Head and Neck Paragangliomas: Overview of Institutional Experience.

Head and neck paragangliomas (HNPGLs) are rare and have high rates of genetic mutations. We conducted a retrospective review of 187 patients with 296 PGLs diagnosed between 1974 and 2023. The mean age of diagnosis was 48.

Peptide Receptor Radionuclide Therapy Using 177Lu-DOTATATE: Nursing Roles in Managing Patients With Gastroenteropancreatic Neuroendocrine Tumors.

Background: Gastroenteropancreatic neuroendocrine tumors (GEP-NETs) are a diverse family of cancers that occur within the gastrointestinal tract and pancreas. Peptide receptor radionuclide therapy (PRRT) via 177Lu-DOTATATE is a newer therapeutic option for certain patients with somatostatin receptor-positive GEP-NETs.

Objectives: This review informs on how oncology nurses treating patients with GEP-NETs receiving PRRT using 177Lu-DOTATATE can facilitate care.

A qualitative study to understand the experience of somatostatin analog treatments from the perspective of patients with neuroendocrine tumors.

Purpose: Neuroendocrine tumors (NETs) negatively impact patients' quality of life. Octreotide long-acting release (LAR) and lanreotide depot are somatostatin analogs (SSAs) approved to treat NETs. The study objective was to explore SSA treatment experiences and preferences of patients with NETs.

Laboratory, Clinical, and Survival Outcomes Associated With Peptide Receptor Radionuclide Therapy in Patients With Gastroenteropancreatic Neuroendocrine Tumors.

Importance: Peptide receptor radionuclide therapy (PRRT) is approved in the US for treatment of gastroenteropancreatic neuroendocrine tumors (NETs), but data on PRRT outcomes within US populations remain scarce.

Objective: To analyze the first 2 years of PRRT implementation at a US-based NET referral center.

Design, Setting, And Participants: This cohort study was conducted using medical records of patients with metastatic NET receiving PRRT from 2018 through 2020 in a NET program at a tertiary referral center.

Presacral neuroendocrine tumors associated with the Currarino syndrome.

Currarino syndrome (CS) is an autosomal dominant syndrome caused by mutations in MNX1 and characterized by anorectal abnormalities, partial sacral agenesis, and presacral masses. The presacral masses are typically benign; however, malignant degeneration can occur, and presacral neuroendocrine tumors (NETs) have been reported in six cases. We report three individuals from two families affected by CS in which multiple individuals developed presacral NETs.

Tumor detection rates in screening of individuals with SDHx-related hereditary paraganglioma-pheochromocytoma syndrome.

Purpose: Minimal data exist regarding the efficacy of screening protocols for individuals with SDHx germline pathogenic variants with hereditary paraganglioma-pheochromocytoma syndrome. This study aimed to evaluate the SDHx-related tumor detection rate in individuals undergoing clinical screening protocols.

Methods: A multicenter retrospective longitudinal observational study was conducted.

Abnormal Pretreatment Liver Function Tests Are Associated with Discontinuation of Peptide Receptor Radionuclide Therapy in a U.S.-Based Neuroendocrine Tumor Cohort.

Background: Peptide receptor radionuclide therapy (PRRT) is effective for treating midgut neuroendocrine tumors (NETs); however, incorporation of PRRT into routine practice in the U.S. is not well studied.

Grade is a Dominant Risk Factor for Metastasis in Patients with Rectal Neuroendocrine Tumors.

Background: Small (< 2 cm) and diminutive (< 1 cm) rectal neuroendocrine tumors (RNETs) are often described as indolent lesions. A large single-center experience was reviewed to determine the incidence of metastasis and the risk factors for its occurrence.

Methods: Cases of RNET between 2010 and 2017 at a single institution were retrospectively reviewed.

Optimizing the Management of Carcinoid Syndrome to Reduce the Impact of Diarrhea.

Diarrhea is often a presenting symptom in patients with neuroendocrine tumors (NETs). Frequently diagnosed in advanced stages, carcinoid syndrome diarrhea negatively impacts patients' well-being and quality of life. This article will review the diagnostic work-up for neuroendocrine tumors and etiology and management of NET-related carcinoid syndrome diarrhea and provide guidance for advanced practitioners, including nurse practitioners, physician assistants, pharmacists, and dieticians, focusing on their role in patient and caregiver education regarding disease, symptom monitoring and management, development of patient-specific treatment, and survivorship plans.

Long-Acting Somatostatin Analogue Safety Monitoring Protocol for Outpatients With Neuroendocrine Tumors.

Somatostatin analogues (SSAs) are widely used in the long-term treatment of neuroendocrine tumors (NETs) and have a relatively favorable safety profile. However, SSAs are associated with specific side effects that are important to monitor. Currently, there is no standardized safety monitoring protocol for health-care professionals to use as a reference when initiating patients on long-acting SSAs.

Efficacy of Peptide Receptor Radionuclide Therapy in a United States-Based Cohort of Metastatic Neuroendocrine Tumor Patients: Single-Institution Retrospective Analysis.

Objectives: The aim of this study was to analyze in a retrospective cohort study the outcomes of a United States-based group of metastatic neuroendocrine tumor (NET) patients who underwent peptide receptor radionuclide therapy (PRRT).

Methods: Twenty-eight patients from a single US NET Center were treated with PRRT. Toxicities were assessed using Common Terminology Criteria for Adverse Events version 4.

Peptide Receptor Radionuclide Therapy-Induced Hepatotoxicity in Patients With Metastatic Neuroendocrine Tumors.

Background: Treatment of metastatic gastroenteropancreatic neuroendocrine tumors (GEP-NETs) with peptide receptor radionuclide therapy (PRRT) is effective in retarding tumor growth. Renal dysfunction, anemia, and thrombocytopenia are well-described treatment-related toxicities. However, hepatotoxicity is not well recognized.

Weight Gain in Zollinger-Ellison Syndrome After Acid Suppression.

Objectives: Zollinger-Ellison syndrome (ZES) is characterized by hypergastrinemia and gastric acid hypersecretion resulting in peptic ulcer disease, diarrhea, and weight loss. Acid secretion can be controlled with medication, and biochemical cure is possible with surgery. Data on how these interventions affect patients' weight are lacking.

PTCH 1 staining of pancreatic neuroendocrine tumor (PNET) samples from patients with and without multiple endocrine neoplasia (MEN-1) syndrome reveals a potential therapeutic target.

Pancreatic neuroendocrine tumors (PNETs) are rare, indolent tumors that may occur sporadically or develop in association with well-recognized hereditary syndromes, particularly multiple endocrine neoplasia type 1 (MEN-1). We previously demonstrated that the hedgehog (HH) signaling pathway was aberrantly up-regulated in a mouse model that phenocopies the human MEN-1 syndrome, Men1l/l;RipCre, and that inhibition of this pathway suppresses MEN-1 tumor cell proliferation. We hypothesized that the HH signaling pathway is similarly upregulated in human PNETs.