Duodenal Gangliocytic Paraganglioma Research Articles

Duodenal gangliocytic paraganglioma: A case report

Introduction: Duodenal gangliocytic paraganglioma (DGP) is a benign tumor that only warrants a surgical resection in the majority of cases. Case Report: We are reporting a case of 42-year-old man who consulted for significant weight loss, fatigue, anemia, and an on–off obstructive jaundice. Imageries showed a 5 cm peri-ampullary duodenal mass. We couldn’t have a diagnosis by endoscopic ultrasound (EUS), so we did a laparoscopic transduodenal tumorectomy and removed the entire mass, which came back as gangliocytic paraganglioma on pathology and immunohistochemistry. Gangliocytic paraganglioma is a benign tumor that most commonly occurs in men and is localized in duodenum. Neuroendocrine tumor, ganglioneuroma, paraganglioma, and schwannoma are the differential diagnoses. Histologic diagnosis is difficult to make. Immunohistochemistry is indispensable for the diagnosis. Often than not, we cannot make diagnosis by a simple biopsy, because the tumor has a submucosal location and three types of cells need to be present for diagnosis: spindle cells, ganglion cells, and epithelial cells. That’s why en-bloc resection of the tumor is frequently needed for accurate diagnosis. Endoscopic resection or laparoscopy is used depending on the characteristics of the tumor. In our case, the 5 cm peri-ampullary tumor warranted a laparoscopic resection. Generally, there is no role for adjuvant therapy in duodenal gangliocytic paraganglioma (DGP). But sometimes, radiotherapy, surgery, somatostatin analog are used for treatment depending on the features of DGP. There is no consensus on follow-up management, but experts agree on the necessity of frequent follow-ups. Conclusion: We believe it is important to include gangliocytic paraganglioma (GP) as a differential diagnosis in patients who present with duodenal masses, weight loss, and general state alteration.

A rare case of Composite Gangliocytoma/Neuroma and Neuroendocrine Tumor (CoGNET) of the Duodenum - A case report

Abstract Introduction/Objective The unique morphologic and clinical features of neuroendocrine tumors have attracted the attention of physicians, most especially pathologists for more than a century. Neuroendocrine tumor of the duodenum is a rare entity and only accounts for ~ 2% of gastrointestinal neuroendocrine tumors. Though it was initially classified as duodenal gangliocytic paraganglioma due to the presence of sustentacular cells and its association with ganglion cells, it has now been reclassified in 2022 WHO classification as “composite gangliocytoma/neuroma and neuroendocrine tumor (CoGNET) due to its clinical features and immunophenotypic characteristics. Hence, we report a rare case of CoGNET in the duodenum. Methods/Case Report We present a 72-year-old female with a past medical history of hypothyroidism, hyperlipidemia, hypertension, deep vein thrombosis, and pulmonary embolism presenting with a 1-day history of abdominal pain and vomiting. Esophagogastroduodenoscopy (EGD) was performed, and it was concerning for a duodenal mass and the specimen was biopsied. Results (if a Case Study enter NA) The biopsy consisted of multiple tan-red hemorrhagic soft tissue, which on microscopic examination showed a triphasic histological pattern which includes neuroendocrine cells, spindle-shaped Schwannian/ sustentacular cells, and neuronal ganglion cells. Immunohistochemical stains performed showed the tumor cells are strongly positive for synaptophysin, and chromogranin; moderately positive with AE1/AE3, and patchy positive with S100. Desmin, CD117, DOG-1, CD34, and GATA were all negative in the tumor cells; the Ki-67 proliferative index was 1%. These morphological and immunophenotypic features are consistent with CoGNET. Conclusion CoGNET is a rare entity in the gastrointestinal tract especially in the duodenum and given its recent reclassification in 2022 WHO classification; pathologists, surgeons, and physicians need to be aware of such rare entities to improve patient care.

