L1CAM Reliably Distinguishes Low-Grade Oncocytic Tumor from Other Eosinophilic Renal Neoplasms: A Multicenter Immunohistochemical Study with Diagnostic Implications.

Low-grade oncocytic tumor (LOT) is a recently described renal neoplasm characterized by indolent clinical behavior, a small nested architecture, and distinctive immunophenotypic features. Its distinction from other eosinophilic renal tumors, such as oncocytoma, eosinophilic chromophobe renal cell carcinoma (E-chRCC), and eosinophilic vacuolated tumor (EVT), can be challenging due to overlapping features. The L1 cell adhesion molecule (L1CAM) is being increasingly recognized as a potential diagnostic marker for LOT.

Tumor-infiltrating immune cells predict the response to somatostatin receptor ligands only in somatotropinomas naïve to medical therapy.

Tumor-infiltrating immune cells (TICs) are important components of the tumor microenvironment (TME). They regulate somatotroph adenoma treatment responses to therapy with somatostatin receptor ligands (SRLs), mediated by soluble factors and cytokines. In this study, we assessed the effect of SRLs treatment on TICs.

In Vivo Versus In Vitro Somatostatin Receptor Expression in Neuroendocrine Neoplasms: A Systematic Review and Meta-Analysis of Correlation Studies.

Well-differentiated neuroendocrine neoplasms (NENs) are characterized by hyperexpression on the cell membrane of somatostatin receptors (SSTRs). The demonstration of SSTRs, mainly the subtype 2 (SSTR2), is the prerequisite for diagnostic and therapeutic strategies with radiolabeled somatostatin analogs (SSAs). SSTRs can be routinely demonstrated in vivo by SSA-positron emission tomography/computed tomography (SSA-PET/CT) and in vitro by immunohistochemistry (IHC).

Appendiceal neuroendocrine tumors in children and adolescents: The European Cooperative Study Group for Pediatric Rare Tumors (EXPeRT) diagnostic and therapeutic recommendations.

Neuroendocrine tumors of the appendix, formerly known as carcinoid tumors, represent a rare entity. They are slow-growing tumors, characterized by an indolent clinical course. In pediatric patients, the 5-year overall survival is estimated to be 100% and the event-free survival slightly less than 100%, with only one reported case of local relapse to date.

Genes of the "regulation of lymphocyte activation" pathway may influence immune cells infiltration in growth hormone secreting pituitary tumors.

Purpose: The tumor microenvironment (TME) may provide a useful framework for understanding the heterogeneous behavior of growth hormone (GH) secreting pituitary adenomas. Although the interest in TME in somatotropinomas has increased exponentially over the last few decades, there is limited elucidation of its mechanisms, particularly in relation to genes expression involved in its regulation.

Methods: A retrospective, observational, single-center study was conducted on 85 subjects: 46 patients diagnosed with acromegaly and 39 controls.

Upfront Oxaliplatin-Fluoropyrimidine Chemotherapy and Somatostatin Analogues in Advanced Well-Differentiated Gastro-Entero-Pancreatic Neuroendocrine Tumors.

(1) Background: GEP-NETs are frequently diagnosed at advanced stage. For well-differentiated somatostatin receptor-positive (SSTR+) NETs, SSA are the preferred first-line therapy. However, in newly diagnosed patients with G2/G3 and a high tumor burden, SSA alone might not be enough; (2) Methods: We conducted a retrospective analysis to assess the effectiveness of combining oxaliplatin-fluoropyrimidine chemotherapy with SSA as an upfront strategy in newly diagnosed metastatic G2/G3 GEP-NET patients treated with oxaliplatin-fluoropyrimidine-based chemotherapy; (3) Results: Between March 2017 and October 2023, 32 pts (19 males, 13 females; M:F = 1.

Clinicopathological Correlates of Hormone Expression-Based Subtypes of Non-Functioning Duodenal/Ampullary Neuroendocrine Tumors: A Multicenter Study of 151 Cases.

Duodenal neuroendocrine tumors (Duo-NETs) may arise in the ampullary and non-ampullary duodenum. Non-functioning Duo-NETs (NF-Duo-NETs), which account for most cases, may express various hormones. Previous studies have suggested that hormone production might be associated with biological aggressiveness.

Cytokines and chemokines modulate the growth of pituitary adenoma/neuroendocrine tumors: preliminary results of a monocenter prospective pilot study.

Introduction: Cytokine and chemokines have been recognized to be involved in the progression and prognosis of pituitary adenoma/neuroendocrine tumors (PAs/PitNETs), also known as pituitary adenomas. We aim to investigate the expression of cytokine and chemokine in PAs/PitNETs, and their association with PAs/PitNETs clinical and biological behavior.

Patients And Methods: A prospective and monocenter study was performed on 16 patients diagnosed for PAs/PitNETs.

Differentiation Versus Grade for Pancreatic Neuroendocrine Neoplasms.

Context.—: Pancreatic neuroendocrine neoplasia (panNEN) is a tumor disease with distinctive morphology and often poses diagnostic challenges.

Objective.

Rapid growing mass of the mandible due to an oral metastasis of thymoma: Case report of an extremely rare localization and review of published cases.

Background: Cancer metastases in the oral cavity are relatively uncommon, occurring in approximately 1-3% of malignant cases. Thymomas and thymic carcinomas are the two main tumors that can affect the thymus, a lymphatic gland that plays an important role in regulating the immune system. The aims of this paper are (i) to describe an extremely rare case of thymoma metastasis to the oral cavity (ii) report all published cases of thymic tumor metastases to the oral cavity.

