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Wen I, Kim LH, Wong M, di Lernia S, Maclean F, Lee A, Lee A, Ganapathy A, Chan D. Renal mucinous cystadenoma in the context of lynch syndrome and colonic neuroendocrine neoplasm: a case report. Oxf Med Case Reports 2025; 2025:omaf012. [PMID: 40162154 PMCID: PMC11952886 DOI: 10.1093/omcr/omaf012] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 10/25/2024] [Revised: 12/26/2024] [Accepted: 01/26/2025] [Indexed: 04/02/2025] Open
Abstract
Renal mucinous cystadenoma (RMC) is an exceptionally rare finding with a poorly understood pathogenesis. Links between RMC and other malignancies are not well described, nor are there known associations with familial cancer disorders. We present the first case of RMC associated with Lynch syndrome (LS) and neuroendocrine neoplasm. A 56-year-old woman presented with iron deficiency leading to a diagnosis of a colonic mixed neuroendocrine-non-neuroendocrine carcinoma. After initial surgery, she experienced local recurrences at 6 and 12 months, treated with resection and adjuvant chemotherapy. Tumours displayed deficient MMR proteins with BRAF positivity, and germline testing confirmed LS. Surveillance subsequently revealed a complex cyst arising from a horseshoe kidney, for which she underwent a partial nephrectomy. Histopathology confirmed this to be renal mucinous cystadenoma arising from the pelvicalyceal system. This case underscores the need for further investigation into RMC pathogenesis and its potential association with LS.
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Affiliation(s)
- Ivy Wen
- Department of Medical Oncology, Royal North Shore Hospital, Reserve Road, St Leonards, 2065, Sydney, New South Wales, Australia
| | - Lawrence H Kim
- Department of Urology, Westmead Hospital, Hawkesbury Road, Westmead, 2145, Sydney, New South Wales, Australia
- University of Sydney, Camperdown, 2050, Sydney, New South Wales, Australia
| | - Matt Wong
- University of Newcastle, University Drive, Callaghan, 2308, New South Wales, Australia
| | - Shannon di Lernia
- Department of General Surgery, Gosford Private Hospital, Burrabil Avenue, Gosford, 2250, New South Wales, Australia
| | - Fiona Maclean
- Anatomical Pathology, Douglass Hanly Moir Pathology, Giffnock Avenue, Macquarie Park, 2113, Sydney New South Wales, Australia
| | - Allen Lee
- Department of Nuclear Medicine, Royal North Shore Hospital, Reserve Road, St Leonards, 2065, Sydney, New South Wales, Australia
| | - Adrian Lee
- Department of Medical Oncology, Royal North Shore Hospital, Reserve Road, St Leonards, 2065, Sydney, New South Wales, Australia
- University of Sydney, Camperdown, 2050, Sydney, New South Wales, Australia
| | - Anu Ganapathy
- Department of Medical Oncology, Royal North Shore Hospital, Reserve Road, St Leonards, 2065, Sydney, New South Wales, Australia
| | - David Chan
- Department of Medical Oncology, Royal North Shore Hospital, Reserve Road, St Leonards, 2065, Sydney, New South Wales, Australia
- University of Sydney, Camperdown, 2050, Sydney, New South Wales, Australia
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Matsushita K, Urakami A, Matsubara M, Akagi A, Urano T, Ishida N, Takaoka M, Fujiwara H, Akiyama T, Yamatsuji T. Mixed neuroendocrine-non-neuroendocrine neoplasms (MiNEN) in the non-ampullary region of the duodenum: a case report. J Surg Case Rep 2025; 2025:rjaf189. [PMID: 40191662 PMCID: PMC11971567 DOI: 10.1093/jscr/rjaf189] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 02/20/2025] [Accepted: 03/15/2025] [Indexed: 04/09/2025] Open
Abstract
Mixed neuroendocrine-non-neuroendocrine neoplasms (MiNENs), a very rare form of neuroendocrine neoplasm (NEN), are associated with poor prognosis. Herein, we present a rare case of duodenal MiNEN composed of neuroendocrine carcinoma (NEC) and adenocarcinoma in a non-ampullary lesion. A 70-year-old male referred to our hospital was found to have duodenal perforation with a tumor in the 2nd duodenal portion based on computed tomography. Biopsy revealed a poorly differentiated adenocarcinoma. Three weeks following emergency surgery for the perforation, pancreaticoduodenectomy of the duodenal tumor was performed. The resected specimen comprised a 45 × 35-mm protruding and circumferential tumor extracted from the 2nd portion, with no involvement of the papilla of Vater. Histopathological examination revealed coexisting poorly differentiated adenocarcinoma and NEC components. The final pathological diagnosis was MiNEN (por2 > sig + NEC). TNM: pT4b (SE), pN2, M0, pStageIIIb. The postoperative course was uneventful, developing peritoneal dissemination and multiple bone metastasis after 5 months, with death 7 months postoperatively.