A case of duodenal gangliocytic paraganglioma treated by endoscopic papillectomy

A case of duodenal gangliocytic paraganglioma treated by endoscopic papillectomy

Immunohistochemical distinction of paragangliomas from epithelial neuroendocrine tumors—gangliocytic duodenal and cauda equina paragangliomas align with epithelial neuroendocrine tumors

Distinction of paraganglioma (PGL) from epithelial neuroendocrine tumors (NETs) can be difficult as they can mimic each other by nested architecture and expression of neuroendocrine markers. In this study, we examined differential diagnostic markers in 262 PGLs (142 adrenal pheochromocytomas and 120 extra-adrenal PGLs), 9 duodenal gangliocytic PGLs and 3 cauda equina PGLs, and 286 NETs (81 GI, 78 pancreatic, 42 thoracic, 37 medullary thyroid carcinomas, and 48 high-grade NETs including 32 small cell carcinomas of lung). While keratin expression was nearly uniform in NETs with the exception of few tumors, extensive keratin expression was seen in only one PGL (<1%)and focal expression in 5% PGLs. GATA3 was present in >90% of PGLs but only in 2% of NETs, usually focally. Tyrosine hydroxylase (TH) was expressed in >90% of adrenal, abdominal, and thoracic PGLs but only in 37% of head and neck PGLs, reflecting their variable catecholamine synthesis. Focal or occasional extensive TH-expression was detected in 10% of NETs. CDX2 was a helpful discriminator seen in 28% of pancreatic and most GI NETs but in no PGLs. SOX10 detected sustentacular cells in 85% of PGLs and 7% of NETs, whereas GFAP detected sustentacular cells mainly in PGLs of neck and was absent in NETs. Duodenal gangliocytic PGLs (n=9) and all cauda equina PGLs (n=3) expressed keratins, lacked GATA3, showed no or minimal TH expression as some NETs, and contained SOX10 and S100 protein-positive spindle cells negative for GFAP. Ganglion-like epithelioid cells were keratin-positive and negative for TH and SOX10 differing from true ganglion cells. We conclude that duodenal gangliocytic and cauda equina PGLs have a NET-like immunoprofile and differ from ordinary PGLs. NETs can be distinguished from PGLs by their expression of keratins and general lack of GATA3, TH, and GFAP-positive sustentacular cells, and sometimes by expression of CDX2 or TTF1.

