|
1
|
Okubo Y. Gangliocytic paraganglioma: An overview and future perspective. World J Clin Oncol 2019; 10:300-302. [PMID: 31572665 PMCID: PMC6766464 DOI: 10.5306/wjco.v10.i9.300] [Citation(s) in RCA: 15] [Impact Index Per Article: 2.5] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 03/02/2019] [Revised: 08/17/2019] [Accepted: 08/21/2019] [Indexed: 02/06/2023] Open
Abstract
Gangliocytic paraganglioma (GP) is rare neuroendocrine tumor (NET) with a good prognosis that commonly arising from duodenum. Although the tumor is characterized by its unique triphasic cells (epithelioid, spindle, and ganglion-like cells), the proportions of these three tumor cells vary widely from case to case, and occasionally, morphological and immunohistochemical similarities are found between GP and NET G1 (carcinoid tumors). Further, GP accounts for a substantial number of duodenal NETs. Therefore, GP continues to be misdiagnosed, most often as NET G1. However, GP has a better prognosis than NET G1, and it is important to differentiate GP from NET G1. In this article, I wish to provide up-to-date clinicopathological information to help oncologists gain better insight into the diagnosis and clinical management of this tumor.
Collapse
Affiliation(s)
- Yoichiro Okubo
- Department of Pathology, Kanagawa Cancer Center, Yokohama, Kanagawa 241-8515, Japan
| |
Collapse
|
|
2
|
Zheng Z, Chen C, Li B, Liu H, Zhou L, Zhang H, Zheng C, He X, Liu W, Hong T, Zhao Y. Biliary Neuroendocrine Neoplasms: Clinical Profiles, Management, and Analysis of Prognostic Factors. Front Oncol 2019; 9:38. [PMID: 30805307 PMCID: PMC6370735 DOI: 10.3389/fonc.2019.00038] [Citation(s) in RCA: 21] [Impact Index Per Article: 3.5] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 11/12/2018] [Accepted: 01/15/2019] [Indexed: 12/12/2022] Open
Abstract
Biliary neuroendocrine neoplasms (NENs) represent <1% of all NENs. The aim of this retrospective study is to present the clinical characteristics, management and prognosis profiles of 28 biliary NEN patients from a large tertiary center, and identify factors related to prognosis. Nine tumors originated from the gallbladder, two from the extrahepatic bile duct and 17 from the ampulla of Vater. One patient was classified as neuroendocrine tumor (NET) Grade 1, three patients were classified as NET Grade 2, 18 were graded neuroendocrine carcinoma (NEC) Grade 3 and six were classified as mixed adenoneuroendocrine carcinoma (MANEC). The overall survival rate and disease-free survival rate did not have statistically significant differences between tumors of different locations or different grading. Recurrence of disease correlated with poor prognosis (p < 0.001). Lymphovascular invasion and invasion beyond the submucosa were related to higher risk of local lymph node metastases. Multivariate analysis identified patient age (p = 0.021) and R0 resection margin (p = 0.027) as independent prognostic factors associated with overall survival. Our study included relatively large numbers of biliary tract NENs with intact follow-up information. Patients with biliary neuroendocrine tumors showed different clinical outcomes according to tumor locations and tumor grades. Achieving R0 resection is important for better prognosis.
Collapse
Affiliation(s)
- Zhibo Zheng
- Department of General Surgery, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences, Beijing, China
| | - Chuyan Chen
- Department of General Surgery, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences, Beijing, China
| | - Binglu Li
- Department of General Surgery, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences, Beijing, China
| | - Hongbo Liu
- School of Public Health, China Medical University, Shenyang, China
| | - Liangrui Zhou
- Department of Pathology, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences, Beijing, China
| | - Hui Zhang
- Department of Pathology, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences, Beijing, China
| | - Chaoji Zheng
- Department of General Surgery, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences, Beijing, China
| | - Xiaodong He
- Department of General Surgery, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences, Beijing, China
| | - Wei Liu
- Department of General Surgery, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences, Beijing, China
| | - Tao Hong
- Department of General Surgery, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences, Beijing, China
| | - Yupei Zhao
- Department of General Surgery, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences, Beijing, China
| |
Collapse
|
|
3
|