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Affiliation(s)
- Kazuki Matsushita
- Department of General Surgery, Kawasaki Medical School General Medical Center, 2-6-1 Nakasange, Kita-ku, Okayama 700-8505, Japan
| | - Atsushi Urakami
- Department of General Surgery, Kawasaki Medical School General Medical Center, 2-6-1 Nakasange, Kita-ku, Okayama 700-8505, Japan
| | - Masaki Matsubara
- Department of General Surgery, Kawasaki Medical School General Medical Center, 2-6-1 Nakasange, Kita-ku, Okayama 700-8505, Japan
| | - Akihisa Akagi
- Department of General Surgery, Kawasaki Medical School General Medical Center, 2-6-1 Nakasange, Kita-ku, Okayama 700-8505, Japan
| | - Takashi Urano
- Department of General Surgery, Kawasaki Medical School General Medical Center, 2-6-1 Nakasange, Kita-ku, Okayama 700-8505, Japan
| | - Naomasa Ishida
- Department of General Surgery, Kawasaki Medical School General Medical Center, 2-6-1 Nakasange, Kita-ku, Okayama 700-8505, Japan
| | - Munenori Takaoka
- Department of General Surgery, Kawasaki Medical School General Medical Center, 2-6-1 Nakasange, Kita-ku, Okayama 700-8505, Japan
| | - Hideyo Fujiwara
- Department of Pathology, Kawasaki Medical School General Medical Center, 2-6-1 Nakasange, Kita-ku, Okayama 700-8505, Japan
| | - Takashi Akiyama
- Department of Pathology, Kawasaki Medical School General Medical Center, 2-6-1 Nakasange, Kita-ku, Okayama 700-8505, Japan
| | - Tomoki Yamatsuji
- Department of General Surgery, Kawasaki Medical School General Medical Center, 2-6-1 Nakasange, Kita-ku, Okayama 700-8505, Japan
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Cui W, Yang C, Ding X, Liu J, Zhang H, Xu H. Fine Needle Aspiration Cytological Diagnosis of Primary Breast Large-Cell Neuroendocrine Carcinoma/Squamous Cell Carcinoma. Diagn Cytopathol 2025; 53:E38-E45. [PMID: 39635966 DOI: 10.1002/dc.25427] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 09/27/2024] [Revised: 11/02/2024] [Accepted: 11/22/2024] [Indexed: 12/07/2024]
Abstract
Primary breast large-cell neuroendocrine carcinoma (LCNEC)/squamous cell carcinoma (SCC), also referred to as mixed neuroendocrine/non-neuroendocrine neoplasms of the breast (Br-MiNENs), represents an exceedingly rare malignancy. We report the first case of primary breast LCNEC/SCC diagnosed via ultrasound-guided fine-needle aspiration (FNA) biopsy of the left supraclavicular and left internal mammary lymph nodes. The patient, a 40-year-old female, underwent a lumpectomy followed by breast-conserving surgery and was diagnosed with primary breast LCNEC. Notably, within merely four months following the breast-conserving surgery, PET-CT revealed lymph node enlargement, prompting the performance of FNA. FNA cytology of metastatic lymph nodes revealed two distinct tumor components, allowing for clear differentiation between LCNEC and SCC in the smear. The diagnosis was further corroborated by immunocytochemical (ICC) staining of the cell blocks. Subsequently, histopathological re-examination of the breast mass revealed occult SCC components comprising less than 1% of the tumor cells. Additionally, the case exhibited triple-negative breast cancer, with PIK3CA, TP53, RB1, and BCL2L11 mutations identified through next-generation sequencing (NGS). Br-MiNEN is exceedingly rare, and its cytological diagnosis poses significant challenges. It is recommended that a detailed and objective description of each tumor component and its proportion be provided. This report provides the first detailed description of the FNA cytology of LCNEC/SCC, thereby enhancing cytopathologists' comprehension of this tumor. Auxiliary studies, including ICC staining and molecular biology assays, are crucial for accurate diagnosis, therapy, and prognosis.
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Affiliation(s)
- Wenjing Cui
- Department of Pathology, State Key Laboratory of Cancer Biology, Xijing Hospital and School of Basic Medicine, Fourth Military Medical University, Xi'an, Shaanxi, China
| | - Changwei Yang
- Department of Emergency Medicine, Xi'an Honghui Hospital, Xi'an, Shaanxi, China
| | - Xiaochen Ding
- Department of Experimental Surgery, Xijing Hospital, Fourth Military Medical University, Xi'an, Shaanxi, China
| | - Jiayan Liu
- Department of Pathology, State Key Laboratory of Cancer Biology, Xijing Hospital and School of Basic Medicine, Fourth Military Medical University, Xi'an, Shaanxi, China
| | - Hongjuan Zhang
- Department of Pathology, State Key Laboratory of Cancer Biology, Xijing Hospital and School of Basic Medicine, Fourth Military Medical University, Xi'an, Shaanxi, China
| | - Hong Xu
- Department of Pathology, State Key Laboratory of Cancer Biology, Xijing Hospital and School of Basic Medicine, Fourth Military Medical University, Xi'an, Shaanxi, China
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Zhou K, Li ZW, Wu Y, Wang ZJ, Wang LQ, Zhou LX, Jia L, Ji K, Yang XS, Zhang J, Wu XJ, Wang AQ, Bu ZD. Lymph node metastatic patterns of gastric carcinoma with a combination of adenocarcinoma and neuroendocrine carcinoma components. World J Gastroenterol 2025; 31:102347. [DOI: 10.3748/wjg.v31.i8.102347] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 10/16/2024] [Revised: 12/14/2024] [Accepted: 01/07/2025] [Indexed: 01/23/2025] Open
Abstract
BACKGROUND Gastric mixed-adenoneuroendocrine carcinoma (G-MANEC) is a subtype of gastric cancer. Building upon prior research findings, we propose that tumours containing both neuroendocrine carcinoma (NEC) and adenocarcinoma (AC) components, with each component ranging from 1% to 99% of the tumour, be classified as a distinct entity. We hereby term this adenoneuroendocrine mixed gastric cancer (G-ANEC). Research on lymph node (LN) involvement in G-MANEC has focused mainly on metastasis status, with limited studies on metastatic composition.
AIM To investigate the LN metastasis patterns of G-ANEC, the clinicopathological features associated with these metastasis patterns, and to explore adjuvant chemotherapy regimens for G-ANEC.
METHODS We analyzed 68 G-ANEC cases treated with radical surgery and confirmed LN metastasis at Peking University Cancer Hospital between August 2012 and June 2022. Utilizing χ2 tests in IBM statistical product and service solutions statistics and R software.