An Unusual Cause of Acute Pancreatitis in a Young Woman

Question: A 27-year-old woman presented to the emergency department with intermittent epigastric pain radiating to the back of one week’s duration. The pain was dull and associated with nausea. Her medical and family history was unremarkable. She denied any alcohol or drug intake. There was no history of fever, yellowish discoloration of the sclera or urine, or weight loss. Upon arrival, she had mild tachycardia (heart rate: 103 beats per minute). Physical examination revealed tenderness in the epigastric and right upper quadrant of the abdomen. The Murphy’s sign was negative, and there were no peritoneal signs. The remainder of the physical examination was unremarkable. Laboratory studies showed an elevated serum aspartate aminotransferase level of 176 IU/l, alanine aminotransferase of 240 IU/l, gamma-glutamyl transpeptidase of 757 IU/L, alkaline phosphatase of 308 IU/l, total bilirubin of 0.6 mg/dL, lipase of 6,641 U/L, amylase of 667 U/L, and high sensitivity C-reactive protein of 3.416 mg/L; white blood cell count, serum bilirubin, triglyceride, and calcium levels were all within normal limits. Contrast-enhanced computed tomography (CT) of the abdomen showed marked peripancreatic fat stranding, no dilatation of the biliary tract or pancreatic duct, and no evidence of regional lymphadenopathy (Figure A). An endoscopic retrograde cholangiopancreatography (ERCP) was carried out to rule out biliary pancreatitis. Duodenoscopy showed a 3-cm pedunculated tumor with a smooth surface and soft texture at the Vater’s papilla (Figure B and C). Endoscopic ultrasonography (EUS) revealed the tumor as smooth-margined, homogenous, hypoechoic lesion arising from the submucosal layer (Figure D). What is your diagnosis of the lesion of the Vater’s papilla?A.CholedochocoeleB.Neuroendocrine tumor (NET)C.Gastrointestinal stromal tumor (GIST)D.Gangliocytic paraganglioma Look on page 621 for the answer and see the Gastroenterology web site (www.gastrojournal.org) for more information on submitting your favorite image(s) to Practical Teaching Cases. The correct answer is D. Gangliocytic paraganglioma (GP) of the Vater’s papilla. The patient underwent surgical wedge resection of the tumor (Figure E). The histopathological examination showed the tumor located in the submucosa (Figure F, H&E, 40×). GP is typically composed of spindle cells, ganglion cells, and epithelioid cells with the appearance of neuroendocrine cells (Figure G, H&E, 200×; Figure H, H&E, 400×). There was no mitosis or necrosis in the tumor. Immunohistochemistry examination revealed that the neuroendocrine cells stained positive for CD56 and chromogranin A, and the spindle cells were immunoreactive for S100 protein. These findings confirmed the diagnosis of GP. Answer A is incorrect because choledochocele is an anechoic lesion on EUS. Gastrointestinal NETs are homogenously hypoechoic lesions arising from the second or third layer, sometimes with muscularis propria expansion (Answer B). The EUS imaging feature of a GIST is a hypoechoic mass arising from the muscularis propria, and larger GIST are sometimes composed of heterogeneous echo patterns and anechoic spaces (Answer C). However, the definitive diagnosis of the lesion requires histology. GP is a rare, generally benign neuroendocrine tumor. However, GP with regional lymph node involvement and distant metastasis have been reported.1Li B. Li Y. Tian X.Y. et al.Malignant gangliocytic paraganglioma of the duodenum with distant metastases and a lethal course.World J Gastroenterol. 2014; 20: 15454-15461Crossref PubMed Scopus (28) Google Scholar The most common site of the tumor is the second portion of the duodenum.2Okubo Y. Nemoto T. Wakayama M. et al.Gangliocytic paraganglioma: a multi-institutional retrospective study in Japan.BMC Cancer. 2015; 15: 269Crossref PubMed Scopus (28) Google Scholar, 3Okubo Y. Wakayama M. Nemoto T. et al.Literature survey on epidemiology and pathology of gangliocytic paraganglioma.BMC Cancer. 2011; 11: 187Crossref PubMed Scopus (74) Google Scholar Gastrointestinal bleeding—followed by abdominal pain and anemia—are the most common symptoms of the disease.3Okubo Y. Wakayama M. Nemoto T. et al.Literature survey on epidemiology and pathology of gangliocytic paraganglioma.BMC Cancer. 2011; 11: 187Crossref PubMed Scopus (74) Google Scholar GPs without distant metastasis are usually treated via endoscopic resection or pylorus-preserving pancreaticoduodenectomy.2Okubo Y. Nemoto T. Wakayama M. et al.Gangliocytic paraganglioma: a multi-institutional retrospective study in Japan.BMC Cancer. 2015; 15: 269Crossref PubMed Scopus (28) Google Scholar The patient recovered uneventfully and had no evidence of tumor recurrence after a surgical wedge resection of the duodenal GP at the one-year follow-up. In conclusion, GP may be an uncommon cause of acute pancreatitis. This rare and benign disease can be treated via endoscopic or surgical resection of the tumor. CME Exam 1: An Unusual Cause of Acute Pancreatitis in a Young WomanGastroenterologyVol. 157Issue 3Preview Full-Text PDF

