Article
Pathology
Bence Kovari, Sandor Turkevi-Nagy, Gregory Y. Lauwers, Kun Jiang
Summary: Adenocarcinomas and noninvasive intraepithelial neoplasms are the most common gallbladder tumors, but neuroendocrine neoplasms can also occur. The latest classification denotes mixed neuroendocrine-non-NENs as MiNENs. MANET, a novel type, has been increasingly recognized and reported in various locations.
INTERNATIONAL JOURNAL OF SURGICAL PATHOLOGY
(2021)
Article
Medicine, Research & Experimental
Klara Wyffels, Charlotte De Wilde, Jacques Van Huysse, Vincent De Wilde
Summary: Neuroendocrine neoplasms (NENs) are a diverse group of tumors originating from enterochromaffin cells, each with distinct biological and clinical characteristics. This case report discusses the rare finding of peritoneal carcinomatosis in a well-differentiated Grade 1 (G1) small intestinal NEN and the lack of understanding of its progression and management. The study presents the case of a 68-year-old woman with advanced NEN and rapidly progressive peritoneal metastatic disease, highlighting the need for a predictive tool for early identification of such patients.
EXPERIMENTAL AND THERAPEUTIC MEDICINE
(2023)
Article
Radiology, Nuclear Medicine & Medical Imaging
Yanqi Zhong, Heng Zhang, Xian Wang, Zongqiong Sun, Yuxi Ge, Weiqiang Dou, Shudong Hu
Summary: This study aimed to assess the radiological features of pancreatic mixed neuroendocrine-non-neuroendocrine neoplasm (MiNEN) using computed tomography (CT) and magnetic resonance imaging (MRI) and compare them with those of pancreatic ductal adenocarcinoma (PDAC) and neuroendocrine tumor (NET). The findings showed that MiNEN has larger size, heterogeneous and cystic components compared with PDAC, and ill-defined irregular margins, progressive enhancement, and adjacent organ involvement compared with NET. Vascular invasion is less commonly seen in MiNEN. Moderate enhancement is more frequently observed in MiNEN than in PDAC or NET. The combination of moderate enhancement and ill-defined irregular margin can be used as valuable features for the prediction of pancreatic MiNEN, with high specificity, sensitivity, and accuracy.
INSIGHTS INTO IMAGING
(2023)
Article
Gastroenterology & Hepatology
Anna Niessen, Simon Schimmack, Tim F. Weber, Philipp Mayer, Frank Bergmann, Ulf Hinz, Markus W. Buechler, Oliver Strobel
Summary: MiNEN are rare and difficult to distinguish preoperatively from pure adenocarcinoma or neuroendocrine neoplasm. Surgical resection may be the treatment of choice for localized tumors.
Review
Oncology
Aasems Jacob, Rishi Raj, Derek B. Allison, Heloisa P. Soares, Aman Chauhan
Summary: The classification of mixed neuroendocrine-non-neuroendocrine neoplasms (MiNEN) is evolving, and current management guidelines are lacking. Recent studies provide insights into factors affecting outcomes and treatment decisions for these rare malignancies. Surgical management is appropriate for early-stage disease, while chemotherapy offers survival benefits in advanced disease or when surgery is not possible. Molecular testing should be used to evaluate patients for clinical trials and targeted treatments.
CURRENT TREATMENT OPTIONS IN ONCOLOGY
(2022)
Article
Oncology
Alex J. Liu, Benjamin E. Ueberroth, Patrick W. McGarrah, Skye A. Buckner Petty, Ayse Tuba Kendi, Jason Starr, Timothy J. Hobday, Thorvardur R. Halfdanarson, Mohamad Bassam Sonbol
Summary: This study evaluated metastatic NET G3 response to different treatment regimens, showing that CAPTEM was the most commonly used treatment with clinically meaningful efficacy and disease control. FOLFOX or PRRT are other potentially active treatment options, while EP has limited activity with short-lived responses in NET G3.
Review
Oncology
Anna Pellat, Anne Segolene Cottereau, Lola-Jade Palmieri, Philippe Soyer, Ugo Marchese, Catherine Brezault, Romain Coriat
Summary: Well-differentiated grade 3 neuroendocrine tumors of the digestive system (NET G-3) have been clearly defined since 2017, but remain a rare category lacking specific data and standardized management. Their differences from other neuroendocrine neoplasms not only involve morphology, but also genotype, aggressiveness, imaging uptake, and treatment response. More research is needed to better understand and manage this specific subgroup of tumors.
