Gastroenteropancreatic Neuroendocrine

Gastroenteropancreatic Neuroendocrine Understanding their impact is crucial for early detection, proper management, and improved patient outcomes.

Overview of Gastrointestinal System

The gastrointestinal system, also known as the digestive system, plays a vital role in the body’s overall health and well-being. It is responsible for breaking down food, absorbing nutrients, and eliminating waste. Understanding the structure and function of this complex system is crucial in recognizing and managing digestive system disorders that may arise.

Anatomy of the Gastrointestinal System:

The gastrointestinal system comprises several organs, starting from the mouth, esophagus, stomach, small intestine, large intestine, rectum, and ending at the anus. Each organ has a specific function in the digestion process, ensuring the body receives the necessary nutrients for energy and growth.


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Function of the Gastrointestinal System:

The primary function of the gastrointestinal system is to break down food into smaller molecules that the body can absorb and utilize. This process involves mechanical digestion, such as chewing and churning, and chemical digestion, where enzymes and stomach acid break down food into nutrients.

The small intestine, in particular, plays a crucial role in absorbing nutrients from the digested food, while the large intestine is responsible for absorbing water and electrolytes and forming solid waste.

Common Digestive System Disorders:

The gastrointestinal system is susceptible to various disorders that can affect its normal functioning. Some common digestive system disorders include:


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  • Gastroesophageal Reflux Disease (GERD): A condition where stomach acid flows back into the esophagus, causing heartburn and other discomforts.
  • Peptic Ulcers: Open sores that develop on the lining of the stomach or small intestine due to bacterial infection or prolonged use of certain medications.
  • Inflammatory Bowel Disease (IBD): A chronic inflammation of the digestive tract, including conditions like Crohn’s disease and ulcerative colitis.
  • Irritable Bowel Syndrome (IBS): A common disorder that affects the large intestine, causing symptoms like abdominal pain, bloating, and changes in bowel habits.
  • Diverticulitis: Inflammation or infection of small pouches that form in the colon wall, known as diverticula.

These conditions can significantly impact an individual’s quality of life and may require medical management, dietary modifications, and lifestyle changes.

Gastrointestinal System Disorders and Gastroenteropancreatic Neuroendocrine Tumors:

Understanding the normal functioning of the gastrointestinal system is essential in comprehending how gastroenteropancreatic neuroendocrine tumors (NETs) can affect this system. NETs are rare tumors that originate in the neuroendocrine cells of the digestive system and can disrupt its normal functioning.

Stay tuned for the next section, where we’ll explore the role of intestinal hormones and their connection to gastroenteropancreatic neuroendocrine tumors.

Gastrointestinal System Disorders Prevalence
Gastroesophageal Reflux Disease (GERD) ≈18-28% of the US population
Peptic Ulcers ≈6 million Americans affected
Inflammatory Bowel Disease (IBD) ≈1.6 million Americans
Irritable Bowel Syndrome (IBS) ≈25-45 million Americans
Diverticulitis ≈200,000 hospitalizations annually

Role of Intestinal Hormones

In the intricate workings of the human body, intestinal hormones play a crucial role in regulating various physiological processes. These hormones are secreted by specialized cells within the gastrointestinal tract, acting as chemical messengers to signal and coordinate essential functions such as digestion, nutrient absorption, and appetite control.

Intestinal hormones, also known as gut hormones, are responsible for maintaining the delicate balance within the digestive system. They ensure the optimal release of digestive enzymes, bile, and stomach acid necessary for breaking down food and absorbing nutrients efficiently. Moreover, these hormones play a vital role in modulating the sensations of hunger and satiety, influencing our eating behaviors.

Gastroenteropancreatic Neuroendocrine Tumors and Hormone Disruption

However, gastroenteropancreatic neuroendocrine tumors can disrupt the production and release of intestinal hormones, causing an imbalance in the body. These rare tumors arise from neuroendocrine cells present in the gastrointestinal tract and pancreas.

