Pulmonary gangliocytic paraganglioma: An under-recognized mimic of carcinoid tumor.

Gangliocytic paragangliomas are rare neoplasms occurring almost exclusively in the ampullary region of the gastrointestinal tract. Although these tumors are not typically considered in the differential diagnosis of primary pulmonary neoplasia, 5 cases of primary pulmonary gangliocytic paragangliomas have been previously reported. Herein we report our experience with 3 additional examples, all referred to our Anatomic Pathology Consultation service. The patients (a 32-year-old man, a 69-year-old woman and a 55-year-old man) each presented with an endobronchial (2 cases) or upper lobe lung mass, ranging from 1.5 to 2.5 cm in maximum dimension. Biopsy and endobronchial debulking specimens demonstrated the classic triphasic morphology of gangliocytic paraganglioma, with epithelial, spindled and ganglion-like cells. By immunohistochemistry, the tumors were positive for keratin, synaptophysin and chromogranin A in the epithelial component, S100 protein and glial fibrillary acidic protein (GFAP) in the Schwannian spindled cells, and synaptophysin in ganglion cells. TTF1 expression was seen in the epithelial components of 2 cases. The Ki-67 labelling index was low (<2%). Primary pulmonary gangliocytic paragangliomas should be distinguished from carcinoid tumors, given the different natural histories and risk stratification approaches for these morphologically similar tumors. Awareness that gangliocytic paraganglioma may occur in the lung and appropriate immunohistochemical studies are key to correct diagnosis.

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

Duodenal Gangliocytic Paragangliomas-Case Series and Literature Review.

Duodenal gangliocytic paragangliomas are rare neuroendocrine tumors primarily localized in the periampullary area. Though mostly asymptomatic, they can present with various symptoms, most often jaundice, anemia and abdominal pain. The present paper is a case series report, describing our personal experience with patients presenting to the Emergency Unit with different symptoms due to duodenal gangliocytic paraganglioma. Endoscopic resection is safe and indicated in most of the cases, being also associated with lower medical costs. EUS plays a central role in the pre-resection management and in surveillance, and immunostaining is decisive to ascertain the tumor histologic origin. In addition to reporting our experience, we researched the literature regarding these rare tumors and performed a comprehensive review.

Histopathological spectrum of duodenal polyps in a retrospective ten-year study.

INTRODUCTION AND AIMS:  Duodenal polyps are rare in patients undergoing upper gastrointestinal endoscopy. The present study is an experience of the histopathological spectrum of the duodenal polyps and its correlation with the clinical and endoscopic findings in a tertiary care centre. MATERIALS AND METHODS: The present study is a 10-year retrospective study from the year 2011 to 2020. All the relevant clinical, endoscopic and radiologic findings were retrieved from the hospital medical records. Old histopathology slides were restained, and wherever required, special stains and immunohistochemistry (IHC) were performed. All the cases were reviewed. The present study mainly included descriptive statistics with categorical and continuous variables. RESULTS: Total 81 cases of duodenal polyps were studied in the period of 10 years. The median age was 48 years. Male: female ratio was 2.2:1. The most common presenting system was abdominal pain. We experienced both solitary and multiple polyps. The majority of the duodenal polyps were non-neoplastic, with unremarkable mucosa or inflammatory type. Unlike previous studies the most common site for the hyperplastic polyp in the present study was the first part of the duodenum. Among the neoplastic polyps, adenomatous polyp was the most common type. Contrary to the previous studies, our study showed the first part of the duodenum as the most common site for the sporadic nonampullary adenomatous duodenal polyps. Of the rare entities, we encountered a single case each of lipomatous polyp and gangliocytic paraganglioma. Among the syndromes we encountered two cases of Peutz-Jeghers syndrome and one case of familial adenomatous polyp in our study population.CONCLUSION Duodenal polyps are a rare finding on endoscopic examinations, though most of them are non-neoplastic in nature, vigilant examination under the microcope is required to rule out any neoplastic pathology and identify the risk of malignancy.

Minimally Invasive Resection of a Gangliocytic Paraganglioma of the Cauda Equina: A Case Report and Review of Literature.

Gangliocytic paraganglioma (GP) is considered a rare neuroendocrine tumor (NET) most often located in the distal half of the duodenum. Insufficient reports describe tumors of this histological type located in the distal parts of the spinal canal, the conus medullaris and cauda equina. To date, nine cases of GP of the cauda equina and one case of GP of conus medullaris have been described. After analyzing all available treatment reports of GP, a study described it as a tumor with an extremely good prognosis in cases of total tumor removal. Here, we present a case of a female patient with a GP at the level of the L4 vertebra treated at Burdenko Neurosurgical Center using a minimally invasive approach through a tubular retractor. The tumor was removed en bloc through an intralaminar opening, and the patient was discharged two days after surgery with total regression of symptoms.

