ICD-10: C82.63
Cutaneous follicle center lymphoma, intra-abdominal lymph nodes
Additional Information
Description
Clinical Description of Cutaneous Follicle Center Lymphoma (ICD-10 Code C82.63)
Overview of Cutaneous Follicle Center Lymphoma
Cutaneous follicle center lymphoma (CFCL) is a subtype of non-Hodgkin lymphoma (NHL) that primarily affects the skin. It is characterized by the proliferation of B-lymphocytes in the follicle center of lymphoid tissue, leading to the formation of tumors. This condition is part of a broader category of follicular lymphomas, which are known for their indolent nature, although they can transform into more aggressive forms over time[1][3].
ICD-10 Code C82.63 Specifics
The ICD-10 code C82.63 specifically refers to cutaneous follicle center lymphoma that has spread to intra-abdominal lymph nodes. This classification indicates that while the primary manifestation of the lymphoma is in the skin, there is also involvement of lymph nodes located within the abdominal cavity. This can complicate the clinical picture and may influence treatment decisions and prognosis[1][2].
Clinical Features
Symptoms and Signs
Patients with cutaneous follicle center lymphoma may present with various symptoms, including:
- Skin Lesions: These can appear as nodules, plaques, or tumors on the skin, often resembling other dermatological conditions. The lesions may be asymptomatic or itchy.
- Lymphadenopathy: Enlargement of lymph nodes, particularly in the abdominal region, may occur as the disease progresses.
- Systemic Symptoms: In some cases, patients may experience systemic symptoms such as fever, night sweats, or weight loss, especially if the lymphoma has advanced[2][3].
Diagnosis
Diagnosis typically involves a combination of clinical evaluation, imaging studies, and histopathological examination. Key diagnostic steps include:
- Biopsy: A skin biopsy is essential to confirm the diagnosis, where tissue samples are examined for the presence of atypical lymphoid cells.
- Imaging: CT scans or PET scans may be utilized to assess the extent of disease, particularly to evaluate intra-abdominal lymph node involvement[1][2].
Treatment Options
The management of cutaneous follicle center lymphoma, especially with intra-abdominal lymph node involvement, may include:
- Radiation Therapy: Often used for localized skin lesions.
- Chemotherapy: Systemic chemotherapy may be indicated, particularly if there is significant lymph node involvement or if the disease is aggressive.
- Targeted Therapy: Newer agents, such as monoclonal antibodies, may be employed based on the specific characteristics of the lymphoma[1][3].
Prognosis
The prognosis for patients with cutaneous follicle center lymphoma can vary widely based on several factors, including the extent of disease at diagnosis, the presence of systemic symptoms, and the response to initial treatment. Generally, the indolent nature of this lymphoma subtype allows for a relatively favorable prognosis, although close monitoring is essential due to the potential for transformation into a more aggressive form[2][3].
Conclusion
Cutaneous follicle center lymphoma (ICD-10 code C82.63) represents a unique clinical challenge due to its presentation in the skin and potential spread to intra-abdominal lymph nodes. Understanding the clinical features, diagnostic approaches, and treatment options is crucial for effective management and improving patient outcomes. Regular follow-up and monitoring are essential to address any changes in disease status and to adapt treatment strategies accordingly.
Clinical Information
Cutaneous follicle center lymphoma (CFCL), classified under ICD-10 code C82.63, is a subtype of non-Hodgkin lymphoma that primarily affects the skin but can also involve lymph nodes, including intra-abdominal lymph nodes. Understanding the clinical presentation, signs, symptoms, and patient characteristics associated with this condition is crucial for diagnosis and management.
Clinical Presentation
Overview of Cutaneous Follicle Center Lymphoma
CFCL is a type of B-cell lymphoma that originates from follicle center (germinal center) B-cells. It is characterized by the presence of skin lesions, which can vary in appearance and may be mistaken for other dermatological conditions. The disease can be indolent, meaning it may progress slowly, or it can transform into a more aggressive form.
Signs and Symptoms
Patients with CFCL may present with a variety of signs and symptoms, including:
- Skin Lesions: The most common manifestation is the presence of skin lesions, which can appear as:
- Plaques
- Nodules
- Tumors
-
Ulcerated lesions
These lesions are often asymptomatic but can be itchy or painful in some cases[1]. -
Lymphadenopathy: Involvement of intra-abdominal lymph nodes may lead to:
- Swelling or enlargement of lymph nodes, which may be palpable during physical examination.
-
Symptoms related to lymph node enlargement, such as abdominal pain or discomfort, depending on the location and extent of lymph node involvement[2].
-
Systemic Symptoms: Although less common, some patients may experience systemic symptoms, including:
- Fever
- Night sweats
- Unexplained weight loss
These symptoms may indicate more advanced disease or transformation to a more aggressive lymphoma[3].
Patient Characteristics
Demographics
- Age: CFCL typically occurs in adults, with a higher incidence in individuals over the age of 50. However, it can occur in younger patients as well[4].
- Gender: There is a slight male predominance in the incidence of follicle center lymphomas, including CFCL[5].
