Thymoma and Thymic Carcinoma: Comprehensive Health Information

This information is provided by SSCHRC to help you understand thymoma and thymic carcinoma and their management. It is written in a patient-friendly and medically accurate style.

1. General Information

Thymoma is a rare tumour that develops in the thymus, a small organ located in the chest, behind the breast bone and above the heart. The thymus is a vital part of the immune system, playing a crucial role in the development of immune cells called lymphocytes.

Thymoma arises when the epithelial cells (the cells forming the outer layer, or capsule) of the thymus undergo a cancerous change, beginning to grow and divide rapidly. While the tumour originates from these epithelial cells, it may also contain a mixture of lymphocytes.

Key Facts and Prognosis

  • • Thymoma is considered rare, with only a few hundred cases typically diagnosed in India each year.
  • • The disease is more common in older individuals, with most patients being over 70 years old.
  • • The five-year survival rate is approximately 70%, but an individual's outlook can vary significantly based on the tumour's subtype, stage, and other factors.
  • • An early-stage thymoma is contained within the capsule, while a more advanced disease has broken out of the capsule.

Types of Thymoma

Thymomas are broadly categorised into three main types based on how the cells appear under a microscope:

Type A

This subtype involves cancerous spindle-shaped epithelial cells. The tumour may contain few or many lymphocytes, depending on the specific cancer. The prognosis for Type A thymoma patients is generally very good.

Type B

There are several sub-types of Type B thymoma, each with a different microscopic appearance. The prognosis for this type is variable.

Type C (Thymic Carcinoma)

This is the most aggressive form of the disease. The cells appear highly abnormal under a microscope, and the cancer has a high rate of metastasis (spreading) to other parts of the body. It is often diagnosed when the disease has already spread.

Associated Conditions and Risk Factors

Because the thymus is part of the immune system, thymoma can sometimes trigger autoimmune diseases, where the body's immune system mistakenly attacks healthy cells.

Myasthenia Gravis (MG)

This is the most common autoimmune disease associated with thymoma, causing muscle weakness, especially in the face, head, and neck. If MG is caused by the thymoma, successful treatment of the cancer can often resolve the MG.

Risk Factors

Few known risk factors exist for thymoma, and it has not been definitively linked to family history or specific lifestyle behaviours. The main factors associated with the disease are:

  • • Age: It is uncommon in children and young adults, becoming more prevalent in middle age and peaking in people in their 70s.

2. Symptoms

Thymoma symptoms vary greatly among individuals, and many patients experience no symptoms at all, particularly in the early stages. Symptoms can be caused by the associated autoimmune disease, Myasthenia Gravis, or by the tumour pressing on nearby organs and structures, such as major blood vessels.

Common Thymoma Symptoms

  • • A persistent cough, sometimes with bloody phlegm or mucus
  • • Constant chest pain or pressure
  • • Shortness of breath
  • • Difficulty swallowing
  • • A hoarse voice
  • • Swelling of the face, neck, upper body, or arms
  • • Muscle weakness
  • • Anaemia
  • • Frequent infections
  • • Unexplained weight loss

Important Note: It is important to note that these symptoms can also be signs of other health problems. Any persistent or concerning symptoms should be discussed with a doctor.

3. Diagnosis and Staging

An accurate and early diagnosis is crucial for successful thymoma treatment. The experts at SSCHRC use advanced equipment and have extensive experience in accurately diagnosing all types of thymoma.

Often, thymoma does not present with symptoms in the early stages, and many cases are discovered incidentally, such as during a chest X-ray performed for an unrelated condition.

Imaging Exams

X-rays, Magnetic Resonance Imaging (MRIs), and CT scans are used to create detailed pictures of the chest. In many cases, doctors can make a strong diagnosis of thymoma based on imaging alone, without the need for a tissue sample.

