MODERN APPROACHES AND RECENT ADVANCES IN NEUROENDOCRINE TUMORS

Modern Approaches and Recent Advances in Neuroendocrine Tumors-kapak

CARCINOID SYNDROME: DIAGNOSIS, TREATMENT AND FOLLOW-UP

Erhan Eröz

Sakarya University Training and Research Hospital, Department of General Surgery, Sakarya, Türkiye

Eröz E. Carcinoid Syndrome: Diagnosis, Treatment and Follow-ıp. In: Gönüllü E, Karaman K, editors. Modern Approaches and Recent Advances in Neuroendocrine Tumors. 1st ed. Ankara: Türkiye Klinikleri; 2025. p.165-173.

ABSTRACT

Carcinoid Syndrome is a clinical condition caused by biologically active substances released by carcinoid tumors. These tumors are most commonly found in the gastrointestinal system, particularly in the small intestine. It is most often associated with midgut neuroendocrine tumors that have metastasized to the liver. Cases of Carcinoid Syndrome have been increasing in recent years, and various treatment options have been developed. Somatostatin analogs can alleviate symptoms in 65-72% of patients, and with higher doses, this effectiveness can rise to 84%. Early diagnosis and treatment are crucial, as complications can reduce three-year survival rates by up to 31%. Clinical Features and Symptoms: Carcinoid Syndrome affects various systems of the body and presents with distinct symptoms. The most common symptoms include flushing and diarrhea. Flushing is typically short-lived and appears on the head, neck, chest, and abdomen. Diarrhea occurs in 60-80% of patients and can happen 4-8 times per day. Other symptoms include shortness of breath, heart valve problems, abdominal pain, and pellagra-like skin changes. Diagnosis: The diagnosis of Carcinoid Syndrome is made through clinical examination, biochemical markers, and disease-specific tests. The most reliable biomarker is Chromogranin A, with 80% sensitivity and 95% specificity. A 24-hour 5-HIAA urine test is considered the gold standard for diagnosis. Imaging methods (CT, MRI, Octreoscan) are also used to locate the tumor. Treatment: Somatostatin analogs are the main treatment for Carcinoid Syndrome. Medications like Octreotide and Lanreotide control symptoms by suppressing hormone production. In addition to standard therapies, drugs such as Telotristat etil and Interferon alpha may also be used. Surgical intervention is important for treating localized tumors, and success rates have improved with minimally invasive techniques. Advanced Treatment Options: New targeted therapies, such as Everolimus and Sunitinib, may effectively control symptoms in patients who do not respond to standard treatments. Peptide receptor radionuclide therapy (PRRT) offers a promising option for advanced-stage tumors. Conclusion: Carcinoid Syndrome can be managed with early diagnosis and appropriate treatment. Somatostatin analogs, targeted therapies, and surgical interventions can improve patients’ quality of life. Regular monitoring is essential for evaluating treatment response and disease progression.

Keywords: Carcinoid tumor; Malignant carcinoid syndrome; Carcinoid tumors, intestinal

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