Octreotide

Synthetic somatostatin analogs ameliorate endothelial injury due to hydrochloric acid treatment.

Saikat Fakir, Khadeja-Tul Kubra, Madan Sigdel +2 more

Endothelial barrier dysfunction is a key feature of lung injury induced by hydrogen chloride gas, which is converted to HCl in tissues. Synthetic somatostatin analogs (SSA), such as Lanreotide and Octreotide, are prescribed in clinics for acromegaly and neuroendocrine tumor treatment. Recent findings suggest that those FDA-approved drugs can ameliorate inflammatory lung disease. This study aims to assess the potential protective effects of SSA against HCl-induced endothelial injury in vitro, utilizing bovine pulmonary artery endothelial cells. Our observations suggest that HCl exposure impairs endothelial cell viability and proliferation, increases paracellular and transendothelial permeability, and induces reactive oxygen species generation, as expected. Octreotide and Lanreotide treatment alleviate the aforementioned toxic effects and exert anti-inflammatory effects in the impaired endothelial cells. Those data inform us on the potential protective effects of SSA in HCl-induced endothelial injury and inflammation, suggesting that they could be repurposed to ameliorate respiratory complications due to toxicant exposure.

Real-World Observational Study of Somatostatin Analogs and Rescue Medication Usage for Neuroendocrine Tumors in Canada.

Daniel Rayson, Myriam Troesch, Callahan LaForty +2 more

Neuroendocrine tumors (NETs) are uncommon malignancies with increasing incidence in Canada. Long-acting somatostatin analogs (SSAs) such as Lanreotide Autogel (LAN-ATG) and Octreotide Long-Acting Release (OCT-LAR) are first-line treatments for well-differentiated GEP-NETs either with or without carcinoid syndrome (CS). Despite their efficacy, many patients require short-acting SSAs for breakthrough symptoms. We investigated the impact of switching between long-acting SSAs on the usage of breakthrough medications and the incidence of above maximum recommended dose (AMRD) prescriptions.

Recurrent ventricular asystole during octreotide infusion in a patient with pancreatic neuroendocrine tumor: a case report.

Nicholaos Mansolas, Ian Nesbitt

Somatostatin analogs such as octreotide are frequently used in the prevention of carcinoid syndrome in the perioperative period following neuroendocrine tumor resection. This is the first reported case of ventricular asystole associated with postoperative intravenous octreotide infusion in a patient with previous congenital cardiac surgery.

Nonmetastatic Pulmonary Carcinoid Presenting With Carcinoid Syndrome Despite Negative 5-HIAA: A Case Report.

Aditya Chauhan, Muhammed Kizilgul, Emilian Racila +1 more

Carcinoid syndrome from pulmonary carcinoids without hepatic metastases is rare and diagnostically challenging when biochemical markers are normal.

The impact of radioligand therapy on prognosis in patients with lung neuroendocrine tumors.

Katarzyna Jóźwik-Plebanek, Marek Saracyn, Adam Daniel Durma +7 more

Evidence on the efficacy and safety of radioligand therapy (RLT) in lung neuroendocrine tumors (LNETs) remains scarce. The limited data available, derived mainly from retrospective analyses are based on small patient cohorts and heterogeneous treatment protocols. The objective of this study was to assess the efficacy and safety of RLT in patients with SSTR-positive LNETs treated with either [¹77Lu]Lu-DOTA-TATE or tandem therapy with [90Y]Y-DOTA-TATE/[¹77Lu]Lu-DOTA-TATE at Polish ENETS Center of Excellence.

Lugol's solution for preoperative management of a TSH/GH-secreting pituitary adenoma with suboptimal response to octreotide: a case report.

Guiliang Peng, Xiaotian Lei, Weiling Leng +4 more

Thyroid-stimulating hormone pituitary adenomas (TSHomas) are a rare cause of central hyperthyroidism, characterized by abnormally high TSH levels, and typically respond to somatostatin analogue (SSA). We report a young patient with SSA-insensitive TSHoma where Lugol's solution facilitated surgical preparation.

