Paraganglioma gangliocítico metastásico: una rara entidad histológica diagnosticada tras una duodenopancreatectomía / Metastatic gangliocytic paraganglioma: a rare histological entity diagnosed after duodenopancreatectomy

Introducción. El paraganglioma gangliocítico es una entidad histológica infrecuente, siendo el duodeno su principal localización extra ganglionar. Caso clínico. Se trata de un varón de 54 años que consulta por dolor abdominal y hemorragia digestiva alta. Se diagnosticó una lesión sospechosa de ampuloma por lo que se realizó una duodenopancreatectomía cefálica. En el análisis histológico, se confirmó el diagnóstico de paraganglioma gangliocítico metastásico por la presencia patognomónica de tres estirpes celulares (epiteliales, ganglionares y Schwann-like). Dado su buen pronóstico, asociado con baja quimiosensibilidad, no recibió tratamiento adyuvante. Resultados. Durante el seguimiento, el paciente no presentó complicaciones tardías, ni signos de recidiva después de un año de la intervención. Conclusión. El paraganglioma gangliocítico es una entidad potencialmente maligna, que requiere un correcto estudio de extensión y un seguimiento estrecho a largo plazo

Introduction. Gangliocytic paraganglioma is a rare histological entity, with the duodenum being its main extra-nodal location. Clinical case. This is a 54-year-old man who presented with abdominal pain and upper gastrointestinal bleeding. A suspicious ampuloma lesion was diagnosed, for which a pancreaticoduodenectomy was performed. In the histological analysis, the diagnosis of metastatic gangliocytic paraganglioma was confirmed by the pathognomonic presence of three cell lines (epithelial, ganglionic, and Schwann-like). Given his good prognosis associated with low chemosensitivity, he did not receive adjuvant treatment. Results. During follow-up, the patient did not present late complications or signs of recurrence one year after the intervention. Conclusion. Gangliocytic paraganglioma is a potentially malignant entity that requires a correct extension study and close long-term follow-up

Clinical, imaging and pathological features of duodenal gangliocytic paraganglioma / 中华肝胆外科杂志

Objective:To study the clinical, imaging and pathological features of duodenal gangliocytic paraganglioma (DGP).Methods:The clinical, imaging and pathological data of patients with DGP treated at the Shandong Provincial Hospital Affiliated to Shandong First Medical University from January 2012 to October 2021 were retrospectively analyzed.Results:Of 8 patients with DGP, there were 7 males and 1 female, with a median age of 52 years (range 37 to 57 years). Five patients were asymptomatic and they were diagnosed on physical examination followed by investigations. Three patients presented with black stools. CT examination showed localized nodular thickening of the duodenum, with enhanced scanning showing obvious progressive contrast enhancement. Endoscopic ultrasonography showed a hypoechoic submucosal lesion in duodenal wall. Histologically, the neoplasm composed of three different cell types which included Schwann cells, epithelioid cells, and ganglioid cells. The Schwann cells expressed NF, NSE and S-100 proteins; the epithelioid cells expressed CK, NSE, Syn and CgA proteins; while the ganglioid cells expressed NSE, Syn, CgA and NF proteins. Endoscopic submucosal dissection was performed in 2 patients and surgical resection was performed in 6 patients.Conclusion:DGP is a rare benign neurogenic tumor which is most commonly found in the duodenum. It has a good prognosis. Imaging and endoscopic examinations demonstrated a submucosal mass. The main treatment are endoscopic resection and local surgical resection.

Gangliocytic Paraganglioma of the Filum Terminale. A Rare Entity

Paragangliomas are rare, benign and slow-growing neuroendocrine tumors that can arise from the adrenal medulla (85­90%) or from the extra-adrenal paraganglia. In the central nervous system (CNS), they can be found at several sites, but more often at the cauda equina and filum terminale region, where they account for between 2.5 and 3.8% of total tumor cases of that region. There are only 8 cases described in the literature that mention the presence of the gangliocytic variant of this entity at the filum terminale. We present the case of a 41-year-old man with chronic lumbar pain refractory to medical treatment, without any associated neurological deficits. Magnetic resonance imaging (MRI) revealed an intradural, extramedullar oval lesion with regular contours and homogeneous caption of contrast at L1 level. He was submitted to surgical treatment, with complete resection of the lesion. The histological analysis revealed a gangliocytic paraganglioma of the filum terminale. At 5 years of follow-up, he remains asymptomatic and without any signs of relapse. These are lesions with an overall good prognosis with gross total resection. Although the recurrence rate is extremely low, prolonged observation is recommended due to the slow-growing nature of the tumor, being estimated that between 1 and 4% can recur even after gross total removal.

