AACE/ACE disease state clinical review: diagnosis and management of midgut carcinoids

Abstract

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.

Fig. 1

Example of large calcified matted mesenteric metastasis from a midgut carcinoid causing venous and lymphatic congestion.

Fig. 2

Comparison of the same patient with metastatic midgut carcinoid in the dome of the liver with different cross-sectional imaging techniques. (A) Computed tomography scan without contrast does not show lesions. (B) Computed tomography scan with arterial contrast showing some lesions. (C) Magnetic resonance imaging with Eovist showing multiple lesions more clearly.

Fig. 3

Comparison of patient with metastatic midgut carcinoid imaged with (A) Octreoscan and (B) ⁶⁸Ga-DOTATATE PET.