Duodenal Gangliocytic Paraganglioma Requiring a Pancreaticoduodenectomy: A Case Report and Review of the Literature. Case Rep Surg 2018; 2018:6292789. [PMID: 30356398 PMCID: PMC6178180 DOI: 10.1155/2018/6292789] [Citation(s) in RCA: 4] [Impact Index Per Article: 0.6] [Reference Citation Analysis] [Abstract] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 05/15/2018] [Accepted: 08/02/2018] [Indexed: 02/06/2023] Open
Abstract
Introduction Duodenal gangliocytic paragangliomas (GPs) are a subclass of duodenal neuroendocrine neoplasms and are exceedingly rare. They have been associated with an indolent behavior; however, they can rarely metastasize. Radical resection like a pancreaticoduodenectomy is sometimes indicated. We hereby present a case requiring major surgery and perform a literature search about this disease. Presentation of Case A 49-year-old Caucasian female, who presented with an upper gastrointestinal bleed, was found to have a hypodense mass in the second/third portion of the duodenum. A biopsy of the mass during upper endoscopy was inconclusive. A pancreaticoduodenectomy was recommended based on the high suspicion for a duodenal adenocarcinoma and was performed successfully. Her final pathology revealed a duodenal gangliocytic paraganglioma. Discussion The majority of duodenal GPs present as gastrointestinal bleeds while others less commonly present with anemia, abdominal pain, duodenal obstructive symptoms, pancreatitis, or abnormal incidental findings on axial abdominal imaging. Duodenal GPs were initially viewed as benign tumors of the duodenum; however, there have been increasing incidence reports of hematogenous and lymphatic metastasis. Appropriate treatment of duodenal GPs is still controversial and ranges from local endoscopic submucosal resection to major radical surgery. Conclusion Duodenal GPs are very rare tumors of the second portion of the duodenum presenting with upper gastrointestinal bleeding and local symptoms of surrounding organs. Local or radical resection is usually recommended to prevent bleeding and the minor risk of metastatic spread.
Collapse
|
|
4
|
Okubo Y, Yoshioka E, Suzuki M, Washimi K, Kawachi K, Kameda Y, Yokose T. Diagnosis, Pathological Findings, and Clinical Management of Gangliocytic Paraganglioma: A Systematic Review. Front Oncol 2018; 8:291. [PMID: 30101131 PMCID: PMC6072869 DOI: 10.3389/fonc.2018.00291] [Citation(s) in RCA: 25] [Impact Index Per Article: 3.6] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 05/24/2018] [Accepted: 07/11/2018] [Indexed: 12/11/2022] Open
Abstract
Background: Although gangliocytic paraganglioma (GP) is considered a rare benign neuroendocrine tumor, cases of mortality have been reported. Occasionally, GP is misdiagnosed as neuroendocrine tumor G1, which is associated with a poorer prognosis than GP. To avoid such misdiagnoses, it is important to understand the clinicopathological characteristics of GP. Thus, herein, we discuss the current literature on the clinicopathological characteristics of GP. Methods: We conducted a systematic review in accordance with the Preferred Reporting Items for Systematic Reviews and Meta-Analyses statement. PubMed and Japana Centra Revuo Medicina searches were used to identify papers describing GP. Inclusion criteria included confirmation of epithelioid, spindle-shaped, and ganglion-like cells in the main article and/or figures and whether the paper was cited in other studies of GP. Data were collected on age, sex, site of the primary lesion, tumor size, treatment, prognosis, lymph node metastasis (LNM), depth of tumor invasion, rate of preoperative diagnosis, and clinical symptoms. Results: In total, 162 papers containing 263 cases of GP met the criteria. The mean age at diagnosis was 53.5 years. The male-to-female ratio was 157:104. The mean tumor size was 25.7 mm. The predominant site of the primary tumor was the duodenum (89.7%). The most common clinical sign of GP was gastrointestinal bleeding (47.9%). Other signs and symptoms of GP included abdominal pain (44.7%), anemia (20.3%), incidental findings (12.9%), nausea (6.9%), weight loss (5.5%), general fatigue (5.1%), jaundice (4.6%), and incidental autopsy findings (5.1%). LNM was observed in 11.4% of patients. Liver metastasis was observed in 1.1% of patients. Depth of tumor invasion (penetrating beyond the submucosal layer or sphincter of Oddi) was by far the most significant risk factor for LNM in patients with GP. This suggests, along with histological heterogeneity, that GP may have hamartomatous characteristics. Furthermore, immunohistochemical expression of progesterone receptor and pancreatic polypeptide were useful in distinguishing between GP and neuroendocrine tumor G1, even in small biopsy specimens. Conclusions: We reveal the clinicopathological characteristics of GP, including risk factors for LNM, differential diagnostic approaches, and improvements in the clinical management of this tumor.In addition, GP may have hamartomatous characteristics.
