Systemic Therapy for Neuroendocrine Neoplasms

2019 ◽  
Vol 03 (01) ◽  
pp. 063-070
Author(s):  
David Chan ◽  
Simron Singh
Keyword(s):  
Carcinoid Syndrome ◽  
Systemic Treatment ◽  
Histological Grade ◽  
Delayed Diagnosis ◽  
Peptide Receptor Radionuclide Therapy ◽  
Somatostatin Analogues ◽  
Neuroendocrine Neoplasms ◽  
Ongoing Research ◽  
Disease Characteristics ◽  
Grade 3

AbstractNeuroendocrine neoplasms (NENs) are heterogeneous malignancies which are becoming more common. Systemic treatment is considered for patients with advanced disease, and treatment decisions are often driven by the histological grade of the tumor and the site of primary. Somatostatin analogues are the first-line option of choice for gastroenteropancreatic NENs but subsequent options may include the targeted agents everolimus and sunitinib as well as peptide receptor radionuclide therapy. Telotristat is a new option for the treatment of refractory carcinoid syndrome diarrhea. Chemotherapy is infrequently used for Grade 1 to 2 NENs (except for the combination of capecitabine and temozolomide) but is the mainstay of therapy for Grade 3 neuroendocrine carcinomas. Bronchial NENs are graded differently and there are few proven options for systemic treatment. Optimal integration of available systemic therapies, the timely recognition of tumor heterogeneity, and the use of nuclear medicine are areas of ongoing research. Finally, the patient experience is impacted by factors such as delayed diagnosis and symptoms of carcinoid syndrome. Clinicians need to account for patient priorities and disease characteristics to individualize therapy choices for patients with advanced NEN.

scholarly journals Challenges in Diagnostic and Systemic Treatment of Metastatic Lung Atypical Carcinoid: a Case Report

2021 ◽  
Vol 1 (1) ◽  
pp. 59-68
Author(s):  
Vlad Mihai Croitoru ◽  
Diana Bogdan ◽  
Ioana Mihaela Dinu ◽  
Monica Ionela Miron ◽  
Irina Mihaela Cazacu ◽  
...  
Keyword(s):  
Radionuclide Therapy ◽  
Carcinoid Syndrome ◽  
Systemic Treatment ◽  
Peptide Receptor Radionuclide Therapy ◽  
Somatostatin Analogues ◽  
Atypical Carcinoid ◽  
Good Clinical Response ◽  
Peptide Receptor ◽  
Metastatic Lung ◽  
Neuroendocrine Lung Tumors

Neuroendocrine lung tumors are rare neoplasms, with an increasing incidence in the last thirty years. Metastatic disease is rarely curable. We present the case of a 65-year-old male patient with atypical functional carcinoid of the lung and synchronous liver metastases beginning with carcinoid syndrome treated with somatostatin analogues, chemotherapy and peptide receptor radionuclide therapy, with a good clinical response and remission of the carcinoid syndrome.

scholarly journals Well Differentiated Grade 3 Neuroendocrine Tumors of the Digestive Tract: A Narrative Review

2020 ◽  
Vol 9 (6) ◽  
pp. 1677
Author(s):  
Anna Pellat ◽  
Romain Coriat
Keyword(s):  
Digestive Tract ◽  
Neuroendocrine Tumors ◽  
Peptide Receptor Radionuclide Therapy ◽  
Small Sample ◽  
World Health ◽  
Neuroendocrine Neoplasms ◽  
Ki 67 ◽  
Metastatic Setting ◽  
Well Differentiated ◽  
Grade 3

The 2017 World Health Organization (WHO) classification of neuroendocrine neoplasms (NEN) of the digestive tract introduced a new category of tumors named well-differentiated grade 3 neuroendocrine tumors (NET G−3). These lesions show a number of mitosis, or a Ki−67 index higher than 20% with a well-differentiated morphology, therefore separating them from neuroendocrine carcinomas (NEC) which are poorly differentiated. It has become clear that NET G−3 show differences not only in morphology but also in genotype, clinical presentation, and treatment response. The incidence of digestive NET G−3 represents about one third of NEN G−3 with main tumor sites being the pancreas, the stomach and the colon. Treatment for NET G−3 is not yet standardized because of lack of data. In a non-metastatic setting, international guidelines recommend surgical resection, regardless of tumor grading. For metastatic lesion, chemotherapy is the main treatment with similar regimen as NET G−2. Sunitinib has also shown some positive results in a small sample of patients but this needs confirmation. Peptide receptor radionuclide therapy (PRRT) and immunotherapy could be future available treatments after ongoing studies. The goal of this review was to sum up the latest data on the epidemiology and management of digestive NET G−3.

scholarly journals Targeted Cancer Therapy: What’s New in the Field of Neuroendocrine Neoplasms?

Cancers ◽  
2021 ◽  
Vol 13 (7) ◽  
pp. 1701
Author(s):  
Anna La Salvia ◽  
Paula Espinosa-Olarte ◽  
Maria Del Carmen Riesco-Martinez ◽  
Beatriz Anton-Pascual ◽  
Rocío Garcia-Carbonero
Keyword(s):  
Treatment Options ◽  
Somatostatin Receptors ◽  
Peptide Receptor Radionuclide Therapy ◽  
Somatostatin Analogues ◽  
Neuroendocrine Neoplasms ◽  
Antiangiogenic Agents ◽  
Drug Conjugates ◽  
Therapeutic Drugs ◽  
Anatomic Distribution ◽  
Pancreatic Origin

