scholarly journals Unmet Needs in Functional and Nonfunctional Pancreatic Neuroendocrine Neoplasms

2018 ◽  
Vol 108 (1) ◽  
pp. 26-36 ◽  
Author(s):  
Robert T. Jensen ◽  
Lisa Bodei ◽  
Jaume Capdevila ◽  
Anne Couvelard ◽  
Massimo Falconi ◽  
...  
Keyword(s):  
Neuroendocrine Tumor ◽  
Unmet Needs ◽  
Advisory Board ◽  
Neuroendocrine Neoplasm ◽  
Neuroendocrine Neoplasms ◽  
Pancreatic Neuroendocrine Neoplasms ◽  
Poorly Differentiated

Recently, the European Neuroendocrine Tumor Society (ENETS) held working sessions composed of members of the advisory board and other neuroendocrine neoplasm (NEN) experts to attempt to identify unmet needs in NENs in different locations or with advanced/poorly differentiated NENs. This report briefly summarizes the main proposed areas of unmet needs in patients with functional and nonfunctional pancreatic NENs.

scholarly journals Tumor Macroscopic Morphology Is an Important Prognostic Factor in Predicting Chemotherapeutic Efficacy and Clinical Outcomes of Patients With Colorectal Neuroendocrine Neoplasms, One Multicenter Retrospective Cohort Study

2021 ◽  
Vol 12 ◽  
Author(s):  
Zhijie Wang ◽  
Ke An ◽  
Rui Li ◽  
Qian Liu
Keyword(s):  
Prognostic Factor ◽  
Locally Advanced ◽  
Progression Free Survival ◽  
Tnm Stage ◽  
Stage Ii ◽  
Neuroendocrine Neoplasm ◽  
Neuroendocrine Neoplasms ◽  
Ki 67 ◽  
Dismal Prognosis ◽  
Poorly Differentiated

Background and AimsLocally advanced and metastatic colorectal neuroendocrine neoplasm (NEN) is a rare disease with a dismal prognosis. We aimed to explore the value of the macroscopic morphology of NENs in the management of TNM stage II-IV colorectal NENs, which has not been fully elucidated in previous reports.MethodsWe retrospectively enrolled 125 eligible patients with TNM stage II-IV colorectal NENs who were diagnosed between 2000 and 2020 from three Chinese hospitals. All were categorized into either protruding or ulcerative NEN groups through endoscopic evaluation of their macroscopic morphology. Clinicopathological data were collected and compared between the two groups. Survival analysis was performed to assess the survival outcomes between the two groups.ResultsA total of 77 and 48 patients had protruding and ulcerative NENs, respectively. Patients with ulcerative NENs had a larger median tumor size (P<0.001) and higher median Ki-67 index (P<0.001), and a larger proportion of these patients had grade G3 disease (P=0.001) and poorly differentiated neoplasms (P=0.001), as well as higher frequencies of T3 and T4 tumors (P=0.006) than patients with protruding NENs. In addition, patients with ulcerative NENs showed a much lower response to first-line chemotherapy [50% (95% CI: 27.3% - 72.7%) versus 20% (95% CI: 3.1% - 36.9%), P=0.03] and a worse 3-year progression-free survival (PFS) rate [19.7% (95% CI: 7.2% - 32.2%) versus 49.5% (95% CI: 37.5% - 61.5%), P=0.001] and 3-year overall survival (OS) rate [30.7% (95% CI: 15.6% - 45.8%) versus 76.9% (95% CI: 66.5% - 87.3%), P<0.001] than those with protruding NENs. The multivariate analysis results indicated that the macroscopic shape of NENs was an independent prognostic factor affecting both PFS (HR = 1.760, 95% CI: 1.024 – 3.026, P = 0.04) and OS (HR = 2.280, 95% CI: 1.123 – 4.628, P = 0.02).ConclusionsUlcerative NENs were more malignant and chemotherapy resistant than protruding NENs. Tumor macroscopic morphology is a valuable prognostic factor for stage II-IV colorectal NENs.

scholarly journals Pancreatic Neuroendocrine Neoplasms: Landscape and Horizon

2020 ◽  
Vol 144 (7) ◽  
pp. 816-828
Author(s):  
Laura H. Tang
Keyword(s):  
Neuroendocrine Neoplasms ◽  
Specific Gene ◽  
Important Distinction ◽  
Pancreatic Neuroendocrine Neoplasms ◽  
Poorly Differentiated Neuroendocrine Carcinoma ◽  
Poorly Differentiated ◽  
Well Differentiated ◽  
Initial Description ◽  
Definition Of ◽  
Hereditary Conditions

