Cardiac Metastases in Patients with Neuroendocrine Tumours: Clinical Features, Therapy Outcomes, and Prognostic Implications

Abstract

<b><i>Background:</i></b> Cardiac metastases (CM) from neuroendocrine tumours (NET) are rare; however, with the introduction of new molecular imaging modalities, such as <sup>68</sup>Ga-DOTATATE PET-CT for NET diagnosis and re-staging, they are now identified more frequently. This study presents a single-institution experience on the NET CM characteristics, management, and prognostic implications. <b><i>Methods:</i></b> Between January 1998 and January 2020, 25 NET patients with CM were treated in our unit. A retrospective review of electronic records was performed. Overall survival (OS) was assessed by the Kaplan-Meier method. Cox regression models were used to evaluate the association of various clinical variables with OS. <b><i>Results:</i></b> The median age in the NET CM cohort was 64 years, with small intestine being the most common primary (84%). Nearly half of the patients suffered either from shortness of breath (48%) or had palpitations (12%). Peptide receptor radionuclide therapy (PRRT) was applied in more than half of the patients (64%), who had an improved trend for a longer median OS compared to those patients who did not receive PRRT (76.0 vs. 14.0 months, <i>p</i> = 0.196). The multivariate analysis demonstrated that concomitant skeletal or pancreatic metastases, as well as N-terminal pro-B-type natriuretic peptide (NT pro-BNP) >2 × upper limit of normal (ULN), were independent poor prognosticators. <b><i>Conclusions:</i></b> Clinical features of NET CM ranged from asymptomatic patients to heart failure. Concomitant bone or pancreatic metastases and NT pro-BNP levels >2 ULN predicted shorter survival time. PRRT serves as a feasible therapy with promising survival benefits; however, more data are needed.

Heterogeneity of Small Intestinal Neuroendocrine Tumors Metastasis: Biologic Patterns of a Series with Virchow’s Node Involvement

“…However, its presence was not found to be a prognostic factor for PFS and/or OS when compared to age- and sex- matched stage IV SI-NET patients. To date, comprehensive descriptions and survival analysis of SI-NET patients with rare metastatic manifestations, including that of VM, are sparse [ 5 , 6 , 16 , 17 , 18 ]. The prevalence of VM among patients with other abdominal and thoracic malignancies has previously been estimated up to 2.8%.…”

“…Importantly, VM was confirmed in octreoscan or DOTATATE PET-CT in all of the patients in this series, as all VMs originated from well-differentiated tumors. Due to the increased availability of new molecular imaging modalities, in particular 68 Ga-DOTATATE PET-CT, the diagnosis of rare metastatic manifestations of NETs is indeed steadily increasing [ 16 , 20 ]. As the prevalence of VM is relatively low and these lesions were clearly recognized in functional imaging with 68 Ga-DOTATATE PET-CT, which is currently applied both at diagnosis and during disease surveillance, we could advocate in favor of routine screening for VM with 68 Ga-DOTATATE PET-CT at baseline in SI-NET patients.…”

Treatment of neuroendocrine tumours with 177Lu‐peptide receptor radionuclide therapy: Challenging clinical scenarios and their management

“…Mild pericarditis occurred in two patients and settled with a short course of oral steroids. No other complications were described 34 …”

“…33 The majority of cardiac metastases appear to arise from midgut NET primaries. 34 Treating patients with 177 Lu-PRRT with cardiac metastases has successfully been reported. In a study of 25 patients with cardiac NET metastases, 15 were treated with 177 Lu-DOTATATE.…”

Cardiovascular Toxicities Secondary to Biotherapy and Molecular Targeted Therapies in Neuroendocrine Neoplasms: A Systematic Review and Meta-Analysis of Randomized Placebo-Controlled Trials

“…On the other hand, carcinoid heart disease, a rare cardiac manifestation involving the right-sided heart valves, constitutes a well-recognized sequela in patients with small intestinal neuroendocrine neoplasms (SI-NENs) often complicating the disease clinical course and eventually leading to right heart failure [ 6 ]. Finally, NEN metastases to the heart are rare, with associated clinical features ranging from asymptomatic patients to heart failure [ 7 ]. All these factors taken together with the cardiovascular side effects of different agents in the therapeutic NEN armamentarium may have a negative impact on patient outcomes, including quality of life and possibly survival outcomes.…”