Laparoscopic resection of duodenal gangliocytic paraganglioma. A case report

Paraganglioma is an exceedingly rare tumour of the duodenum that arises in close proximity to the ampulla of Vater. To date a total of 133 cases of duodenal paraganglioma have been reported in the literature; of these, 27 (20%) were histologically gangliocytic paragangliomas. This neoplasm generally behaves in a benign fashion, although instances of recurrence and/or lymph node metastasis have been described. The treatment consists in endoscopic polypectomy or surgical resection in relation to the histological features and the macroscopic extent of the neoplasm. We present a case of a benign duodenal gangliocytic paraganglioma treated by a laparo-endoscopic approach. We report a case of gangliocytic paraganglioma in a 75-year-old woman admitted to the General Surgery Division of Aosta Regional Hospital (Aosta-Italy), complaining of melaena and anaemia. Upper gastrointestinal endoscopy followed by enteroscopy with a video-capsula, revealed a pedunculated neoplasm in the second portion of the duodenum, with ulceration of the overlying mucosa. Multiple biopsies were performed during the endoscopic examination and showed the cellular pattern of benign paraganglioma. After stabilisation of the patient's clinical status, we performed a resection of the neoplasm via a laparoscopic transduodenal approach and a concomitant intraoperative duodenoscopy. The histological features showed a gangliocytic paraganglioma without a malignant cell pattern. The size of the neoplasm was 4 cm. The resection margins were free of neoplastic infiltration. The postoperative stay was 9 days and there were no intraoperative or postoperative complications. The patient is currently in good health without any tumour recurrence. Transduodenal laparoscopic resection with intraoperative duodenoscopy is a valuable treatment for benign gangliocytic paraganglioma of the duodenum which is unresectable by upper gastrointestinal endoscopy. This approach affords the advantages of the minimally invasive technique and fulfils the surgical tenets of the open transduodenal approach, if en bloc resection of the neoplasm with the adjacent duodenal wall is performed.     

Duodenal gangliocytic paraganglioma as a radiological moving defect

BACKGROUND: Gangliocytic paraganglioma of the duodenum is a peculiar neuroendocrine tumor, often revealed as an incidental radiographic finding or as a result of gastrointestinal hemorrhage, due to frequent ulceration of the overlying mucosa. Most lesions are pedunculated and submucosal with distinctive histology, consisting of endocrine, ganglion and spindle-shaped Schwann cells. METHODS: A case of a duodenal gangliocytic paraganglioma is reported in a 50-year-old woman presenting with episodes of melena. Enteroclysis revealed a pedunculated tumor observed either in the second or third portion of the duodenum. Upper gastrointestinal endoscopy revealed a pedunculated ulcerated tumor which was subsequently confirmed by computed tomography. The tumor was simply excised through a duodenotomy. RESULTS: The size of the tumor was 4.5 x 3 x 1.5 cm. Immunohistochemical and ultrastructural features of the tumor classified it as a typical duodenal gangliocytic paraganglioma with positive cellular reaction for neuron-specific enolase, chromogranin, somatostatin, gastrin and S100. CONCLUSION: Forty-six months after surgery, the patient is well with no evidence of recurrence. The majority of the reported duodenal gangliocytic paragangliomas were of benign nature. However, the fact that in 4 isolated cases there was lymph node involvement indicates that thorough investigation for lymph node metastases is needed, as well as thorough postoperative follow-up.     

Gangliocytic Paraganglioma: Case Report and Review of the Literature

Gangliocytic paraganglioma is a rare tumor, which occurs nearly exclusively in the second portion of the duodenum. Generally, this tumor has a benign clinical course, although rarely, it may recur or metastasize to regional lymph nodes. Only one case with distant metastasis has been reported. We present a case of duodenal gangliocytic paraganglioma treated first by local resection followed by pylorus-preserving pancreaticoduodenectomy. Examination of the first specimen revealed focal nuclear pleomorphism and mitotic activity, in addition to the presence of three characteristic histologic components: epithelioid, ganglion, and spindle cell. In the subsequent pancreaticoduodenectomy specimen, there was no residual tumor identified in the periampullary area, but metastatic gangliocytic paraganglioma was present in two of seven lymph nodes. This case report confirms the malignant potential of this tumor. We review the published literature on gangliocytic paragangliomas pursuing a malignant course. We conclude that surgical therapy of these neoplasms should not be limited to local resection, as disease recurrence, lymph node involvement, and rarely distant metastasis may occur.     

