in an organoid pattern, or are aggregated into fascicles or surrounding ganglion cells (10). Using immunohistochemical studies, epitheloid cells are positive for low-molecular weight keratin, chromogranin, pancreatic polypeptide, neuron-specific enolase, serotonin, somatostatin… Spindle cells are always strongly positive for S-100, but also stain with neuron-specific enolase, neurofilaments and vimentine. Ganglion cells are reactive to neuron-specific enolase (1-3,13), neurofilaments and synaptophysin (10). Therefore, epithelial and ganglion cells have neuroendocrine features (10). Ultrastructural studies show neurosecretory granules and neurofilaments, supporting its neuroendocrine nature (10).

The histogenesis of this tumor is no clear. It has been suggested that the tumor arises from an embryonic celiac ganglion or from pluripotential intestinal stem cells, or that the lesion is due to a hammartomatous proliferation of endodermal cells at the ventral primordium of the pancreas, and neuroectodermally derived ganglion and Schwann cells. However, the finding of regional lymph node metastasis in several reported cases suggest that PG is a true neoplasm (1,4,5).

PG usually have a benign course, but cases of lymph node metastasis and local recurrence have been reported (5,6,9). In such cases, the lesion is usually larger than the tumor of the present case, ranging from 2 to 9 cm, and there are usually local invasion or tumor emboli in the submucosal lymph nodes. If there is evidence or suspicion of lymph node spread, the tumor should be surgically excised. No cases of distant metastasis or of mortality secondary to this tumor have been reported.

The histopathological diagnosis using biopsy specimens taken from the tumor prior to excision is difficult because of its submucosal location. Ecoendoscopy is not useful for the precise diagnosis of this tumor because of the absence of a pathognomonic pattern (7,14,15). However, it provides accurate information about the local and regional staging of this tumor.

This report describes a patient with a history of recurrent obstructive jaundice and an episode of acute cholangitis. CT and MRI did no identify the papillary lesion, which was revealed by ECPR instead. Biopsy specimens were diagnostic as a result of previous sphincterectomy, which allowed access to the submucosa.

The treatment of PG includes the complete excision of the submucosal lesion (2, 4). It is usually performed by surgery, but endoscopic resection has also been carried out with no evidence of recurrence in subsequent years (2,7). Surgical treatment with lymph node dissection is recommended for large lesions or when there is suspicion of lymph node involvement.

Endoscopic resection is recommended for small duodenal PGs that are localized and with no lymphatic spread (7). Tumors of the papilla can be resected by endoscopic ampullectomy (16). Endoscopic resections of PGs located at the minor papilla have also been reported (7). In the present case, the high surgical risk of the patient, the small size of the peripampullary lesion, and the absence of lymph node involvement were the reasons to perform an endoscopic ampullectomy. It was carried out with mild transient bleeding and no other complications. There have been no signs of recurrence at 1-year follow-up.

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