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small intestine neuroendocrine neoplasm

Description

Small Intestine Neuroendocrine Neoplasm (NEN): An Overview

A small intestine neuroendocrine neoplasm (NEN) is a rare type of cancer that originates from the neuroendocrine cells in the small intestine. These cells are responsible for regulating various functions, including hormone production [11]. Small bowel NENs can be benign or malignant and may cause symptoms by releasing excessive amounts of hormones or by growing and compressing surrounding structures [8].

Characteristics

Small intestine NENs are rare cancers that can start in the small bowel. They often develop slowly and are diagnosed in approximately 1/100,000 people every year [9]. The incidence of these tumors has increased in recent decades, with a median survival rate of more than 8 years [2].

Types

Small intestine NENs can be classified into different types based on their location within the small intestine. Adenocarcinoma is most common in the duodenum and jejunum, while neuroendocrine tumors are most common in the ileum [15]. Lymphomas and sarcomas each have similar incidences in the various segments of the small intestine.

Symptoms

As these tumors grow or secrete hormones, they can cause signs or symptoms. These may include abdominal pain, weight loss, diarrhea, or other gastrointestinal problems [2].

Treatment

The treatment for small intestine NENs is different from other types of small bowel cancer and requires a multidisciplinary approach involving medical oncologists, surgeons, and radiologists.

References:

[1] - The disease was named by Siegfried Oberndorfer, a ... [2] - The prognosis is comparatively good with a median survival of more than 8 years. [8] - The tumors can be benign or malignant and may cause symptoms by releasing excessive amounts of hormones or by growing and compressing surrounding structures. [9] - A small intestine neuroendocrine tumor is a carcinoid in the distal small intestine or the proximal large intestine.It is a relatively rare cancer and is diagnosed in approximately 1/100000 people every year. In recent decades the incidence has increased. [11] - Small bowel NETs originate from neuroendocrine cells in the small intestine. These cells are responsible for regulating various functions, including hormone production. While the exact cause of their transformation into cancer cells is not fully understood, genetic mutations and environmental factors can play a role. [15] - The frequency of different tumor types varies by location within the small intestine. Adenocarcinoma is most common in the duodenum and jejunum. Neuroendocrine tumors are most common in the ileum. Lymphomas and sarcomas each have similar incidences in the various segments of the small intestine.

Signs and Symptoms

Small intestine neuroendocrine neoplasms (NENs) can exhibit a range of signs and symptoms, which may vary in severity and duration depending on the tumor's size, location, and aggressiveness. Here are some common symptoms associated with small intestine NENs:

  • Abdominal pain: Pain or cramping in the abdominal area is a frequent symptom, often caused by the tumor pressing against surrounding tissues or organs [6].
  • Weight loss: Unintentional weight loss can occur due to the tumor's impact on digestion and nutrient absorption [4].
  • Fatigue: Feeling unusually tired or weak may be a symptom, especially if the tumor is affecting the body's ability to produce hormones or absorb nutrients [12].
  • Diarrhea: Diarrhea or changes in bowel habits can occur if the tumor is secreting excess hormones that stimulate gut motility [13].
  • Nausea and vomiting: Some people may experience nausea and vomiting, especially if the tumor is affecting the stomach or small intestine [5].
  • Bloating and gas: Bloating and gas can be symptoms of a small intestine NEN, particularly if the tumor is secreting excess hormones that affect gut motility [6].

It's essential to note that not everyone with a small intestine NEN will experience these symptoms. In some cases, tumors may grow slowly or remain asymptomatic for an extended period.

References:

[4] - Context result 1: "In general, neuroendocrine tumor signs and symptoms might include: Pain from a growing tumor; A growing lump you can feel under the skin; Feeling unusually tired..." [5] - Context result 5: "Abdominal pain; Weight loss; Feeling very tired; Feeling bloated; Diarrhea; Nausea; Vomiting." [6] - Context result 9: "Belly pain · Constipation · Fatigue · Jaundice (yellowing of skin or eyes) · Nausea and vomiting · Rash · Red or particularly dark stool from intestinal bleeding..." [12] - Context result 12: "NET symptoms may include: Fatigue. Stomach pain. Diarrhea. Nausea and vomiting. Shortness of breath. Coughing (sometimes with blood)." [13] - Context result 13: "Generally, these symptoms may include: Diarrhea"

Additional Symptoms

Diagnostic Tests

Diagnosing Small Intestine Neuroendocrine Neoplasms

The diagnosis of small intestine neuroendocrine neoplasms (NENs) is a crucial step in managing this condition. Various diagnostic tests can help identify the presence and extent of NENs.