Risk Factors
- Immunosuppression: Patients with compromised immune systems, such as those with HIV/AIDS or those undergoing immunosuppressive therapy, are at increased risk for developing lymphomas, including CFCL[6].
- Chronic Skin Conditions: Individuals with a history of chronic skin conditions or previous skin cancers may have a higher risk of developing CFCL[7].
Clinical Course
- Indolent Nature: Many patients experience a slow progression of the disease, with skin lesions being the primary concern for years before any lymph node involvement is noted[8].
- Transformation Potential: While CFCL is generally indolent, there is a risk of transformation to a more aggressive form of lymphoma, which can significantly alter the clinical course and prognosis[9].
Conclusion
Cutaneous follicle center lymphoma, particularly with intra-abdominal lymph node involvement, presents with distinctive skin lesions and potential systemic symptoms. Understanding the clinical presentation and patient characteristics is essential for timely diagnosis and appropriate management. Regular monitoring and follow-up are crucial, especially in patients with risk factors or those showing signs of disease progression. If you suspect CFCL or have concerns about skin lesions, consulting a healthcare professional for further evaluation is recommended.
Approximate Synonyms
Cutaneous follicle center lymphoma (CFL) is a subtype of non-Hodgkin lymphoma that primarily affects the skin and can involve lymph nodes. The ICD-10 code C82.63 specifically refers to cutaneous follicle center lymphoma with involvement of intra-abdominal lymph nodes. Here are some alternative names and related terms associated with this condition:
Alternative Names
- Follicular Lymphoma: While this term generally refers to a broader category of lymphomas, it can sometimes be used interchangeably with cutaneous follicle center lymphoma, particularly when discussing its follicular nature.
- Primary Cutaneous Follicle Center Lymphoma: This term emphasizes the primary cutaneous origin of the lymphoma.
- Cutaneous B-cell Lymphoma: Since CFL is a type of B-cell lymphoma, this broader term may be used in clinical discussions.
- Skin Follicle Center Lymphoma: A descriptive term that highlights the skin involvement and follicular characteristics of the lymphoma.
Related Terms
- Non-Hodgkin Lymphoma (NHL): A general category that includes various types of lymphomas, including follicular lymphoma and cutaneous lymphomas.
- Lymphadenopathy: Refers to the enlargement of lymph nodes, which can occur in CFL, especially when intra-abdominal lymph nodes are involved.
- B-cell Neoplasm: A term that encompasses various malignancies arising from B-cells, including CFL.
- Lymphoma: A general term for cancers that originate in the lymphatic system, which includes both Hodgkin and non-Hodgkin lymphomas.
- Stage IV Lymphoma: If the lymphoma has spread to intra-abdominal lymph nodes, it may be classified as stage IV, indicating advanced disease.
Conclusion
Understanding the alternative names and related terms for ICD-10 code C82.63 can enhance communication among healthcare professionals and improve patient education. These terms reflect the nature of the disease and its classification within the broader context of lymphomas. If you have further questions or need more specific information, feel free to ask!
Diagnostic Criteria
Cutaneous follicle center lymphoma (CFCL), classified under ICD-10 code C82.63, is a subtype of non-Hodgkin lymphoma that primarily affects the skin and can involve lymph nodes, including intra-abdominal lymph nodes. The diagnosis of CFCL involves a combination of clinical evaluation, histopathological examination, and imaging studies. Below are the key criteria used for diagnosis:
Clinical Evaluation
-
Symptoms: Patients may present with skin lesions that can appear as nodules, plaques, or tumors. These lesions are often asymptomatic but can be itchy or painful in some cases. Systemic symptoms such as fever, weight loss, or night sweats may also be present, although they are less common in CFCL compared to other lymphomas.
-
Physical Examination: A thorough examination of the skin and lymphatic system is essential. The presence of enlarged lymph nodes, particularly in the cervical, axillary, or inguinal regions, may indicate lymphatic involvement.
Histopathological Examination
-
Biopsy: A skin biopsy is crucial for diagnosis. The biopsy should be taken from an affected area and examined microscopically. Pathologists look for characteristic features of follicle center lymphoma, including:
- Follicular Architecture: The presence of neoplastic follicles that resemble normal germinal centers.
- Cell Type: The predominant cell type is typically centrocytes and centroblasts, which are derived from B-lymphocytes. -
Immunophenotyping: Immunohistochemical staining is performed to identify specific markers. CFCL typically expresses:
- CD10: A marker for germinal center B-cells.
- BCL6: Another marker associated with germinal center B-cells.
- BCL2: Often overexpressed in follicle center lymphomas, indicating a block in apoptosis.
Imaging Studies
-
CT or PET Scans: Imaging studies may be utilized to assess the extent of disease, particularly if intra-abdominal lymph nodes are suspected to be involved. These scans help in identifying lymphadenopathy and any other organ involvement.
-
Ultrasound: In some cases, ultrasound may be used to evaluate lymph nodes and assess for any abdominal masses.