Distinct cases of gangliocytic paraganglioma in the duodenum: Two case reports.

Gangliocytic paraganglioma (GP) is a rare, benign neuroendocrine tumor that commonly arises in the second portion of the duodenum. Despite its favorable prognosis, there have been instances of lymph node and liver metastasis as well as 1 reported fatal case. The immunohistochemical and morphological resemblance between GP and neuroendocrine tumor G1 makes it critical to properly recognize and differentiate between the 2. In this article, we present 2 distinct cases of GP: a 70-year-old male with a GP tumor in the ampulla, and a 46-year-old male with a GP near the ampulla whose tumor was excised using a robotic Whipple procedure. We focus on optimizing diagnosis and management through the application of radiological modalities and pathological analysis.

Surgical treatment for a rare case of duodenal gangliocytic paraganglioma accompanied with lymph node metastasis.

We report a rare case of duodenal gangliocytic paraganglioma accompanied with lymph node metastasis. An asymptomatic 58-year-old woman was admitted for the evaluation of abnormal liver enzyme test results. Abdominal computed tomography and upper gastrointestinal endoscopy revealed a 27-mm pedunculated tumor of the papilla of Vater. Adenocarcinoma of the papilla with muscularis propria invasion was suspected; therefore, pancreaticoduodenectomy with lymph node dissection was performed. There were no perioperative complications. A definitive diagnosis of gangliocytic paraganglioma of the papilla of Vater with lymph node metastases was confirmed by pathological examinations, which is a rare condition. The patient was discharged on her 16th postoperative day, and no recurrence or metastases were identified during the 18-month follow-up period. We herein discuss the surgical treatment for this rare case and present a review of related literature.

Is Gangliocytic Paraganglioma Designated as a Subtype of Composite Paragangliomas and Originated From Pancreas Islet? A Case Report and Review of Literature.

Gangliocytic paraganglioma (GP) is quite rare, and origin and entity remain to be elucidated. A 51-year-old man presented with GP as a sessile polyp with a smooth surface that measured about 1 cm in diameter in the descending portion of duodenum. Pathological examination displayed that a neoplasm was predominantly located in the submucosa and infiltrated mucosa focally. The tumor consisted of epithelioid, ganglion-like, and spindle cells admixing in a haphazard way. The epithelioid cells resembled paraganglioma in cytological and architectural features. The ganglion-like cells were scattered and merged with the bland spindle cells in fascicular clusters, which resembled ganglioneuroma. Synaptophysin (Syn), microtubule-associated protein-2 (MAP-2), and chromogranin A (CgA) were positive in the epithelioid and ganglion-like cells in variety, and neurofilament (NF) staining highlighted the ganglion-like cells. S-100 and SOX-10 were positive in the spindle cell proliferation and around the epithelioid cells. Progesterone receptor (PR) was positive in the epithelioid cells. The polyp was resected, and no adjuvant therapy was given. The patient remained with no recurrence in 2 years' follow-up. Origin of GP is presumed to be related to pancreas islet. GP is distinguished from neuroendocrine tumor (NET) G1 and designated as paraganglioma-ganglioneuroma, a kind of composite paragangliomas.

Anaplastic Sarcoma of the Kidney With Heterologous Ganglioneuroblastic Differentiation: Another DICER1-Associated Tumor.

Anaplastic sarcoma of the kidney (ASK) is a rare renal tumor for which less than thirty cases have been described in the literature to date. Diagnosis of ASK is primarily based on histology, which features solid spindle cell neoplastic islands arranged in a fascicular pattern, prominent anaplastic nuclear morphology, brisk mitoses, and multiple multiloculated cysts lined by hobnail epithelium reminiscent of cystic nephroma. Chondroid or rhabdomyocytic differentiation is often present within the sarcoma. It has been recently suggested that this tumor entity belongs to the DICER1 syndrome tumors based on identification of DICER1 mutations. We report on a case of this rare tumor found in a twenty-month-old female. In addition to the typical histologic findings of ASK, this case also displayed heterologous neuroblastic-gangliocytic differentiation, which has not been previously described in the literature. TP53 and BRAF v600E had aberrant immunostaining. Chromosomal microarray and genomic sequencing revealed loss of chromosome 10 p15.3-p11.2 and both somatic and germline DICER1 mutations, consistent with recent research and further supporting the classification of this tumor within the DICER1 syndrome associated tumors.