Article
Oncology
Hiroyuki Kuwano, Takehiko Yokobori, Munenori Ide, Hiroshi Saeki, Makoto Sohda, Makoto Sakai, Tomonori Yoshida, Kengo Kuriyama, Kyoichi Ogata, Hiroomi Ogawa, Takuhisa Okada, Tatsuya Miyazaki, Shunsuke Takahashi, Ken Shirabe
Summary: This study evaluated the histopathological findings of superficial carcinomas adjacent to neuroendocrine neoplasms (NENs) in the esophagogastrointestinal tract. The results showed that superficial carcinomas adjacent to NENs in the esophagus were squamous cell carcinoma/dysplasia, while those in the stomach and large intestine were adenocarcinoma. This suggests a potential bystander effect of NENs on resident epithelium.
Review
Oncology
Ugo Grossi, Alessandro Bonis, Emma Carrington, Enrico Mazzobel, Giulio Aniello Santoro, Laura Cattaneo, Giovanni Centonze, Gaetano Gallo, Andrea Kazemi Nava, Maurizio Romano, Gian Luca Di Tanna, Giacomo Zanus
Summary: This study evaluates the prevalence and survival rate of mixed adeno-neuroendocrine carcinomas (MANEC) in the lower gastrointestinal tract, revealing MANEC as a clinically aggressive pathological entity with a predominant occurrence in the appendix.
Article
Oncology
Lei Lou, Fengzhu Lv, Xin Wu, Yuehong Li, Xianghong Zhang
Summary: MiNENs are rare tumors that combine high-grade neuroendocrine and non-neuroendocrine components, and are associated with poor prognoses. Deficiency in MMR is significantly correlated with early TNM stages and better prognoses in MiNEN patients.
Article
Pathology
Jingci Chen, Pengyan Wang, Ke Lv, Weixun Zhou
Summary: This case report presents a rare mixed neuroendocrine-non-neuroendocrine neoplasm of the pancreas, with genetic alterations identified by next-generation sequencing. The study found shared somatic mutations in both components and the patient remained uneventful 21 months after surgery. The research suggests that KRAS mutation may be a driver event underlying the occurrence of such mixed neoplasms.
DIAGNOSTIC PATHOLOGY
(2021)
Review
Endocrinology & Metabolism
Stefano La Rosa
Summary: The increasing knowledge of neuroendocrine neoplasms has led to changes in nomenclature, particularly concerning high-grade NENs and MiNENs, causing confusion among pathologists and clinicians. The review aims to clarify diagnostic criteria and discuss the role of Ki67 labelling index in defining grade 3 NENs, as well as review the evolution of concepts and terminology of MiNENs.
ENDOCRINE PATHOLOGY
(2021)
Article
Oncology
Daniela Pereira, Daley White, Michael Mortellaro, Kun Jiang
Summary: This study reports an unusual case of colonic microsatellite instability mixed neuroendocrine tumor (MiNEN) and reviews the literature. The tumor was composed of neuroendocrine cells and mucin-containing signet ring-like cells, with multiple carcinogenic mutations identified. Due to the advanced stage and adhesion to adjacent organs, surgical resection was aborted, and immunotherapy was initiated. The tumor went into remission after 30 months of treatment, and the patient remains asymptomatic.
Article
Oncology
Jiwon Koh, Soo Kyung Nam, Yoonjin Kwak, Gilhyang Kim, Ka-Kyung Kim, Byung-Chul Lee, Sang-Hoon Ahn, Do Joong Park, Hyung-Ho Kim, Kyoung Un Park, Woo Ho Kim, Hye Seung Lee
Summary: In this study, targeted DNA sequencing was conducted on tumor samples from 21 patients to investigate the pathogenesis of gastric mixed adenoneuroendocrine carcinoma (MANEC) and pure neuroendocrine carcinoma (NEC). Mutational profiles of MANECs/NECs were compared with other tumors, revealing unique mutational profiles and underlying protein networks for gastric MANEC and pure NEC. The research suggests a possible clonal origin of ADC and NEC components of MANEC, with differentially altered genes associated with specific signaling pathways.
JOURNAL OF PATHOLOGY
(2021)
Letter
Oncology
Fengxia He, Xue He, Menghan Cui, Yan Wang
Summary: This study reports a rare case of mixed neuroendocrine-non-neuroendocrine neoplasm (MiNENs) with squamous cell carcinoma (SCC) component in the rectum, and investigates the molecular landscape of MiNENs. The patient was initially diagnosed with rectal small cell neuroendocrine carcinoma (SCNEC), followed by mixed SCNEC and SCC in the same site 6 months later. Samples from the three histologic subtypes were subjected to next-generation sequencing (NGS). Multiple liver metastases occurred in the following month after surgery. The patient died of ketoacidosis 1 year after initial diagnosis of the tumor.
