The standard management remains surgical resection, with tyrosine kinase inhibitors (TKIs) in RET mutation-positive or RET mutation-negative metastatic cases and/or Lutathera peptide receptor radionuclide therapy (PRRT) used in disseminated disease, and external beam radiotherapy for locally aggressive or infiltrative retaining a limited role...Of the 80 MTC patients reviewed, this case series highlights 10 atypical presentations in nine cases : 3 unusual tumors along with MTC, namely chondrosarcoma, carcinoma prostrate, and ectopic Cushing's syndrome; 4 unusual associations or presenting manifestations: pneumoconiosis masquerading as lung metastasis, Marfanoid habitus in MEN-2A and VHL spectrum disease, 1 with skull metastasis, and 2 cases with TKI-related complications in the form of glomerulonephritis and one patient displayed Marfanoid habitus with a RET mutation but without MEN2B or fibrillin gene mutation, while another developed bowel perforation secondary to lenvatinib therapy emphasizing diagnostic and therapeutic challenges and rare tumor associations...Management of MTC remains complex due to therapy-related complications and resistance. Molecular diagnostics enable better risk stratification and personalized care.

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.

P2, N=130, Recruiting, National Cancer Institute (NCI) | Trial completion date: Jan 2027 --> Jan 2033 | Trial primary completion date: Jan 2026 --> Jan 2030

P3, N=288, Recruiting, RayzeBio, Inc. | Trial primary completion date: Dec 2025 --> Dec 2026

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.

P2, N=153, Recruiting, RTOG Foundation, Inc. | Not yet recruiting --> Recruiting

P1/2, N=140, Active, not recruiting, Novartis Pharmaceuticals | Recruiting --> Active, not recruiting | N=200 --> 140

P1, N=65, Active, not recruiting, Novartis Pharmaceuticals | Recruiting --> Active, not recruiting | Trial completion date: Jul 2026 --> Jul 2027

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.

P2, N=1, Terminated, Imperial College London | N=106 --> 1 | Trial completion date: Mar 2029 --> Oct 2025 | Recruiting --> Terminated | Trial primary completion date: Sep 2028 --> Oct 2025; The study has closed earlier than planned due to one of the funders decision to withdraw the financial support for the study.

P2, N=30, Recruiting, National Cancer Institute (NCI) | Trial completion date: Nov 2025 --> Nov 2026 | Trial primary completion date: Nov 2025 --> Nov 2026

The patient, who was unresponsive to RAI therapy, was being evaluated for suitability for 177 Lu-DOTATATE therapy...Muscle metastases are extremely rare for PTC. This case exemplifies the different levels of tumoral affinity shown by aggressive variants of PTC across three distinct imaging modalities: 68 Ga-DOTATATE PET/MRI, 18 F FDG PET/CT, and whole-body iodine scintigraphy.