Neuroendocrine tumors (NETs) are a heterogeneous family of neoplasms of increasing incidence and high prevalence due to their relatively indolent nature. Their wide anatomic distribution and their characteristic ability to secrete hormonally active substances pose unique challenges for clinical management. They are also characterized by the common expression of somatostatin receptors, a target that has been extremely useful for diagnosis and treatment (i.e., somatostatin analogues (SSAs) and peptide-receptor radionuclide therapy (PRRT)). Chemotherapy is of limited use for NETs of non-pancreatic origin, and the only approved targeted agents for advanced progressive NETs are sunitinib for those of pancreatic origin, and everolimus for lung, gastrointestinal and pancreatic primaries. Despite recent therapeutic achievements, thus, systemic treatment options remain limited. In this review we will discuss the state-of-the-art targeted therapies in the field of NETs, and also future perspectives of novel therapeutic drugs or strategies in clinical development, including recently presented results from randomized trials of yet unapproved antiangiogenic agents (i.e., pazopanib, surufatinib and axitinib), PRRT including both approved radiopharmaceuticals (177Lu-Oxodotreotide) and others in development (177Lu-Edotreotide, 177Lu-Satoreotide Tetraxetan), immunotherapy and other innovative targeted strategies (antibody-drug conjugates, bites,…) that shall soon improve the landscape of personalized treatment options in NET patients.

scholarly journals Peptide receptor radionuclide therapy with 177Lu-DOTATATE for symptomatic control of refractory carcinoid syndrome

2021 ◽  
Author(s):  
Wouter T Zandee ◽  
Tessa Brabander ◽  
Anela Blažević ◽  
Noémie S Minczeles ◽  
Richard A Feelders ◽  
...  
Keyword(s):  
Radionuclide Therapy ◽  
Carcinoid Syndrome ◽  
Tertiary Care ◽  
Somatostatin Analogs ◽  
Peptide Receptor Radionuclide Therapy ◽  
Progression Free Survival ◽  
Neuroendocrine Neoplasms ◽  
Care Hospital ◽  
Acid Excretion ◽  
Peptide Receptor

Abstract Context Peptide receptor radionuclide therapy (PRRT) with 177Lu-DOTATATE results in an increase of progression-free survival and quality of life in patients with progressive well-differentiated neuroendocrine neoplasms (NENs). Objective To study the effect of 177Lu-DOTATATE in patients with carcinoid syndrome and radiologically stable or newly diagnosed disease treated solely for the purpose of symptom reduction. Design Retrospective cohort study Setting Tertiary care hospital Patients 22 patients with a metastatic midgut NEN, elevated urinary 5-hydroxyindolacetic acid excretion and flushing and/or diarrhea despite treatment with a somatostatin analog, without documented disease progression Intervention PRRT with 177Lu-DOTATATE (intended cumulative dose: 29.6 GBq) with a primary aim to reduce symptoms. Results After PRRT, mean bowel movement frequency (BMF) decreased from 6.1 ± 3.4 to 4.6 ± 3.6 per day (p=0.009). Flushes decreased from 4.3 ± 2.9 to 2.4 ± 2.7 flushes per day (p=0.002). A decrease of BMF of more than 30% occurred in 47% of patients with baseline BMF of 4 or more (n=17). In patients with ≥2 episodes of flushing a day (n=15), 67% of patients had more than 50% decrease of daily flushing. A decrease in urinary 5-hydroxyindolacetic acid excretion of more than 30% was seen in 56% of patients. The EORTC-C30 diarrhea subscale score showed a trend towards improvement by an average of 16.7 ± 33.3 points (p=0.11). Conclusion PRRT with 177Lu-DOTATATE effectively reduced diarrhea and flushing in patients with carcinoid syndrome and can be considered for symptomatic treatment of carcinoid syndrome insufficiently controlled with somatostatin analogs.

scholarly journals Peptide Receptor Radionuclide Therapy in Grade 3 Neuroendocrine Neoplasms: Safety and Survival Analysis in 69 Patients

2018 ◽  
Vol 60 (3) ◽  
pp. 377-385 ◽  
Author(s):  
Jingjing Zhang ◽  
Harshad R. Kulkarni ◽  
Aviral Singh ◽  
Karin Niepsch ◽  
Dirk Müller ◽  
...  
Keyword(s):  
Survival Analysis ◽  
Radionuclide Therapy ◽  
Peptide Receptor Radionuclide Therapy ◽  
Neuroendocrine Neoplasms ◽  
Peptide Receptor ◽  
Grade 3

scholarly journals NON SURGICAL TREATMENT OF CARCINOID LUNGS TUMORS

2021 ◽  
Vol 9 (12) ◽  
pp. 647-651
Author(s):  
Bourhafour Mouna ◽  
◽  
Chekrine Tarik ◽  
Bouchbika Zineb ◽  
Benchakroun Nadia ◽  
...  
Keyword(s):  
Peptide Receptor Radionuclide Therapy ◽  
Somatostatin Analogues ◽  
Neuroendocrine Neoplasms ◽  
Low Grade ◽  
Intermediate Form ◽  
Typical Carcinoid ◽  
Grade Malignancy ◽  
Long Acting ◽  
Non Surgical Treatment ◽  
Systemic Therapies

Typical carcinoid lungs tumors are neuroendocrine bronchopulmonary tumors with a low-grade malignancy, and an atypical carcinoid is an intermediate form of these tumors. Their systemic treatment is greatly influenced by therapeutic evidence derived from the more frequent gastroenteropancreatic neuroendocrine neoplasms. Currently, systemic therapies for lung carcinoids, aiming at controlling tumor growth include long acting somatostatin analogues (SSAs), peptide receptor radionuclide therapy, chemotherapy and molecular-targeted therapy.

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