ICD-10: C85.26

ICD-10 code C85.26 refers specifically to mediastinal (thymic) large B-cell lymphoma involving intrapelvic lymph nodes. This classification falls under the broader category of non-Hodgkin lymphoma (NHL), particularly large B-cell lymphomas. Below are alternative names and related terms associated with this specific diagnosis.

Alternative Names

1. Thymic Large B-Cell Lymphoma: This term emphasizes the origin of the lymphoma in the thymus gland, which is located in the mediastinum.
2. Primary Mediastinal B-Cell Lymphoma (PMBCL): While PMBCL typically refers to a specific subtype of diffuse large B-cell lymphoma, it is often used interchangeably with mediastinal large B-cell lymphoma.
3. Large B-Cell Lymphoma of the Thymus: This name highlights the specific type of lymphoma and its location.
4. Intrapelvic Large B-Cell Lymphoma: This term focuses on the involvement of the intrapelvic lymph nodes, indicating the spread of the disease.

Related Terms

1. Non-Hodgkin Lymphoma (NHL): A broader category that includes various types of lymphomas, including large B-cell lymphomas.
2. Diffuse Large B-Cell Lymphoma (DLBCL): This is the most common subtype of non-Hodgkin lymphoma and includes various presentations, including those that may arise in the mediastinum.
3. Lymphoma: A general term for cancers that originate in the lymphatic system, which includes both Hodgkin and non-Hodgkin lymphomas.
4. Thymoma: Although distinct from lymphoma, thymomas can occur in the thymus and may be confused with thymic lymphomas due to their anatomical proximity.
5. Lymphadenopathy: This term refers to the enlargement of lymph nodes, which can be a symptom of lymphoma, including C85.26.

Clinical Context

Mediastinal large B-cell lymphoma is characterized by the presence of large B-cell lymphomas in the mediastinum, often presenting with symptoms such as chest pain, cough, or respiratory distress due to mass effect. The involvement of intrapelvic lymph nodes indicates a more advanced stage of the disease, which may require specific treatment approaches, including chemotherapy, radiation therapy, or targeted therapies.

Understanding these alternative names and related terms can aid healthcare professionals in accurately diagnosing and discussing this specific type of lymphoma, ensuring appropriate treatment and management strategies are employed.

ICD-10 code C85.26 refers to Mediastinal (thymic) large B-cell lymphoma, intrapelvic lymph nodes. This classification falls under the broader category of non-Hodgkin lymphoma (NHL), specifically large B-cell lymphoma, which is a type of aggressive lymphoma characterized by the proliferation of large B lymphocytes.

Clinical Description

Definition

Mediastinal large B-cell lymphoma is a subtype of diffuse large B-cell lymphoma (DLBCL) that primarily arises in the mediastinum, which is the central compartment of the thoracic cavity. This condition can also involve lymph nodes located in the intrapelvic region, indicating a more advanced stage of the disease.

Epidemiology

This type of lymphoma is more common in young adults, particularly in males, and is often associated with a mediastinal mass that can lead to symptoms due to compression of surrounding structures. The prognosis can vary based on several factors, including the stage at diagnosis and the patient's overall health.

Symptoms

Patients with mediastinal large B-cell lymphoma may present with a variety of symptoms, including:
- Chest pain: Due to the mass effect on thoracic structures.
- Cough: Resulting from airway compression.
- Shortness of breath: Caused by pleural effusion or lung involvement.
- Fever, night sweats, and weight loss: Common systemic symptoms associated with lymphoma.
- Abdominal symptoms: If intrapelvic lymph nodes are involved, patients may experience abdominal pain or discomfort.

Diagnosis

Diagnosis typically involves a combination of imaging studies (such as CT scans or PET scans) and histopathological examination of lymph node biopsies. Immunophenotyping is crucial for confirming the diagnosis, as it helps differentiate large B-cell lymphoma from other types of lymphomas and neoplasms.

Staging

The staging of large B-cell lymphoma follows the Ann Arbor classification, which assesses the extent of disease based on the number of lymph node regions involved and the presence of extranodal disease. The involvement of intrapelvic lymph nodes indicates a more advanced stage, which can influence treatment decisions.

Treatment

The treatment for mediastinal large B-cell lymphoma typically involves:
- Chemotherapy: The standard regimen often includes a combination of cyclophosphamide, doxorubicin, vincristine, and prednisone (CHOP), sometimes with the addition of rituximab (R-CHOP) for CD20-positive cases.
- Radiation therapy: This may be used in conjunction with chemotherapy, especially in localized cases or to address residual disease after chemotherapy.
- Stem cell transplant: In cases of relapsed or refractory disease, high-dose chemotherapy followed by autologous stem cell transplantation may be considered.

Prognosis

The prognosis for patients with mediastinal large B-cell lymphoma can vary significantly based on factors such as age, performance status, and response to initial treatment. Generally, early-stage disease has a better prognosis compared to advanced-stage disease.

