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Diagnosis and Treatment for Neuroendocrine Tumors in NJ, CT, MD, and the Washington, D.C., Area

Neuroendocrine tumors (NETs) are relatively uncommon, with about 12,000 diagnoses per year in the United States. However, the rate of NET diagnoses is rising by about 5% annually. This increase is largely credited to diagnostic advances that are enabling physicians to identify NETs that may not have been recognized without the sophisticated methods now available.

Serving patients in New Jersey, Connecticut, Maryland, and the Washington, D.C., area, Regional Cancer Care Associates (RCCA) is home to expert medical oncologists who are well-versed in diagnosing and treating neuroendocrine cancer, along with other forms of cancer and benign blood disorders.

What Is a Neuroendocrine Tumor?

Scattered throughout the body, neuroendocrine cells connect the endocrine and nervous systems, combining the characteristics of hormone-producing endocrine cells and nerve cells. Neuroendocrine tumors begin forming in these specialized cells, and though this type of cancer is uncommon, several types and subtypes of NET display symptoms often mistaken for minor medical conditions.

Oncologist examining cells under a microscope

About Neuroendocrine Cells

Complex in nature, the neuroendocrine system regulates the body’s physiological or behavioral state. Its components include the following:

  • Glands, including the pituitary gland
  • Hypothalamus and extrahypothalamic regions
  • Neurons and neurochemicals
  • Non-endocrine tissues
  • Hormones and humoral signals

This system also is home to specialized neuroendocrine cells, which control the endocrine system’s release of hormones. Aside from enabling hormones to coordinate various bodily functions, the neuroendocrine system also creates its own hormones, which regulate functions within the organs where they live.

Types of Neuroendocrine Tumors

Cancer can develop if neuroendocrine cells divide and multiply uncontrollably, forming tumors that can metastasize, or spread to other parts of the body, if not caught and treated early. NETs are classified into two categories and include several types:

Classification Based on Location

  • Gastrointestinal (GI) NETs: The most common location for NETs is the GI tract. The large intestine accounts for about 20% of cases, while the small intestine accounts for around 19% and the appendix for about 4%.
  • Pancreatic NETs: Formerly known as islet cell tumors, less than 10% of NETs occur in the pancreas, which is a gland in the abdomen.
  • Pulmonary NETs: NETs developing in the lungs or bronchial system, formerly known as carcinoid tumors, account for about 30% of cases.

Classification Based on Hormone Secretion

  • Functioning NETs: These tumors cause symptoms because they create their own hormones in excessive amounts.
  • Non-functioning NETs: These tumors do not cause symptoms even if they create their own hormones.

Causes and Risk Factors

NETs can develop in people at any age, although they are most common between ages 50 and 60. Females are slightly more likely than males to develop NETs. When it comes to racial risk factors, Caucasians are generally more likely to have neuroendocrine cancer than Black people. Further, the following inherited syndromes have been linked to NETs:

  • Multiple endocrine neoplasia type 1 and 2 (MEN1 and MEN2)
  • Neurofibromatosis type 1
  • Tuberous sclerosis complex
  • Von Hippel-Lindau syndrome

Other diseases and conditions also can increase the risk for NETs, such as:

  • Hormonal imbalances
  • Acquired immune deficiency syndrome (AIDS)
  • Human immunodeficiency virus (HIV)
  • Suppressed immunity from organ transplants

Signs and Symptoms of a Neuroendocrine Tumor

Symptoms of NETs differ based on where the malignant cells are located. They may include:

  • Carcinoid syndrome: This is the result of GI and lung NETs and consists of a group of symptoms, such as:
    • Flushing of the neck and head
    • Abdominal cramping
    • Diarrhea
    • Fatty, foul-smelling stool
    • Swelling in the feet and legs
    • Wheezing and shortness of breath
    • Low libido and erectile dysfunction
    • Jaundice (yellowing of the skin/eyes)
  • Hormone-related symptoms: Glucagon, a hormone created by the pancreas to regulate blood glucose levels, can cause additional symptoms. If blood glucose levels are high, people with NETs might experience more frequent urination and increased thirst or hunger. If blood glucose levels are low, NETs may cause sweating, fainting, dizziness, and trembling.
  • Other effects: These may include weakness, fatigue, unexplained changes in body weight, skin lesions, areas of thin skin, and heart palpitations.

