SSTR positive

P=N/A, N=9, Enrolling by invitation, University of California, Davis | Trial completion date: Mar 2026 --> Sep 2026 | Trial primary completion date: Sep 2025 --> Sep 2026

In this prematurely closed phase II study, 177Lu-Dotatate/capecitabine did not improve ORR or prolong PFS and OS in advanced NET patients compared to 177Lu-Dotatate alone and was associated with reduced QALYs.

The benefit of adjuvant therapy in well-differentiated tumors is unclear, whereas thymic neuroendocrine carcinomas often require multimodal approaches, including platinum-etoposide chemotherapy and radiotherapy...Somatostatin analogues are widely used in indolent or peptide receptor-positive tumors, whereas everolimus and, more recently, cabozantinib represent options for progressive disease...Despite promising therapeutic options, robust prospective data remain limited. The integration of TNENs into basket trials, the molecular refinement of prognostic subgroups (e.g., NET G3), and the conduct of dedicated multicenter prospective studies are urgently needed to define optimal treatment algorithms and improve clinical outcomes in these rare entities.

Due to biological heterogeneity and limited evidence, tNEPC requires individualised, interdisciplinary management. This review summarises current insights into the pathogenesis, diagnosis, and therapeutic strategies of tNEPC and provides an outlook on future developments.

Considering that SSTR expression is also present in various other tumors-including pheochromocytomas, paragangliomas, meningiomas, and medullary thyroid carcinomas-there is increasing interest in expanding the use of PRRT to other SSTR-positive malignancies. This review aimed to present evidence, explore ongoing clinical research, and highlight emerging directions for 177Lu-DOTATATE therapy beyond gastroenteropancreatic NETs.

The whole-body washout rate derived from pretreatment SSTR imaging is a strong, practical predictor for outpatient eligibility following 177Lu-DOTATATE TRT. Incorporating this simple, noninvasive marker into clinical workflow could support individualized discharge planning and improve patient access under strict radiation safety regulations.

P=N/A, N=30, Recruiting, Fondazione Policlinico Universitario Agostino Gemelli IRCCS

This case highlights the diagnostic challenges associated with OOM, the utility of somatostatin receptor-based PET/CT in tumor localization, and the importance of considering TIO in patients with unexplained hypophosphatemia and osteomalacia. Prompt recognition and surgical intervention can lead to complete resolution of symptoms and prevent long-term skeletal complications.

P=N/A, N=1014, Active, not recruiting, Advanced Accelerator Applications | Trial completion date: Jun 2028 --> Sep 2027 | Trial primary completion date: Jun 2028 --> Sep 2027

The postoperative course was uneventful, and follow-up imaging at eight months showed no recurrence. This unique case of synchronous SDHB-deficient gallbladder paraganglioma and periduodenal NET illustrates cross-lineage tumorigenesis within a single germline background and emphasizes the value of SDH immunohistochemistry and genotype-guided surveillance.

The tumor-to-bone marrow absorbed dose ratio was in the same range for [161Tb]Tb-DOTA-LM3 as for [177Lu]Lu-DOTATOC. The administration of 1 GBq of [161Tb]Tb-DOTA-LM3 was safe for all patients, without relevant adverse events.

This includes work that led to the FDA approvals of immune checkpoint inhibitors in multiple rare pediatric tumor types, the NTRK inhibitors larotrectinib, entrectinib, and repotrectinib for children and adults with solid tumors with NTRK fusions, the ALK inhibitor crizotinib in children and adults with ALK-positive inflammatory myofibroblastic tumors, and the radioligand LUATHERA for adolescents and adults with somatostatin receptor-positive gastroenteropancreatic neuroendocrine tumors. Despite these advances, the study of rare pediatric cancers faces multiple challenges including a limited number of patients for efficient and well-powered clinical trials and a dearth of financial incentives. Ongoing, coordinated efforts are needed to continue the advancement of novel treatments and improve survival and minimize late effects.