Periampullary Gangliocytic Paraganglioma

Introduction: Gangliocytic paraganglioma (GP) is rare and difficult to be differentiated from other periampullary neoplasms. The clinical characteristics and optimal treatment of periampullary GPs have not been clarified. Method: The data pool for the analysis comprised of cases of periampullary GP encountered in our institution and sporadic cases reported in the English literature. Result: A total of 117 cases with periampullary GP were studied, including 2 from our institute, and among them, duodenal GP was the most common (53.0%). GP size ranged from 0.7 cm to 19.0 cm, with a median of 2.2 cm. The most common presenting symptom for overall periampullary GPs was epigastric pain in 49.5% cases, followed by gastrointestinal bleeding in 35.4% cases. Most (84.1%) of the periampullary GPs were benign, whereas 15.9% were malignant. Metastasis was noted in 26.3% of periampullary GPs, with 14.5% showing lymph node metastasis and 1.8% showing liver metastasis. Of the periampullary GP cases included, 30.1% were treated with pancreaticoduodenectomy, 40.6% with local excision, and 17.7% with endoscopic resection. Conclusion: Periampullary GP should be considered as a tumor with malignant potential. Endoscopic resection is the treatment of choice for most of the duodenal GPs, whereas pancreaticoduodenectomy is recommended for GPs with possible malignancy, such as large size, with submucosal invasion, or pancreatic GP.

Cytological Diagnosis of Duodenal Gangliocytic Paraganglioma on Ultrasound-Guided Fine Needle Aspirate: A Case Report with Review of the Literature

Cytological Diagnosis of Duodenal Gangliocytic Paraganglioma on Ultrasound-Guided Fine Needle Aspirate: A Case Report with Review of the Literature

Successful Endoscopic Resection of a Rare Minor Ampullary Duodenal Gangliocytic Paraganglioma: A Case Report

Duodenal gangliocytic paragangliomas (DGP) are extremely rare neuroendocrine tumors. We present a case of a successful endoscopic resection of an incidental DGP. A 61-year-old Caucasian male with a history of cholecystectomy, iron deficiency anemia and GERD was incidentally found to have a 1.3cm periampullary duodenal polyp without any lymphadenopathy on a routine CT scan. The findings were confirmed with an abdominal MRI. He denied abdominal pain, early satiety, nausea, diarrhea, constipation, flushing, wheezing, weight loss, melena or any family history of gastrointestinal malignancies. An EUS examination showed markedly enlarged minor papilla with a hypoechoic mass and intact muscularis propria. A prominent pancreatic duct (PD) was also visualized. The EUS was followed by an ERCP to resect the polyp and a 5Fx4cm stent was placed in the PD due to high suspicion of pancreatic divisum. The specimen was a tan-colored, well-circumscribed 1.3x0.8x1cm soft, polypoid mass. Histologically, it was mostly a submucosal mass with a focal mucosal component. It exhibited a triple cellular differentiation with the epithelioid tumor cells arranged in nests along with occasional ganglion-like cells and surrounding scant spindle cells without atypia. The margins were clean. The epithelioid cells stained strongly positive for synaptophysin and chromogranin, and weakly positive for keratin. They were negative for S100, gastrin and somatostatin. The spindle cells stained positive for S100, synaptophysin and were negative for keratin. The mitotic active was low with a Mib-1 proliferative index of 3%. A 6-month follow up EGD with minor ampullary biopsy showed no signs of dysplasia or malignancy. DGPs are extremely rare and non-functional neuroendocrine tumors. They are twice more prevalent in males and usually found in the 6th to 7th decades of life. They are mostly benign tumors with excellent prognosis post resection. However, there have been about 20 case reports with lymph node metastasis and 1 report of fatal metastatic DGP. Traditionally, pancreaticoduodenectomy has been the treatment of choice when lymph nodes are involved. However, endoscopic resection, as in this case, remains a safe alternative for a localized DGP. In the lack of official guidelines, more studies are required to understand the nature of the disease, optimal treatment and follow up duration.Figure: Duodenal Gangliocytic Paraganglioma, ultrasound view.Figure: Duodenal Gangliocytic Paraganlioma, endoscopic view.Figure: The ampulla post resection.

Endoscopic resection as a treatment for duodenal gangliocytic paraganglioma

Endoscopic resection as a treatment for duodenal gangliocytic paraganglioma

Gangliocytic paraganglioma: a multi-institutional retrospective study in Japan.