Context.— Since the initial description of pancreatic endocrine physiology and the recognition of islet cell tumors in the 1800s, there have been noteworthy advances in the pathobiology of pancreatic neuroendocrine neoplasms (PanNENs), and definition of the important distinction between well-differentiated neuroendocrine tumor (PanNET) and poorly differentiated neuroendocrine carcinoma (PanNEC). The evolving knowledge has resulted in a continuous update in terminology, classification, and grading system for this group of neoplasms. Pancreatic neuroendocrine tumors associated with hereditary conditions have been linked to unique molecular and genetic events, and sporadic PanNETs have specific gene signatures. Based on accumulative experience and knowledge, therapeutic strategies have been defined for this group of neoplasms. Objective.— To review the evolution and description of the pathologic-genomic evolution of PanNENs, and to facilitate accurate pathologic interpretation for the corresponding clinical management. Data Sources.— Literature review of published studies and author's own work. Conclusions.— Evolving experience and knowledge have established subtypes of pancreatic neuroendocrine neoplasms, based on their genotype and phenotype. Accurate pathologic interpretation of the specific neoplasm has significant implications for therapy and prognosis.

VHL-RELATED NEUROENDOCRINE NEOPLASMS AND BEYOND: AN ISRAELI SPECIALIZED CENTER REAL-LIFE REPORT

2020 ◽  
Vol 26 (10) ◽  
pp. 1131-1142
Author(s):  
Auryan Szalat ◽  
Kira Oleinikov ◽  
Avital Nahmias ◽  
Vardiella Meiner ◽  
Simona Ben-Haim ◽  
...  
Keyword(s):  
Central Nervous System ◽  
Nervous System ◽  
Neuroendocrine Tumor ◽  
Neuroendocrine Neoplasms ◽  
Pancreatic Neuroendocrine Neoplasms ◽  
Von Hippel Lindau ◽  
Mutation Carriers ◽  
Long Term Follow Up

Objective: Von Hippel-Lindau (VHL) syndrome is a rare and complex disease. In 1996, we described a 3 generation VHL 2A kindred with 11 mutation carriers. We aim to share our experience regarding the long-term follow-up of this family and the management of all our other VHL patients focusing on frequently encountered neuroendocrine neoplasms: pheochromocytoma/paraganglioma and pancreatic neuroendocrine neoplasms (PNEN). Methods: All VHL patients in follow-up at our tertiary center from 1980 to 2019 were identified. Clinical, laboratory, imaging, and therapeutic characteristics were retrospectively analyzed. Results: We identified 32 VHL patients in 16 different families, 7/16 were classified as VHL 2 subtype. In the previously described family, the 4 initially asymptomatic carriers developed a neuroendocrine tumor; 7 new children were born, 3 of them being mutation carriers; 2 patients died, 1 due to metastatic PNEN-related liver failure. Pheochromocytoma was frequent (22/32), bilateral (13/22;59%), often diagnosed in early childhood when active screening was timely performed, associated with paraganglioma in 5/22, rarely malignant (1/22), and recurred after surgery in some cases after more than 20 years. PNEN occurred in 8/32 patients (25%), and was metastatic in 3 patients. Surgery and palliative therapy allowed relatively satisfactory outcomes. Severe disabling morbidities due to central-nervous system and ophthalmologic hemangiomas, and other rare tumors as chondrosarcoma in 2 patients and polycythemia in 1 patient were observed. Conclusion: A multidisciplinary approach and long-term follow-up is mandatory in VHL patients to manage the multiple debilitating morbidities and delay mortality in these complex patients. Abbreviations: CNS = central nervous system; HIF = hypoxia inducible factor; PGL = paraganglioma; PHD = prolyl hydroxylase; Pheo = pheochromocytoma; PNEN = pancreatic neuroendocrine neoplasms; PNET = pancreatic neuroendocrine tumor; RCC = clear cell renal cell carcinoma; VHL = Von Hippel-Lindau

scholarly journals Platinum-Based Treatment for Well- and Poorly Differentiated Pancreatic Neuroendocrine Neoplasms