An unusual case of duodenal obstruction-gangliocytic paraganglioma

Gangliocytic paragangliomas are rare tumors located in the gastrointestinal tract that are considered to be benign. They are composed of spindle-shaped cells, epithelioid cells, and ganglion-like cells. They usually present with abdominal pain, and/or gastrointestinal bleeding, and occasionally with obstructive jaundice. We report a case of obstruction in a 17-year-old female, which on histology was found to be a gangliocytic paraganglioma, with an extremely unusual presentation. Intraoperatively, the patient was found to have local tumor extension and regional lymph node invasion, and so she underwent a pylorus-preserving pancreaticoduodenectomy, with local lymph node clearance. We discuss the management of this unusual case and review the literature.     

Gangliocytic paraganglioma of the duodenum.

In spite of possessing microscopic features of invasiveness, the clinical behaviour of duodenal gangliocytic paraganglioma is benign. The operation of choice for removal of the tumour is local excision. This conservative approach was used in the case of a 70-year-old white women who presented with occult gastrointestinal bleeding. The unusual histologic features of this woman's tumour are discussed. Although the tumour appears to be aggressive no recurrence has been reported to date following adequate local excision of a duodenal gangliocytic paraganglioma.     

Duodenal gangliocytic paraganglioma,successfully treated by local surgical excision-a case report

IntroductionDuodenal gangliocytic paragangliomas are rare neoplasms often arising in proximity to the major duodenal papilla of Vater. These neoplasms are considered to have a benign behavior with lymph node metastases being a rare phenomenon and distant metastatic disease even more so. Resection of the tumor is the only definitive therapy.Presentation of caseA 67 year old male presented to a referring hospital with symptoms of fatigue and malaise. Evaluation with CT imaging revealed a 3.1 cm intraluminal mass situated grossly at the junction of the third with the fourth portion of the duodenum. The tumor was found to be situated near the ampulla of Vater and was excised through a longitudinal duodenotomy followed by myotomy of the sphincter of Oddi.DiscussionComplete resection of duodenal gangliocytic paragangliomas by surgical or endoscopic means is the only potential cure. Endoscopic removal is the first option and is both safe and adequate. Ηowever, localized excision may be utilized instead in those cases in which endoscopic removal is not possible or cannot achieve negative margins. Recurrent disease after complete resection is unlikely.ConclusionCases of duodenal gangliocytic paragangliomas are best managed with endoscopic resection. However, local surgical excision remains as a second-choice procedure. Adjuvant chemotherapy and radiotherapy are unnecessary after complete excision.     

Pulmonary gangliocytic paraganglioma: case report and comparative immunohistochemical study of related neuroendocrine neoplasms

The authors report a case of gangliocytic paraganglioma of the lung, which has not yet been described in a pulmonary neoplasm. A 75-year-old man underwent right middle and lower lobe lobectomy. A slightly yellowish mass was located at the bifurcation between the lower and middle lobe bronchus, protruding into the truncus intermedius. The neoplastic cells were composed of three cellular elements: uniform endocrine cells in a Zellballen arrangement, large ganglion-like cells within the nests of endocrine cells, and spindle-shaped cells arranged in streams to surround the nests. Each component exhibited the characteristic immunohistochemical properties, which were similar to those of the corresponding neuroendocrine neoplasms: Endocrine cells were positive for CAM 5.2, chromogranin A, and synaptophysin, like carcinoid tumor; ganglion-like cells were positive only for neurofilament, like ganglioneuroma; and spindle-shaped cells were positive for neurofilament and S-100 protein, like paraganglioma. These results agreed with those in gangliocytic paraganglioma of the duodenum. Pulmonary gangliocytic paraganglioma is similar to that in the duodenum, and is a hamartomatous proliferation of epithelial endocrine and neuronal cells of the bronchus.     

