Intestinal neuroendocrine tumors (NETs) are rare, slow-growing neoplasms arising from enterochromaffin cells, capable of secreting vasoactive substances that may cause carcinoid syndrome (CS) and clinically significant valvular heart disease. These tumors are often diagnosed at advanced stages due to nonspecific gastrointestinal symptoms, and distant metastases, particularly to the liver and lymph nodes, are common at presentation. Hormonal dysregulation can lead to chronic diarrhea, flushing, and bronchospasm, while carcinoid heart disease (CHD) contributes substantially to morbidity and mortality, typically affecting right-sided valves, with left-sided involvement being uncommon and more frequently associated with intracardiac shunts than exceptionally high serotonin exposure. Recent advances in management emphasize a multidisciplinary approach integrating systemic therapy, surgery, and targeted radionuclide treatment. Somatostatin analog therapy remains the cornerstone for controlling hormonal symptoms and slowing tumor progression. Aggressive cytoreductive surgery to achieve hormonal stabilization prior to surgical valve replacement has been associated with improved survival, symptomatic relief, and cardiac function. Targeted peptide receptor radionuclide therapy provides additional treatment for residual or metastatic disease, enhancing biochemical and radiological control. We report the case of a 61-year-old woman with chronic diarrhea, weight loss, and recurrent flushing, diagnosed with a well-differentiated ileal NET with extensive hepatic metastases and severe right-sided valvular disease with mild left-sided involvement. She underwent somatostatin analog therapy to control hormonal symptoms, cytoreductive surgery, and surgical replacement of the pulmonary and tricuspid valves. Subsequent targeted peptide receptor radionuclide therapy further reduced tumor burden and stabilized biochemical markers. This combined multimodal strategy resulted in sustained clinical improvement, normalization of neuroendocrine markers, and long-term oncologic stability, as the patient remains asymptomatic and oncologically stable after four years of follow-up from initial presentation. This case highlights the importance of early recognition, comprehensive evaluation, and a multidisciplinary strategy in advanced NETs. Coordinated care integrating endocrinology, oncology, cardiology, nuclear medicine, and surgery can significantly improve survival, hormonal control, and quality of life in patients with complex metastatic disease.

Port site metastasis is an uncommon but clinically relevant complication, particularly associated with minimally invasive surgery for abdomino-pelvic malignancies, often indicating poor prognosis and necessitating prompt evaluation for potential surgical intervention. Proposed mechanisms include direct tumor implantation, aerosolization during pneumoperitoneum, surgical wound contamination, and immune alterations. In this report, we describe a case of a 48-year-old man who developed SSTR-expressing port site metastases, occurring 4 years and 4 months following laparoscopic resection of an ileal neuroendocrine tumor (NET). This case highlights the importance of long-term SSTR-based PET imaging surveillance in NET patients following minimally invasive surgery, even in the absence of symptoms or early biochemical relapse.

We aim to describe a patient diagnosed with a rare and aggressive metastatic pattern of an ileal neuroendocrine tumor (NET). A 53-year-old male patient was referred to our cancer care center following the diagnosis of an ileal NET for further evaluation. As part of the initial diagnostic workup, 68Ga-DOTATOC positron emission tomography/computed tomography was conducted. The scan revealed a fulminant metastatic pattern of somatostatin-positive lesions affecting the axial and peripheral skeleton, liver, and a few lesions involving the brain leptomeninges and right extra-orbital eye muscle. Despite the prompt diagnosis and systematic therapeutic approach offered according to institutional guidelines, the patient did not achieve an optimal clinical response, resulting in his demise 18 months after his initial diagnosis. This case is shared with the primary aim of raising awareness of such an unreported aggressive metastatic pattern and encouraging further clinical investigations for patients with similar aggressive metastatic patterns to achieve optimal therapy and care.

Granular cell tumors are rare neoplasms originating from Schwann cells found in various organs. GCTs are seldom reported in the gastrointestinal tract. Pre-operative detection and diagnosis of colonic GCTs is challenging since the tumors are mainly asymptomatic, small, slow-growing, and submucosal. Most of these tumors are benign in histopathology and behavior. Recently, there has been greater insight into the varying presentations and behaviors of colonic GCTs with atypical histopathologic features. To contribute, we describe a GCT (2.3 cm) at the ileocecal junction found incidentally during follow-up for an excised ileal neuroendocrine tumor in a 65-year-old woman. Our GCT had an unusual focal atypia and infiltrative behavior into the pericolonic adipose tissue without metastasizing to the lymph nodes. These features are important since GCTs have a propensity for local recurrence if incompletely excised, which could have been easily missed. Even though GCTs with atypical features have low rates of recurrence and metastasis, they require close and careful attention in the absence of specific management guidelines due to potential aggressive behavior.

This case report follows a 63-year-old female patient with a history of a gastroenteropancreatic (GEP) neuroendocrine tumor of the terminal ileum who developed ovarian metastasis and later progressed to peritoneal carcinomatosis. The patient was found to have worsening metastasis on CT that was subsequently confirmed with (68Ga)-DOTATATE PET/CT imaging. This case outlines the rare metastatic nature of a primary ileal neuroendocrine tumor and emphasizes the efficacy of (68Ga)-DOTATATE PET/CT imaging in the localization, progression, and treatment of neuroendocrine metastatic disease.