BackgroundGangliocytic paraganglioma (GP) is an extremely rare benign tumor that commonly arises from the second part of the duodenum. Since GP exhibit neither prominent mitotic activity nor Ki-67 immunoreactivity, this tumor is often misdiagnosed as neuroendocrine tumor (NET) G1 (carcinoid tumor). However, patients with GP may have a better prognosis than patients with NET G1. This fact emphasizes the importance of differentiating GP from NET G1, but few studies have reported the epidemiology and histopathology of GP because of its rarity. To differentiate GP from NET G1 with ease, we conducted a multi-institutional retrospective study analyzing the morphometric and immunohistochemical features of this tumor.MethodsSince only a limited number of patients with GP could be identified in our institute, we conducted a multi-institutional retrospective study of GP in Japan, which was approved by the Ethics Committee of our medical institute. The obtained tissue sections underwent detailed morphometric and immunohistochemical analyses. Additionally, to differentiate GP from NET G1 with ease, immunohistochemical findings were compared.ResultsIn our examination of 12 cases of duodenal GP, we found that epithelioid cells of GP exhibited positive reactivity for progesterone receptor and pancreatic polypeptide, whereas tumor cells of NET G1 were completely negative reactivity for both. Additionally, although GP is considered to be an extremely rare NET, we found that four (40.0%) of the ten patients at our institute with duodenal NET G1 actually had GP.ConclusionsAlthough GP is regarded as a rare NET, our results suggest that it accounts for a substantial percentage of duodenal NETs. Additionally, confirmation of immunoreactivity for progesterone receptor and pancreatic polypeptide can assist in differentiating GP from NET G1.

内視鏡的切除術を施行した十二指腸乳頭部gangliocytic paragangliomaの1例

内視鏡的切除術を施行した十二指腸乳頭部gangliocytic paragangliomaの1例

Duodenal gangliocytic paraganglioma in a dog: an unusual cause for posthepatic obstructive jaundice

Gangliocytic paraganglioma is a neoplasm with no reported cases in veterinary medicine and rarely described in humans that usually occurs in the second portion of the duodenum. The name is derived from its unique histologic features of carcinoid tumor, paraganglioma, and ganglioneuroma. We report on the clinical and cytohistological features of a duodenal gangliocytic paraganglioma in a miniature poodle dog with intestinal obstruction and severe jaundice. Upper gastrointestinal fibreoptic endoscopy was performed, and cytologic and histologic samples were obtained. Microscopically, a triphasic (epithelioid, spindle, and ganglion-like cells) neoplasm was observed. The dog died and was submitted to necropsy. Grossly, a firm mass was detected in the submucosa of the ampullary region and was associated with regional lymph node enlargement and obstruction of the major duodenal papilla. The present report is the first description of such tumor in animals, which has to be included in the differential diagnosis of intestinal obstructions and posthepatic jaundice in dogs.

Duodenal gangliocytic paraganglioma, a rare entity among GEP-NET: a case report with immunohistochemical and molecular study

Gastroenteropancreatic neuroendocrine tumors are the most incident neuroendocrine tumors. In the new WHO classification (2010) the embryological derivation of each neoplastic entity is one of the most important parameters. Gangliocytic Paraganglioma is a tumor originating in the hindgut, a rare neoplasm, generally affecting the second portion of the duodenum, the majority of which are benign.Cases of gangliocytic paraganglioma with local metastasis or local recurrence have also been reported.We describe a GP in a 48-year-old caucasian male with an unusual site (4th portion of duodenum) and an interesting immunohistochemical and molecular pattern. In particular, we examined the expression of some neuroendocrine markers and a marker of neuronal differentiation, NeuroD1, whose expression can help to better understand the nature of this neoplasia.Virtual slidesThe virtual slides for this article can be found here: http://www.diagnosticpathology.diagnomx.eu/vs/3720959161096807

Recurrent episodes of acute pancreatitis due to duodenal gangliocytic paraganglioma: report of a case.

