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Gastrointestinal neuroendocrine tumors

Riccardo Ariotti, Stefano Partelli, Francesca Muffatti, Valentina Andreasi, Francesca Della Sala, Massimo Falconi
Neuroendocrine gastro-entero-pancreatic neoplasms (GEP-NENs) constitute a heterogeneous group of tumors, whose incidence has increased over the years. The most frequent site for primary disease is the stomach followed by small and large intestine, and pancreas. In the last decade, a dramatic growing in the incidence of small, incidental GEP-NENs has been recorded. In parallel, an increasing attitude toward more conservative approaches instead of surgical management has being widely spreading. This is particularly true for small, asymptomatic, pancreatic NEN as for these tumor forms an active surveillance has proven to be safe and feasible...
March 12, 2018: Reviews in Endocrine & Metabolic Disorders
Mohamed E Salem, Alberto Puccini, Axel Grothey, Derek Raghavan, Richard M Goldberg, Joanne Xiu, W Michael Korn, Benjamin A Weinberg, Jimmy J Hwang, Anthony F Shields, John L Marshall, Philip A Philip, Heinz-Josef Lenz
The efficacy of immunotherapy varies widely among different gastrointestinal cancers. Response to immune checkpoint inhibitors is shown to correlate with tumor mutation load (TML), mismatch repair deficiency (dMMR) status, and programmed cell death-ligand 1 (PD-L1) expression. Herein, we quantify TML, dMMR, and PD-L1 expression and determine their interrelationship in gastrointestinal cancers. Here, a total of 4125 tumors from 14 different gastrointestinal cancer sites were studied using validated assays. Next-generation sequencing (NGS) was performed on genomic DNA isolated from formalin-fixed paraffin-embedded (FFPE) tumor specimens using the NextSeq platform...
March 9, 2018: Molecular Cancer Research: MCR
Silvia Uccella, Stefano La Rosa, Marco Volante, Mauro Papotti
Neuroendocrine neoplasms (NENs) are a heterogeneous group of epithelial neoplastic proliferations that irrespective of their primary site share features of neural and endocrine differentiation including the presence of secretory granules, synaptic-like vesicles, and the ability to produce amine and/or peptide hormones. NENs encompass a wide spectrum of neoplasms ranging from well-differentiated indolent tumors to highly aggressive poorly differentiated neuroendocrine carcinomas. Most cases arise in the digestive system and in thoracic organs, i...
March 9, 2018: Endocrine Pathology
Maria João Marques Ribeiro, Teresa Alonso, Pablo Gajate, Javier Molina, Arantzazu Barquin, Cristian Perna, Enrique Grande
Chemotherapy is considered "state of the art" for the treatment of poorly differentiated neuroendocrine neoplasms. Unfortunately, there is no standard effective post-first-line treatment for relapsing high-grade gastroenteropancreatic neuroendocrine neoplasms. We report the case of a patient with a gastric neuroendocrine carcinoma stage IV, with massive gastrointestinal bleeding at diagnosis. After the first line of platin-based chemotherapy a major tumoral response was documented, but the patient relapsed after 4 months...
January 2018: Autopsy & Case Reports
Alia Zehani, Sarra Aloui, Ines Chelly, Slim Haouet, Nidhameddine Kchir
BACKGROUND: Neuroendocrine tumors (NETs) of the gastrointestinal tract are a heterogeneous group of tumors which have different malignant potential and evolution. The World Health Organization (WHO) has set up a new classification of gastrointestinal NETs in 2010. However, it does not evaluate the risk of malignancy for each tumor. AIM: To evaluate the prognostic impact of the WHO classification in 2010 by reclassifying the tumor according to new recommendations and to identify histoprognostic factors to better predict changes...