The abnormal growth of these tumor cells can lead to the overproduction or underproduction of intestinal hormones, interfering with the normal functioning of the digestive system and hormonal regulation. As a result, individuals with gastroenteropancreatic neuroendocrine tumors may experience a range of symptoms, including diarrhea, abdominal pain, flushing, and hormonal imbalances.

It is important to note that the specific effects of gastroenteropancreatic neuroendocrine tumors on intestinal hormones can vary depending on the location and characteristics of the tumor. Different tumors may produce distinct hormones, leading to unique symptom profiles and clinical manifestations.

Understanding the intricate relationship between intestinal hormones and gastroenteropancreatic neuroendocrine tumors is crucial for developing effective diagnostic and therapeutic strategies. By targeting these hormone disruptions, healthcare professionals can better manage the symptoms and complications associated with these tumors, optimizing patient outcomes.

Understanding Neuroendocrine Neoplasms

In this section, we will explore neuroendocrine neoplasms, shedding light on their characteristics, classification, and diagnostic methods. Neuroendocrine neoplasms are a group of tumors that arise from neuroendocrine cells throughout the body. These cells have the ability to produce hormones, which regulate various bodily functions.

Neuroendocrine neoplasms can occur in different organs, including the gastrointestinal tract, pancreas, lungs, and more. However, for the purpose of this article, we will focus specifically on gastroenteropancreatic neuroendocrine tumors (GEP-NETs), which encompass those occurring in the digestive system and pancreas.

Classifying neuroendocrine neoplasms can be challenging due to their heterogeneous nature. However, they are commonly categorized into well-differentiated and poorly-differentiated tumors based on their histological features. Well-differentiated tumors are further classified into three subtypes: low-grade, intermediate-grade, and high-grade. These classifications play a critical role in determining the prognosis and treatment options for patients.

Diagnosing neuroendocrine neoplasms requires a multidisciplinary approach, involving various imaging techniques and laboratory tests. Imaging modalities such as computed tomography (CT) scans, magnetic resonance imaging (MRI), and somatostatin receptor scintigraphy (SRS) can help visualize the tumors and identify their location and extent. Additionally, blood tests that measure specific tumor markers can provide valuable information for diagnosis and monitoring.

Neuroendocrine Neoplasm Classification Description
Well-Differentiated Tumors These tumors have a distinct appearance under the microscope and are further classified based on their histological features and grade.
Poorly-Differentiated Tumors These tumors lack the characteristic features of well-differentiated tumors and are often aggressive in nature.

Early detection of neuroendocrine neoplasms is crucial for better patient outcomes. It allows for timely treatment interventions and reduces the risk of complications. As we move forward in this article, we will explore the role of pancreatic islet cells and discuss the diagnosis, staging, treatment options, and management of gastroenteropancreatic neuroendocrine tumors (GEP-NETs).

Role of Pancreatic Islet Cells

The role of pancreatic islet cells in hormone production and the development of hormone-producing tumors is crucial in the context of gastroenteropancreatic neuroendocrine tumors (NETs). Pancreatic islet cells, also known as islets of Langerhans, are small clusters of cells scattered throughout the pancreas. They play a vital role in regulating blood glucose levels and maintaining overall hormonal balance in the body.

These specialized cells produce different hormones, including insulin, glucagon, somatostatin, and pancreatic polypeptide, each with its specific function. Insulin, produced by beta cells, helps regulate glucose metabolism by promoting the uptake of glucose from the bloodstream into cells. Glucagon, on the other hand, is produced by alpha cells and stimulates the liver to release stored glucose, thereby increasing blood sugar levels when necessary.

In the case of gastroenteropancreatic neuroendocrine tumors, the normal functioning of pancreatic islet cells is disrupted. Hormone-producing tumors, also known as functional NETs, develop in the pancreatic islet cells and begin to produce excessive amounts of hormones. The type of hormone produced depends on the specific type of tumor present.

These hormone-producing tumors can cause a range of symptoms and complications. For example, insulinomas, which are hormone-producing tumors that produce excessive levels of insulin, can lead to episodes of low blood sugar (hypoglycemia). Glucagonomas, on the other hand, produce excessive glucagon, leading to elevated blood sugar levels and symptoms such as weight loss, skin rashes, and diarrhea.