Gangliocytic paraganglioma is a rare tumor, almost always located in the second portion of the duodenum, and manifested with upper gastrointestinal bleeding and abdominal pain. To date, only one case of duodenal gangliocytic paraganglioma presented with recurrent acute pancreatitis has been reported in the literature.We present a 72-year-old woman admitted to the hospital due to recurrent episodes of acute pancreatitis. Paraclinical examinations showed a polypoid mass in the second portion of duodenum which was removed surgically by local excision. The preoperative differential diagnosis was suggestive with gastrointestinal stromal tumor or adenoma. The histopathology examination revealed a duodenal gangliocytic paraganglioma. After a follow up period of seventeen months the patient remained without clinical evidence of tumor recurrence.Our case report draws attention to the need for including in our differential diagnosis of recurrent acute pancreatitis the mechanical obstruction of the pancreatic duct due to this tumor.

A case of a recurrent acute pancreatitis caused by duodenal gangliocytic paraganglioma

A case of a recurrent acute pancreatitis caused by duodenal gangliocytic paraganglioma

Endoscopic Resection as a Possible Radical Treatment for Duodenal Gangliocytic Paraganglioma: A Report of Four Cases

Gangliocytic paraganglioma (GP) is a rare, benign tumor which is usually found in the duodenum. We here report four recent cases of GP, with successful endoscopic resection in three cases, including a lesion on the ampulla of Vater. In all cases, each lesion had a stalk that facilitated removal using an endoscopic approach. Endoscopic mucosal resection is a feasible and safe treatment if the location, depth, and lymph node status are all favorable and is also helpful for definite diagnosis of unknown duodenal mass. To avoid morbidity resulting from open surgical resection, careful inspection for the peduncle of the GP will help determine the feasibility of endoscopic resection.

A Gangliocytic Paraganglioma Presenting as a Submucosal Tumor in the Ampulla

A gangliocytic paraganglioma (GP) is a rare benign neuroendocrine tumor. However, its origin remains unclear. It is seen most frequently in the second portion of the duodenum. At endoscopy, a GP characteristically appears as a pedunculated nodular submucosal tumor with erosions and surface ulcers. The histological diagnosis is usually made from endoscopic biopsies showing the presence of epithelioid, spindle, and ganglion cells. We experienced a case of GP in a 38-year-old female who was referred because of a possible ampullary tumor. The endoscopic images showed a 1.5-cm, oval ampullary tumor covered with normal mucosa. An endoscopic biopsy showed chronic duodenitis. The tumor was removed by an endoscopic papillectomy. We report a case of duodenal GP that presented as a submucosal tumor that was treated with a papillectomy and review the literature. (Korean J Med 2014;86:603-607)

十二指腸carcinoid tumorと術前診断された十二指腸gangliocytic paragangliomaの1例

十二指腸carcinoid tumorと術前診断された十二指腸gangliocytic paragangliomaの1例

Duodenal gangliocytic paraganglioma with lymph node metastasis and an 8-year follow-up

Gangliocytic paraganglioma (GP) is a rare tumor, usually located in the second part of the duodenum. On pathological examination, GP is characterized by the association of the histological features of paragangliomas, ganglioneuromas, and carcinoid tumors. Classical clinical presentations are upper gastrointestinal bleeding and abdominal pain. Preoperative diagnosis is difficult because of the submucosal site of the tumor, with usually negative mucosal biopsies. Endoscopic ultrasound helps establish the diagnosis and allows lymph node staging, which will guide the choice of the treatment. If GPs usually follow a benign course, metastatic spread to regional lymph nodes treated by surgical resection alone has been reported. We report a case of GP with lymph node metastases treated by duodenopancreatectomy with long-term disease-free survival, suggesting that surgical resection is a reasonable approach for metastatic GPs.

A Rare Cause of Melaena-Duodenal Gangliocytic Paraganglioma

Gangliocytic paraganglioma (GP), a rare tumour that arises most frequently from the periampullary area, is considered to be a benign neoplasm with a potential for lymphatic spread. Distant metastases are rare [1]. We report a case of a 51 year old female who presented with abdominal pain, anaemia and melaena. Endoscopy, push enteroscopy and biopsy revealed a periampullary neuroendocrine tumour. Immunohistochemistry of the pancreatic duodenectomy specimen demonstrated a duodenal gangliocytic paraganglioma with no lymph node metastases. We review the literature on this rare tumour and the current treatment protocol.