Gangliocytic paraganglioma of the pancreas with bone metastasis

We report the case of a 50-years-old man who underwent sequential curative resection for gangliocytic paraganglioma of the pancreas with metachronous sternal metastasis. Gangliocytic paraganglioma are mainly located on the duodenum but several localizations have been reported, including on the digestive tract. Locoregional lymph node metastases are possible and prove their malignant potential but, to our knowledge, this observation is the first documented case of distant metastasis. Their usually good prognosis supports complete resection of these tumors.     

Duodenal gangliocytic paraganglioma. An immunohistochemical and ultrastructural study and a hypothesis concerning its origin

We report the immunohistochemical and ultrastructural features of three duodenal gangliocytic paragangliomas and compare them with duodenal carcinoid, extra-adrenal paraganglioma, pheochromocytoma, and ganglioneuroma. The gangliocytic paraganglioma is characterized by polygonal or columnar epithelial cells, ganglion cells, and spindle cells. The epithelial cells stained for neurofilament, neuron-specific enolase, pancreatic polypeptide, and somatostatin in three cases; leu-enkephalin, molluskan cardioexcitatory peptide, and vasoactive intestinal peptide in two; and glucagon and insulin in one case each. The ganglion cells were positive for leu-enkephalin, neurofilament, neuron-specific enolase, pancreatic polypeptide, and somatostatin in three cases, and glucagon in one. The spindle cells stained for neurofilament, neuron-specific enolase, and S-100 protein. Although there was some overlap in immunoreactivity between the gangliocytic paraganglioma and the other tumors examined, our data indicate that the gangliocytic paraganglioma is a distinctive lesion. We propose that it is a hyperplastic or neoplastic proliferation of 1) endodermally derived epithelial cells originating from the ventral primordium of the pancreas, 2) neuroectodermal ganglion cells, and 3) neuroectodermal spindle cells (Schwann cells).     

Gangliocytic paraganglioma of the ampulla of Vater

A case of a bleeding duodenal gangliocytic paraganglioma (GPG) of the Papilla Vateri is reported. The 47 year old patient was treated successfully by partial pancreaticoduodenectomy. Histologically the GPG is characterized by three types of cells: epitheloid cells, ganglion-like cells and spindle cells. So far 81 cases are reported in the literature. Most cases of GPGs are benign, metastasis or recurrence is possible. This case showed an infiltrative growth pattern indicating the true neoplastic nature of this tumor.     

Aorto-Enteric Fistula: a Dilemma for the Endoscopist as a Rare Cause of Gastro-Intestinal Bleeding

A 78-year-old man with a history of aorta-femoral graft operation was admitted to the hospital with symptoms of syncope, melena and haematemesis. He reported several episodes of melena during the previous year for which he underwent repeated gastro-intestinal endoscopic examinations, which were unable to show the site of the gastrointestinal bleeding. The third upper gastro-intestinal endoscopic examination disclosed a yellowish ulcerative lesion with irregular borders in the third part of the duodenum, which was considered to be a fistula, between the aorta and the duodenum. The patient underwent an explorative operation that revealed an intact aortic graft, firmly adherent to the duodenal wall, and the duodenum that was eroded in the third portion. The duodenum was transected and a duodenoduo-denostomy was performed. Although re-bleeding did not occur, the patient died of sepsis eight days after the operation. Aorto-enteric fistulae can be missed due to the common practice of limiting the endoscopic examination to the second part of the duodenum and not considering them in the differential diagnosis of gastro-intestinal bleeding because of their rarity. Possibly, a number of prior endoscopic examinations may be inconclusive until a correct diagnosis is reached in most of the cases.     