Gangliocytic Paraganglioma of the Gastrointestinal Tract: A Case Report of Cecal Origin.

INTRODUCTION: Gangliocytic paraganglioma is an extremely rare tumor that is composed of epithelioid, ganglion, and spindle cells. It has a predilection site in the duodenum, especially the periampullary region. The clinical course is usually benign, but a few cases of metastasis to lymph nodes or distant sites have been noted. Several case reports or small series reviews of gangliocytic paraganglioma have been published, but so far, cecal gangliocytic paraganglioma has not been described. CASE DESCRIPTION: A 61-year-old man underwent an abdominal Computed Tomography (CT) scan for known GB stones, which revealed an incidental cecal mass. The patient underwent wedge resection of the cecum for diagnostic and therapeutic purposes. Histopathological examination of the cecal mass identified a gangliocytic paraganglioma. There was no evidence of metastasis in the dissected regional lymph nodes. No additional treatment, such as adjuvant chemotherapy or radiotherapy, was administered, and the patient underwent regular follow-up without any complications. CONCLUSION: The most common radiologic finding in gangliocytic paraganglioma is a circumscribed, enhancing subepithelial mass. Herein, we report an uncommon case of cecal-origin gangliocytic paraganglioma. With knowledge of the radiologic and histologic characteristics of gangliocytic paraganglioma, appropriate management can be provided without unnecessary radical resection.

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 leading to duodeno-jejunal intussusception: A case report.

The intussusception of the small bowel is rarely encountered in adult patients and is frequently associated with a lead point that is often malignant. In a 69-year-old female patient with an episode of gastrointestinal (GI) bleeding, computed tomography (CT) showed a duodenal-jejunal intussusception caused by an intraluminal mass. Open polypectomy and reduction of intussusception were performed and the diagnosis of gangliocytic paraganglioma was made at pathological evaluation. It would be important to consider neoplasms like gangliocytic paraganglioma in the setting of adult small bowel intussusception.

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.

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 very rare cause for upper gastrointestinal bleeding: Case report with review of literature.

INTRODUCTION: Gangliocytic paraganglioma is a rare tumors mainly arises from the second part of the duodenum. The tumor is generally benign, but some cases are associated with lymph node or distant metastasis. It must be differentiated from other tumors. The main treatment is good local surgical resection. Most patients have good prognosis after an appropriate resection. CASE PRESENTATION: A 47-year-old male patient presented with recurrent attacks of epigastric pain and melena. The general examination revealed pallor with no jaundice. The abdomen was soft with no tenderness. There were no stigmata of chronic liver diseases. The hemoglobin level was 8 gm/dl with normal liver and renal functions. The patients received compatible blood. Endoscopy showed a large pedunculated polyp with ulceration in the second part of the duodenum just distal to ampulla of Vater. During surgery, the anterior wall of duodenum was opened, and a 4 cm pedunculated polyp was excised which was arising from the posterior wall of the duodenum just distal to the ampulla of Vater. The frozen section examination revealed a benign lesion. The final histopathology result was consistent with the diagnosis of gangliocytic paraganglioma. The patient had smooth post-operative recovery and no postoperative complications. CONCLUSIONS: Gangliocytic paraganglioma is a rare tumor which mostly affects the second part of the duodenum. The tumor is considered benign but the possibility of the malignancy with lymphatic and distant metastases must be excluded before any surgery. The management plan must be addressed carefully and continuous follow up is recommended.

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.

Endoscopic resection of gangliocytic paraganglioma of the duodenum: a case report.

Duodenal gangliocytic paragangliomas are extremely rare. A 79-year-old woman underwent gastrointestinal endoscopy for screening prior to resection of gallbladder carcinoma. Gastrointestinal endoscopy revealed a 5-mm submucosal tumor in the second portion of the duodenum. Contrast-enhanced computed tomography revealed no tumor or metastasis. Endoscopic ultrasonography revealed low echo pattern of the tumor. Histopathological examination of the biopsy specimen revealed proliferation of three types of cells (epithelioid cells, spindle cells, and ganglion cells). Immunohistochemical examination revealed that the tumor was positive for S-100 and synaptophysin. The preoperative diagnosis was gangliocytic paraganglioma. The tumor was completely resected by endoscopic mucosal resection (EMR). In conclusion, an early stage of gangliocytic paraganglioma of the duodenum could be resected using EMR.