Transcatheter Valve Replacement in Carcinoid Heart Disease: A Potential Change of Paradigm.

Marie-Anne Barbier, Laura Gerard, Daniel Grinberg +4 more

To evaluate the feasibility and early clinical outcomes of transcatheter valve replacement in high-surgical-risk patients with carcinoid heart disease.

One-Stop Transcatheter Pulmonary and Tricuspid Valve Replacement for Carcinoid Heart Disease Treatment.

Nicolas Veas, Fernando J Verdugo, Jaime Álvarez +5 more

Carcinoid heart disease is a complex consequence of functional neuroendocrine tumors, characterized by progressive thickening and degeneration of the right-sided valvular apparatus, leading to valvular and heart failure. Management is challenging, requiring multidisciplinary considerations regarding tumor disease management, heart failure pharmacotherapy, and heart valve replacement in individuals with acceptable performance status.

Identification of Pancreatic Neuroendocrine Tumor During Evaluation for Severe Valvulopathy in a Patient With a History of Lung Carcinoid Tumor: A Case Report.

Zhan Rong, Cassie Liu, Christopher Loh +4 more

A 36-year-old man with a history of resected typical lung carcinoid tumor and bicuspid aortic valve presented with acute decompensated heart failure. Workup revealed severe mitral valvulopathy and incidentally identified an enhancing pancreatic head mass with additional lesions on imaging. Liver biopsy was benign, while endoscopic ultrasound-guided biopsy of the pancreatic mass showed a well-differentiated, WHO grade 1 pancreatic neuroendocrine tumor (NET) positive for chromogranin, synaptophysin, and CD56, with Ki-67 < 3%. Serum 5-hydroxyindoleacetic acid (5-HIAA) and chromogranin A were elevated. Positron emission tomography (PET)-DOTATATE demonstrated somatostatin receptor-avid lesions in the pancreas, liver, bone, and lymph nodes, consistent with metastatic neuroendocrine neoplasm. The patient underwent aortic valve replacement followed by long-acting octreotide therapy. Concurrent duodenal biopsies revealed celiac disease, and his gastrointestinal symptoms improved with a gluten-free diet. This case represents an exceptionally rare scenario of pancreatic neuroendocrine tumor in a patient with prior lung carcinoid tumor and raises the critical question of pancreatic metastasis versus a second primary NET in the absence of hereditary syndromes. It underscores the need for heightened vigilance for second primary malignancies (SPMs) and atypical metastatic patterns in patients with a history of NETs and highlights the importance of multidisciplinary evaluation for accurate classification and optimal management.

Exploring the carcinoid crisis: insights from a cancer-specific centre.

Maribel Del Olmo-García, Grace Kong, HuiLi Wong +5 more

To assess the prevalence, pre-procedure biomarkers, and management of carcinoid crisis (CC) in a cancer-specific hospital.

Concurrent head and neck paragangliomas and pulmonary carcinoid tumor: A rare clinical entity.

Faria Nisar, Hoo Jung Rhim, Roman Finocchiaro +1 more

We report the first documented case of a 61-year-old female presenting with concurrent multiple head and neck paragangliomas (HNPGLs) and a pulmonary carcinoid tumor, representing an unprecedented association of synchronous neuroendocrine tumors (NETs). This rare coexistence presented unique diagnostic challenges requiring multimodal imaging, including FDG PET-CT, contrast-enhanced CT, MRI, and Cu-64 DOTATATE PET-CT for comprehensive tumor characterization. Management employed a tailored multidisciplinary approach: surgical resection of the dominant carotid body tumor with excellent local control, stereotactic radiation therapy for remaining cervical lesions (45 Gy), and active surveillance with octreotide therapy for the pulmonary carcinoid. At 24-month follow-up, all lesions remained stable with no evidence of progression or new neurological deficits. Despite declining genetic testing, the patient's multiple paragangliomas strongly suggest hereditary predisposition, emphasizing the critical importance of genetic counseling in such presentations. The successful management approach provides a framework for similar complex presentations and highlights the potential for shared molecular mechanisms underlying synchronous neuroendocrine tumorigenesis.

