2026-07-11 | Exceptional Response of Necrolytic Migratory Erythema to Octreotide in a Patient with Glucagonoma: A Case Report and Scoping Review.
Glucagonomas are rare neuroendocrine neoplasms (NENs) that often present with locoregional involvement at diagnosis and are characterized by variable survival outcomes and diverse therapeutic approaches. We present a clinical case of glucagonoma associated with necrolytic migratory erythema (NME), demonstrating an exceptional response to octreotide. Additionally, a scoping review was conducted to evaluate the efficacy of somatostatin analogs (SSAs) in managing NME. Through a meticulous search on MEDLINE, we unearthed a collection of case reports and series that shed light on patients with glucagonoma and neuroendocrine tumors (NETs) who received treatment with somatostatin analogs (SSAs). A thorough extraction and analysis of pertinent data was conducted to deepen our understanding of these cases. A 66-year-old woman consulted with a 4-year history of migratory, pruritic, and painful vesiculobullous skin lesions consistent with NME. Laboratory tests revealed serum glucagon levels exceeding 500 pg/mL, and magnetic resonance imaging identified a pancreatic mass with hepatic metastases. Octreotide therapy resulted in the complete resolution of cutaneous lesions within 4 days of the first dose. She chose not to undergo surgery or other treatment options, and as a result, she passed away due to the progression of her disease. Out of a total of 1,712 reviewed cases, 25 met the inclusion criteria. Among these cases, 56% of the patients were female, and the mean age at diagnosis was 59.74 years. Clinical improvement in NME was observed in 80% of patients treated with SSAs, with an average time to resolution of dermatological symptoms being 1.41 months. The median overall survival for these patients was 11 months. Although SSAs demonstrated notable efficacy in the resolution of NME, their impact on overall survival remains limited. These results emphasize the importance of ongoing research and innovation in treating NENs to improve long-term patient outcomes.
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2026-07-09 | An Integrative RNA Spliceosomic Landscape of Pancreatic Neuroendocrine Tumors Identifies Clinically Relevant Molecular Subgroups.
Alterations in alternative splicing are emerging as a novel cancer hallmark, offering new insights into tumor biology. However, integrative analyses of splicing are still scarce, particularly in rare cancers like pancreatic neuroendocrine tumors (PanNETs), whose striking heterogeneity complicates patient diagnosis and treatment. Here, we provide the first comprehensive characterization of the RNA splicing landscape in PanNETs through integrative analysis of RNA-seq data from 174 tumor samples. We identified three robust spliceosomic groups (SPN1, SPN2, SPN3) each associated with unique clinical and molecular characteristics. SPN1 displayed intermediate clinical behavior alongside enhanced mTOR signaling; SPN2 was characterized by a less secretory phenotype, enrichment in alpha-cell markers and somatostatin receptors, increased metastasis, and frequent mutations in MEN1 and DAXX/ATRX genes; in contrast, SPN3 was composed mainly by low grade tumors with beta-cell marker expression and the lowest mutational rate, yet it also contained all the highly proliferative neoplasms. Moreover, each group had a specific alternative splicing events signature, revealing an unprecedented discovery: the association between the expression profile of the splicing machinery and its product, the splicing variants. We provide a detailed characterization of the molecular and functional consequences of the splice variants defining each of the spliceosomic groups. These findings underscore the previously unrecognized yet significant impact of RNA splicing on PanNET heterogeneity and suggest that detailed splicing profiles could serve as valuable tools for identifying novel biomarkers and therapeutic targets. Thus, beyond providing crucial insights into PanNET molecular biology, our study offers a foundation for future studies exploring personalized therapeutic strategies based on splicing features.
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