WORLD JOURNAL OF SURGICAL ONCOLOGY
(2023)
Letter
Endocrinology & Metabolism
Ozgur Mete, C. Christofer Juhlin
ENDOCRINE PATHOLOGY
(2023)
Review
Endocrinology & Metabolism
Silvia Uccella, Matthias Dottermusch, Lori Erickson, Julia Warmbier, Kathleen Montone, Wolfgang Saeger
Summary: A variety of inflammatory conditions, including infectious agents and autoimmune processes, can directly affect the endocrine glands, leading to dysfunction and potentially mimicking neoplastic processes. These disorders are often under-recognized clinically and diagnosed based on pathological samples. Therefore, pathologists need to understand the pathogenesis, morphological features, clinicopathological correlates, and differential diagnosis of these diseases. This review focuses on the morphological and clinicopathological features of infectious diseases, autoimmune disorders, drug-induced inflammatory reactions, IgG4-related disease, and other inflammatory disorders of the endocrine system, aiming to provide a practical guide for pathologists.
ENDOCRINE PATHOLOGY
(2023)
Article
Endocrinology & Metabolism
Amedeo Sciarra, Silvia Uccella, Philippe Hiroz, Ian Fournier, Vincent Soubeyran, Giovanna Finzi, Stefano La Rosa
Summary: We report a case of a 63-year-old male patient with a gastric nodule diagnosed as a type 3 well-differentiated gastric neuroendocrine tumor (NET). Despite the absence of (In-111)Octreotide uptake, the patient was diagnosed with amphicrine carcinoma with pancreatic acinar cell and neuroendocrine features (pT1b). The patient remained disease-free after 18 months without additional oncological treatment.
ENDOCRINE PATHOLOGY
(2023)
Article
Endocrinology & Metabolism
Sylvia L. Asa, Ozgur Mete, Nicole D. Riddle, Arie Perry
Summary: This study identified a rare type of pituitary neuroendocrine tumors (PitNETs) that express both PIT1 and SF1 transcription factors. These tumors exhibit variable clinical and morphological features, commonly presenting as large tumors with growth hormone excess and occasionally coexisting with other types of PitNETs.
ENDOCRINE PATHOLOGY
(2023)
Review
Endocrinology & Metabolism
Sylvia L. Asa, Silvia Uccella, Arthur Tischler
Summary: The assessment of cell differentiation in endocrine neoplasms involves the identification of cell structure, specific transcription factors, hormones, and enzymes. Differentiation status serves as prognostic and predictive factors for endocrine neoplasms. Hormones can act as biomarkers for clinical surveillance and loss of differentiated hormone production can indicate dedifferentiation and aggressiveness of the tumor. Differentiated endocrine cells express targets for therapy, providing opportunities for targeted treatment.
ENDOCRINE PATHOLOGY
(2023)
Letter
Cell Biology
Andrea Remo, Federica Grillo, Alessandro Vanoli, Paola Parente, Luca Mastracci, Valentina Angerilli, Emanuele Damiano Urso, Francesca Bergamo, Matteo Fassan
Review
Medicine, General & Internal
Alessandro Vanoli, Paola Parente, Matteo Fassan, Luca Mastracci, Federica Grillo
Summary: Gut inflammation disrupts gastrointestinal homeostasis and is associated with cancerogenesis. Various chronic inflammatory disorders of the tubular gastrointestinal tract have been linked to an increased risk of neoplasms, including epithelial tumors (esophageal, gastric, small bowel, and colorectal) and lymphomas. Studies have investigated the mechanisms and changes in the microenvironment and microbiome that lead to genetic and/or epigenetic alterations and eventually tumorigenesis, typically following an inflammation-dysplasia-carcinoma sequence.
INTERNAL AND EMERGENCY MEDICINE
(2023)
Article
Pathology
Valentina Angerilli, Paola Parente, Michela Campora, Clara Ugolini, Serena Battista, Paola Cassoni, Alessandro Gambella, Francesco Cavallin, Giuseppe De Lisi, Alessandro Vanoli, Federica Grillo, Luca Mastracci, Matteo Fassan
Summary: In this study, the clinicopathological and molecular features of HER2-low gastric/gastro-oesophageal junction cancers were investigated. It was found that HER2-low expression was more common in biopsy specimens and varied among different institutions. If further research confirms the effectiveness of novel anti-HER2 agents in HER2-low gastro-oesophageal cancers, the interpretation of HER2 status may need to be revised.
