omatostatin receptors are expressed in the majority of welldifferentiated metastatic neuroendocrine tumors (NET), rendering them amenable to peptide receptor radionuclide therapy (PRRT) with radiolabeled somatostatin analogues. [1][2][3][4] Peptide receptor radionuclide therapy is a tumor-targeted treatment that uses radiation to induce tumor cell death via β particle-emitting radionuclide, most commonly lutetium-177 ( 177 Lu) or yttrium 90 ( 90 Y), linked to a somatostatin peptide analog, usually dotatate or dotatoc. 177 Lu is a radionuclide with a more favorable toxicity profile compared with 90 Y. 5 To our knowledge, the phase III, NETTER-1 trial 6 was the first randomized clinical trial (RCT) to demonstrate a progressionfree survival benefit and suggestive of overall survival benefit in patients with well-differentiated NETs randomized to 177 Lu-dotatate compared with higher dose of long-acting octreotide, eventually leading to the US Food and Drug Administration approval of 177 Lu-dotatate for somatostatin-positive NET. Hematological toxicity is the most common subacute adverse event with 177 Lu PRRT with nadir usually occurring 4 to 6 weeks after therapy. 7,8 In addition, permanent hematologic dysfunction has been reported in multiple studies with therapy-related myeloid neoplasms (t-MN) and bone marrow failures. 1,6,[9][10][11][12] However, the incidence rates along with the clinical features of t-MN are variable across studies. While RCTs may be adequate for reporting short-term toxicity, information on rare longterm adverse events (such as t-MN) are unlikely to be sufficiently reported in RCTs because many trials set criteria that exclude patient groups who are potentially more likely to be harmed. Therefore, the systematic evaluation of new or rare adverse events may require the inclusion of other study designs: cohort, case-control, cross-sectional, and even case series. In this systematic review, we sought to review the current evidence regarding the incidence and IMPORTANCE Peptide receptor radionuclide therapy (PRRT) is a tumor-targeted treatment that uses radiation to induce tumor cell death in neuroendocrine tumors (NET) via β particle-emitting radionuclide linked to a somatostatin peptide analog. Therapy-related myeloid neoplasm (t-MN) has been reported as a potential long-term and frequently lethal adverse event after PRRT. However, the incidence, time of diagnosis, and nature of t-MN is unclear. Therefore, a systematic review is helpful to study the incidence and characteristics of t-MN after PRRT in patients with NET.OBJECTIVE To systematically evaluate the literature and report the incidence, time of diagnosis, and nature of t-MN after PRRT.EVIDENCE REVIEW MEDLINE, Embase, Scopus, Web of Science, and Cochrane Central Register of Controlled Trials for articles and abstracts reporting studies of different designs studying more than 1 patient (randomized clinical trials, prospective phase I or phase II, retrospective studies, and case series) were searched from database inception through April...