Lymphangioma of the duodenum: Report of a case

A case of lymphangioma of the duodenum with a successful preoperative diagnosis is reported herein. A 76-year-old man who complained of tarry stool was found to have a hemorrhagic polypoid tumor in the third portion of the duodenum. The tumor was diagnosed histologically as cavernous lymphangioma by endoscopic biopsy. Since such a tumor is essentially benign, a partial resection of the duodenum including the tumor was performed. Therefore, an endoscopic biopsy seems to be valuable in the diagnosis of duodenal lymphangioma.     

Gangliocytic paraganglioma of the bronchus: a case report with follow-up and ultrastructural assessment

We report a case of gangliocytic paraganglioma of bronchus. A 54-year-old woman underwent bronchoscopy following two episodes of right lower lobe pneumonia over the previous 5 months with unresolved chest radiographic changes. A computerized tomographic scan showed a right lower lobe endobronchial lesion, and at bronchoscopy there was a mass partly occluding the lumen of the bronchus. The biopsy and subsequent bronchoscopic resection showed a tumor with morphologic, immunohistochemical, and ultrastructural features of paragangliomatous, gangliocytic, and Schwann cell differentiation consistent with a gangliocytic paraganglioma. The lesion was treated conservatively with bronchoscopic resection and laser therapy. Histopathologic examination of recurrent tumor at 6 months showed features consistent with paraganglioma. Ten months after initial diagnosis, there was no bronchoscopic evidence of residual tumor. The occurrence of gangliocytic paraganglioma in diverse sites gives cause for the reappraisal of the histogenesis of this fascinating lesion. The variable morphology of this lesion may be an expression of the potential for divergent differentiation of a pluripotent stem cell.     

Upper digestive haemorrhage of a rare cause: benign duodenal schwannoma

The upper gastrointestinal bleeding is a common cause of admission to hospital. Neurogenic tumors of the duodenum are extremely rare and represent an unusual cause of gastrointestinal hemorrhage. The treatment is endoscopic or surgical total excision. In case of ulcerated large tumors situated periampullary pancreaticoduodenectomy (Whipple's or Traverso-Longmire's) represents an efficient solution. In addition, their high index of malignancy justifies this trend. We present a case of schwannoma of the second portion of the duodenum with several episodes of upper GI bleeding treated by Trasverso-Longmire pancreaticoduodenectomy.     

Villous tumors of the duodenum.

Five cases of villous tumors of the duodenum are reported, all of which involve the ampulla of Vater. Three of the five lesions contained either infiltrating carcinoma or carcinoma in situ. Although preoperative endoscopic biopsy was performed on all tumors no malignancy was identified. Frozen sections done at the time of operation on the three patients with carcinoma also failed to identify malignancy. One patient underwent pancreaticoduodenectomy and four patients had local excision of the tumor. Three of the patients treated with local excision developed recurrence and two subsequently had pancreaticoduodenectomy. Because of the difficulty in making an accurate diagnosis and the chance of recurrence when local excision is employed, strong consideration should be given to pancreaticoduodenectomy as the initial form of treatment of these lesions.     

Two case of bilateral approach in laparoscopic pancreas-sparing distal duodenectomy for duodenal neoplasms arising from the distal duodenum