RESULTS We identified three distinct LN metastasis patterns in G-ANEC that were significantly associated with the NEC proportion, tumour invasion depth, Lauren classification, and tumour location (P values: 0.008, 0.015, 0.01, and 0.004, respectively). When the SOX/XELOX regimen was applied for adjuvant chemotherapy, patients with LN metastasis comprising only AC exhibited better overall survival (OS) (94.25 ± 11.07 months vs 54.36 ± 11.36 months) than did those with NEC. When LN metastasis components contained NEC, there was a trend towards improved OS (64 ± 10.77 months vs 54.35 ± 11.36 months) and disease-free survival (71.28 ± 9.92 months vs 66.28 ± 11.93 months) in patients treated with the etoposide and cisplatin compared to those receiving the SOX/XELOX regimen.
CONCLUSION We found a significant correlation between the NEC percentage, tumour invasion depth, Lauren classification, and tumour location and LN metastasis patterns in G-ANEC. For G-ANEC, a lower proportion of NEC or AC in the primary lesion does not preclude the possibility of these components metastasizing to the LNs. Different adjuvant chemotherapy regimens should be administered on the basis of the varying components of LN metastasis in patients with G-ANEC.
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Affiliation(s)
- Kai Zhou
- Department of Gastrointestinal Surgery, Peking University Cancer Hospital and Institute, Beijing 100142, China
| | - Zhong-Wu Li
- Department of Pathology, Key Laboratory of Carcinogenesis and Translational Research (Ministry of Education), Peking University Cancer Hospital and Institute, Beijing 100142, China
| | - Yan Wu
- Department of Pathology, Key Laboratory of Carcinogenesis and Translational Research (Ministry of Education), Peking University Cancer Hospital and Institute, Beijing 100142, China
| | - Zhi-Jie Wang
- Department of Gastrointestinal Surgery, Peking University Cancer Hospital and Institute, Beijing 100142, China
| | - Ling-Qian Wang
- Department of Gastrointestinal Surgery, Peking University Cancer Hospital and Institute, Beijing 100142, China
| | - Li-Xin Zhou
- Department of Pathology, Key Laboratory of Carcinogenesis and Translational Research (Ministry of Education), Peking University Cancer Hospital and Institute, Beijing 100142, China
| | - Ling Jia
- Department of Pathology, Key Laboratory of Carcinogenesis and Translational Research (Ministry of Education), Peking University Cancer Hospital and Institute, Beijing 100142, China
| | - Ke Ji
- Department of Gastrointestinal Surgery, Peking University Cancer Hospital and Institute, Beijing 100142, China
| | - Xue-Song Yang
- Department of Gastrointestinal Surgery, Peking University Cancer Hospital and Institute, Beijing 100142, China
| | - Ji Zhang
- Department of Gastrointestinal Surgery, Peking University Cancer Hospital and Institute, Beijing 100142, China
| | - Xiao-Jiang Wu
- Department of Gastrointestinal Surgery, Peking University Cancer Hospital and Institute, Beijing 100142, China
| | - An-Qiang Wang
- Department of Gastrointestinal Surgery, Peking University Cancer Hospital and Institute, Beijing 100142, China
| | - Zhao-De Bu
- Department of Gastrointestinal Surgery, Peking University Cancer Hospital and Institute, Beijing 100142, China
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Tang J, Wei S, Tang G, Zhao P. Development of a Nomogram-Based Online Calculator for Predicting Cancer-Specific Survival in Patients With Digestive Tract Mixed Neuroendocrine-Non-Neuroendocrine Neoplasms (MiNENs): An Analysis of the SEER Database. Cancer Rep (Hoboken) 2025; 8:e70156. [PMID: 39972644 PMCID: PMC11839491 DOI: 10.1002/cnr2.70156] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 10/27/2024] [Revised: 01/28/2025] [Accepted: 02/07/2025] [Indexed: 02/21/2025] Open
Abstract
AIMS Mixed neuroendocrine-non-neuroendocrine neoplasms (MiNENs) represent a rare and heterogeneous subgroup of neoplasms that typically consist of a neuroendocrine (NE) component, most commonly neuroendocrine carcinoma (NEC), alongside a non-neuroendocrine (non-NE) component. They commonly occur in the digestive tract, and their prognosis is influenced by multiple factors. This article aimed to identify factors that affect the cancer-specific survival (CSS) of MiNENs and develop an effective nomogram-based online calculator to validate its effectiveness. METHODS The clinical, pathological, epidemiological, and survival data of patients with digestive tract MiNENs were collected from the Surveillance, Epidemiology, and End Results (SEER) database spanning from 2000 to 2020. Then, the dataset was divided into a training cohort and a validation cohort. The χ2 test or Fisher's exact test was utilized to assess differences in demographic and clinicopathological characteristics between the two groups. Kaplan-Meier survival curves and log-rank tests were employed to conduct survival analysis. Additionally, univariate and multivariate Cox regression analyses were performed to identify potential prognostic factors and develop nomograms and an online calculator for predicting CSS at 1, 3, and 5 years. Lastly, the predictive ability of the online calculator was subsequently compared with the sixth edition of the American Joint Committee on Cancer (AJCC) TNM staging system using the Harrell concordance index (C-index), the area under the receiver operating characteristic curve (AUC), calibration curve, and decision curve analysis (DCA). RESULTS A total of 330 patients were randomly assigned to two groups, namely, the training cohort (n = 231) and the validation cohort (n = 99). The log-rank test revealed a significant association between the lower cumulative survival and age ≥ 65 years, poor tumor grade, lack of surgical treatment, TNM stages III and IV, and distant metastasis. In the training cohort, a nomogram incorporating grade, surgery, TNM stage, and tumor metastasis was developed, which demonstrated favorable calibration and discriminatory capabilities. Compared to TNM staging, the nomogram exhibited satisfactory performance in predicting 1-year, 3-year, and 5-year CSS rates. The C-index value was 0.787 in the training cohort and 0.738 in the validation cohort, respectively. In the training cohort, the nomogram achieved an AUC of 85.81%, 85.86%, and 87.32% for 1-year CSS, 3-year CSS, and 5-year CSS, respectively. In contrast, these AUC values were 78.46%, 81.50%, and 83.88% in the validation cohort, respectively. CONCLUSIONS The developed online calculator offers a novel approach to predicting the prognosis of patients with digestive tract MiNENs. Indeed, it can accurately predict the CSS of these patients over 1, 3, and 5 years, thereby assisting in enhancing prognosis and formulating appropriate treatment strategies.