Gangliocytic paraganglioma of the spine

Paragangliomas are rare, encapsulated, benign neuroendocrine tumors that can arise from the adrenal medulla or extra-adrenal paraganglia. Extra-adrenal paragangliomas may develop a gangliocytic component with ganglion cells (Gangliocytic paragangliomas). Nearly 25%of cauda equina paragangliomas are gangliocytic paragangliomas. Here, we describe the case of a 35-year-old male who presented with weakness of both lower limbs over the last two months. Radiological findings were suggestive of myxopapillary ependymoma. However, the histopathological examination revealed a tumor with cells arranged in sheets, papillae, lobules, and around vessels forming pseudo rosettes. Ganglion cells were seen in small groups and, also singly. Tumor cells were immunopositive for chromogranin, synaptophysin, and S-100. Ganglion cells were immunopositive for synaptophysin, NSE, and NFP. A final histological diagnosis of Gangliocytic paraganglioma (WHO grade I) was made. To date, only nine gangliocytic paraganglioma cases have been previously reported, and to the best of our knowledge, this is the largest gangliocytic paraganglioma.

Duodenal gangliocytic paraganglioma: A rare cause for gastrointestinal polyp

Duodenal gangliocytic paragangliomas are rare neoplasms often arising in the duodenum in close proximity to the ampulla of Vater. These neoplasms are considered to have a benign behavior with lymph node metastases being a rare phenomenon and distant metastases even more so. Although a standardized treatment has not been determined, a margin-free tumor resection seems to be the best treatment modality. We report herein the case of a 36-year-old female who presented with abdominal pain and was found to have a polyp in the second part of duodenum which was excised endoscopically. Histopathology and immunohistochemistry revealed characteristic features of this rare tumor.

A Gangliocytic Paraganglioma of the Ampulla of Vater Removed by Endoscopic Resection / 대한췌담도학회지

Gangliocytic paraganglioma (GP) is an extremely rare tumor that mostly occurs in the periampullary area of the duodenum. It is characterized by benign behavior and favorable outcomes, but sometimes shows regional lymph node dissemination. GP consist of three characteristic histological components: epithelioid, spindle, and ganglion cells. Therefore, it is often misdiagnosed as a neuroendocrine tumor when only endoscopic forceps biopsy is performed. The clinical management of GP has not yet been standardized. This case report describes an asymptomatic patient who was initially diagnosed with a grade-1 neuroendocrine tumor, but was confirmed as having benign GP after endoscopic papillectomy. Complete en-bloc resection was performed for this patient, without any significant adverse events. At a 6-month follow-up assessment, the patient remained asymptomatic and there was no evidence of recurrence.

Simultaneous Manifestation of Gangliocytic Paraganglioma and Heterotopic Pancreas of Ampulla of Vater Treated by Endoscopic Resection / 대한췌담도학회지

Gangliocytic paraganglioma is an uncommon tumor of digestive system that is usually found in the second portion of duodenum. It is generally considered benign tumor, although few reports of local recurrences and regional lymph node metastases have been made. Gangliocytic paraganglioma is characterized by its histologic pattern including ganglion cells, spindle cells and epithelioid cells. Heterotopic pancreas, also known as ectopic pancreas, is a pancreatic tissue appeared outside of its normal location lacking anatomic or vascular connection with the pancreas. In duodenum, it is a relatively unusual lesion that may be found incidentally during surgery or endoscopy. We present a case of 39-year-old woman with gangliocytic paraganglioma combined with heterotopic pancreas in the ampulla of Vater successfully treated by endoscopic resection.

