Thymoma type B

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ORPHA:263317C37D15.0
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3Specialists8Treatment centers

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Overview

Thymoma type B is a neoplasm arising from the epithelial cells of the thymus, a small organ located in the anterior mediastinum (the front part of the chest, behind the breastbone). The thymus plays a critical role in the development of the immune system, particularly in the maturation of T-lymphocytes. Thymoma type B is classified according to the World Health Organization (WHO) histological classification and is further subdivided into type B1, B2, and B3, based on the proportion of neoplastic epithelial cells relative to lymphocytes and the degree of atypia of the epithelial cells. Type B1 thymomas are lymphocyte-rich and resemble normal thymic cortex, type B2 contains a more equal mixture of epithelial cells and lymphocytes, and type B3 is predominantly composed of epithelial cells with few lymphocytes and may exhibit more aggressive behavior. Many patients with thymoma type B are asymptomatic at diagnosis, with the tumor discovered incidentally on chest imaging. When symptoms occur, they may include chest pain, cough, shortness of breath, and superior vena cava syndrome due to compression of mediastinal structures. A hallmark feature of thymomas, particularly types B1 and B2, is their strong association with autoimmune paraneoplastic syndromes, most notably myasthenia gravis, which causes muscle weakness and fatigue. Other associated conditions include pure red cell aplasia, hypogammaglobulinemia, and various other autoimmune disorders. The clinical behavior of type B thymomas ranges from relatively indolent (B1) to more locally invasive or potentially metastatic (B3). Treatment of thymoma type B depends on the stage at diagnosis and the histological subtype. Complete surgical resection is the primary treatment and offers the best chance of cure for encapsulated or early-stage tumors. For invasive or advanced-stage disease, multimodal therapy including neoadjuvant or adjuvant chemotherapy and/or radiation therapy may be employed. Platinum-based chemotherapy regimens are commonly used. Long-term follow-up is essential due to the risk of late recurrence, even many years after initial treatment. Management of associated autoimmune conditions, particularly myasthenia gravis, is an important component of overall patient care.

Inheritance

Sporadic

Usually appears on its own, not inherited from a parent

Age of Onset

Adult

Begins in adulthood (age 18 or older)

Orphanet ↗NORD ↗

Treatments

No FDA-approved treatments are currently listed for Thymoma type B.

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No actively recruiting trials found for Thymoma type B at this time.

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Specialists

3 foundView all specialists →
SM
Shivaani Kummar, MD
PORTLAND, OR
Specialist
PI on 5 active trials
JM
Jeffrey W. Andrey, M.D.
LA JOLLA, CA
Specialist
PI on 1 active trial

Treatment Centers

8 centers
🏥 NORD

Baylor College of Medicine Rare Disease Center

Baylor College of Medicine

📍 Houston, TX

🏥 NORD

Stanford Medicine Rare Disease Center

Stanford Medicine

📍 Stanford, CA

🔬 UDN

NIH Clinical Center Undiagnosed Diseases Program

National Institutes of Health

📍 Bethesda, MD

🔬 UDN

UCLA UDN Clinical Site

UCLA Health

📍 Los Angeles, CA

🔬 UDN

Baylor College of Medicine UDN Clinical Site

Baylor College of Medicine

📍 Houston, TX

🔬 UDN

Harvard/MGH UDN Clinical Site

Massachusetts General Hospital

📍 Boston, MA

🏥 NORD

Mayo Clinic Center for Individualized Medicine

Mayo Clinic

📍 Rochester, MN

👤 Mayo Clinic Center for Individualized Medicine

🏥 NORD

UCLA Rare Disease Day Program

UCLA Health

📍 Los Angeles, CA

Travel Grants

No travel grants are currently matched to Thymoma type B.

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Community

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Latest news about Thymoma type B

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Caregiver Resources

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Learn how rare disease patients may qualify for SSDI/SSI benefits.

Common questions about Thymoma type B

What is Thymoma type B?

Thymoma type B is a neoplasm arising from the epithelial cells of the thymus, a small organ located in the anterior mediastinum (the front part of the chest, behind the breastbone). The thymus plays a critical role in the development of the immune system, particularly in the maturation of T-lymphocytes. Thymoma type B is classified according to the World Health Organization (WHO) histological classification and is further subdivided into type B1, B2, and B3, based on the proportion of neoplastic epithelial cells relative to lymphocytes and the degree of atypia of the epithelial cells. Type B1

How is Thymoma type B inherited?

Thymoma type B follows a sporadic inheritance pattern. Genetic counseling can help families understand recurrence risk and testing options.

At what age does Thymoma type B typically begin?

Typical onset of Thymoma type B is adult. Age of onset can vary across affected individuals.

Which specialists treat Thymoma type B?

3 specialists and care centers treating Thymoma type B are listed on UniteRare, sourced from ClinicalTrials.gov principal investigators, published research, and the NPPES NPI registry.