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Affiliation(s)
- Jing Tang
- Department of GastroenterologyGuangyuan Central HospitalGuangyuanChina
| | - Siqi Wei
- Department of GastroenterologyGuangyuan Central HospitalGuangyuanChina
| | - Guobin Tang
- Department of GastroenterologyGuangyuan Central HospitalGuangyuanChina
| | - Ping Zhao
- Department of GastroenterologyGuangyuan Central HospitalGuangyuanChina
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Pavlidis ET, Galanis IN, Pavlidis TE. Critical considerations for the management of gastrointestinal mixed neuroendocrine non-neuroendocrine neoplasms and pure neuroendocrine carcinomas. World J Gastrointest Oncol 2024; 16:4559-4564. [PMID: 39678788 PMCID: PMC11577359 DOI: 10.4251/wjgo.v16.i12.4559] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 08/10/2024] [Revised: 09/25/2024] [Accepted: 10/18/2024] [Indexed: 11/12/2024] Open
Abstract
Mixed neuroendocrine non-neuroendocrine neoplasms constitute rare tumors that are located mainly in the gastrointestinal (GI) tract and have high degrees of malignancy, and the frequency of these tumors has been increasing. They consist of a neuroendocrine neoplastic component with another component of adenocarcinoma usually and have a dismal prognosis. The rare GI pure neuroendocrine carcinoma is highly aggressive and requires complex and extensive management since a genetic distinction exists between it and GI non-neuroendocrine neoplasms, which are generally slow-growing lesions. The most common GI-mixed neuroendocrine non-neuroendocrine neoplasms are colorectal, followed by gastric, mainly in the gastroesophageal junction. Current imaging modalities of nuclear medicine and radiology play important roles in the accuracy of diagnosis. Liquid biopsy may contribute to early detection and timely diagnosis. Ultrasonography, either endoscopic or abdominal, is a technique that contributes to a diagnosis; additionally, contrast-enhanced ultrasonography is very helpful in follow-up appointments. Histopathology establishes a definite diagnosis and stage by evaluating the cell differentiation grade and the cell proliferation index Ki67. The genetic profile can be valuable in diagnosis and gene therapy. Surgical resection with wide lymphadenectomy, whenever possible, and adjuvant chemotherapy constitute the main therapeutic management strategies. Targeted therapy and immunotherapy achieve encouraging results.
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Affiliation(s)
- Efstathios T Pavlidis
- The 2nd Department of Propaedeutic Surgery, Hippokration General Hospital, School of Medicine, Aristotle University of Thessaloniki, Thessaloniki 54642, Greece
| | - Ioannis N Galanis
- The 2nd Department of Propaedeutic Surgery, Hippokration General Hospital, School of Medicine, Aristotle University of Thessaloniki, Thessaloniki 54642, Greece
| | - Theodoros E Pavlidis
- The 2nd Department of Propaedeutic Surgery, Hippokration General Hospital, School of Medicine, Aristotle University of Thessaloniki, Thessaloniki 54642, Greece
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Liu P, Zhang Y. A rare early gastric mixed neuroendocrine-non-neuroendocrine neoplasms misdignosed to early gastric cancer. REVISTA ESPANOLA DE ENFERMEDADES DIGESTIVAS 2024. [PMID: 39588958 DOI: 10.17235/reed.2024.10933/2024] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Track Full Text] [Subscribe] [Scholar Register] [Indexed: 11/27/2024]
Abstract
Recently, mixed neuroendocrine-non-neuroendocrine neoplasms (MiNENs) have attracted more attention. However, early gastric MiNEN is uncommon, and there are rare reports on the characteristics of it. As its clinical and endoscopic features are resemble to early gastric cancer (EGC), it can be easily misdiagnosed. The purpose of this article is to elaborate the endoscopic and pathological characteristics of early gastric MiNEN, which is of great significance for distinguishing it from EGC. A 67-year-old male patient underwent EGD revealing a 0-IIc+IIa lesion on the lesser curvature of the lower stomach body. The biopsy pathology indicated the lesion was an EGC. Endoscopic submucosal dissection (ESD) was subsequently applied to remove the lesion. According to postoperative pathological, the lesion was diagnosed as early MiNEN (tubular adenocarcinoma 50%, neuroendocrine carcinoma 50%), pT1b (submucosa 600 μm), pUL (1), Ly (0), v (1), pHM0, pVM0. And after that, surgical resection were added. A follow-up CT scan and endoscopy performed six months later revealed no metastasis or recurrence. This case highlights that early gastric MiNEN could mimic ECG. We deemed that ME-NBI combined with pathological biopsy could contribute to the identification of early gastric MiNEN.