Staging
The staging of CFCL is essential for determining the extent of the disease and guiding treatment. The Ann Arbor staging system is commonly used, which considers the number of involved lymph node regions and the presence of systemic symptoms.
Conclusion
The diagnosis of cutaneous follicle center lymphoma, particularly with intra-abdominal lymph node involvement, requires a comprehensive approach that includes clinical assessment, histopathological confirmation through biopsy, immunophenotyping, and imaging studies. Accurate diagnosis is crucial for effective management and treatment planning for patients with this type of lymphoma. If you have further questions or need more specific information, feel free to ask!
Treatment Guidelines
Cutaneous follicle center lymphoma (CFCL), particularly when associated with intra-abdominal lymph nodes, is a subtype of non-Hodgkin lymphoma (NHL) that requires a tailored treatment approach. The ICD-10 code C82.63 specifically refers to this condition, which is characterized by the proliferation of B-cells in the skin and lymphatic tissues. Here’s an overview of standard treatment approaches for this specific lymphoma type.
Overview of Cutaneous Follicle Center Lymphoma
CFCL is a type of indolent (slow-growing) lymphoma that primarily affects the skin but can also involve lymph nodes, including those in the abdomen. It is part of the broader category of follicular lymphomas, which are known for their relatively good prognosis compared to more aggressive forms of lymphoma. However, treatment is necessary when the disease progresses or causes symptoms.
Standard Treatment Approaches
1. Observation and Monitoring
For patients with asymptomatic CFCL or those with limited disease, a "watchful waiting" approach may be adopted. This involves regular monitoring without immediate treatment, as many patients may not require intervention for extended periods.
2. Local Therapies
For localized skin lesions, local treatments can be effective:
- Topical Chemotherapy: Agents such as chlorambucil or mechlorethamine can be applied directly to the skin lesions.
- Radiation Therapy: Localized radiation can be used to target specific skin lesions, particularly if they are symptomatic or causing discomfort.
3. Systemic Therapies
When the disease involves intra-abdominal lymph nodes or is more widespread, systemic therapies are often indicated:
- Chemotherapy: Regimens may include combinations of drugs such as cyclophosphamide, vincristine, prednisone, and rituximab (R-CHOP). Rituximab, a monoclonal antibody targeting CD20 on B-cells, is particularly effective in treating various forms of NHL, including CFCL.
- Immunotherapy: In addition to rituximab, newer agents like lenalidomide may be considered, especially in relapsed cases.
- Targeted Therapy: Agents targeting specific pathways involved in lymphoma growth, such as ibrutinib (a Bruton's tyrosine kinase inhibitor), may be utilized in certain cases.
4. Stem Cell Transplantation
For patients with relapsed or refractory CFCL, particularly those with significant intra-abdominal involvement, hematopoietic stem cell transplantation (HCT) may be considered. This approach is typically reserved for younger patients or those with a good performance status, as it involves intensive pre-transplant conditioning.
5. Clinical Trials
Participation in clinical trials may also be an option for patients, especially those with advanced disease or those who have not responded to standard therapies. Trials may offer access to novel therapies that are not yet widely available.
Conclusion
The treatment of cutaneous follicle center lymphoma with intra-abdominal lymph node involvement is multifaceted and should be personalized based on the patient's overall health, disease stage, and specific symptoms. Regular follow-up and monitoring are crucial, as the disease can often be indolent. Collaboration with a multidisciplinary team, including oncologists, dermatologists, and hematologists, is essential to optimize treatment outcomes and manage any potential complications associated with the disease or its treatment.
Related Information
Description
Clinical Information
- Cutaneous follicle center lymphoma affects the skin
- Can also involve lymph nodes and intra-abdominal lymph nodes
- Typically occurs in adults over 50 years old
- Male predominance in incidence of follicle center lymphomas
- Increased risk with immunosuppression and chronic skin conditions
- Indolent nature, but potential for transformation to aggressive form
- Skin lesions are the primary manifestation
- Lymphadenopathy can occur, leading to swelling and discomfort
Approximate Synonyms
- Follicular Lymphoma
- Primary Cutaneous Follicle Center Lymphoma
- Cutaneous B-cell Lymphoma
- Skin Follicle Center Lymphoma
- Non-Hodgkin Lymphoma
- Lymphadenopathy
- B-cell Neoplasm
Diagnostic Criteria
- Skin lesions appear as nodules, plaques, or tumors
- Lymph nodes may be enlarged in cervical, axillary, or inguinal regions
- Follicular architecture with neoplastic follicles present
- Centrocytes and centroblasts are predominant cell types
- CD10 marker is expressed on germinal center B-cells
- BCL6 and BCL2 markers associated with germinal center B-cells
- Systemic symptoms such as fever, weight loss, or night sweats present
Treatment Guidelines
- Observation for asymptomatic patients
- Local therapy for skin lesions
- Systemic chemotherapy for intra-abdominal involvement
- Rituximab as first-line treatment
- Immunotherapy with lenalidomide for relapsed cases
- Targeted therapy with ibrutinib in certain cases
- Hematopoietic stem cell transplantation for relapsed disease
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