P-P22 NET Profit: Considering quality of life assessment in patients treated with SSAs for NETs

Background Somatostatin Analogue (SSA) therapy of neuroendocrine tumours (NETs) leads to pancreatic exocrine insufficiency (PEI). PEI symptoms include diarrhoea, abdominal discomfort, bloating, and steatorrhea, which negatively impact quality of life (QoL). NETs (and the sequelae of carcinoid syndrome) however, have similar symptomatology to PEI, with a comparably negative impact on QoL. QoL tools exist to assess PEI and its response to enzyme therapy; however, we hypothesise that PEI symptom scale scores will be unreliable in SSA-induced PEI due to the concurrent improvement of the carcinoid symptoms. Methods Adult patients commencing SSA therapy for NETs were recruited from December 2020. Qualitative assessments of the impact of SSAs and pancreatic exocrine function on patient QoL were performed before and during (at 8 weeks) therapy. The Pancreatic Exocrine Insufficiency Questionnaire (PEI-Q) was used to capture patient-reported assessment of relevant symptoms. Health-related QoL was assessed using the European Organisation Research and Treatment of Cancer Quality of Life Questionnaire (EORTC QLC)-C30, supplemented by the EORTC GI.NET.21. Patients were specifically asked about steatorrhea at both assessments. Results Seven patients completed the study. 5-HIAA levels were raised in 4/7 patients, indicative of carcinoid syndrome, secondary to the NET. Pancreatic exocrine function reduced after SSA therapy in all patients (data reported elsewhere) but paradoxically PEI-Q symptom scale scores reduced (median decrease from baseline: -18.5%, range: -1.5- -55.6%; p = 0.018) (Figure 1). According to PEI-Q, all seven patients would meet the criteria for a mild PEI at baseline, and two would be considered severe. One patient reported steatorrhea after commencing SSA-therapy. No changes in relevant domains of EORTC questionnaires were statistically significant, including functional, symptomatic, and overall health scores. Conclusions The PEI-Q tool is not useful for assessing SSA-related PEI due to substantial symptom overlap with the tumour itself and SSA therapy. The decrease in symptom score is likely due to improvement of carcinoid syndrome, independent of PEI. Although the cardinal PEI symptom is steatorrhea, there is no distinction between that and diarrhoea in the EORTC tool, and confounding aetiologies of diarrhoea in NET patients may further complicate assessment. Current available QoL measures are of limited use in the setting of SSA-related PEI, and care should be taken if evaluating PEI or response of PEI to treatment.

Neuroendocrine disorders

This chapter begins by covering the neuroendocrine system, then neuroendocrine tumours. Carcinoid syndrome, insulinomas, gastrinomas, glucagonomas, and VIPomas are all described, as well as other rare functional pancreatic NETs.

Heidinger syndrome - Carcinoid syndrome

Carcinoid heart disease was first described in 1954. It develops in over 70% of carcinoid syndrome patients. Up to 20% of patients with carcinoid syndrome have pronounced carcinoid heart disease at diagnosis. Cardiac involvement is usually well tolerated by patients. Symptoms are associated with secretion of serotonin, tachykinin and other vasoactive substances in the systemic circulation. Deposition of fibrous tissue along the tricuspid annulus leads to ring constriction and is the basis of tricuspid stenosis. Primary insular carcinoid tumor of the ovary is a very rare tumor that accounts for less than 1% of all carcinoid cases. We present a case of a 62-year-old woman, demonstrating shortness of breath and fatigue, diarrhea, redness and progressive swelling on both lower legs. After single-photon emission computed tomography we found pathologically increased somatostatin expression in a tumor formation in the pelvis. After removal of the primary focus, the patient received a prosthetic tricuspid valve with good clinical results.

Surgical Options for Pulmonary Valve Pathology in the Current Era

Pulmonary valve pathology occurs mostly in the settings of congenital heart disease whether primary or as the result of repair of a variety of congenital heart defects. Acquired pulmonary valve disorders, albeit rare, can occur in the settings of endocarditis, tumors, carcinoid syndrome, or rheumatic fever. Surgical options include repair and replacement of pulmonary valve. Several options for replacement are available, which can be tailored based on the patient’s clinical profile and the primary valve pathology. In this chapter, we present the surgical options that are currently available for pulmonary valve disorders and the current outcomes.