Octreotide ameliorates Bisphenol A-induced testicular toxicity via autophagy-inflammation pathway modulation.

Basma B Morad, Ola M Salem, Rasha Osama El-Esawy +1 more

IntroductionTesticular toxicity commonly manifests as impaired spermatogenesis and testicular atrophy. Bisphenol A (BPA), a commonly used organic plasticizer, negatively affects sperm parameters, hormonal levels, and fertility. Octreotide (OCT), a somatostatin analog, was originally used to treat acromegaly, carcinoid tumors, vasoactive intestinal peptide-secreting, and growth hormone-secreting tumors. OCT has demonstrated potential therapeutic properties beyond its traditional use in endocrine disorders. We hypothesized that OCT would attenuate BPA-induced testicular damage through its anti-inflammatory, anti-oxidant, and anti-autophagic properties. This study aimed to assess the mechanisms of BPA-induced testicular toxicity and evaluate the ameliorative effects of OCT.MethodsForty adult male Sprague Dawley (SD) rats were randomly assigned to four equal groups: group1: saline control, group2: dimethyl sulfoxide (DMSO) vehicle, group3: BPA-treated, and group4: BPA + OCT-treated. Treatments were administered for 4 weeks. Sperm count, testicular weight, serum testosterone, lactate dehydrogenase (LDH), alkaline phosphatase (ALP), total antioxidant capacity (TAC), testicular levels of malondialdehyde (MDA), tumor necrosis factor-alpha (TNFα), Beclin-1 (BECN-1), Microtubule-associated proteins 1A/1B light chain 3A (MAP1LC3A/LC3), mammalian target of rapamycin (M-TOR), histopathological examination, apoptotic index, and Johnson's score were assessed.ResultsBPA administration significantly impaired spermatogenesis, reduced serum testosterone and TAC, and increased MDA, TNFα, and autophagy-related markers, along with histopathological damage of testis. Co-treatment with OCT mitigated these effects by improving sperm parameters, hormone levels, oxidative stress markers, inflammatory cytokines, and testicular histology.DiscussionThe findings suggest that OCT exerts a protective effect against BPA-induced testicular toxicity, through its anti-inflammatory, antioxidant, and autophagy-modulating properties. OCT may offer therapeutic potential in mitigating BPA-induced testicular toxicity.

Medical treatment in acromegaly: a network meta-analysis.

Chrysi Kaparounaki, Mirela-Diana Ilie, Dario De Alcubierre +6 more

Acromegaly is a rare disorder caused by a growth hormone-secreting pituitary adenoma. Clinical trial evidence for its management is limited. This study compared medical treatments for acromegaly through a network meta-analysis, assessing biochemical and radiological responses.

Paltusotine: The first selective nonpeptide agonist of somatostatin receptor 2 (SSTR2) for the treatment of acromegaly.

Yueyi Sun, Daoran Lu, Jianjun Gao

Acromegaly is an endocrine disorder characterized by abnormal enlargement of the extremities and internal organs resulting from excessive secretion of growth hormone (GH) by the pituitary gland, which in turn leads to elevated levels of insulin-like growth factor 1 (IGF-1). Approximately 45% of patients remain biochemically uncontrolled after surgery and require long-term treatment with injectable somatostatin analogs such as octreotide or lanreotide. These polypeptide drugs generally require monthly administration to maintain disease control; however, many patients experience recurrence of symptoms towards the end of the dosing interval. Moreover, injection-site pain and local reactions are common, significantly impacting patients' quality of life. On September 25, 2025, the U.S. Food and Drug Administration (FDA) approved paltusotine, the first once-daily oral, nonpeptide somatostatin receptor 2 (SSTR2) agonist for the treatment of acromegaly. By enabling oral rather than injectable therapy, paltusotine reduces the treatment burden and enhances patient adherence. With its rapid onset and durable biochemical control, this novel agent has the potential to reshape the current paradigm of acromegaly pharmacotherapy and offer patients a more convenient and effective treatment option. Nevertheless, its long-term safety and efficacy warrant further evaluation in real-world clinical settings.