Diagnosing Neuroendocrine Tumors

The physicians at Regional Cancer Care Associates diagnose NETs in several ways. First, they confirm the location, size, and other tumor characteristics, as well as verify whether the tumor is malignant or benign. Tests used to diagnose NETs include:

  • Physical examination to detect lumps, changes in skin color, enlarged organs, or other signs of cancer
  • Imaging techniques such as computed tomography (CT) scans, magnetic resonance imaging (MRI), positron emission tomography (PET) scans, bone scans, and ultrasounds
  • Laboratory tests to evaluate urine, blood, and DNA for irregularities or genetic mutations
  • Biopsies and histopathology to examine the bodily fluids, cells, and tissue in question under a microscope 

Staging and Grading of Neuroendocrine Tumors

Staging is the process of establishing the tumor’s size and location, identifying how far cancer has progressed at or near its site of origin, and determining whether the cancerous cells have metastasized.

Meanwhile, grading assesses the cancer cells, considering how they appear under a microscope and at what rate they’re dividing (if at all). Both are vital for deciding on the best treatment options, as NETs can be found anywhere in the body.

Oncologists use the TNM system when evaluating tumors for staging and grading. This acronym stands for:

  • T – tumor: What is the size, shape, and physical condition of the original tumor?
  • N – node: Has cancer spread to lymph nodes?
  • M – metastasis: Has the cancer spread to other parts of the body?

After evaluation, physicians may categorize NETs by the following stages, grades, or degrees of differentiation:

NET Stages

  • Stage I: A small tumor that hasn’t spread beyond its point of origin.
  • Stage II: The tumor is growing but is still confined to its point of origin.
  • Stage III: The tumor may have reached lymph nodes nearby, or it may be growing outside of the originating point into nearby organs or blood vessels.
  • Stage IV: The cancer has spread throughout the body.

NET Grades

  • Grade 1 – low-grade tumor: Cancer cells are dividing at a low rate and growing slowly.
  • Grade 2 – intermediate-grade tumor: Cancer cells are dividing at a faster speed.
  • Grade 3 – high-grade tumor: Cancer cells have reached a high rate of division and are growing rapidly.

Degrees of Differentiation

  • Well-differentiated: Cells appear to be more healthy than cancerous.
  • Poorly-differentiated: Cells look more like cancer.

Treatment Options for Neuroendocrine Tumors

At Regional Cancer Care Associates, several state-of-the-art treatment options are available for patients with NETs. These may include, but are not limited to, the following:

Surgical Intervention

Surgery is the most common treatment for NET and often is the first course of action. The type of surgery depends on the tumor’s primary location, size, stage, and grade, as well as the patient’s symptoms and overall health.

Targeted Therapies

Targeted therapy involves the use of innovative, newly developed cancer drugs, which target proteins and other substances on the surface of or within NETs that drive their division and growth.

Radiation Therapy

High-energy particles or rays from an external source are delivered to the cancerous tissue in a highly precise manner to kill cancer cells.

Peptide Receptor Radionuclide Therapy (PRRT)

Through high doses of radiation, PRRT destroys tumors and slows their growth while reducing the symptoms of NETs.

Immunotherapy

Immunotherapy harnesses the power of the body’s own immune system to recognize and destroy cancer cells.

Complementary and Alternative Medicine

Although these approaches won’t destroy NETs, modalities such as acupuncture, light therapy, and even meditation can help diminish the symptoms of NETs and the side effects of related treatments.

Find Outstanding Neuroendocrine Tumor Care at Regional Cancer Care Associates

Beyond offering diagnosis and treatment options for NETs, Regional Cancer Care Associates also understands how challenging living with cancer can be. That’s why the team of medical oncologists offers a host of resources for supportive care and quality of life, from symptom management and psychosocial support to nutrition, patient support groups, and advocacy efforts. For more information about neuroendocrine tumor care, contact Regional Cancer Care Associates today in New Jersey, Connecticut, Maryland, or the Washington, D.C., area. Patients are treated at more than 20 convenient locations in the region.

Every neuroendocrine cancer diagnosis is different, but you can feel confident in your prognosis when you trust your care to the oncologists at Regional Cancer Care Associates. Our compassionate, experienced team will explain all available treatment options and work with you to create the best treatment plan for your needs. To learn more or to schedule an appointment, contact one of our Connecticut, Maryland, or New Jersey locations today.

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