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Affiliation(s)
- Pengwei Liu
- Gastroenterology, The First Affiliated Hospital of Wannan Medical College, China
| | - Yan Zhang
- Gastroenterology, The First Affiliated Hospital of Wannan Medical College
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Elmore LR, Drymon A, Toca A, Gritsiuta AI, Gilleland W. Collision tumor of the cecum and ileocecal valve composed of mucinous adenocarcinoma and neuroendocrine tumor: a case report. AME Case Rep 2024; 8:109. [PMID: 39380877 PMCID: PMC11459393 DOI: 10.21037/acr-24-87] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 04/22/2024] [Accepted: 07/26/2024] [Indexed: 10/10/2024]
Abstract
Background Collision tumors of the gastrointestinal (GI) tract are thought to be uncommon, with those of the colon being rare with very few cases reported in current literature. There are three proposed theories regarding the etiology of collision tumors currently, including the "double primaries", the "biclonal malignant transformation", and the "tumor-to-tumor carcinogenesis" theories. Prognosis of collision tumors remains unclear. To our knowledge, this is the fifth case of a collision carcinoma involving the cecum and ileocecal valve and the first report of a collision carcinoma including both mucinous adenocarcinoma and neuroendocrine tumor of the cecum and the ileocecal valve. The aim of this paper is to explore the history of collision tumors and associated nomenclature, defined diagnostic criteria, and proposed theories for etiology in addition to patient presentation, approach to diagnosis, treatment options, and prognosis. Case Description We present the case of an 83-year-old female who presented to the emergency room with a 4-month history of cramping abdominal pain associated with nausea, emesis, and decreased appetite with associated weight loss. Diagnostic imaging demonstrated a bowel obstruction secondary to a mass in the cecum and she underwent an exploratory laparotomy with right hemicolectomy. She was found to have a collision carcinoma of the cecum and ileocecal valve containing both mucinous adenocarcinoma and neuroendocrine tumor. Diagnosis was confirmed post-operatively with pathologic examination and immunohistochemical testing. Conclusions Diagnosing collision tumors upon patient presentation is exceedingly difficult as the symptoms are often identical to other neoplasms of the GI tract and vary based on location of the tumor. It is thought that the true prevalence of collision tumors is underestimated due to history of changing nomenclature, unclear diagnostic criteria, unreported cases, and unrecognized cases. Furthermore, new advances in immunohistochemical evaluation have allowed for better characterization of these neoplasms. With clarification regarding nomenclature, diagnostic criteria and expanding awareness, it is our hope that this leads to an increase in reported cases, allowing for an expanded discussion and resulting growth of literature and further studies. Further knowledge regarding the pathogenesis, treatment, and prognosis is needed.
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Affiliation(s)
- Leslie R. Elmore
- Department of Surgery, University of Pittsburgh Medical Center, Pittsburgh, PA, USA
| | - Alexandra Drymon
- Department of Surgery, University of Pittsburgh Medical Center, Pittsburgh, PA, USA
| | - Angel Toca
- Department of Pathology, University of Pittsburgh Medical Center, Pittsburgh, PA, USA
| | - Andrei I. Gritsiuta
- Department of Surgery, University of Pittsburgh Medical Center, Pittsburgh, PA, USA
| | - William Gilleland
- Department of Surgery, University of Pittsburgh Medical Center, Pittsburgh, PA, USA
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Kawakami M, Nakazato H, Tokisawa H, Tomiyama T, Miyagi J, Nagayoshi S, Tamashiro K, Yoshimi N, Ohmine Y. Preoperative Diagnostic Dilemma in Rapidly Progressive Mixed Neuroendocrine-Nonendocrine Neoplasm of the Ascending Colon: A Case Report. Case Rep Gastroenterol 2024; 18:361-366. [DOI: 10.1159/000539978] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 01/05/2025] Open
Abstract
Introduction: Mixed neuroendocrine-nonendocrine neoplasms (MiNENs) of the digestive tract are rare. They contain both neuroendocrine and nonendocrine components. We here report a rare case of a metastatic MiNEN originating in the ascending colon. Case Presentation: An 80-year-old man presented with abdominal pain and vomiting. Colonoscopy showed an obstructing mass in the ascending colon, and a biopsy resulted in diagnosis of an adenocarcinoma. A CT scan revealed multiple liver metastases. Thus, our diagnosis was stage IV colon adenocarcinoma (cT4aN1bM1a according to TNM Classification of Malignant Tumors). The primary lesion was resected to alleviate symptoms. Histopathological and immunohistochemical examination revealed a well-differentiated adenocarcinoma and a small-cell neuroendocrine carcinoma, leading to the diagnosis of a MiNEN. The patient declined postoperative treatment and died approximately 2 months after the surgery. Conclusion: The incidence of MiNENs may be underestimated because they lack typical symptoms or imaging features and are therefore often only diagnosed after resection. Awareness of colonic MiNENs and further accumulation of cases are necessary to improve the outcomes.
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Shenoy S. Mixed neuroendocrine and adenocarcinoma of gastrointestinal tract: A complex diagnosis and therapeutic challenge. World J Gastrointest Oncol 2024; 16:2295-2299. [PMID: 38994166 PMCID: PMC11236242 DOI: 10.4251/wjgo.v16.i6.2295] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 01/08/2024] [Revised: 03/05/2024] [Accepted: 04/10/2024] [Indexed: 06/13/2024] Open
Abstract
In this editorial we comment on the manuscript describing a case of adenocarcinoma mixed with a neuroendocrine carcinoma of the gastroesophageal junction. Mixed neuroendocrine and non-neuroendocrine neoplasms of the gastrointestinal system are rare heterogeneous group of tumors characterized by a high malignant potential, rapid growth, and poor prognosis. Due to the rarity of these cancers, the standard therapy is poorly defined. The diagnosis of these tumors is based on combination of morphological features, immunohistochemical and neuroendocrine and epithelial cell markers. Both endocrine and epithelial cell components can act independently of each other and thus, careful grading of each component separately is required. These cancers are aggressive in nature and the potential of each component has paramount importance in the choice of treatment and response. Regardless of the organ of origin, these tumors portend poor prognosis with increased proportion of neuroendocrine component. Multidisciplinary services and strategies are required for the management of these mixed malignancies to provide the best oncological outcomes. The etiopathogenesis of these mixed tumors remains obscure but poses interesting question. We briefly discuss a few salient points in this editorial.