Posology for 177Lu-DOTATATE Therapy in Neuroendocrine Tumor: Effectiveness of Dose Reduction and Cycle Delay for Improving Therapy Tolerability.

Ivan E Wang, Jasmine Patterson, Azadeh Akhavanallaf +8 more

Approval of 177Lu-DOTATATE for the treatment of neuroendocrine tumors (NETs) requires monitoring for therapeutic efficacy and safety. A retrospective review was conducted on 173 patients who received 177Lu-DOTATATE at the University of Michigan to identify if posology changes would improve therapy tolerance and completion. Patients were followed up to 3 years following their last cycle of treatment to determine long-term adverse events. Dose reduction (from 7.4 GBq to 3.7 GBq) was used in 13 patients, and cycle delay (8±1 wk to >16-week gap) was used in 36 patients, leading to 82% completion of 4 cycles of therapy. Dose reduction led to higher rates for completing all 4 cycles of therapy, whereas cycle delay led to worsened outcomes. Amino acid infusions led to limited adverse events with nausea, vomiting, and fatigue, even with antiemetic prophylaxis. Carcinoid crisis was observed in 1.2% of patients, which was similar to the findings in the NETTER-1 trial. Acute decline in neutrophils and platelets was observed; however, these blood values rebounded. A decline in renal function (4.3 mL/min/1.73 m2 over 4 years compared with untreated patients) suggests a potential long-term risk for renal toxicity; however, it could also be associated with changes in therapy. When adverse events were severe due to underlying disease, dose reduction, or cycle delay did not adequately impact therapy completion, and required further therapy monitoring. Long-term occurrence of myelodysplasia syndrome was 1.8%, comparable to reported occurrences requiring further monitoring. Our analysis reinforces that 177Lu-DOTATATE is an effective, well-tolerated treatment for NETs with low incidences of long-term adverse events.

Hematological Malignancy in a Hypophysectomised Acromegalic Patient Under 4-Year Therapy with Somatostatin Analogues: From a Rib Lump Underlying Bone Plasmatocytoma Features to Multiple Myeloma.

Mihaela Stanciu, Alina Cătană, Ruxandra Paula Ristea +4 more

Acromegaly is associated with a higher risk of certain malignancies, but not hematological neoplasia, although multiple myeloma (MM) was found in very limited cases. We aim to present such a case, adding a particular presentation with co-occurrence of a plasmocytoma. A 52-year-old male with acromegaly confirmed at 46 (MRI: pituitary macroadenoma of 12 × 11 × 10 mm) underwent hypophysectomy followed by 3 years of octreotide LAR then lanreotide depot. After another 6 months, he experienced a rapidly growing, painful lump in the right lateral thoracic area confirmed by CT as a 9-cm osteolytic lesion at the third rib. Core biopsy revealed plasmocytoma of the bone and medullary biopsy confirmed MM. Plasmacytoma was managed with 10 radiotherapy sessions, with favorable outcome and mass resorption; MM was managed with a VRD regimen, followed by autologous hematopoietic stem-cell transplantation. Six months after sFLC normalization and plasmacytoma resorption, complete remission was reported. In the meantime, lanreotide was continued, with complete acromegaly control. To conclude, what started as a rather typical scenario for an otherwise rare condition, as is acromegaly in the general population (but not so rare for endocrinologists), turned into an unexpected and more severe outcome. Noting this exceptional association, we pinpoint that further research is needed for understanding the dual acromegaly-MM relationship.

Paltusotine versus octreotide: different effects on radioligand uptake in neuroendocrine tumours.

Katharina Wang, Christoph J Auernhammer, Simon Lindner +19 more

Somatostatin receptor analogues are well-established in the treatment of metastatic gastro-enteropancreatic neuroendocrine tumours (GEP-NETs), especially for symptom control in patients with the carcinoid syndrome, and to control tumour growth. However, they need to be discontinued before peptide receptor radionuclide therapy (PRRT) as they may saturate the somatostatin receptor 2 (SSTR2) and prevent binding of the radioactive ligand.

