SSTR positive

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.

Early results support α-PRRT as a potential first- or second-line therapeutic option. Ongoing phase III trials will be critical to confirm its long-term safety, survival outcomes, and role in routine clinical practice.

In 2018, the Food and Drug Administration (FDA) approved lutetium-177 (177Lu) DOTATATE (LUTATHERA, Advanced Accelerator Applications [Novartis]) for the treatment of somatostatin receptor positive gastroenteropancreatic neuroendocrine tumors (GEP-NETs)...Less than 5 years later, in March 2022, 177Lu vipivotide tetraxetan (PLUVICTO, Advanced Accelerator Applications) received FDA approval for the treatment of prostatespecific membrane antigen-positive castration-resistant metastatic prostate cancer...As more radiotheranostic agents and applications get adopted in clinical practice, interventional radiologists are likely to get exposed to this field in a way or another. In this article, we discuss the fundamentals of radiotheranostic therapy and explore the expanding role interventional radiology (IR) is expected to play as an essential partner in modern oncology practice.

However, the expansion of the repertoire of studied and approved theranostics promises to make these highly effective treatments available to more patients. The following review intends to provide an overview of current questions about theranostics with relevance to surgical oncology.

Short-course corticosteroid prophylaxis to prevent tumor flare reactions in high-risk patients with neuroendocrine tumors treated with 177Lu-DOTATATE did not appear to decrease the incidence of tumor flare reactions compared to previously reported numbers. Randomized, placebo-controlled trials looking at the use of corticosteroids to prevent tumor flare reactions in patients treated with 177Lu-DOTATATE are needed to fully elucidate the safety and efficacy of corticosteroids used in this setting and to determine the impact on treatment outcomes.

P3, N=332, Active, not recruiting, Camurus AB | Trial completion date: Dec 2027 --> Jul 2028 | Trial primary completion date: Dec 2025 --> Jul 2026

The combination of PRRT and chemotherapy may enhance treatment efficacy by targeting both somatostatin receptor-positive and FDG-avid tumor cells. By addressing the lack of prospective data on the efficacy of PRRT combined with chemotherapy in NETs, this trial aims to provide valuable insights into the benefits and risks of this combination. The results of this study have the potential to significantly impact the treatment strategies for patients with aggressive NETs, potentially improving the outcomes and quality of life of this patient population.

PRRT with [¹⁷⁷Lu]Lu-DOTA-TATE is a well-tolerated treatment option for selected patients with advanced MTC, including those receiving first-line therapy and those with low SSTR expression. In our limited cohort, bone metastases were associated with shorter PFS, and discordant imaging/biochemical responses are common. Functional imaging-guided selection and individualized response assessment are essential for optimal management.

Radioligand therapy (RLT) with [177Lu]Lu-DOTA-TATE was authorized to treat well-differentiated (G1 and G2) unresectable or metastatic, somatostatin receptor (SSTR)-positive GEP-NETs, in progression after SSA...Thus, the scientific board agreed that RLT should always be considered in SSTR-positive GEP-NETs. Graphical Abstract available for this article.

P=N/A, N=164, Active, not recruiting, Advanced Accelerator Applications | Trial completion date: Apr 2025 --> Mar 2026 | Trial primary completion date: Apr 2025 --> Mar 2026

During follow-up, 13 patients died (survival range 5-30 months); 22 patients were alive (follow-up range 1-18 months). Our retrospective analysis shows that [225Ac]Ac-DOTA-LM3 PRRT is relatively safe concerning acute and long-term toxicity and bears promising survival outcomes in patients progressing after [177Lu]Lu-DOTATATE or [177Lu]Lu-DOTATOC PRRT.

Elevated IL-33 and IL-4 favor the development of a type 2 immune response associated with unfavored therapeutic outcome, while increased sST2 mitigates the IL-33's effect in responders, contributing to a more favorable response. These findings emphasize IL-33 as an important biomarker of early response in NET patients undergoing PRRT.