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Affiliation(s)
- Santosh Shenoy
- Department of General Surgery, Kansas City VA Medical Center, University of Missouri - Kansas City, Kansas City, MO 64128, United States
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11
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Díaz-López S, Jiménez-Castro J, Robles-Barraza CE, Ayala-de Miguel C, Chaves-Conde M. Mixed neuroendocrine non-neuroendocrine neoplasms in gastroenteropancreatic tract. World J Gastrointest Oncol 2024; 16:1166-1179. [PMID: 38660639 PMCID: PMC11037054 DOI: 10.4251/wjgo.v16.i4.1166] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 12/06/2023] [Revised: 01/17/2024] [Accepted: 02/18/2024] [Indexed: 04/10/2024] Open
Abstract
Mixed neuroendocrine non-neuroendocrine neoplasms (MiNENs) are a heterogeneous group of malignant neoplasms that can settle in the gastroenteropancreatic tract. They are composed of a neuroendocrine (NE) and a non-NE component in at least 30% of each tumour. The non-NE component can include different histological combinations of glandular, squamous, mucinous and sarcomatoid phenotypes, and one or both of the components can be low-or high grade malignant. Recent changes in the nomenclature of these neoplasms might lead to great deal of confusion, and the lack of specific clinical trials is the main reason why their management is difficult. The review aims to clarify the definition of MiNEN and analyze available evidence about their diagnosis and treatment options according to their location and extension through careful analysis of the available data. It would be important to reach a general consensus on their diagnosis in order to construct a classification that remains stable over time and facilitates the design of clinical trials that, due to their low incidence, will require long recruitment periods.
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Affiliation(s)
- Sebastián Díaz-López
- Medical Oncology Department, Hospital Universitario Valme, Seville 41014, Andalucía, Spain
| | | | | | - Carlos Ayala-de Miguel
- Medical Oncology Department, Hospital Universitario Valme, Seville 41014, Andalucía, Spain
| | - Manuel Chaves-Conde
- Medical Oncology Department, Hospital Universitario Valme, Seville 41014, Andalucía, Spain
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12
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Liu L, Li Q, Liu W, Qiu Z, Wu Z, Yu D, Deng W. Gastric mixed neuroendocrine non-neuroendocrine neoplasms. Front Oncol 2024; 14:1335760. [PMID: 38655135 PMCID: PMC11036886 DOI: 10.3389/fonc.2024.1335760] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 12/05/2023] [Accepted: 03/26/2024] [Indexed: 04/26/2024] Open
Abstract
The uncommon tumour known as gastric mixed neuroendocrine-non-neuroendocrine neoplasms (G-MiNENs) is made up of parts of neuroendocrine carcinoma and adenocarcinoma. The biological and clinical features are different from those of gastric adenocarcinoma. Their pathophysiology, diagnostic standards, and clinical behaviour have all been the subject of lengthy debates, and their nomenclature has undergone multiple changes. Its emergence has created new challenges in the classification and diagnosis of gastric tumours. This review will update information on the topic, covering molecular aspects, diagnostic criteria, treatment, and prognostic factor discovery. It will also provide a historical context that will aid in understanding the evolution of the idea and nomenclature of mixed gastric tumours. Additionally, it will provide the reader a thorough understanding of this difficult topic of cancer that is applicable to real-world situations.
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Affiliation(s)
- Li Liu
- Department of General Surgery, Renmin Hospital of Wuhan University, Wuhan, Hubei, China
| | - Qian Li
- Department of Ultrasound Imaging, Hubei Cancer Hospital, Tongji Medical College, Huazhong University of Science and Technology, Wuhan, Hubei, China
| | - Wenxuan Liu
- Department of General Surgery, Renmin Hospital of Wuhan University, Wuhan, Hubei, China
| | - Zhendong Qiu
- Department of General Surgery, Renmin Hospital of Wuhan University, Wuhan, Hubei, China
| | - Zhongkai Wu
- Department of General Surgery, Renmin Hospital of Wuhan University, Wuhan, Hubei, China
| | - Danli Yu
- Department of Ultrasound Imaging, Hubei Cancer Hospital, Tongji Medical College, Huazhong University of Science and Technology, Wuhan, Hubei, China
| | - Wenhong Deng
- Department of General Surgery, Renmin Hospital of Wuhan University, Wuhan, Hubei, China
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13
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Ashindoitiang JA, Nwagbara VIC, Ipeh TU, Chukwu B, Obanife HO, Eyong AO, Asuquo ME. Retroperitoneal mixed neuroendocrine non-neuroendocrine neoplasm: A case report and review of literature. Rare Tumors 2024; 16:20363613241242383. [PMID: 38525086 PMCID: PMC10958797 DOI: 10.1177/20363613241242383] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 01/14/2023] [Accepted: 03/08/2024] [Indexed: 03/26/2024] Open
Abstract
The existence of both neuroendocrine and non-neuroendocrine histology in variable proportion in a lesion has been described by the World Health Organisation (WHO) as mixed neuroendocrine and non-neuroendocrine neoplasm (MiNEN). The pathogenesis of this tumour remains controversial but molecular studies point towards a common monoclonal origin. Tumours are classified as functioning and nonfunctioning based on substances secreted. The nonfunctioning tumours may be discovered due to its local effect. Presented is a 66-year-old male with an intra-abdominal mass, underwent laparotomy and excision biopsy with transient right lower limb lymphoedema. Histology confirmed retroperitoneal MiNEN with no evidence of tumour recurrence 12 months following surgery. MiNENs should be considered as a differential diagnosis in patients with intra-abdominal mass. Surgical resection is recommended as this may offer the best treatment option.