Insulin-like growth factor-I and symptoms of acromegaly according to time since somatostatin receptor ligand injection.

Ilan Remba-Shapiro, Júnia R O L Schweizer, Alberto Moscona-Nissan +9 more

Somatostatin receptor ligands (SRLs) are the mainstay of pharmacotherapy for acromegaly. Patients report symptoms toward the end of the injection cycle. However, the correlation between insulin-like growth factor-I (IGF-I) and patient-reported outcomes (PROMs) has not been evaluated in this context.

Ectopic Cushing's syndrome in a patient with DIPNECH and metastatic lung carcinoid.

Sara Ribeiro, Telma Moreno, Maria Lume +7 more

Diffuse idiopathic pulmonary neuroendocrine cell hyperplasia (DIPNECH) is a rare precursor to lung carcinoids. We report a case of ACTH-dependent Cushing's syndrome in a 73-year-old female patient with metastatic lung carcinoid arising on a background of DIPNECH. She presented with lower limb oedema, hypokalaemia, hypertension, and de novo diabetes. Clinical suspicion for hypercortisolism was confirmed by abnormal cortisol tests. A thoracic CT scan showed multiple lung nodules suggestive of DIPNECH and biopsy of one of the nodules identified an ACTH-expressing carcinoid tumour. A PET-Ga-68-DOTATOC revealed pulmonary and multiple tumour lesions in the ganglia, bone and liver with overexpression of somatostatin receptors. A liver biopsy demonstrated involvement by a well-differentiated neuroendocrine neoplasia, consistent with metastasis. Hypercortisolism was managed with octreotide and metyrapone, but the patient succumbed to complications 14 months post-diagnosis. This case suggests DIPNECH's potential to progress to hyperfunctioning, metastatic carcinoids and highlights the necessity for vigilant long-term surveillance and early intervention.

Surgical treatment and somatostatin experience in growth hormone-secreting pituitary macroadenoma due to novel AIP mutation.

Kıymet Karagöz, Emine Şeyma Eken, Gülin Karacan Küçükali +9 more

Somatotropinomas are extremely rare in children and frequently associated with genetic causes. Among pituitary gigantism, approximately 30 % are attributed to the aryl hydrocarbon receptor-interacting protein (AIP) gene mutations, whereas other genetic causes are less common. These mutations cause more aggressive tumors that are challenging to control with a single intervention and often exhibit resistance to somatostatin analogs (SSAs). Our aim is to present a pediatric patient with a somatotropinoma due to a novel AIP variant who responded positively to SSA therapy following a single surgical intervention.

A Case of Acromegaly With Parasellar Meningioma With Oculomotor Palsy Responding to and Maintained With Oral Octreotide Therapy.

Muhammed Kizilgul, Ammar Ahmed, Lindsey Sloan +3 more

Meningiomas are the most frequently occurring primary central nervous system tumors. Recommended treatment options for large, growing, or symptomatic meningiomas typically involve surgical removal (when possible) and/or radiation therapy. There is no sufficient evidence of effective systemic therapies for recurrent meningioma.

Dual PET Imaging with [68Ga]Ga-DOTA-TOC and [18F]FDG to Localize Neuroendocrine Tumors of Unknown Origin.