June 2017: La Tunisie Médicale
Saoussen Antit, Lilia Zakhama, Sarra Chenik, Mohamed Saadi, Ahmed Houmed, Samar Mohamed, Elhem Boussabeh, Moez Thameur, Soraya Ben Youssef
Carcinoid tumors are rare, mainly located in the gastrointestinal tract particularly in the small intestine. Cardiac involvement, fairly frequent and usually affecting right sided heart valves, had a poor prognosis that is improved by an earlier detection and valve surgery. We report the case of a 50-years old woman with neuroendocrine tumor and liver metastases, in whom carcinoid involvement of the right heart was diagnosed following exertional dyspnea. She had dilated right cavities, severe tricuspid regurgitation, mild tricuspid stenosis and a moderate pulmonary stenosis...
May 2017: La Tunisie Médicale
David L Chan, Simron Singh
Carcinoid syndrome occurs in 20% of patients with neuroendocrine tumors, and serotonin is usually the main causative hormonal peptide. Carcinoid syndrome, and particularly diarrhea, can significantly impact patients' quality of life. Somatostatin analogs (SSAs) are the mainstay of treatment, but are unable to ameliorate symptoms in all patients due to dose-limiting side effects and tachyphylaxis. Telotristat is a novel oral inhibitor of tryptophan hydroxylase, which is the rate-limiting enzyme in serotonin synthesis...
2018: Therapeutics and Clinical Risk Management
Rita Linhas, David Tente, Nuno China, Sara Conde, Ana Barroso
RATIONALE: Carcinoid tumors are derived from neuroendocrine cells and are most frequently found in the gastrointestinal tract and bronchopulmonary system. They are generally characterized by an indolent clinical course but may in some instances spread to regional lymph nodes or to distant sites. Subcutaneous metastases of carcinoid tumors are extremely rare; there are only few cases reported in the literature and the site of the primary tumor was mainly the gastrointestinal tract. Also, the diagnosis of this type of lesions many years after the surgical resection of the pulmonary carcinoid (PC) could be a challenge for clinicians...
January 2018: Medicine (Baltimore)
Hanwen Zhang, Ananda Kumar Kanduluru, Pooja Desai, Afruja Ahad, Sean D Carlin, Nidhi Tandon, Wolfgang Weber, Philip S Low
Neurokinin 1 receptor (NK1R) is expressed in gliomas and neuroendocrine malignancies, and represents a promising target for molecular imaging and targeted radionuclide therapy. The goal of this study was to synthesize and evaluate a novel NK1R ligand (NK1R-NOTA) for targeting NK1R-expressing tumors. Using carboxymethyl moiety linked to L-733060 as starting reagent, NK1R-NOTA was synthesized in a 3-step reaction, and then labeled with64 Cu (or67 Ga for in vitro studies) in the presence of CH3 COONH4 buffer. The radioligand affinity and cellular uptake was evaluated with NK1R-transduced HEK293 cells (HEK293-NK1R) and NK1R non-transduced HEK293 cells (HEK293-WT) and its xenografts...
February 21, 2018: Bioconjugate Chemistry
Parthik Patel, Karina Galoian
Neuroendocrine tumors (NETs) are a very heterogeneous group that are thought to originate from the cells of the endocrine and nervous systems. These tumors develop in a number of organs, predominantly in the gastrointestinal and pulmonary systems. Clinical detection and diagnosis are reliable at the late stages when metastatic spread has occurred. However, traditional conventional therapies such as radiation and chemotherapy are not effective. In the majority of cases even surgical resection at that stage is unlikely to produce promising reusults...
March 2018: Oncology Letters
Katiuscha Merath, Fabio Bagante, Eliza W Beal, Alexandra G Lopez-Aguiar, George Poultsides, Eleftherios Makris, Flavio Rocha, Zaheer Kanji, Sharon Weber, Alexander Fisher, Ryan Fields, Bradley A Krasnick, Kamran Idrees, Paula M Smith, Cliff Cho, Megan Beems, Carl R Schmidt, Mary Dillhoff, Shishir K Maithel, Timothy M Pawlik
BACKGROUND: The risk of recurrence after resection of non-metastatic gastro-entero-pancreatic neuroendocrine tumors (GEP-NET) is poorly defined. We developed/validated a nomogram to predict risk of recurrence after curative-intent resection. METHODS: A training set to develop the nomogram and test set for validation were identified. The predictive ability of the nomogram was assessed using c-indices. RESULTS: Among 1477 patients, 673 (46%) were included in the training set and 804 (54%) in y the test set...