IntroductionLaparoscopic pancreas-sparing distal duodenectomy is a less invasive surgical therapy; however, the anatomical complexity of the duodenum increases the difficulty of laparoscopic procedures. We introduce our technique for laparoscopic pancreas-sparing distal duodenectomy for distal duodenal tumors.Presentation of casesA first patient was 47-year-old woman who had 30 mm of duodenal tumor which located in third portion of duodenum. A second patient was 66-year-old man who had 35 mm of submucosal tumor which located in the third portion of duodenum. Laparoscopic pancreas-sparing duodenectomy was performed using bilateral approach for both cases. We began by dissecting an avascular area on the right side of the transverse mesocolon to mobilize the second and third portions of the duodenum with the uncinate process of the pancreas. Next, from the left side, the jejunum and the fourth portion of the duodenum were fully mobilized orally from the surrounding tissue, connecting the dissection plane with the right-side area. The jejunum and duodenum were cut with a linear stapler. Intracorporeal reconstruction was performed in an overlapped manner. We performed this procedure in two patients. Operative time was 326 and 370 min, respectively. Patients were discharged on postoperative days 9–12 without postoperative complications.DiscussionDuodenal tumors are found increasingly often because of developments in endoscopic technology and techniques; therefore, establishing safe surgical procedures for duodenal tumor excision is imperative. Our surgical approach was simple and safe procedure.ConclusionLaparoscopic pancreas-sparing distal duodenectomy with a bilateral approach is a useful approach without wide mobilization of duodenum.     

Giant gangliocytic paraganglioma of the filum terminale. Case report

The clinical and pathological features of a giant cauda equina paraganglioma arising from the intradural filum terminale is described. Scattered mature large neurons characterized the tumor as a gangliocytic paraganglioma. Histologically, these neoplasms have considerable similarity with ependymoma and the diagnosis can be easily missed unless special techniques are employed.     

A case of vesical paraganglioma with intrapelvic multiplicity

A 35-year-old woman, who had a past history of hypertension due to paraganglioma of the urinary bladder and in the pelvis, was referred to us 12 years after the initial diagnosis of paraganglioma. Before coming to us and during the follow-up after enucleation of myoma uteri, she was again diagnosed as having a bladder tumor by a gynecologist. Cystoscopy revealed a non-papillary broad-based tumor, measuring 2 cm in diameter, on the trigone of the urinary bladder. Magnetic resonance imaging also showed two solid tumors, each measuring 2 cm in diameter, on the bilateral peri-vesical sides in the pelvis. Endocrinologic examination disclosed increased levels of serum and urinary catecholamines. Histopathologic examination revealed that the bladder tumor specimen obtained by transurethral resection was paraganglioma. She underwent partial cystectomy and resection of these intrapelvic tumors via a retroperitoneal approach. These tumors were histopathologically diagnosed as multiple paragangliomas. She has been followed up for 10 months after operation without any evidence of recurrence. Herein, we report this rare case of vesical paraganglioma and present a brief review of the relevant literature.     

Aorticopulmonary paraganglioma

We describe a 61-year-old woman with an anterior mediastinal paraganglioma. The patient complained of a chronic cough that had lasted about 6 months. Her chest computed tomography (CT) showed a highly enhanced mediastinal tumor in the aorticopulmonary window. We surgically resected this tumor thorough a left anterior thoracotomy. Although numerous feeding vessels and firm adhesions with the pericardium hindered the procedure, macroscopically complete resection was achieved. Postoperatively, the tumor was diagnosed as an aorticopulmonary paraganglioma. As these highly enhanced tumors in the anterior mediastinum on CT images are unusual, a differential diagnosis including mediastinal hemangioma, epithelioid hemangioendothelioma, Castleman’s disease, choriocarcinoma, metastatic tumor (especially from renal cell carcinoma), and paraganglioma should be considered.     

Duodenal Stromal Tumor: Report of a Case

Gastrointestinal stromal tumors are rare tumors of the gastrointestinal (GI) tract that arise from primitive mesenchymal cells. Gastrointestinal stromal tumors account for approximately 80% all of gastrointestinal mesenchymal tumors. Duodenal stromal tumors (DSTs) manifest with unexplained melena, pain, bleeding, anemia, sometimes a partial duodenal obstruction and, rarely, with obstructive jaundice. If the tumor is successfully treated, its prognosis is usually good because of its non-aggressive nature. If resected, the prognosis is favorable in a majority of cases, and it is much better than in carcinomas of the duodenum. In this article, we report a case of DST originating from the first and second portion of the duodenum. Our patient did not have any problems postoperatively and remained symptom-free at 18 months after surgery.