Pancreas ◽  
2021 ◽  
Vol 50 (2) ◽  
pp. 138-146
Author(s):  
Alessandra Pulvirenti ◽  
Nitya Raj ◽  
Sara Cingarlini ◽  
Antonio Pea ◽  
Laura H. Tang ◽  
...  
Keyword(s):  
Neuroendocrine Neoplasms ◽  
Pancreatic Neuroendocrine Neoplasms ◽  
Poorly Differentiated

scholarly journals Neuroendocrine Tumors: Clinical, Histological and Immunohistochemical Perspectives and Case Report—Mature Teratoma in a 16-Year-Old Girl

2021 ◽  
Vol 28 (3) ◽  
pp. 373-386
Author(s):  
Elżbieta Sowińska-Przepiera ◽  
Dariusz Starzyński ◽  
Anhelli Syrenicz ◽  
Ireneusz Dziuba ◽  
Barbara Wiszniewska ◽  
...  
Keyword(s):  
Neuroendocrine Tumor ◽  
Neuroendocrine Tumors ◽  
Histopathological Examination ◽  
Mature Teratoma ◽  
Young Patients ◽  
The Body ◽  
Systemic Review ◽  
Neuroendocrine Neoplasm ◽  
Neuroendocrine Neoplasms ◽  
Heterogeneous Structure

A mature teratoma is a germinal neoplasm that differentiates from embryonic multipotent cells into three germ layers. There may also be glandular tissue. The literature describes a total of 658 cases of ovarian neuroendocrine neoplasms, mainly in women over 40 years of age. The authors, together with a systemic review, present a case of a 16-year-old girl diagnosed with and treated for a neuroendocrine tumor. Case description: A 16-year-old girl visited the Paediatric Gynaecology Outpatient Clinic because of abdominal pains that intensified during menstruation. Standard painkillers and diastolic drugs were ineffective. An ultrasound examination revealed a large tumor with a heterogeneous structure in her right ovary. A sparing operation was carried out. During laparotomy, the lesion was enucleated, leaving healthy tissue. Histopathological examination revealed the typical features of teratoma, as well as the coexistence of a G1 neuroendocrine tumor. Immunohistochemical examination (IHC) showed the presence of markers characteristic for this type of tumor. The patient requires constant monitoring in the Endocrinology and Oncological Gynaecology Clinic. Conclusion: Tissue of neuroendocrine neoplasm within a teratoma is rare in this age group of patients; thus, there are currently no standards for long-term follow-up. This case adds to the body of evidence and demonstrates a possible good prognosis with non-aggressive behavior in G1 neuroendocrine tumors and teratomas in young patients.

scholarly journals Neuroendocrine neoplasms of the pancreas: diagnosis and pitfalls

2021 ◽  
Author(s):  
Björn Konukiewitz ◽  
Moritz Jesinghaus ◽  
Atsuko Kasajima ◽  
Günter Klöppel
Keyword(s):  
Neuroendocrine Tumors ◽  
Chromogranin A ◽  
Molecular Profile ◽  
Neuroendocrine Neoplasms ◽  
Histological Differentiation ◽  
Pancreatic Neuroendocrine Neoplasms ◽  
Poorly Differentiated ◽  
Well Differentiated ◽  
Diagnostic Pitfalls ◽  
Neuroendocrine Carcinomas

AbstractCommon to neuroendocrine neoplasms of the pancreas is their expression of synaptophysin, chromogranin A, and/or INSM1. They differ, however, in their histological differentiation and molecular profile. Three groups can be distinguished: well-differentiated neuroendocrine neoplasms (neuroendocrine tumors), poorly differentiated neuroendocrine neoplasms (neuroendocrine carcinomas), and mixed neuroendocrine-non-neuroendocrine neoplasms. However, the expression of synaptophysin and, to a lesser extent, also chromogranin A is not restricted to the neuroendocrine neoplasms, but may also be in a subset of non-neuroendocrine epithelial and non-epithelial neoplasms. This review provides the essential criteria for the diagnosis of pancreatic neuroendocrine neoplasms including diagnostic clues for the distinction of high-grade neuroendocrine tumors from neuroendocrine carcinomas and an algorithm avoiding diagnostic pitfalls in the delineation of non-neuroendocrine neoplasms with neuroendocrine features from pancreatic neuroendocrine neoplasms.

scholarly journals Case report: composite pancreatic intraductal papillary mucinous neoplasm and neuroendocrine tumor: a new mixed neuroendocrine-non-neuroendocrine neoplasm?