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Affiliation(s)
- John Adi Ashindoitiang
- Department of Surgery, University of Calabar/University of Calabar Teaching Hospital, Calabar
| | | | - Theophilus Ugbem Ipeh
- Department of Pathology, University of Calabar/University of Calabar Teaching Hospital, Calabar
| | - Ben Chukwu
- Department of Surgery, University of Calabar Teaching Hospital, Calabar
| | - Henry Olaleye Obanife
- Department of Surgery, University of Calabar/University of Calabar Teaching Hospital, Calabar
| | | | - Maurice Efana Asuquo
- Department of Surgery, University of Calabar/University of Calabar Teaching Hospital, Calabar
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14
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Verdasca FR, Ferreira R, Montenegro A, Mendes JL, Furtado I, Escaleira R, Fernandes V, Seladas M, Cristovão M, da Luz R, Guerreiro I. Mixed Neuroendocrine-Non-Neuroendocrine Neoplasms of the Rectum: A Case Report. Case Rep Oncol 2024; 17:587-595. [PMID: 39015645 PMCID: PMC11249797 DOI: 10.1159/000538384] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 01/08/2024] [Accepted: 03/04/2024] [Indexed: 07/18/2024] Open
Abstract
Introduction Mixed neuroendocrine-non-neuroendocrine neoplasms (MiNEN) represent roughly 1-2% of all colorectal malignancies. Given the rareness and heterogeneity of these mixed tumors, recognition and accurate diagnosis remain a challenge. In the absence of established guidelines, they are treated according to the standard of care for pure neuroendocrine carcinomas or adenocarcinomas from similar sites of origin. Case Presentation We herein report a case of a rectal MiNEN in a 55-year-old male. He underwent colonoscopy for rectal bleeding and mucus emission, which revealed a vegetating lesion located approximately 8 cm from the anal verge, corresponding to a moderately differentiated low-grade adenocarcinoma of the rectum. Computed tomography scan and magnetic resonance imaging uncovered the presence of lung, lymph node, and subcutaneous implant metastases. The biopsy of the cutaneous implant showed neuroendocrine carcinoma Ki-67 90%. The patient underwent systemic chemotherapy. Conclusion High-grade MiNEN tumors are the most commonly encountered in clinical practice and have an aggressive biological behavior. Little is known about the genetic drivers of this neoplasm and its pathogenesis remains controversial. Clinical and pathological awareness of this rare entity is a key step to design future targeted therapies and improve treatment options. The aim of this case report is to further our understanding regarding the clinical presentation, radiological features, pathology, management, and prognosis of MiNEN.
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Affiliation(s)
| | - Rita Ferreira
- Oncology Department, Unidade Local de Saúde São José, Lisboa, Portugal
| | | | - José Leão Mendes
- Oncology Department, Unidade Local de Saúde São José, Lisboa, Portugal
| | - Ivánia Furtado
- Oncology Department, Unidade Local de Saúde São José, Lisboa, Portugal
| | - Rui Escaleira
- Oncology Department, Unidade Local de Saúde São José, Lisboa, Portugal
| | - Válter Fernandes
- Oncology Department, Unidade Local de Saúde São José, Lisboa, Portugal
| | - Marta Seladas
- Oncology Department, Unidade Local de Saúde São José, Lisboa, Portugal
| | - Miguel Cristovão
- Pathology Department, Unidade Local de Saúde São José, Lisboa, Portugal
| | - Ricardo da Luz
- Oncology Department, Unidade Local de Saúde São José, Lisboa, Portugal
| | - Inês Guerreiro
- Oncology Department, Unidade Local de Saúde São José, Lisboa, Portugal
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15
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Zhang L, Wang X, Wang Y, Zeng Y, Li L. Clinicopathological characteristics of co-existing or mixed colorectal cancer and neuroendocrine tumor: Report of five cases. Open Life Sci 2023; 18:20220774. [PMID: 38152582 PMCID: PMC10751993 DOI: 10.1515/biol-2022-0774] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 05/24/2023] [Revised: 10/14/2023] [Accepted: 10/27/2023] [Indexed: 12/29/2023] Open
Abstract
Coexisting or mixed type of colorectal tumors has been rarely reported. This study was designed to investigate clinicopathological characteristics of co-existing or mixed colorectal adenocarcinoma and highly differentiated neuroendocrine tumor (NET-G1). To do that, clinicopathological characteristics of five cases of co-existing or mixed colorectal adenocarcinoma and NET-G1 admitted to our institution between 2017 and 2021 were retrospectively analyzed and literature review was conducted. Four patients were male and one female, aged 62-75 years old. Among them, four cases were diagnosed with rectal cancer and one case of colon cancer. Gross examination found that one patient was diagnosed with multiple colon polyps including three malignant polyps, and the remaining four cases of ulcerous masses. The tumors infiltrated into the muscle layer in two cases, and three cases with tumors infiltrating into surrounding adipose tissues. Microscopic examination revealed one patient developed poorly differentiated adenocarcinoma and four cases of moderately differentiated adenocarcinoma. Four patients had adenocarcinoma and NET-G1 in colon, and one case of adenocarcinoma in colon and NET-G1 in appendix. To conclude, co-existing or mixed colorectal tumors are extremely rare in clinical settings. Clinicopathological characteristics of five cases of co-existing or mixed adenocarcinoma and NET-G1 are diverse and adenocarcinoma is more aggressive in most affected patients.