Conclusion

ICD-10 code C85.26 encapsulates a specific and clinically significant subtype of large B-cell lymphoma that presents unique challenges in diagnosis and management. Understanding the clinical features, treatment options, and prognostic factors associated with this condition is essential for healthcare providers involved in the care of patients with lymphoma. Early diagnosis and appropriate treatment are critical for improving outcomes in affected individuals.

Mediastinal (thymic) large B-cell lymphoma, classified under ICD-10 code C85.26, is a subtype of diffuse large B-cell lymphoma (DLBCL) that primarily arises in the mediastinum, particularly involving the thymus gland. This lymphoma can also affect intrapelvic lymph nodes, which may complicate its clinical presentation. Below is a detailed overview of the clinical presentation, signs, symptoms, and patient characteristics associated with this condition.

Clinical Presentation

General Characteristics

Mediastinal large B-cell lymphoma typically presents in young adults, with a higher incidence in males than females. The median age at diagnosis is often in the late 20s to early 30s, although it can occur in older adults as well. Patients may present with a variety of symptoms depending on the extent of the disease and the specific lymphatic regions involved.

Signs and Symptoms

1. Respiratory Symptoms:
   - Cough: Patients may experience a persistent cough due to compression of the airways or lung tissue.
   - Dyspnea: Shortness of breath can occur if the tumor compresses the trachea or major bronchi.
   - Chest Pain: This may arise from pleural involvement or direct tumor invasion.

2. Mediastinal Mass Effects:
   - Swelling: A noticeable swelling in the neck or chest may be observed due to enlarged lymph nodes.
   - Superior Vena Cava Syndrome: This can occur if the mass compresses the superior vena cava, leading to facial swelling, distended neck veins, and upper extremity edema.

3. Systemic Symptoms:
   - Fever: Patients may present with unexplained fevers, often classified as "B symptoms."
   - Night Sweats: Profuse sweating during the night is common.
   - Weight Loss: Unintentional weight loss can occur due to the malignancy's metabolic demands.

4. Intrapelvic Symptoms:
   - Abdominal Pain: If intrapelvic lymph nodes are involved, patients may experience abdominal discomfort or pain.
   - Bowel Obstruction: Enlarged lymph nodes in the pelvis can lead to gastrointestinal symptoms, including obstruction.

5. Lymphadenopathy:
   - Peripheral Lymphadenopathy: Patients may have enlarged lymph nodes in other regions, such as the neck, axilla, or groin.

Patient Characteristics

• Demographics: The condition is more prevalent in young adults, particularly those in their 20s and 30s, with a male predominance.
• Immunocompromised Status: Patients with weakened immune systems, such as those with HIV/AIDS or those on immunosuppressive therapy, may be at higher risk for developing this lymphoma.
• Associated Conditions: There may be a correlation with autoimmune diseases or prior history of lymphoproliferative disorders.

Diagnostic Considerations

Diagnosis typically involves imaging studies such as CT scans or PET scans to assess the extent of the disease, followed by biopsy of the mediastinal mass or affected lymph nodes to confirm the diagnosis histologically. Immunophenotyping and genetic studies may also be performed to characterize the lymphoma further.

Conclusion

Mediastinal (thymic) large B-cell lymphoma, particularly when involving intrapelvic lymph nodes, presents with a range of respiratory, systemic, and localized symptoms. Understanding the clinical presentation and patient characteristics is crucial for timely diagnosis and management. Early recognition of symptoms and appropriate imaging studies can significantly impact treatment outcomes for affected individuals.

Mediastinal (thymic) large B-cell lymphoma, classified under ICD-10 code C85.26, is a subtype of non-Hodgkin lymphoma characterized by the proliferation of large B-cells in the mediastinum, particularly affecting the thymus gland. The diagnosis of this specific lymphoma type involves a combination of clinical evaluation, imaging studies, and histopathological examination. Below is a detailed overview of the criteria used for diagnosis.

Clinical Presentation

Symptoms

Patients with mediastinal large B-cell lymphoma may present with a variety of symptoms, including:
- Chest pain: Often due to the mass effect of the tumor on surrounding structures.
- Cough: Resulting from airway obstruction or irritation.
- Dyspnea: Difficulty in breathing due to mediastinal mass.
- Fever, night sweats, and weight loss: Common systemic symptoms associated with lymphoma.

Physical Examination

• Lymphadenopathy: Enlarged lymph nodes may be palpable, particularly in the cervical or axillary regions.
• Mediastinal mass: Detected through physical examination or imaging.

Imaging Studies

Radiological Evaluation

1. Chest X-ray: May reveal a mediastinal mass or enlarged lymph nodes.
2. CT Scan: A contrast-enhanced CT scan of the chest is crucial for assessing the size, location, and extent of the mediastinal mass and any involvement of adjacent structures.
3. PET Scan: Positron emission tomography (PET) can help evaluate metabolic activity and detect any distant disease, which is important for staging.