2021 ◽  
Vol 16 (1) ◽  
Author(s):  
Jingci Chen ◽  
Pengyan Wang ◽  
Ke Lv ◽  
Weixun Zhou
Keyword(s):  
Neuroendocrine Tumor ◽  
Intraductal Papillary Mucinous Neoplasm ◽  
Histopathological Examination ◽  
Abdominal Ultrasound ◽  
Genetic Alterations ◽  
Neuroendocrine Neoplasm ◽  
Neuroendocrine Neoplasms ◽  
Pancreatic Lesion ◽  
Partial Pancreatectomy ◽  
Mucinous Neoplasm

Abstract Background Mixed neuroendocrine-non-neuroendocrine neoplasms (MiNEN) of the pancreas are extremely rare. Their pathogenesis and molecular landscape are largely unknown. Here, we report a case of mixed pancreatic intraductal papillary mucinous neoplasm (IPMN) and well-differentiated neuroendocrine tumor (NET) and identify its genetic alterations by next-generation sequencing (NGS). Case presentation A fifty-year-old male was admitted into the hospital for evaluation of a pancreatic lesion detected during a routine examination. Abdominal ultrasound indicated a hypoechoic mass of 2.6 cm at the head of the pancreas. Malignancy was suspected and partial pancreatectomy was performed. Thorough histopathological examination revealed a mixed IPMN-NET. In some areas, the two components were relatively separated, whereas in other areas IPMN and NET grew in a composite pattern: The papillae were lined with epithelial cells of IPMN, and there were clusters of NET nests in the stroma of papillary axis. NGS revealed shared somatic mutations (KRAS, PCK1, MLL3) in both components. The patient has been uneventful 21 months after the surgery. Conclusions Our case provides evidence of a common origin for mixed IPMN-NET with composite growth features. Our result and literature review indicate that KRAS mutation might be a driver event underlying the occurrence of MiNEN. We also recommend the inclusion of mixed non-invasive exocrine neoplasms and neuroendocrine neoplasms into MiNEN.

scholarly journals Mixed Neuroendocrine/Non-neuroendocrine Neoplasm (MiNEN) of the Ovary Arising from Endometriosis: Molecular Pathology Analysis in Support of a Pathogenetic Paradigm

2021 ◽  
Author(s):  
Roberta Maragliano ◽  
Laura Libera ◽  
Ileana Carnevali ◽  
Valeria Pensotti ◽  
Giovanna De Vecchi ◽  
...  
Keyword(s):  
Large Cell ◽  
Neuroendocrine Neoplasm ◽  
Molecular Profile ◽  
Neuroendocrine Neoplasms ◽  
Fish Analysis ◽  
Sequencing Analysis ◽  
Endometrioid Carcinoma ◽  
Preneoplastic Lesions ◽  
Ovarian Endometriosis ◽  
Neuroendocrine Carcinomas

AbstractPrimary ovarian neuroendocrine neoplasms (Ov-NENs) are infrequent and mainly represented by well-differentiated forms (neuroendocrine tumors — NETs — or carcinoids). Poorly differentiated neuroendocrine carcinomas (Ov-NECs) are exceedingly rare and only few cases have been reported in the literature. A subset of Ov-NECs are admixed with non-neuroendocrine carcinomas, as it occurs in other female genital organs, as well (mostly endometrium and uterine cervix), and may be assimilated to mixed neuroendocrine/non-neuroendocrine neoplasms (MiNENs) described in digestive and extra-digestive sites. Here, we present a case of large cell Ov-NEC admixed with an endometrioid carcinoma of the ovary, arising in the context of ovarian endometriosis, associated with a uterine endometrial atypical hyperplasia (EAH). We performed targeted next-generation sequencing analysis, along with a comprehensive immunohistochemical study and FISH analysis for TP53 locus, separately on the four morphologically distinct lesions (Ov-NEC, endometrioid carcinoma, endometriosis, and EAH). The results of our study identified molecular alterations of cancer-related genes (PIK3CA, CTNNB1, TP53, RB1, ARID1A, and p16), which were present with an increasing gradient from preneoplastic lesions to malignant proliferations, both neuroendocrine and non-neuroendocrine components. In conclusion, our findings underscored that the two neoplastic components of this Ov-MiNEN share a substantially identical molecular profile and they progress from a preexisting ovarian endometriotic lesion, in a patient with a coexisting preneoplastic proliferation of the endometrium, genotypically and phenotypically related to the ovarian neoplasm. Moreover, this study supports the inclusion of MiNEN in the spectrum ovarian and, possibly, of all gynecological NENs, among which they are currently not classified.

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