Ali Zaidi, Pavithraa Ravi, Ingrid Bloise +8 more

Neuroendocrine tumors of unknown primary (CUP-NET) present a diagnostic challenge when conventional imaging fails to localize the primary tumor. This study aimed to evaluate the diagnostic value of concurrent [68Ga]Ga-DOTA-TOC and [18F]FDG PET/CT imaging in localizing primary tumors in patients with histologically confirmed CUP-NET. Thirty-four patients underwent both imaging modalities as part of a prospective imaging protocol after negative conventional imaging or [111In]In-octreotide scintigraphy. Primary tumor detection rates were assessed, and imaging characteristics compared between the two modalities. The overall localization rate was 58.9% (20/34). Of these, 90% (18/20) of primary tumors were identified solely by [68Ga]Ga-DOTA-TOC PET/CT, with the remaining two visualized by both modalities. [18F]FDG PET/CT did not independently localize any primary tumors. Identified primaries were limited to grade 1 (60%) or grade 2 (40%) tumors, predominantly in the small intestine (95%). Among localized cases, 45% (9/20) underwent surgical resection and 15% (3/20) became eligible for peptide receptor radionuclide therapy. [68Ga]Ga-DOTA-TOC PET/CT demonstrated superior detection of metastatic lesions compared to [18F]FDG PET/CT (97.1% vs. 70.6%, p = 0.006). No significant survival differences were observed between patients with localized versus non-localized primaries. These findings support the value of [68Ga]Ga-DOTA-TOC PET/CT for identifying primary tumors in CUP-NET. Further research is warranted to explore the role of [18F]FDG PET/CT in high-grade NETs.

Real-world drug safety study of somatostatin analogs based on the food and drug administration adverse event reporting system database.

Wangyu Ye, Zhen Liu

Somatostatin analogs (SSAs), synthetic peptides mimicking endogenous somatostatin, are widely used to treat neuroendocrine tumors and acromegaly. However, their use is often accompanied by adverse events (AEs) that may compromise patient safety. This study systematically evaluated AE signals associated with SSAs using the FDA Adverse Event Reporting System (FAERS) database to inform clinical practice.

A Rare Case of Neuroendocrine Tumor Presenting as Isolated Scalp Swelling With Paroxysmal Symptoms.

Vivek Kumar Dubey, Kush Pathak, Kavita Sijwali

We report a 36-year-old male with a nine-year history of recurrent left frontal scalp swelling following minor trauma, associated with local warmth, sweating, and a syncopal episode during fine-needle aspiration cytology (FNAC). Histopathology confirmed grade 2 neuroendocrine tumor (NET) with a Ki-67 index of 8-10%. 68Ga-DOTANOC positron emission tomography and computed tomography (PET-CT) showed no evidence of residual, recurrent, or primary somatostatin receptor-expressing disease, confirming this as a rare case of isolated scalp NET without an identifiable primary source. This is, to our knowledge, the first reported case of its kind. The patient's syncope post-FNAC highlights the risk of carcinoid-like crisis from lesion manipulation. This case underscores the importance of a high index of suspicion, thorough immunohistochemical workup, and consideration of peri-procedural octreotide prophylaxis.

Adherence, duration and healthcare costs in a real-world population of patients with acromegaly.

Tiffany P Quock, Eunice Chang, Ashis K Das +5 more

Aim: The aim of this study was to describe treatment patterns among patients with acromegaly who are newly treated with acromegaly medical therapy. Materials & methods: Data from IQVIA Pharmetrics Plus® Database from 1 January 2013 to 30 June 2023 were used to identify patients with acromegaly who started a new acromegaly medical therapy and observe their treatment patterns. Patients were required to have at least 12 months of data without any acromegaly therapy (medication or surgery) prior to the index date and at least 6 months of follow-up. Comorbidities were measured during the baseline period. Adherence, persistence, medication and switching were measured during follow-up. Results: A total of 453 patients with acromegaly who were newly treated with acromegaly medical therapy and had no evidence of acromegaly therapy for at least 12 months were identified. Among these patients, 46.1% (n = 206) were treated with cabergoline as their index treatment, 24.5% (n = 111) with injectable octreotide, 15.0% (n = 68) with lanreotide, 5.5% (n = 25) with bromocriptine, 4.9% (n = 22) with pegvisomant, 2.2% (n = 10) with pasireotide, 1.1% (n = 5) with oral octreotide, 0.4% (n = 2) with cabergoline + octreotide, and 0.2% (n = 1) with cabergoline + lanreotide. By the end of the follow-up period, 54.3% (n = 246) were not on any treatment, 19.6% (n = 89) remained on the index treatment, and the remaining 26.0% (n = 118) switched to another treatment. Conclusion: This study contributed to the growing evidence that patients with acromegaly are not well-served by current therapeutic options, as indicated by high rates of treatment discontinuation, switching and add-on therapy. However, treatment switching and add-on therapy represent ongoing efforts to optimize patient care toward more effective and tolerable treatments. Expanded treatment options may serve an unmet need in this patient population.