To help visualize the different types of hormone-producing tumors and their effects on pancreatic islet cells, we’ve provided the following table:

Type of Hormone-Producing Tumor Hormone Produced Associated Symptoms
Insulinoma Insulin Hypoglycemia, confusion, weakness
Glucagonoma Glucagon Hyperglycemia, weight loss, skin rashes, diarrhea
Somatostatinoma Somatostatin Abdominal pain, diarrhea, weight loss
Pancreatic polypeptide tumor Pancreatic polypeptide Abdominal pain, diarrhea, weight loss

As shown in the table, different hormone-producing tumors can lead to varied symptoms. The specific hormonal imbalance caused by these tumors can have a significant impact on the overall health and well-being of individuals with gastroenteropancreatic neuroendocrine tumors.

Treatment Options for Gastroenteropancreatic Neuroendocrine Tumors

Effective treatment for gastroenteropancreatic neuroendocrine tumors often involves a multidisciplinary approach. The treatment plan may include various strategies, such as surgery, medical therapies, and targeted treatments.

Surgery is the primary treatment option for localized tumors that haven’t spread to other parts of the body. Surgical procedures can involve removing the tumor, a portion of the pancreas, or other affected organs. In cases where the tumor has spread or cannot be completely removed, other treatment options such as medical therapies or targeted treatments may be considered.

Medical therapies for gastroenteropancreatic neuroendocrine tumors aim to control symptoms, slow down tumor growth, and improve overall quality of life. These therapies can include hormone therapy, chemotherapy, targeted drug therapy, and peptide receptor radionuclide therapy (PRRT). The choice of medical therapy depends on factors such as the tumor type, stage, and individual patient characteristics.

Targeted treatments have emerged as a promising approach for gastroenteropancreatic neuroendocrine tumors. These treatments specifically target the underlying molecular abnormalities in NETs, inhibiting tumor growth and progression. Examples of targeted drugs used in the treatment of NETs include somatostatin analogs, mTOR inhibitors, and tyrosine kinase inhibitors.

It’s important to note that treatment plans for gastroenteropancreatic neuroendocrine tumors should be tailored to each individual case. The decision regarding the most appropriate treatment approach should be made in consultation with a knowledgeable healthcare team.

Diagnosis and Staging

Accurate diagnosis and staging of gastroenteropancreatic neuroendocrine tumors (NETs) is crucial for effective treatment planning and prognosis. Various diagnostic methods are employed to detect these tumors and determine the extent of their spread within the body.

Symptoms and Clinical Evaluation

When patients present with symptoms suggestive of gastroenteropancreatic NETs, a thorough clinical evaluation is conducted. This includes a detailed medical history, physical examination, and assessment of symptoms such as abdominal pain, diarrhea, flushing, or hormonal disturbances. Further investigations are then initiated based on the initial assessment.

Laboratory Tests

Laboratory tests play a vital role in the initial evaluation of gastroenteropancreatic NETs. Blood tests may be performed to measure levels of various markers, such as chromogranin A and serotonin, which can indicate the presence of NETs and provide information about tumor activity.

Imaging Techniques

Imaging techniques are essential for detecting gastroenteropancreatic NETs and determining their localization and extent. Different imaging modalities may be employed, including:

  • Computed Tomography (CT): This non-invasive imaging technique uses X-rays to create detailed cross-sectional images of the body, allowing for the visualization of tumors and their involvement with surrounding structures. CT scans can help in assessing tumor size, location, and potential metastases.
  • Magnetic Resonance Imaging (MRI): MRI utilizes powerful magnets and radio waves to generate detailed images of the body. It can provide valuable information about tumor characteristics and involvement with nearby organs and blood vessels.
  • Positron Emission Tomography (PET) Scan: PET scans involve the injection of a radioactive substance that accumulates in areas with high metabolic activity, such as tumors. By detecting the radiation emitted by these substances, PET scans can identify the presence and spread of gastroenteropancreatic NETs.
  • Octreotide Scintigraphy: This imaging technique uses a radioactive substance called octreotide, which binds to somatostatin receptors commonly found on neuroendocrine tumor cells. By detecting the radioactive signals emitted by the bound octreotide, octreotide scintigraphy can identify the location and extent of gastroenteropancreatic NETs.

