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AACE/ACE disease state clinical review: diagnosis and management of midgut carcinoids
Eric H Liu1, Carmen C Solorzano1, Laurence Katznelson2
1Division of Surgical Oncology and Endocrine Surgery, Vanderbilt University, Nashville, Tennessee.
Objective:
Neuroendocrine tumors (NETs) are a collection of complex tumors that arise from the diffuse endocrine system, primarily from the digestive tract. Carcinoid tumors most commonly originate from the small intestine. These tumors are either referred to as small intestinal neuroendocrine tumors or midgut carcinoids (MGCs). The purpose of this review article is to survey the diagnostic and therapeutic pathways for patients with MGC and provide an overview of the complex multidisciplinary care involved in improving their quality of life, treatment outcomes, and survival.
Methods:
The current literature regarding the diagnosis and management of MGCs was reviewed.
Results:
Dry flushing and secretory diarrhea are the hallmarks of the clinical syndrome of MGC. Managing MGC requires attention to the overall symptom complex, including the physical effects of the tumor and biomarker levels. The somatostatin analogs (SAs) octreotide and lanreotide are highly efficacious for symptomatic improvement. MGCs require resection to encompass the primary tumor and mesenteric lymph node metastases and should include cholecystectomy if the patient is likely to receive SA therapy. Debulking of liver metastasis by resection in combination with ablative therapies and other liver-directed modalities may help palliate symptoms and hormonal overproduction in carefully selected patients. Quality of life is an important measure of patients' perception of the burden of their disease and impact of treatment modalities and may be a useful guide in deciding changes in therapy to alter apparent health status.
Conclusion:
MGC is a challenging malignancy that requires the input of a multidisciplinary team to develop the best treatment plan. Consultation with expert centers that specialize in NETs may also be indicated for complex cases. With expert care, patients can be cured or live with the disease and enjoy good quality of life.
Insights
Midgut carcinoids (MGCs) are challenging neuroendocrine tumors. Effective management involves a multidisciplinary approach, focusing on symptom control, surgical resection, and specialized therapies to improve quality of life and survival.
Area of Science:
- Gastroenterology
- Oncology
- Endocrinology
Background:
- Neuroendocrine tumors (NETs) originate from the diffuse endocrine system.
- Midgut carcinoids (MGCs), a type of NET, commonly arise from the small intestine.
Purpose of the Study:
- To review diagnostic and therapeutic pathways for MGC.
- To provide an overview of multidisciplinary care for MGC patients.
Main Methods:
- Literature review on MGC diagnosis and management.
Main Results:
- Hallmarks of MGC include flushing and secretory diarrhea.
- Somatostatin analogs (octreotide, lanreotide) improve symptoms.
- Resection of primary tumor and lymph nodes, plus cholecystectomy for SA therapy, is indicated.
- Debulking liver metastases can palliate symptoms and hormonal overproduction.
- Quality of life is crucial for guiding therapy changes.
Conclusions:
- MGC management requires a multidisciplinary team and expert center consultation.
- With expert care, MGC patients can achieve cure or live with good quality of life.