Availability and characteristics of clinical trials for neuroendocrine tumors: Is there an unmet need?

Philipp Melhorn, Amirhossein Sadeghi, Markus Raderer +1 more

Therapeutic advances in neuroendocrine tumors (NET) have been incremental over recent decades. Similar to other rare diseases, the number of trials that can be offered to NET patients is generally limited.

A Royal Flush: Carcinoid Heart Disease Complicated by Severe Tricuspid and Pulmonic Valve Regurgitation.

Syed Rafay H Zaidi, Nina Appareddy, Wallacy Garcia +4 more

Carcinoid heart disease (CHD) is a severe complication of metastatic neuroendocrine tumors (NETs), leading to fibrotic degeneration of right-sided heart valves. A 38-year-old male presented with progressive dyspnea, fatigue, abdominal bloating, diarrhea, and facial flushing. Imaging revealed hepatic metastases and mesenteric lymphadenopathy, and biochemical markers confirmed a NET of likely gastrointestinal origin. Echocardiography showed torrential tricuspid regurgitation, severe pulmonary insufficiency, right ventricular dilation, and a patent foramen ovale (PFO). The patient was started on long-acting lanreotide for carcinoid syndrome and optimized on heart failure therapy. Due to severe valvular dysfunction, he underwent tricuspid and pulmonic valve replacement with bioprosthetic valves and PFO closure under perioperative octreotide infusion. The patient also underwent transthoracic liver debulking and ablation, small bowel resection, and mesenteric dissection. Postoperatively, he showed symptomatic improvement and remains under multidisciplinary surveillance. This case highlights the importance of early recognition, multidisciplinary management, and surgical intervention in CHD to optimize outcomes. Early initiation of somatostatin analog therapy, guideline-directed medical therapy for heart failure, and timely surgical intervention can significantly improve symptom burden and survival.

Diagnostic Dilemma of a Neuroendocrine Tumour Complicated by Simultaneous Retroperitoneal Fibrosis and Carcinoid Heart Disease in a Perimenopausal Woman.

Mohammad Adjmal Rummun, Nosamudiana Obazee, Mimi Leung +5 more

Carcinoid tumours are rare, slow-growing neuroendocrine neoplasms that often remain asymptomatic until metastatic spread or the development of carcinoid syndrome. Carcinoid syndrome is characterised by flushing, diarrhoea, and bronchospasm due to the secretion of vasoactive hormones. These tumours commonly arise in the gastrointestinal tract but can also occur in other organs, namely, the lungs, genitourinary tract, and pancreas. Retroperitoneal fibrosis (RPF), a rare inflammatory disease, involves chronic inflammation leading to fibrous scarring and compression of surrounding structures like the ureters and blood vessels. Carcinoid heart disease secondary to fibrous valve thickening can also occur and causes high morbidity and mortality. This case report highlights a 52-year-old woman who developed a rare complication of RPF along with carcinoid heart disease secondary to a carcinoid tumour. Her symptoms, initially misdiagnosed as menopausal, included a four-year history of diarrhoea, uncontrolled hypertension, and flushing. She was admitted with abdominal pain, acute kidney injury, and bilateral hydronephrosis. Imaging and biochemical tests revealed a primary ileocecal carcinoid tumour with hepatic metastases, RPF causing ureteric obstruction, and elevated chromogranin A/B and urinary 5-hydroxyindoleacetic acid (5-HIAA) levels. Cardiac involvement included severe tricuspid regurgitation, pulmonary hypertension, and impaired left ventricular function, consistent with carcinoid heart disease. Management involved nephrostomy placement following failed bilateral ureteric stenting, octreotide infusions to prevent carcinoid crisis, and symptomatic control with lanreotide. The patient continues to receive multidisciplinary care from cardiology, urology, and oncology. This case underscores the complexity of diagnosing and managing carcinoid tumours with atypical presentations and rare complications like RPF.