February 15, 2018: Journal of Surgical Oncology
Kohei Ono, Eisuke Shiozawa, Nobuyuki Ohike, Tomonori Fujii, Hideki Shibata, Tetsuya Kitajima, Koichiro Fujimasa, Naoko Okamoto, Yukiko Kawaguchi, Tasuku Nagumo, Sakiko Tazawa, Mayumi Homma, Toshiko Yamochi-Onizuka, Tomoko Norose, Hitoshi Yoshida, Masahiko Murakami, Gensyu Tate, Masafumi Takimoto
The WHO 2010 classification divides gastrointestinal neuroendocrine neoplasms (GI-NENs) into neuroendocrine tumor (NET) G1, NET G2, neuroendocrine carcinoma (NEC) and mixed adenoendocrine carcinoma (MANEC) groups. A total of 136 cases of GI-NENs diagnosed at our hospitals as gastrointestinal carcinoids, endocrine cell carcinomas and NENs over the last 11 years, using the WHO 2010 classification were assessed. Among the 136 cases, 88.2% (120/136) were classified into the NET group (NET G1/G2) and 11.8% (16/136) were classified into the NEC group (NEC/MANEC)...
February 2018: Oncology Letters
Ken Sugezawa, Hiroaki Saito, Yusuke Kono, Yuki Murakami, Yuji Shishido, Hirohiko Kuroda, Tomoyuki Matsunaga, Yoji Fukumoto, Tomohiro Osaki, Yoshiyuki Fujiwara
A 47-year-old man with progressive anemia possibly due to digestive tract bleeding was referred to our hospital. Contrast-enhanced computed tomography of the abdomen showed a 2-cm tumor lesion arising near the small intestine. Enteroscopy revealed a 3-cm submucosal tumor at the ileum. A gastrointestinal stromal tumor of the small intestine was suspected, and the patient underwent surgery. During the operation, a diverticulum approximately 60 cm orad to the terminal ileum and a tumor at the top of the diverticulum were observed...
December 2017: Yonago Acta Medica
Andreas Blesl, Elisabeth Krones, Marion J Pollheimer, Johannes Haybaeck, Ulrike Wiesspeiner, Rainer W Lipp, Patrizia Kump
The antiproliferative treatment options for neuroendocrine tumors (NET)/neuroendocrine carcinomas of the gastrointestinal tract critically depend on the proliferation rate, evaluated by immunohistochemical staining for Ki-67. According to their grading, tumors are treated with somatostatin analogs, mTOR inhibitors, or cytotoxic substances. This case illustrates downgrading of a primarily highly proliferative NET achieved by a variation of cytotoxic chemotherapy regimens, followed by a combination therapy using everolimus together with lanreotide...
September 2017: Case Reports in Oncology
Shingo Nakashima, Shoichiro Hikami, Yasuhito Izumiya, Toshifumi Tsuji, Sousuke Komiyama, Soujin Sai, Sadao Kawakami
We report a case of duodenal neuroendocrine tumor(NET)G1 resected by laparoscopic-endoscopic cooperative surgery (LECS). A 78-year-old woman underwent upper gastrointestinal endoscopy, revealing an 8 mm, rising tumor on the anterior wall of the duodenal bulb. The tumor was pathologically diagnosed as a G1 duodenal NET, by biopsy. Endoscopic ultrasonography showed the tumor might invade the submucosal layer. We performed LECS. The endoscopist marked the dissection line around the tumor and penetrated the duodenal wall...