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Affiliation(s)
- Ling Zhang
- Department of Pathology, Guang’anmen Hospital, China Academy of Chinese Medical Sciences, Beijing100053, China
| | - Xiaoling Wang
- Department of Radiology, Guang’anmen Hospital, China Academy of Chinese Medical Sciences, Beijing100053, China
| | - Yun Wang
- Department of Pathology, Guang’anmen Hospital, China Academy of Chinese Medical Sciences, Beijing100053, China
| | - Yan Zeng
- Department of Pathology, Guang’anmen Hospital, China Academy of Chinese Medical Sciences, Beijing100053, China
| | - Li Li
- Department of Pathology, Guang’anmen Hospital, China Academy of Chinese Medical Sciences, Beijing100053, China
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16
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Shi JG, Zhao L, Yang Q. Mixed tumor of nasal cavity: A case report. Asian J Surg 2023; 46:1684-1685. [PMID: 36334996 DOI: 10.1016/j.asjsur.2022.09.122] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 09/15/2022] [Accepted: 09/26/2022] [Indexed: 04/03/2023] Open
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17
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Pang S, Song J, Zhang K, Wang J, Zhao H, Wang Y, Li P, Zong Y, Wu Y. Case report: Coexistence of sigmoid tumor with unusual pathological features and multiple colorectal neuroendocrine tumors with lymph node metastases. Front Oncol 2023; 13:1073234. [PMID: 36994194 PMCID: PMC10040595 DOI: 10.3389/fonc.2023.1073234] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Grants] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 10/24/2022] [Accepted: 02/20/2023] [Indexed: 03/18/2023] Open
Abstract
The coexistence of adenocarcinoma and neuroendocrine neoplasm (NEN) in the same tumor is rare. What is rarer is that the neuroendocrine component is a well-differentiated neuroendocrine tumor (NET) Grade (G) 1. Most colorectal NETs are single, but multiple neuroendocrine tumors (M-NETs) are rare. Well-differentiated NETs rarely metastasize. Here, we present a unique case of a synchronous sigmoid tumor and multiple colorectal NETs with lymph node metastases. The sigmoid tumor consisted of adenocarcinoma and NET G1. The metastatic component was NET G1. A 64-year-old man underwent a colonoscopy for persistent changes in bowel habits and positive fecal occult blood for 1 year. An ulcerative lesion, which was diagnosed as colon cancer, was seen in the sigmoid colon. In addition, scattered lesions could be seen in the colon and rectum. Surgical resection was performed. Pathological findings suggested that the ulcerative lesion was composed of 80% adenocarcinoma and 20% neuroendocrine component (NET G1), while the remaining lesions were consistent with NET G1. At the same time, 11 lymph nodes around the resected intestinal segment were invaded by NET G1. The prognosis of the patient was good. After 13 months of follow-up, no recurrence and no metastasis were found. We hope to provide a reference and improve our understanding of the clinicopathological features and biological behavior of these unique tumors. We also aim to emphasize the importance of radical surgery and individualized treatment.
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Affiliation(s)
- Shu Pang
- Department of General Practice, Beijing Friendship Hospital, Capital Medical University, Beijing, China
| | - Jiugang Song
- Department of Gastroenterology, Beijing Friendship Hospital, Capital Medical University, Beijing, China
| | - Kun Zhang
- Department of Pathology, Beijing Friendship Hospital, Capital Medical University, Beijing, China
| | - Jia Wang
- Department of Ultrasonography, Beijing Friendship Hospital, Capital Medical University, Beijing, China
| | - Haiying Zhao
- Department of Gastroenterology, Beijing Friendship Hospital, Capital Medical University, Beijing, China
| | - Yongjun Wang
- Department of Gastroenterology, Beijing Friendship Hospital, Capital Medical University, Beijing, China
| | - Peng Li
- Department of Gastroenterology, Beijing Friendship Hospital, Capital Medical University, Beijing, China
| | - Ye Zong
- Department of Gastroenterology, Beijing Friendship Hospital, Capital Medical University, Beijing, China
- *Correspondence: Ye Zong, ; Yongdong Wu,
| | - Yongdong Wu
- Department of Gastroenterology, Beijing Friendship Hospital, Capital Medical University, Beijing, China
- *Correspondence: Ye Zong, ; Yongdong Wu,
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18
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Pavlidis ET, Pavlidis TE. Molecular factors, diagnosis and management of gastrointestinal tract neuroendocrine tumors: An update. World J Clin Cases 2022; 10:9573-9587. [PMID: 36186187 PMCID: PMC9516923 DOI: 10.12998/wjcc.v10.i27.9573] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 06/28/2022] [Revised: 07/16/2022] [Accepted: 08/17/2022] [Indexed: 02/05/2023] Open
Abstract
The prevalence of gastrointestinal neuroendocrine tumors (GI-NETs) is increasing, and despite recent advances in their therapy, it remains inadequate in patients with advanced well-differentiated neuroendocrine tumors. These tumors present many challenges concerning the molecular basis and genomic profile, pathophysiology, clinicopathological features, histopathologic classification, diagnosis and treatment. There has been an ongoing debate on diagnostic criteria and clinical behavior, and various changes have been made over the last few years. Neuroendocrine carcinoma of the gastrointestinal system is a rare but highly malignant neoplasm that is genetically distinct from gastrointestinal system neuroendocrine tumors (NETs). The diagnosis and management have changed over the past decade. Emerging novel biomarkers and metabolic players in cancer cells are useful and promising new diagnostic tools. Progress in positron emission tomography-computerized tomography and scintigraphy with new radioactive agents (64Cu-DOTATATE or 68Ga-DOTATATE) replacing enough octreoscan, has improved further the current diagnostic imaging. Promising results provide targeted therapies with biological agents, new drugs, chemotherapy and immunotherapy. However, the role of surgery is important, since it is the cornerstone of management. Simultaneous resection of small bowel NETs with synchronous liver metastases is a surgical challenge. Endoscopy offers novel options not only for diagnosis but also for interventional management. The therapeutic option should be individualized based on current multidisciplinary information.
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Affiliation(s)
- Efstathios Theodoros Pavlidis
- Department of 2nd Surgical Propedeutic, Hippocration Hospital, Aristotle University of Thessaloniki, School of Medicine, Thessaloniki 54642, Greece
| | - Theodoros Efstathios Pavlidis
- Department of 2nd Surgical Propedeutic, Aristotle University of Thessaloniki, School of Medicine, Thessaloniki 54642, Greece
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