Simultaneous Manifestation of Gangliocytic Paraganglioma and Heterotopic Pancreas of Ampulla of Vater Treated by Endoscopic Resection / 대한췌담도학회지

Gangliocytic paraganglioma is an uncommon tumor of digestive system that is usually found in the second portion of duodenum. It is generally considered benign tumor, although few reports of local recurrences and regional lymph node metastases have been made. Gangliocytic paraganglioma is characterized by its histologic pattern including ganglion cells, spindle cells and epithelioid cells. Heterotopic pancreas, also known as ectopic pancreas, is a pancreatic tissue appeared outside of its normal location lacking anatomic or vascular connection with the pancreas. In duodenum, it is a relatively unusual lesion that may be found incidentally during surgery or endoscopy. We present a case of 39-year-old woman with gangliocytic paraganglioma combined with heterotopic pancreas in the ampulla of Vater successfully treated by endoscopic resection.

Paraganglioma gangliocítico en la tercera porción del duodeno / Gangliocytic paraganglioma in the third portion of the duodenum

El paraganglioma gangliocítico es un tumor neuroendocrino no epitelial, originado de la cresta neural, que afecta en la gran mayoría de los casos la segunda porción del duodeno, sin embargo puede presentarse en cualquiera de sus porciones e inclusive originarse en el yeyuno proximal, en el apéndice y en otros sitios menos frecuentes. Por su localización y la tendencia a ulcerarse, estas lesiones pueden manifestarse por sangrado digestivo, ictericia, obstrucción o anemia como en el caso que describiremos más adelante, y muchas veces son encontradas de forma incidental durante procedimientos endoscópicos o autopsias. El paraganglioma gangliocítico es un tumor benigno de pronóstico favorable, aunque se han reportado algunos casos de recurrencia, invasión linfática o metástasis a distancia. Histológicamente el paraganglioma gangliocítico está compuesto por varios patrones celulares, los cuales pueden ser corroborados mediante inmunohistoquímica y su tratamiento debe ir dirigido a la resección local o radical en algunos casos.

Gangliocytic paraganglioma is a non-epithelial, neuroendocrine tumor that originates from the neural crest. In the majority of cases, it affects the second part of the duodenum, however it may occur in any of its parts and even originate in the proximal jejunum, the appendix and other less frequent places. Because of its location and high tendency to ulcerate, these lesions can appear as gastrointestinal bleeding, jaundice, obstruction and anemia, as in the case described herein. Also, many times they are found incidentally during endoscopy or autopsy. Gangliocytic paraganglioma is a benign tumor, with a favorable prognosis, but some cases of recurrence, lymphatic invasion and metastasis have been reported. Histologically gangliocytic paraganglioma is composed by various cell patterns which can be found by immunohistochemistry and in some cases its treatment consists of radical or local resection.

A Gangliocytic Paraganglioma Presenting as a Submucosal Tumor in the Ampulla / 대한내과학회지

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.

Gangliocytic paraganglioma: A rare presentation as intestinal intussusception.

Gangliocytic paragangliomas are rare benign neoplastic lesions of neuroendocrine origin occurring primarily in peri-ampullary region. Commonly occurring as small submucosal lesions, presentation as a large polyp with acute ileo-ileal intussusception in a 62-year-old woman is rare and is described in this case report.

A case of juxtapapillary gangliocytic paraganglioma treated with endoscopic resection / 대한내과학회지

Gangliocytic paraganglioma is a rare tumor that is usually seen in the duodenum. This neoplasm generally behaves in a benign fashion, although instances of recurrence and lymph node metastasis have been described. We experienced a case of incidentally found gangliocytic paraganglioma treated with endoscopic resection. A 61-year-old man was referred because of submucosal tumor adjacent to the major papilla. Endoscopic biopsy revealed paraganglioma. Abdominal CT showed that there was no evidence of a duodenal mass or lymphadenopathy. This tumor was resected endoscopically by electrosurgical snare polypectomy. The excised tumor measured 1.0 cm in diameter. Histopathological examination revealed a benign gangliocytic paraganglioma and the resection margins were free of tumor. At the 6-month follow-up, the patient was still asymptomatic and no residual tumor was detected at the resection site.