Collapse
Affiliation(s)
- Yoichiro Okubo
- Department of Pathology, Kanagawa Cancer Center, Kanagawa, Japan
| | | | | | | | | | | | | |
Collapse
|
|
5
|
Okubo Y, Kasajima R, Suzuki M, Miyagi Y, Motohashi O, Shiozawa M, Yoshioka E, Washimi K, Kawachi K, Kameda Y, Yokose T. Risk factors associated with the progression and metastases of hindgut neuroendocrine tumors: a retrospective study. BMC Cancer 2017; 17:769. [PMID: 29145818 PMCID: PMC5693490 DOI: 10.1186/s12885-017-3769-4] [Citation(s) in RCA: 6] [Impact Index Per Article: 0.8] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Grants] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 07/06/2017] [Accepted: 11/09/2017] [Indexed: 12/14/2022] Open
Abstract
BACKGROUND The worldwide incidence of neuroendocrine tumors (NETs) has increased remarkably, with the hindgut being the second most common site for such tumors. However, the mechanisms underlying progression and metastasis of hindgut NETs are unclear. A retrospective study was conducted to elucidate these mechanisms. METHODS Clinicopathological data of cases of hindgut NET between April 1996 and September 2015 were analyzed, retrospectively. Patients with neuroendocrine carcinoma were excluded. Formalin-fixed paraffin-embedded tissues of hindgut NET cases were subjected to detailed morphometric and immunohistochemical analyses. Statistical analyses were performed using the non-parametric Mann-Whitney U test, Spearman's correlation coefficient, and chi-squared test. Multivariate logistic regression analysis was conducted as appropriate for the data set. RESULTS Fifty-six hindgut NET cases were considered. Microvessel density and lymphatic microvessel density were identified as significant risk factors for venous and lymphatic invasion. There was a positive correlation between microvessel density and the maximum tumor diameter. Multivariate logistic regression analysis revealed that the maximum tumor diameter alone was an independent predictor of lymph node metastasis, whereas lymphovascular invasion and MVD was not the predictor of lymph node metastasis. There were no significant correlations between the Ki-67 labeling index and any of the parameters evaluated including age, sex, the maximum tumor diameter, venous invasion, lymphatic invasion, microvessel density, lymphatic microvessel density, and lymph node metastasis. CONCLUSIONS Angiogenic mechanisms may play important roles in the progression of hindgut NET. Otherwise, the maximum tumor diameter alone was an independent predictor of lymph node metastasis in hindgut NETs. Moreover, our study raises the question of whether the presence of lymphovascular invasion, in endoscopically obtained hindgut NET tissues, is an absolute indication for additional surgery or not.
Collapse
Affiliation(s)
- Yoichiro Okubo
- Department of Pathology, Kanagawa Cancer Center, 2-3-2, Nakao, Asahi-Ku, Yokohama, Kanagawa 241-8515 Japan
| | - Rika Kasajima
- Molecular Pathology and Genetics Division, Kanagawa Cancer Center Research Institute, 2-3-2, Nakao, Asahi-Ku, Yokohama, Kanagawa 241-8515 Japan
| | - Masaki Suzuki
- Department of Pathology, Kanagawa Cancer Center, 2-3-2, Nakao, Asahi-Ku, Yokohama, Kanagawa 241-8515 Japan
| | - Yohei Miyagi
- Molecular Pathology and Genetics Division, Kanagawa Cancer Center Research Institute, 2-3-2, Nakao, Asahi-Ku, Yokohama, Kanagawa 241-8515 Japan
| | - Osamu Motohashi
- Department of Gastroenterology, Kanagawa Cancer Center, 2-3-2, Nakao, Asahi-Ku, Yokohama, Kanagawa 241-8515 Japan
| | - Manabu Shiozawa
- Department of Gastrointestinal Surgery, Kanagawa Cancer Center, 2-3-2, Nakao, Asahi-Ku, Yokohama, Kanagawa 241-8515 Japan
| | - Emi Yoshioka
- Department of Pathology, Kanagawa Cancer Center, 2-3-2, Nakao, Asahi-Ku, Yokohama, Kanagawa 241-8515 Japan
| | - Kota Washimi
- Department of Pathology, Kanagawa Cancer Center, 2-3-2, Nakao, Asahi-Ku, Yokohama, Kanagawa 241-8515 Japan
| | - Kae Kawachi
- Department of Pathology, Kanagawa Cancer Center, 2-3-2, Nakao, Asahi-Ku, Yokohama, Kanagawa 241-8515 Japan
| | - Yoichi Kameda
- Department of Pathology, Kanagawa Cancer Center, 2-3-2, Nakao, Asahi-Ku, Yokohama, Kanagawa 241-8515 Japan
| | - Tomoyuki Yokose
- Department of Pathology, Kanagawa Cancer Center, 2-3-2, Nakao, Asahi-Ku, Yokohama, Kanagawa 241-8515 Japan
| |
Collapse
|