Biopsy and Pathology

A biopsy is often necessary to confirm the diagnosis of gastroenteropancreatic NETs. During a biopsy, a small sample of tissue or cells is obtained from the tumor through minimally invasive procedures, such as endoscopy or fine-needle aspiration. Pathologists then examine the tissue sample under a microscope to determine the tumor type, grade, and other important characteristics.

Staging

Staging refers to the process of determining the extent and spread of gastroenteropancreatic NETs. It helps guide treatment decisions and predict prognosis. The staging system commonly used for NETs is the TNM (Tumor, Node, Metastasis) system, which takes into account the size of the primary tumor, involvement of nearby lymph nodes, and presence of distant metastases.

It is important to note that gastroenteropancreatic NETs can have a wide range of behavior, with some being slow-growing and localized, while others may be more aggressive and have spread to distant sites. Accurate diagnosis and staging allow for individualized treatment plans tailored to each patient’s specific situation.

Diagnostic Method Advantages Disadvantages
Computed Tomography (CT) High-resolution images, helps assess tumor size and extent Involves exposure to ionizing radiation
Magnetic Resonance Imaging (MRI) No ionizing radiation, provides detailed anatomical information May not be suitable for patients with certain metal implants
Positron Emission Tomography (PET) Scan Can detect small metastases, useful for assessing tumor activity Costly, limited availability
Octreotide Scintigraphy Targets neuroendocrine tumor cells specifically Lower spatial resolution compared to other imaging modalities

Treatment Options

When it comes to managing gastroenteropancreatic neuroendocrine tumors (NETs), there are various treatment options available. The choice of treatment depends on factors such as the tumor location, size, stage, and overall health of the patient. Here, we explore some of the main treatment modalities for gastroenteropancreatic NETs: surgery, medical therapies, and novel approaches.

Surgery

Surgery plays a crucial role in the treatment of gastroenteropancreatic NETs, especially for localized tumors. The goal of surgery is to remove the tumor and any nearby lymph nodes that may be affected. The type of surgery performed depends on the specific location of the tumor. Some common surgical procedures for gastroenteropancreatic NETs include:

  • Resection: This involves removing the tumor and a margin of healthy tissue around it.
  • Whipple procedure: Also known as a pancreaticoduodenectomy, this procedure involves removing the head of the pancreas, the duodenum, a portion of the bile duct, and the gallbladder.
  • Hepatectomy: This surgery removes a portion of the liver affected by NET metastasis.

Medical Therapies

In cases where surgery may not be feasible or when the tumor has spread beyond the primary site, medical therapies are an important treatment option for gastroenteropancreatic NETs. These therapies aim to control tumor growth, alleviate symptoms, and improve overall survival. Some commonly used medical therapies include:

  • Somatostatin analogs: These drugs help to control the release of hormones produced by the tumor, thereby reducing symptoms and slowing down tumor growth.
  • Targeted therapies: These medications specifically target the molecular pathways involved in tumor growth, blocking their activity and inhibiting tumor progression.
  • Radiolabeled somatostatin analogs: Also known as peptide receptor radionuclide therapy (PRRT), this treatment uses a radioactive substance attached to a somatostatin analog to deliver targeted radiation directly to the tumor cells.

Novel Approaches

Advancements in research have led to the development of novel approaches for treating gastroenteropancreatic NETs. These emerging therapies show promising results in improving patient outcomes. Some of the innovative treatment approaches currently being explored include:

  • Immunotherapy: This approach harnesses the body’s immune system to recognize and attack cancer cells. Immune checkpoint inhibitors and peptide vaccines are among the immunotherapy strategies being investigated.
  • Peptide receptor radionuclide therapy: Building on the success of radiolabeled somatostatin analogs, researchers are exploring the use of different radioactive substances and combination therapies to enhance treatment effectiveness.
  • Molecular targeted therapy: By targeting specific genetic mutations or aberrant signaling pathways, molecular targeted therapies aim to inhibit tumor growth and improve disease control.

