Endocrine Abstracts

Background: Octreotide LAR is a long-acting somatostatin analogue used in the management of metastatic neuroendocrine tumors (NETs), with antiproliferative and symptom control effects. It requires intramuscular (IM) injection. Missed IM injections can cause subcutaneous nodules (SCNs) on radiologic images. We reviewed the rates of SCNs in a real-world cohort of NETs receiving octreotide LAR and explored treatment outcomes.Methods: Patients with gastroint...

Background: We report a case of a patient with an ileocecal neuroendocrine tumor (NET) metastatic to both breasts, for whom the initial clinical presentation was chronic diarrhea Breast metastases were initially suspected by a 68-Gallium DOTANOC PET/CT and were confirmed by histopathology. We also performed a comprehensive (non-systematic) literature review through which we identified 116 cases of NENs metastatic to breast.Methods: Case report and compre...

Background: Metastatic neuroendocrine neoplasms (NENs) constitute a heterogeneous group of uncurable cancers with limited treatment options. Lenvatinib is an oral multiple kinase inhibitor that showed activity in gastroenteropancreatic neuroendocrine tumors in the phase II Talent Trial at a starting dose of 24 mg per day. Although a confirmatory phase III study was never conducted, the drug is available for use off-label.Methods: We retrospectively revie...

Background: Metastatic neuroendocrine carcinomas (NECs) have a poor prognosis, and standard first-line chemotherapy combines etoposide (E) and platinum (P) has limited benefit. Currently, there is no standard second-line therapy. Irinotecan-based regimens have shown benefits; hence we explored nanoliposomal Irinotecan (nal-IRI) efficacy in this patient population.Methods: In this open-label, single-arm, multi-center Phase 2 study, advanced GEP or unknown...

Background: Metastatic pheochromocytomas and paragangliomas are orphan neuroendocrine tumors. 50% of these tumors are associated with germline mutations of the SDHB gene. SDHB related pheochromocytomas and paragangliomas and many apparently sporadic tumors exhibit abnormal angiogenesis. This trial assessed the efficacy and safety of Cabozantinib, a potent, antiangiogenic, tyrosine kinase inhibitor. Methods: The Natalie trial is a single arm phase 2 clini...

Background: Somatostatin analogues (SSAs) are the standard of care first line therapy for metastatic well-differentiated neuroendocrine tumors (NETs). They are approved for both alleviating hormone-related symptoms for functional tumors and inhibiting tumor growth. Their anti-proliferative benefit in the refractory setting is debatable due to lack of supportive literature. This systematic review compared efficacy of targeted therapies (Everolimus, Sunitinib, Surufatinib, Lenva...

Background: Treatment options are limited in patients with metastatic neuroendocrine neoplasms (NEN). Combination temozolomide and nivolumab showed encouraging objective response rates of 35.7% in advanced NEN regardless of site of origin, with response rates of 64% in lung NEN and 67% in pancreatic NEN (NCT03728361). We present updated survival data on follow-up from a phase II trial of combination nivolumab and temozolomide in patients with advanced NEN.<p class="abstext...

Background: Paltusotine is an investigational oral, once-daily, small molecule somatostatin receptor type 2 agonist in development for the treatment of acromegaly and carcinoid syndrome associated with neuroendocrine tumors.Methods: This multicenter, open-label, phase 1 study enrolled participants with mild, moderate, and severe hepatic impairment based on Child-Pugh classification and matched healthy participants. A single 20-mg oral dose of paltusotine...

Background: The expression of BCL-2, an anti-apoptotic protein, has been implicated in the aggressive behavior of NENs. Dysregulation of BCL-2 expression, controlled by the pRb1-E2F1 axis, has been linked to the development of various cancers, including NENs. Studies have shown that higher BCL-2 expression is associated with poorer survival outcomes in NEN patients.Methods: We report a unique case of a patient with a pancreatic neuroendocrine tumor who h...

Background: Delta-like ligand 3 (DLL3) is highly expressed on small-cell lung cancer (SCLC) tumors and neuroendocrine carcinomas (NECs). BI 764532 is a DLL3/CD3 IgG-like T-cell engager with potent preclinical activity. NCT04429087 is an ongoing phase I dose-escalation trial of BI 764532 in adults with locally advanced/metastatic DLL3-positive (confirmed centrally) SCLC, extrapulmonary NEC, or large cell neuroendocrine lung carcinoma. Here we focus on patients with extrapulmona...

Background: Low- (G1) and intermediate-grade (G2) neuroendocrine tumors (NET) are defined by lower mitotic rates and genetic mutations that rarely cause transformation to high-grade (G3) neoplasm. Yet a proportion of patients with de novo low-grade NETs progress to G3 disease or even neuroendocrine carcinoma (NEC). We aimed to highlight cases of low-grade G1 and G2 NETs that eventually transformed to G3 pathology on repeat biopsy and describe characteristics that portend high-...

Background: Gastroenteropancreatic neuroendocrine tumors (GEP-NETs) are a relatively rare group of tumors. Traditionally, management of unresectable GEP-NETs was performed by in-person assessment at specialized regional cancer centers. The COVID-19 pandemic resulted in an unprecedent shift towards virtual care. The aim of this study is to evaluate the use of virtual care for GEP-NETs after the COVID-19 pandemic at a high-volume academic cancer center. Me...

Background: Pancreatic acinar cell carcinoma (PACC) is a rare cancer, comprising less than 1% of all pancreatic cancers and often presents as a mixed malignancy with a neuroendocrine component. Limited data exists on its clinical and molecular characteristics. This retrospective study aims to provide additional insights to better understand and characterize the disease.Methods: Patients from the Mayo Clinic with pathology records denoting pancreatic acin...

Background: G3 NETs are a recently recognized entity and although the treatment recommendations largely follow those for grade 1 and 2 NETs, the outcomes of therapy are inferior. Little is known about the optimal therapy sequencing but small studies have suggested a benefit of multikinase inhibitors. Somatostatin analogs, PRRT and chemotherapy have all been reported to have activity but later lines of therapy are needed. In this study, we sought to evaluate the activity of cab...

Background: Immune checkpoint inhibitor (ICI) maintenance therapy is the standard of care for frontline management of extensive-stage small cell lung cancer (ES SCLC). However, the overall survival benefit of the addition of ICI maintenance to frontline ES SCLC treatment is modest and further improvement is needed. We hypothesized that the addition of poly (ADP-ribose) polymerase inhibition to ICI maintenance therapy for patients with platinum-sensitive ES SCLC could improve t...

Background: G3NETs demonstrate variable clinical behavior (Ki-67>20-100%). Treatment extrapolated from grade 1/2 (G1/2) NETs and neuroendocrine carcinomas depending on clinicopathologic features. Therapy for treatment-emergent G3NETs remains especially ill-defined.Methods: Retrospective chart review of patients with de novo G3NETs or progressing from low-to-high grade (L-H) (G1/2 at diagnosis and G3 >=3 months later). Treatment patterns compared ...

Background: Gastric neuroendocrine tumors (gNETs) are relatively uncommon. Represent up to 23% of all digestive neuroendocrine neoplasms. The majority are incidentally diagnosed from histology of ‘simple’ gastric polyps identified at endoscopy. There are 3 groups of gNETs: types 1, 2 and 3 with different pathogeneses, biological and clinical behaviors. It is worth mentioning that recent reports suggest a fourth type of gNETs1. Type I represents 75–80%...