November 2017: Gan to Kagaku Ryoho. Cancer & Chemotherapy
Tomoki Kido, Yuta Morimoto, Hiroshi Toda, Kei Yoshino, Hiroshi Imano, Toshiki Sasaki, Yoshihiko Kimura, Reijiro Saito, Kazuhiko Endo
A 64-year-old man was admitted to our hospital because of epigastralgia. Gastrointestinal endoscopyrevealed a submucosal tumor with ulceration in the upper bodyof the stomach. The tumor was histologicallydiagnosed as a neuroendocrine carcinoma. CT showed that the tumor had directlyinfiltrated the pancreas and splenic vessels. The patient underwent onlyan exploratorylaparotomybecause the tumor seemed to involve the celiac artery. Chemotherapywas conducted using CPT-11/ CDDP. After 15 courses of chemotherapy, a significant tumor reduction was obtained...
November 2017: Gan to Kagaku Ryoho. Cancer & Chemotherapy
Yuto Kitano, Jun Kinoshita, Katsunobu Oyama, Koichi Okamoto, Seisho Sakai, Isamu Makino, Keishi Nakamura, Hironori Hayashi, Masafumi Inokuchi, Tomoharu Miyashita, Hidehiro Tajima, Hiroyuki Takamura, Itasu Ninomiya, Sachio Fushida, Tetsuo Ohta
Gastric cancer rarely contains neuroendocrine component. This mixed tumor is defined as neuroendocrine carcinoma (NEC), mixed adenoneuroendocrine carcinoma(MANEC)and so on according to the WHO classification. We report a patient with esophagogastric junction cancer with neuroendocrine differentiation(NED). The patient was 54-year-old man who diagnosed of esophagogastric junction cancer at the medical examination. Upper gastrointestinal endoscopy revealed a type 3 tumor at esophagogastric junction, and the pathological findings were poorly differentiated adenocarcinoma with focal positivity of chromogranin A...
November 2017: Gan to Kagaku Ryoho. Cancer & Chemotherapy
B Sipos
Neuroendocrine neoplasms (NENs) are infrequent in sensory organs. There are well-differentiated neuroendocrine neoplasms that should be classified as neuroendocrine tumors, in analogy to their gastrointestinal counterparts, however the nomenclature is inconsistent. The best defined entities are neuroendocrine tumors in the middle ear and ectopic pituitary adenoma in the sphenoid region. Poorly differentiated NENs most often arise in the olfactory organ and nasal cavity that are represented by olfactory neuroblastomas and poorly differentiated neuroendocrine carcinomas...
February 1, 2018: Der Pathologe
Nicholas Manguso, Jeffrey Johnson, Attiya Harit, Nicholas Nissen, James Mirocha, Andrew Hendifar, Farin Amersi
Small bowel neuroendocrine tumors (SBNET) account for most gastrointestinal neuroendocrine tumors. Patients often present with late-stage disease; however, there is little information regarding factors that contribute to recurrence. Database review identified 301 patients diagnosed with SBNET between 1990 and 2013. Univariate analysis included patients who underwent complete resection. Survival was estimated by the Kaplan-Meier method. A total of 147 patients met study criteria. Average age was 60 years (range 21-91); 49 per cent were male...
October 1, 2017: American Surgeon
Yen-Po Chin, Wei-Fu Lai, Meng-Ting Chiang, Shih-Chieh Chang
RATIONALE: Esophageal neuroendocrine tumors (NETs) are a rare type of esophageal tumor which are usually positive for chromogranin A, synaptophysin, and CD56 in tumor immunohistochemical staining. The most common symptoms of esophageal NETs are gastrointestinal symptoms such as dysphagia and/or abdominal discomfort. While esophageal NETs have the potential for distant metastasis, there have only been a few reports of brain metastasis originating from esophageal NET. PATIENT CONCERNS: We report the case of a 60-year-old Taiwanese female who initially presented with a 1 month history of painless forehead and bilateral neck masses...
December 2017: Medicine (Baltimore)
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