Biopsy

This procedure involves obtaining a small tissue sample from the suspected tumour, which is then studied under a microscope by pathologists. The biopsy is often performed by a radiologist using image guidance (live image) to precisely guide a needle to the tumour site. Occasionally, an open biopsy requiring surgery may be necessary.

Blood Tests

While blood tests cannot directly diagnose thymoma, they are valuable in helping doctors understand a patient's symptoms, especially in checking for autoimmune conditions like myasthenia gravis.

Thymoma Staging

Cancer staging describes the size of the primary tumour and how far the cancer has spread within the body. Staging is vital for the care team to develop an appropriate treatment plan and estimate the patient's prognosis.

TNM Staging System

The TNM staging system is commonly used for many cancers and is based on three main factors:

  • T (Tumour): The size of the primary tumour and whether it has invaded nearby tissues.
  • N (Nodes): The spread of the cancer to nearby lymph nodes.
  • M (Metastasis): Whether the cancer has spread to distant parts of the body.

Stage I

Cancer is confined solely within the thymus and is entirely surrounded by the organ's capsule.

Stage II

Cancer has spread through the capsule and into the surrounding fat or the lining of the chest cavity.

Stage III

Cancer has spread to nearby organs in the chest, such as the lung, the sac around the heart (pericardium), or large blood vessels connected to the heart.

Stage IV

This advanced stage is divided into:

  • Stage IVA: Cancer has spread widely around the lungs or heart.
  • Stage IVB: Cancer has spread to the blood or lymph system.

4. Treatment

Successful thymoma treatment requires an experienced care team. Given that only a few hundred cases are diagnosed annually, the disease is rare for most physicians. However, the doctors at SSCHRC are members of a top-ranked cancer centre and treat a substantial number of thymoma patients each year, providing them with deep skill and expertise for newly diagnosed, metastatic, or recurrent disease.

Our doctors collaborate in multidisciplinary teams to discuss each patient's unique case. They coordinate a comprehensive, tailored treatment plan that may combine surgery, radiation therapy, and cancer drugs. SSCHRC also offers clinical trials for new treatment options, including targeted therapy and immunotherapy, for patients at all stages.

Surgery

For thymomas smaller than four centimetres, surgical removal of the entire thymus, known as a thymectomy, is the standard approach.

  • Minimally Invasive Surgery: For small cancers, a thymectomy is often performed using surgical robots through three to four small incisions under and to the side of the breast. This results in less pain, faster recovery, and shorter hospital stays compared to traditional surgery.
  • Sternotomy: Larger tumours typically require a sternotomy, a procedure where the surgeon opens the middle of the chest by cutting through the breast bone. This procedure involves a hospital stay of several days and a recovery period of several weeks.

Chemotherapy

This treatment uses drugs to kill rapidly dividing cells, including cancer cells. It can be used before surgery (neoadjuvant) to shrink the tumour or in combination with radiation therapy if surgery is not an option.

Patients with larger tumours are often given chemotherapy before surgery to shrink the tumour, which increases the likelihood of a successful surgical procedure.

Radiation Therapy

This uses powerful energy beams to destroy cancer cells. It is typically given after surgery, or in combination with chemotherapy if surgery is not feasible. Treatment options include:

  • Intensity-Modulated Radiotherapy (IMRT): This is tailored to the specific shape of the tumour and is often used for cancers that have spread to nearby lymph nodes.
  • Volumetric-Modulated Arc Therapy (VMAT): A type of IMRT where the machine rotates around the patient in an arc, allowing for more precise tumour irradiation and potentially shorter treatment times.

Advanced Disease Treatment

If the tumour has spread beyond the primary site, patients may receive a combination of radiation therapy and cancer drugs (like chemotherapy). Surgery may still be performed at the primary and distant sites to help relieve symptoms and improve patient outcomes, in coordination with other therapies.

Thymic Carcinoma (Type C): As this is typically diagnosed at an advanced stage, patients are treated with chemotherapy, radiation therapy, and potentially surgery.