JOURNAL OF CLINICAL PATHOLOGY
(2023)
Article
Oncology
Francesca Magnoli, Deborah Marchiori, Sofia Facchi, Vittoria Martin, Leonardo Campiotti, Michele Merli, Fausto Sessa, Maria Grazia Tibiletti, Silvia Uccella
Summary: This study investigated the frequency of BCL2 rearrangement in follicular lymphomas (FLs) from northwestern Italy, evaluated their clinicopathological features, and explored alternative genetic aberrations in BCL2-negative FLs. The results showed that BCL2 rearrangements were found in 39 of the 76 FL cases. Additionally, other genetic rearrangements were identified in BCL2-negative FLs. The study suggests that BCL2 rearrangement is not a constant finding in FL and alternative genetic aberrations exist in BCL2-negative cases.
Article
Genetics & Heredity
Andrea Remo, Federica Grillo, Luca Mastracci, Michele Simbolo, Matteo Fassan, Maria Paola Cecchini, Giuseppe Miscio, Antonio Sassano, Paola Parente, Alessandro Vanoli, Giovanna Sabella, Guido Giordano, Emanuele Damiano Urso, Luigi Cerulo, Aldo Scarpa, Francesco Fiorica, Massimo Pancione
Summary: A recent study explores the genetic and immunophenotypic characteristics of rhabdoid colorectal tumors (RCTs). The findings reveal that RCTs exhibit mismatch repair-deficient phenotypes, CK7-/CK20-/CDX2- marker phenotype, and aberrant activation of the MAPK pathway, particularly with BRAF V600E mutations. While SMARCB1/INI1 expression is normal, the ciliogenic markers CROCC and β-tubulin are globally altered in RCTs. Overall, these results indicate that primary ciliogenesis and MAPK pathway activation contribute to the aggressiveness of RCTs, suggesting a potential therapeutic target.
Article
Oncology
Marco Lucioni, Sara Fraticelli, Giovanni Santacroce, Arturo Bonometti, Nicola Aronico, Roberta Sciarra, Marco Vincenzo Lenti, Paola Ilaria Bianchi, Giuseppe Neri, Monica Feltri, Benedetto Neri, Giuseppina Ferrario, Roberta Riboni, Gino Roberto Corazza, Alessandro Vanoli, Luca Arcaini, Marco Paulli, Antonio Di Sabatino
Summary: This retrospective study aimed to describe the clinical-pathological characteristics of intestinal T-cell lymphomas (ITCL) and possibly identify their hallmarks. The study included 28 patients, with most cases associated with celiac disease. The presence of CD, peripheral lymphocytosis, advanced Lugano clinical stage, and the histological subtype ITCL-NOS were significantly associated with worse survival.
Article
Cell Biology
Stefano Marletta, Maria Salatiello, Liron Pantanowitz, Claudio Bellevicine, Massimo Bongiovanni, Emanuela Bonoldi, Gisele De Rezende, Guido Fadda, Paolo Incardona, Enrico Munari, Fabio Pagni, Esther Diana Rossi, Giovanni Tallini, Giancarlo Troncone, Clara Ugolini, Elena Vigliar, Albino Eccher
Summary: The study validates the utility of whole slide imaging (WSI) in thyroid fine needle aspiration (FNA) using the Delphi method. It shows broad consensus among physicians that WSI is reliable and safe for primary diagnosis in thyroid cytopathology.
Book Review
Endocrinology & Metabolism
Ozgur Mete
ENDOCRINE PATHOLOGY
(2023)
Article
Cell Biology
Paola Parente, Maria C. Macciomei, Anna M. Buccoliero, Luisa Santoro, Barbara Cafferata, Delfina Bifano, Jacopo Ferro, Alessandro Vanoli, Matteo Fassan, Valentina Angerilli, Rita Alaggio, Luca Mastracci, Maria D'Armiento, Federica Grillo, Paola Francalanci
Summary: Very early-onset inflammatory bowel disease (VEO-IBD) refers to IBD-like symptoms in children before the age of 6 years, including both 'pure' IBD and monogenic diseases (MDs). MD patients may have histological features similar to IBD, making diagnosis challenging.
Review
Endocrinology & Metabolism
Elena Russo, Mathilda Guizzardi, Luca Canali, Francesca Gaino, Andrea Costantino, Gherardo Mazziotti, Andrea Lania, Silvia Uccella, Luca Di Tommaso, Fabio Ferreli, Luca Malvezzi, Giuseppe Spriano, Giuseppe Mercante
Summary: This meta-analysis showed no association between NLR, PLR, LMR and DFS in differentiated thyroid carcinoma (DTC). However, more prospective data are needed to better define the association between inflammatory status and prognosis of DTC.
REVIEWS IN ENDOCRINE & METABOLIC DISORDERS
(2023)