Periampullary Gangliocytic Paraganglioma Successfully Treated by Endoscopic Mucosal Resection / 대한소화기내시경학회지

Gangliocytic paraganglioma is a rare tumor that occurs nearly exclusively in the second portion of the duodenum. This tumor is usually considered to be benign. So, endoscopic resection may be the best procedure to facilitate the diagnosis and for treatment to avoid an unnecessary, invasive operation. To the best of our knowledge, this is the first report of a periampullary gangliocytic paraganglioma that was successfully treated by endoscopic mucosal resection in Korea. We report here a case of a 45-year-old man who was found to have a periampullary gangliocytic paraganglioma. This tumor was adequately treated by endoscopic mucosal resection.

Two Cases of Endoscopic Resection of Submucosal Tumor of the Minor Papilla / 대한소화기내시경학회지

Tumors of the minor papilla are very rare and these tumors have generally been known as neuroendocrine tumors such as carcinoid tumor and somatostatinoma. As these are mostly submucosal tumors, their diagnosis is difficult by just performing endoscopic forceps biopsy, but diagnosis is possible by surgery or endoscopic resection. EUS and ERCP is an essential tool for the diagnosis of these tumors, and abdominal CT or MRI is also useful because there is the possibility of malignant tumors such as carcinoid tumor. For our present two cases, screening endoscopy revealed the polypoid lesion of the minor papilla. EUS disclosed that the submucosal tumor was limited to the submucosal layer and no abnormality was found from the abdominal CT and ERCP. Herein, we performed endoscopic resection for making the diagnosis and treatment. Histologically, these 2 tumors were diagnosed as ectopic pancreas and gangliocytic paraganglioma, respectively.

Gangliocytic Paraganglioma of the Duodenum

Gangliocytic paragangliomas are rare benign neuroendocrine tumors that arise mainly in the duodenum. A total of 3 cases of duodenal gangliocytic paraganglioma have been reported in Korea. The authors encountered another case of a gangliocytic paraganglioma in the duodenum. A 48-year old man presented with a mass in the duodenum that had been found incidentally in a medical checkup. The endoscopic examination revealed a 3.5x2.2 cm sized polypoid mass in the second portion of the duodenum. The patient underwent a polypectomy in the form of a wedge resection under general anesthesia. Microscopically, the tumor was composed of epithelial cells forming paraganglioma like patterns, spindle cells and ganglion cells. Gangliocytic paragangliomas are believed to be benign tumors but regional lymph node metastasis and recurrence can occur in rare cases. During the 27-month follow-up after treatment, there was no evidence of recurrence or lymph node metastasis. We report this case to help improve the understanding of this rare disease.

A Case of Gangliocytic Paraganglioma of the Ampulla of Vater Presenting as Jaundice / 대한소화기내시경학회지

Gangliocytic paraganglioma is a rare and benign gastrointestinal tumor that usually arises in the second portion of the duodenum. Histogenesis of this tumor is yet unclear. It is incidentally found during radiographic examinations or during endoscopy looking for the cause of gastrointestinal hemorrhage, which usually manifests as mucosal ulcerations. To our knowledge, there was only one case of duodenal gangliocytic paraganglioma presenting with melena in Korea. We experienced a case of gangliocytic paraganglioma arising from the ampulla of Vater, presenting as obstructive jaundice, which was subsequently removed surgically. Thus, we report this case with a review of literatures.

Gangliocytic Paraganglioma

Gangliocytic paraganglioma (GP) is a rare benign tumor that is usually seen in the duodenum. It shows unique histologic features that are composed of a carcinoid or paraganglioma-like appearance, ganglion cells, and Schwann cells. The common presenting symptoms are abdominal pain, gastrointestinal bleeding and obstruction. The lesion can sometimes be asymptomatic and they are discovered incidentally. We experienced a case of incidentally found GP in a 73 year-old-man who had a colon cancer with liver metastasis. During the preoperative workup, a submucosal tumor was found in the duodenal papilla. The frozen diagnosis of the duodenal mass was GP, which was confirmed by the permanent sections and immunohistochemical staining. Pathologists should be alert to recognize and diagnose this rare, but benign disease, especially in the patient suffering with gastrointestinal cancer.