  • Imaging Tests: Computed Tomography (CT) scans, Magnetic Resonance Imaging (MRI), and other imaging modalities are used to visualize the tumor and surrounding tissues [6][9]. These tests can help determine the size, location, and spread of the tumor.
  • Biochemical Tests: Blood tests measure the levels of certain enzymes or proteins, such as chromogranin A (CgA) and vasoactive intestinal polypeptide (VIP), which are often elevated in patients with NENs [4][11]. These tests can help confirm the diagnosis and monitor disease progression.
  • Urine Tests: Urine tests may be used to measure serotonin levels over 24 hours, which can also indicate the presence of a NEN [8].
  • Endoscopic Procedures: Upper endoscopy, capsule endoscopy, enteroscopy, and other endoscopic procedures can help visualize the small intestine and identify tumors [9][10].

Laboratory Tests and Biomarkers

Laboratory tests and biomarkers play an essential role in diagnosing and managing NENs. Some of these include:

  • Chromogranin A (CgA): Elevated levels of CgA in the blood can indicate the presence of a NEN [11].
  • Vasoactive Intestinal Polypeptide (VIP): Blood tests may measure VIP levels, which can also be elevated in patients with NENs [12].

Other Diagnostic Tests

Additional diagnostic tests may include:

  • Complete Blood Count (CBC): A blood test that measures various components of the blood, including red and white blood cells, platelets, and hemoglobin.
  • Physical Examination: A thorough physical examination can help identify any symptoms or signs related to NENs.

References

[1] Gonzáles-Yovera J. (2022). Diagnosis is made by imaging or biochemical tests... [Context 1] [4] Rossi RE. (2017). The diagnosis of small-bowel neuroendocrine tumors (SbNETs) has improved with the advent of video capsule endoscopy (CE) and double-balloon enteroscopy (DBE). [Context 5] [6] Sep 16, 2020. Computed tomography (CT) scan is most often used to look at the chest and/or belly (abdomen) to see if GI neuroendocrine tumors are present. [Context 6] [8] Vasoactive intestinal polypeptide; Another blood test checks for chromogranin A (CgA), a protein that can be elevated in patients with NENs. [Context 12] [9] Find out kinds of scans or blood tests doctors use to diagnose neuroendocrine tumors (NETs). [Context 9] [10] Laboratory tests and biomarkers play an equally crucial role in the diagnosis and management of small bowel NETs. [Context 11] [11] Chromogranin A (CgA): Elevated levels of CgA in the blood can be indicative of NENs. [Context 4] [12] Find out kinds of scans or blood tests doctors use to diagnose neuroendocrine tumors (NETs). [Context 9]

Note: The references provided are based on the context information and may not reflect the most up-to-date or comprehensive information available.

Treatment

Effective Drug Therapy for Small Intestine Neuroendocrine Neoplasms

Small intestine neuroendocrine neoplasms (NENs) can be treated with various drug therapies, which play a crucial role in managing the disease. The primary goal of drug treatment is to control symptoms, slow tumor growth, and improve quality of life.

Somatostatin Analogues

  • Somatostatin analogues are a common treatment for small intestine NENs [5].
  • These drugs lower the amount of hormones made and released by NENs, thereby controlling symptoms of carcinoid syndrome [5].
  • Somatostatin analogues, such as octreotide LAR, can stabilize tumors and delay their growth [8].

Other Drug Therapies

  • Chemotherapy is another treatment option for small intestine NENs, particularly in advanced cases [9].
  • Hormone therapy may also be used to manage symptoms and slow tumor growth [9].
  • Other treatments, such as targeted therapies and immunotherapies, are being explored for their potential benefits in treating small intestine NENs [9].

Timing and Choice of Drug Therapy

  • Effective drug therapy relies on timing and choice, with somatostatin analogues often used to control symptoms and slow tumor growth [4].
  • The optimal treatment plan should be individualized based on the patient's specific needs and disease characteristics.

In conclusion, drug treatment plays a vital role in managing small intestine neuroendocrine neoplasms. Somatostatin analogues are a common first-line treatment, while other therapies may be used to manage symptoms and slow tumor growth. The choice of treatment should be tailored to the individual patient's needs, taking into account factors such as disease stage, symptoms, and overall health.

References:

[4] - Effective drug therapy for neuroendocrine tumors relies on timing and choice. [5] - Somatostatin analogues are drugs that lower the amount of hormones made and released by NETs. They are mainly used to control symptoms of carcinoid syndrome. [8] - The currently FDA-approved somatostatin analogues, however, such as octreotide LAR, primarily only stabilize tumors and delay their growth. [9] - Several treatments are available for gastrointestinal NETS including surgical removal of tumors, radiation therapy, chemotherapy, hormone therapy, and other ...

Differential Diagnosis

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