Histopathological Examination

Biopsy

• Tissue Sampling: A definitive diagnosis requires a biopsy of the mediastinal mass or affected lymph nodes. This can be performed via:
• Fine-needle aspiration (FNA): Less invasive but may not provide sufficient tissue for diagnosis.
• Core needle biopsy: Provides a larger sample for histological analysis.
• Surgical biopsy: May be necessary for a comprehensive evaluation.

Immunophenotyping

• Flow Cytometry: Used to analyze the surface markers on the cells, confirming the B-cell lineage and identifying specific markers associated with large B-cell lymphoma (e.g., CD19, CD20, CD22).
• Immunohistochemistry: Staining techniques help differentiate between various types of lymphomas and confirm the diagnosis of large B-cell lymphoma.

Genetic Studies

• Cytogenetic Analysis: Detection of chromosomal abnormalities, such as the presence of the MYC gene rearrangement, can provide additional diagnostic and prognostic information.

Staging and Classification

Ann Arbor Staging System

• The Ann Arbor classification is used to stage the lymphoma, which is essential for determining treatment options. Staging involves assessing the number of lymph node regions involved and whether the disease has spread to extranodal sites.

Revised European-American Lymphoma (REAL) Classification

• This classification system helps in categorizing lymphomas based on histological features, which is crucial for treatment planning.

Conclusion

The diagnosis of mediastinal (thymic) large B-cell lymphoma (ICD-10 code C85.26) is a multifaceted process that requires careful clinical assessment, imaging studies, and histopathological confirmation. The integration of these diagnostic criteria ensures accurate identification of the disease, which is vital for effective management and treatment planning. If you have further questions or need more specific information, feel free to ask!

Mediastinal (thymic) large B-cell lymphoma (PMBCL), classified under ICD-10 code C85.26, is a subtype of non-Hodgkin lymphoma characterized by the presence of large B-cell lymphomas primarily located in the mediastinum. This condition can also involve intrapelvic lymph nodes, complicating its management. The treatment approaches for PMBCL typically involve a combination of chemotherapy, radiation therapy, and, in some cases, targeted therapies or stem cell transplantation. Below is a detailed overview of the standard treatment modalities.

Standard Treatment Approaches

1. Chemotherapy

Chemotherapy remains the cornerstone of treatment for PMBCL. The most commonly used regimens include:

• R-CHOP: This regimen combines Rituximab (a monoclonal antibody) with Cyclophosphamide, Doxorubicin, Vincristine, and Prednisone. R-CHOP is often the first-line treatment for aggressive B-cell lymphomas, including PMBCL, due to its effectiveness in inducing remission[1][2].

• DA-EPOCH-R: This is a dose-adjusted regimen that includes Etoposide, Prednisone, Vincristine, Cyclophosphamide, and Doxorubicin, along with Rituximab. It is particularly beneficial for patients with high-risk features or those who may not respond adequately to R-CHOP[3].

2. Radiation Therapy

Radiation therapy is frequently employed in conjunction with chemotherapy, especially for localized disease. It is particularly effective in targeting residual disease in the mediastinum after chemotherapy. The typical approach includes:

• Involved Field Radiation Therapy (IFRT): This technique focuses on the specific areas affected by lymphoma, minimizing exposure to surrounding healthy tissues. Radiation is usually administered after the completion of chemotherapy to consolidate the response[4].

3. Targeted Therapy

In certain cases, targeted therapies may be considered, especially for patients with specific genetic markers or those who have relapsed. Options include:

• Brentuximab vedotin: This antibody-drug conjugate targets CD30 and may be used in cases where traditional chemotherapy regimens fail[5].

• CAR T-cell therapy: For relapsed or refractory cases, chimeric antigen receptor (CAR) T-cell therapy has shown promise, particularly in aggressive B-cell lymphomas[6].

4. Stem Cell Transplantation

For patients with high-risk disease or those who do not achieve complete remission with initial therapy, autologous stem cell transplantation may be considered. This approach involves harvesting the patient’s stem cells, administering high-dose chemotherapy, and then reinfusing the stem cells to restore bone marrow function[7].

Conclusion

The management of mediastinal (thymic) large B-cell lymphoma, particularly when involving intrapelvic lymph nodes, requires a multidisciplinary approach tailored to the individual patient’s disease characteristics and overall health. The combination of chemotherapy, radiation therapy, and potential use of targeted therapies or stem cell transplantation provides a comprehensive strategy aimed at achieving remission and improving patient outcomes. Ongoing clinical trials and research continue to refine these treatment protocols, offering hope for improved efficacy and reduced toxicity in the management of this challenging lymphoma subtype.

References

1. Billing and Coding: Radiation Therapies (A59350).
2. Billing and Coding: Genetic Testing for Oncology (DA59123).
3. Medical Drug Clinical Criteria.
4. Radioimmunotherapy in the Treatment of Non-Hodgkin Lymphoma.
5. ICD - O International Classification of Diseases for Oncology.
6. Primary Mediastinal Large B-cell Lymphoma - PMC - PubMed Central (PMC).
7. How I Diagnose Primary Mediastinal (Thymic) Large B-Cell Lymphoma.