Ileal Neuroendocrine Tumor: Signs, Causes, And How To Treat

An ileal neuroendocrine tumor is a type of rare cancer that develops in the cells of the small intestine, specifically the ileum. These tumors are part of a group called neuroendocrine tumors, which affect the neuroendocrine system responsible for producing hormones.

The exact cause of ileal neuroendocrine tumors is not fully understood, but factors like genetic mutations or family history may play a role in their development. While these tumors are uncommon, early detection and proper management are crucial for the best possible outcomes. If you have concerns about your health or are experiencing concerning symptoms, it's important to consult with a healthcare provider for a proper evaluation and guidance.

What Are the Symptoms of Ileal Neuroendocrine Tumor

Patients with an ileal neuroendocrine tumor may experience a range of symptoms, including abdominal pain, diarrhea, flushing of the skin, unintended weight loss, fatigue, and sometimes, the development of a palpable mass in the abdomen.

These tumors can also lead to hormonal changes that manifest as symptoms such as wheezing, rapid heartbeat, low blood pressure, or changes in blood sugar levels. It is essential for individuals experiencing these symptoms to consult with a healthcare provider for a proper evaluation and diagnosis.

Abdominal pain is a common symptom of ileal neuroendocrine tumors, often persistent and located in the lower abdomen.
Diarrhea or changes in bowel habits can occur due to the excess hormone production associated with ileal neuroendocrine tumors.
Flushing, characterized by sudden reddening of the face and neck, is a symptom commonly seen in patients with ileal neuroendocrine tumors.
Unexplained weight loss may occur in individuals with ileal neuroendocrine tumors due to metabolic changes and malabsorption.
Fatigue and weakness are frequent symptoms of ileal neuroendocrine tumors, often impacting daily activities and quality of life.

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Causes of Ileal Neuroendocrine Tumor

These tumors originate from the neuroendocrine cells in the ileum, a part of the small intestine. While the exact cause is often unknown, certain risk factors may contribute to their development. These include genetic predisposition, such as in individuals with multiple endocrine neoplasia type 1 (MEN1) syndrome, exposure to certain chemicals or toxins, inflammatory bowel disease, and a history of radiation therapy to the abdomen.

Additionally, abnormalities in certain genes may play a role in the formation of these tumors. Understanding these potential causes can aid in early detection and management of ileal neuroendocrine tumors.

Genetic mutations, such as in MEN1 or MEN2 syndromes, can predispose individuals to develop ileal neuroendocrine tumors.
Exposure to certain carcinogens or environmental toxins may increase the risk of developing ileal neuroendocrine tumors.
Chronic inflammation in the gastrointestinal tract, particularly in the ileum, can contribute to the development of neuroendocrine tumors.
Overgrowth of neuroendocrine cells in the ileum due to unknown factors can lead to the formation of tumors.
Immunodeficiency states or conditions that weaken the immune system may play a role in the development of ileal neuroendocrine tumors.

Types Of Ileal Neuroendocrine Tumor

Ileal neuroendocrine tumors can be categorized into various types based on their behavior and characteristics. These types include well-differentiated neuroendocrine tumors (NETs), which are slow-growing and often less aggressive, and poorly differentiated neuroendocrine carcinomas (NECs), which are more aggressive and have a higher tendency to spread.

Additionally, there are subtypes within these categories, such as typical carcinoid tumors, atypical carcinoid tumors, small cell neuroendocrine carcinomas, and large cell neuroendocrine carcinomas. Each type requires specific treatment approaches tailored to its behavior and progression.

Well-differentiated neuroendocrine tumors (NETs) of the ileum are the most common type of ileal neuroendocrine tumor.
Carcinoid tumors, a subtype of NETs, typically originate in the ileum.
Ileal neuroendocrine carcinomas are aggressive tumors with a high potential for metastasis.
Functioning ileal neuroendocrine tumors can produce hormones that lead to specific symptoms.
Non-functioning ileal neuroendocrine tumors do not produce hormones but can still cause symptoms due to their size or location.
Mixed neuroendocrine-non-neuroendocrine neoplasms (MiNENs) are rare tumors that contain both neuroendocrine and non-neuroendocrine components in

Risk Factors

Ileal neuroendocrine tumors can develop due to various risk factors. These tumors are more commonly seen in individuals with certain genetic syndromes, such as multiple endocrine neoplasia type 1 (MEN1) and neurofibromatosis type 1 (NF1). Additionally, a family history of neuroendocrine tumors, particularly in the small intestine, can increase the risk of developing these tumors.

Other potential risk factors include older age, exposure to certain chemicals or radiation, and inflammatory bowel diseases. It is crucial for individuals with these risk factors to undergo regular screening and monitoring to detect any potential tumors at an early stage.

Family history of neuroendocrine tumors increases the risk of developing an ileal neuroendocrine tumor.
Certain genetic syndromes, such as multiple endocrine neoplasia type 1 (MEN1), can predispose individuals to ileal neuroendocrine tumors.
Chronic inflammatory conditions affecting the gastrointestinal tract may elevate the likelihood of developing an ileal neuroendocrine tumor.
Exposure to certain environmental toxins or radiation may be a risk factor for the development of ileal neuroendocrine tumors.
Older age is associated with an increased risk of developing ileal neuroendocrine tumors.

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Diagnosis of Ileal Neuroendocrine Tumor

Initially, a comprehensive medical history is taken, followed by a physical examination. Imaging studies such as CT scans, MRI, and somatostatin receptor scintigraphy can help locate the tumor. Blood tests to measure specific markers like chromogranin A levels are also utilized. Additionally, a tissue biopsy may be necessary for a definitive diagnosis.

Collaborating with specialists like endocrinologists, gastroenterologists, and oncologists is essential throughout the diagnostic process to ensure a thorough evaluation and accurate diagnosis of an ileal neuroendocrine tumor.

Imaging tests such as CT scans and MRIs can help identify Ileal neuroendocrine tumors.
Blood tests to check hormone levels and tumor markers may aid in the diagnosis.
Endoscopic procedures like upper endoscopy or colonoscopy can visualize the tumor directly.
Biopsy of the tumor tissue is crucial for confirming the presence of an Ileal neuroendocrine tumor.
Genetic testing may be recommended to assess the genetic makeup of the tumor for personalized treatment strategies.

Treatment for Ileal Neuroendocrine Tumor

Treatment options for ileal neuroendocrine tumors typically involve a multidisciplinary approach tailored to the individual patient's case. Surgery is often the primary treatment to remove the tumor and surrounding tissue.

Additional treatments may include somatostatin analogs to control hormone secretion, targeted therapies like everolimus or sunitinib to inhibit tumor growth, and peptide receptor radionuclide therapy (PRRT) for advanced cases. Chemotherapy may be used in cases where the tumor is aggressive or has spread to other parts of the body.

Close monitoring and follow-up care are essential to track the tumor's response to treatment and manage any side effects.

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