Treatment Comparison

To better understand the different treatment options for gastroenteropancreatic NETs, let’s compare their key features:

Treatment Option Advantages Potential Side Effects
Surgery Offers the potential for complete tumor removal; effective for localized NETs Infection, bleeding, pain, impaired organ function
Medical Therapies Controls symptoms, slows tumor growth, improves survival; suitable for advanced NETs Nausea, diarrhea, fatigue, hormone imbalance
Novel Approaches Promising outcomes, potential for personalized therapy Varies depending on the specific approach; more research needed

It is important to note that treatment plans for gastroenteropancreatic NETs are tailored to each individual based on their specific circumstances. The choice of treatment will be made in collaboration between the patient and their healthcare team, taking into account factors such as tumor characteristics, overall health, and treatment goals.

Managing Symptoms and Complications

Gastroenteropancreatic neuroendocrine tumors (NETs) can present with a variety of symptoms and complications that can significantly impact a patient’s quality of life. Effective management strategies are crucial in alleviating symptoms, reducing complications, and improving overall well-being. This section provides an overview of supportive care measures, lifestyle modifications, and other strategies that can help patients cope with the challenges posed by gastroenteropancreatic NETs.

Symptom Management

Patients with gastroenteropancreatic NETs may experience a range of symptoms, including abdominal pain, diarrhea, flushing, wheezing, and weight loss. The goal of symptom management is to alleviate discomfort, minimize disease-related complications, and enhance daily functioning.

  • Dietary Modifications: Working with a registered dietitian can help patients identify trigger foods and make dietary adjustments to reduce symptoms such as diarrhea and abdominal pain. Recommendations may include consuming smaller, more frequent meals and avoiding spicy or fatty foods.
  • Medical Therapies: Medications such as somatostatin analogs, histamine blockers, and proton pump inhibitors may be prescribed to control symptoms and manage hormone overproduction.
  • Pain Management: In cases where NETs cause significant pain, pain medications and palliative care interventions can be employed to improve comfort and quality of life.

Complication Management

Gastroenteropancreatic NETs can lead to various complications, depending on the location and stage of the tumor. Effective management of these complications is crucial to prevent further health deterioration.

  • Hormone Overproduction: Hormone-producing NETs can result in the overproduction of specific hormones, leading to symptoms such as flushing, wheezing, and heart palpitations. Medications, including somatostatin analogs and targeted therapies, may be prescribed to control hormone secretion and alleviate related symptoms.
  • Obstruction or Carcinoid Crisis: Tumors located in the gastrointestinal tract can cause obstruction, leading to symptoms like bloating, nausea, and vomiting. In severe cases, a carcinoid crisis can occur, characterized by severe flushing, low blood pressure, and difficulty breathing. Immediate medical interventions, such as surgery, stent placement, or emergency medications, may be necessary to manage these complications.
  • Liver Function: Gastroenteropancreatic NETs often metastasize to the liver, potentially causing hepatomegaly and impairing liver function. Treatment options include liver-directed therapies, such as embolization, radiofrequency ablation, or surgical resection, to reduce tumor burden and improve liver function.

Early detection and close monitoring of gastroenteropancreatic NETs are essential for timely intervention and effective complication management. Your healthcare team will work closely with you to develop an individualized treatment plan that focuses on managing your specific symptoms and minimizing complications.

Supportive Measures Lifestyle Modifications Other Strategies
Regular follow-up appointments with healthcare team Physical activity and exercise Psychological support and counseling
Pain management techniques Stress management techniques Complementary and alternative therapies
Dietary adjustments Smoking cessation Participation in support groups

Prognosis and Survival Rates

Understanding the prognosis and survival rates of patients diagnosed with gastroenteropancreatic neuroendocrine tumors (NETs) is crucial for both patients and their healthcare providers. The prognosis of NETs can vary significantly based on several factors that influence the disease course and patient outcomes.