The Rare Entity of Basaloid Thymic Carcinoma: A Multicentric Retrospective Analysis from the Italian Collaborative Group for ThYmic MalignanciEs (TYME).

Background: thymic basaloid carcinoma (BTC) is an extremely rare tumor, and very little data are available on BTC's biology, clinical behavior, drug sensitivity, and patient outcomes.

Methods: We performed a retrospective observational study on patients diagnosed with BTC in 11 referral centers of TYME. All BTC diagnoses were reviewed by the referring pathologist.

Environmental maternal exposures and the risk of premature birth and intrauterine growth restriction: The Generation Gemelli study protocol of newborn exposome.

Background: The study of women exposures and child outcomes occurring in the first 1,000 days of life since conception enhances understanding of the relationships between environmental factors, epigenetic changes, and disease development, extending beyond childhood and spanning the entire lifespan. Generation Gemelli is a recently launched case-control study that enrolls mother-newborns pairs in one of the largest university hospitals in Italy, in order to examine the association between maternal environmental exposures and intrauterine growth restriction (IUGR) and the risk of premature birth. The study will also evaluate the association of maternal exposures and the health and growth of infants and children up to 24 months of age.

IGF-I levels during standard Lanreotide dose predicts biochemical outcome of high-frequency regimen in acromegaly.

Introduction: First-generation somatostatin receptor ligands (fg-SRLs) are the cornerstone of acromegaly treatment. Additional benefits were shown using high dose (HD) or high frequency (HF), relatively short-term regimens. Although several predictors of response to standard dose (SD)-fg-SRLs were reported, outcome biomarkers for HF administration are not yet available.

Neuroendocrine neoplasms of the breast: a multicenter retrospective Italian study.

Background: The aim of our retrospective study was to describe the immunophenotypic characteristics of neuroendocrine breast neoplasm (BNEN), a peculiar and rare type of breast carcinoma.

Methods: Pathological data from 51 patients affected by BNEN were retrospectively evaluated, and 44 patients were considered eligible (7 patients with incomplete data were excluded). The patients were followed in six different Italian hospitals between 2009 and 2019.

LCM-RNAseq Highlights Intratumor Heterogeneity and a lncRNA Signature from Archival Tissues of GH-Secreting PitNETs.

Background: This study explores the potential for hidden variations within seemingly uniform regions of growth hormone-secreting pituitary neuroendocrine tumors (GH-PitNETs). We employed archived tissue samples using Laser Capture Microdissection Sequencing (LCM-RNAseq) to probe the molecular landscape of these tumors at a deeper level.

Methods: A customized protocol was developed to extract, process, and sequence small amounts of RNA from formalin-fixed, paraffin-embedded (FFPE) tissues derived from five patients with GH-secreting PitNETs and long-term follow-up (≥10 years).

Screening and surveillance practices for Multiple Endocrine Neoplasia type 1-related Neuroendocrine Tumours in European Neuroendocrine Tumor Society Centers of Excellence (ENETS CoE)-An ENETS MEN1 task force questionnaire study.

Multiple Endocrine Neoplasia type 1 (MEN1) Clinical Practice Guidelines (2012) are predominantly based on expert opinion due to limited available evidence at the time, leaving room for interpretation and variation in practices. Evidence on the natural course of MEN1-related neuroendocrine tumours (NET) and the value of screening programs has increased and new imaging techniques have emerged. The aim of this study is to provide insight in the current practices of screening and surveillance for MEN1-related NETs in ENETS Centers of Excellence (CoEs).

Atypical Presentation of Splenic Tissue Mimicking Neuroendocrine Tumor Relapse.

A 61-year-old man underwent splenopancreasectomy for a 3-cm neuroendocrine tumor of the body of the pancreas (G2, pT1 pN0, Ki67 3%). Five months after surgery 68 Ga-DOTATOC PET/CT showed increased radiotracer uptake in a solid tissue of the splenic fossa, possibly referring to a splenosis nodule. After 19 months, a further 68 Ga-DOTATOC PET/CT showed a significant functional and dimensional increase of the previously detected tissue and the appearance of a new finding in the left lateral abdominal wall.

Critical updates in neuroendocrine tumors: Version 9 American Joint Committee on Cancer staging system for gastroenteropancreatic neuroendocrine tumors.

The American Joint Committee on Cancer (AJCC) staging system for all cancer sites, including gastroenteropancreatic neuroendocrine tumors (GEP-NETs), is meant to be dynamic, requiring periodic updates to optimize AJCC staging definitions. This entails the collaboration of experts charged with evaluating new evidence that supports changes to each staging system. GEP-NETs are the second most prevalent neoplasm of gastrointestinal origin after colorectal cancer.

The Pathogenic RET Val804Met Variant in Acromegaly: A New Clinical Phenotype?

Several genetic investigations were conducted to identify germline and somatic mutations in somatotropinomas, a subtype of pituitary tumors. To our knowledge, we report the first acromegaly patient carrying a pathogenic variant: c.2410G>A (rs79658334), p.

Pancreas as an Unusual Metastatic Site of Medullary Thyroid Carcinoma: A Case of Very Long-term Follow-up Under Prolonged Treatment with Somatostatin Analogues.

Background: Pancreatic metastases from medullary thyroid carcinoma (MTC) are exceptional. Imaging and treatment based on somatostatin receptors may play a role, though the evidence is unconvincing.

Case Presentation: We have, herein, documented a unique case of metastatic MTC, where pancreatic metastasis was identified by 68Ga-PET/CT, with the disease showing very slow progression during treatment with lanreotide autogel.