Factors Influencing Prognosis

The prognosis of gastroenteropancreatic NETs depends on the tumor grade, stage, location, and extent of metastasis. Well-differentiated NETs generally have a more favorable prognosis compared to poorly differentiated tumors. Additionally, the presence of specific genetic mutations, such as mutations in the MEN1 or VHL genes, can impact prognosis. It is important to note that the clinical behavior of gastroenteropancreatic NETs can be highly variable, making accurate prognostication challenging.

Survival Rates

The survival rates of patients with gastroenteropancreatic NETs can vary depending on the stage at diagnosis and the extent of tumor spread. According to current data, the overall 5-year survival rate for patients with localized NETs is estimated to be around 75%. However, for patients with metastatic disease, the 5-year survival rate drops to approximately 30%. These statistics highlight the importance of early detection and intervention to improve patient outcomes.

Ongoing Research

Ongoing research efforts are focused on improving the prognosis and survival rates for patients with gastroenteropancreatic NETs. Researchers are studying novel therapeutic approaches, such as targeted therapies and immunotherapies, to enhance treatment efficacy and patient outcomes. Furthermore, clinical trials are exploring the use of combination therapies and personalized treatment approaches that consider the specific characteristics of the tumor and individual patient factors.

It is important for patients with gastroenteropancreatic NETs to consult with their healthcare team to fully understand their individual prognosis and survival expectations. The management of NETs requires a multidisciplinary approach, and close collaboration between medical oncologists, surgeons, endocrinologists, and other healthcare professionals is crucial to optimize patient care and outcomes.

Advances in Research and Future Directions

Gastroenteropancreatic neuroendocrine tumors (NETs) have been the focus of extensive research in recent years. Scientists and healthcare professionals are dedicated to uncovering new insights into the biology and treatment of these tumors, with the ultimate goal of improving patient outcomes.

Current research efforts have shed light on the genetic and molecular mechanisms underlying gastroenteropancreatic NETs. This has led to the development of targeted therapies that show promise in inhibiting tumor growth and reducing the burden of the disease for patients. Additionally, ongoing studies are exploring the potential of immunotherapy and combination treatment approaches to further enhance treatment efficacy.

The future of gastroenteropancreatic NET management holds exciting possibilities. Researchers are focusing on unraveling the complexities of tumor heterogeneity, which will pave the way for more personalized and precise treatment strategies. Moreover, advancements in diagnostic techniques, such as molecular imaging and liquid biopsies, offer the potential for early detection and monitoring of tumor progression.

Emerging fields of research, like metabolomics and artificial intelligence, are also playing a crucial role in improving gastroenteropancreatic NET management. By analyzing metabolic profiles and harnessing the power of machine learning, scientists aim to identify novel therapeutic targets and develop predictive models for treatment response. These cutting-edge approaches hold the promise of revolutionizing the field and transforming the prognosis for patients with gastroenteropancreatic NETs.

FAQ

What are gastroenteropancreatic neuroendocrine tumors (NETs)?

Gastroenteropancreatic neuroendocrine tumors, also known as NETs, are tumors that develop in the digestive system and the hormone-producing cells of the pancreas. These tumors can be benign or malignant and have the potential to produce hormones, leading to various symptoms and complications.

How do gastroenteropancreatic neuroendocrine tumors affect the gastrointestinal system?

Gastroenteropancreatic neuroendocrine tumors can affect the gastrointestinal system by causing symptoms such as abdominal pain, diarrhea, and nausea. They can also lead to digestive system disorders, including malabsorption and bowel obstruction, depending on their location and size.

What role do intestinal hormones play in the body?

Intestinal hormones, produced by various cells in the gastrointestinal tract, play a crucial role in regulating digestion, nutrient absorption, and gut motility. They also contribute to the overall function of the digestive system. Gastroenteropancreatic neuroendocrine tumors can disrupt the production of these hormones, leading to imbalances and related complications.


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