ICD-10: C82.65

Cutaneous follicle center lymphoma, lymph nodes of inguinal region and lower limb

Additional Information

Description

Cutaneous follicle center lymphoma (CFCL) is a subtype of non-Hodgkin lymphoma that primarily affects the skin. The ICD-10 code C82.65 specifically refers to CFCL localized to the lymph nodes of the inguinal region and lower limb. Below is a detailed clinical description and relevant information regarding this condition.

Clinical Description of Cutaneous Follicle Center Lymphoma

Overview

Cutaneous follicle center lymphoma is characterized by the proliferation of B-lymphocytes that typically arise from the follicle center (germinal center) of lymphoid tissue. This lymphoma is often indolent, meaning it tends to grow slowly, and it primarily manifests in the skin, although it can involve lymph nodes and other organs.

Symptoms

Patients with CFCL may present with various symptoms, including:
- Skin Lesions: These can appear as plaques, nodules, or tumors on the skin, often resembling other dermatological conditions.
- Lymphadenopathy: Enlargement of lymph nodes, particularly in the inguinal region and lower limbs, may occur as the disease progresses.
- Pruritus: Some patients may experience itching in the affected areas.

Diagnosis

Diagnosis typically involves:
- Histopathological Examination: A biopsy of the skin lesions is essential for confirming the diagnosis. Pathological examination reveals atypical lymphoid cells in the dermis and epidermis.
- Imaging Studies: Imaging techniques such as CT scans or PET scans may be used to assess lymph node involvement and the extent of the disease.

Staging

The staging of CFCL is crucial for determining the appropriate treatment approach. The Ann Arbor staging system is commonly used, which considers the number of affected lymph nodes and the presence of systemic symptoms.

Specifics of ICD-10 Code C82.65

Code Definition

  • ICD-10 Code: C82.65
  • Description: Cutaneous follicle center lymphoma, lymph nodes of inguinal region and lower limb.

This code is used to classify cases where the lymphoma is specifically localized to the lymph nodes in the inguinal region and lower limbs, indicating a more advanced stage of the disease compared to localized skin involvement alone.

Treatment Options

Treatment for CFCL may vary based on the extent of the disease and may include:
- Observation: In cases where the disease is asymptomatic and indolent.
- Radiation Therapy: Often used for localized skin lesions or involved lymph nodes.
- Chemotherapy: Systemic treatment may be necessary for more advanced cases.
- Immunotherapy: Targeted therapies, such as monoclonal antibodies, may be employed.

Prognosis

The prognosis for patients with CFCL can vary widely based on factors such as the extent of the disease, the patient's overall health, and response to treatment. Generally, CFCL has a better prognosis compared to more aggressive forms of lymphoma.

Conclusion

Cutaneous follicle center lymphoma, particularly when involving the lymph nodes of the inguinal region and lower limb (ICD-10 code C82.65), represents a specific clinical scenario within the broader category of non-Hodgkin lymphomas. Understanding the clinical features, diagnostic criteria, and treatment options is essential for effective management and improved patient outcomes. Regular follow-up and monitoring are crucial for managing this condition, given its potential for progression.

Diagnostic Criteria

Cutaneous follicle center lymphoma (CFCL), classified under ICD-10 code C82.65, is a subtype of non-Hodgkin lymphoma that primarily affects the skin and can involve lymph nodes, particularly in the inguinal region and lower limbs. 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

  1. 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.
  2. Physical Examination: A thorough examination of the skin and lymph nodes is essential. Enlarged lymph nodes in the inguinal region or lower limbs may be noted.

Histopathological Criteria

  1. Biopsy: A skin biopsy is crucial for diagnosis. The biopsy should show:
    - Follicle Center Cell Morphology: The presence of neoplastic follicle center (germinal center) cells, which are typically large and atypical.
    - Infiltration Pattern: The infiltration of the dermis and subcutaneous tissue by these atypical cells, often with a follicular or diffuse pattern.
    - Immunophenotyping: Immunohistochemical staining is performed to confirm the diagnosis. The neoplastic cells typically express:
    • CD10
    • BCL6
    • BCL2 (often in a t(14;18) translocation)
    • CD19 and CD20 (B-cell markers)

Imaging Studies

  1. CT or PET Scans: Imaging may be used to assess the extent of disease, particularly to evaluate lymph node involvement in the inguinal region and lower limbs. These scans help determine if there is any systemic involvement.

Additional Diagnostic Criteria

  1. Exclusion of Other Conditions: It is essential to rule out other types of lymphomas or skin conditions that may mimic CFCL, such as primary cutaneous B-cell lymphoma or other skin malignancies.
  2. Staging: The Ann Arbor staging system may be utilized to determine the extent of the disease, which is important for treatment planning.

Conclusion

The diagnosis of cutaneous follicle center lymphoma (ICD-10 code C82.65) relies on a combination of clinical findings, histopathological analysis, and imaging studies. Accurate diagnosis is critical for appropriate management and treatment planning, as CFCL can vary significantly in its clinical behavior and response to therapy. If you suspect CFCL, it is advisable to consult a specialist in hematology or oncology for further evaluation and management.

Treatment Guidelines

Cutaneous follicle center lymphoma (CFCL), classified under ICD-10 code C82.65, is a subtype of non-Hodgkin lymphoma that primarily affects the skin and can involve lymph nodes, particularly in the inguinal region and lower limbs. The treatment approaches for this condition can vary based on several factors, including the stage of the disease, the patient's overall health, and specific characteristics of the lymphoma. Below is a detailed overview of standard treatment strategies for CFCL.

Overview of Cutaneous Follicle Center Lymphoma

CFCL is characterized by the proliferation of B-cells in the skin and can manifest as localized or disseminated disease. It is often indolent, meaning it may grow slowly, but it can also transform into a more aggressive form. The involvement of lymph nodes, particularly in the inguinal region and lower limbs, can complicate the treatment approach.

Standard Treatment Approaches

1. Observation and Monitoring

For patients with early-stage CFCL that is asymptomatic and localized, a "watchful waiting" approach may be adopted. This involves regular monitoring without immediate treatment, as some cases may not require intervention if the disease is stable.

2. Topical Therapies

Topical treatments are often the first line of therapy for localized skin lesions. These may include:

  • Corticosteroids: Topical steroids can help reduce inflammation and control symptoms.
  • Chemotherapy Creams: Agents such as mechlorethamine (nitrogen mustard) can be applied directly to the skin lesions.
  • Immunomodulators: Topical agents like imiquimod may stimulate the immune response against the lymphoma cells.

3. Radiation Therapy

Localized radiation therapy is effective for patients with localized CFCL, particularly when lesions are symptomatic or causing discomfort. Radiation can target specific areas, such as the inguinal lymph nodes, to reduce tumor burden and alleviate symptoms.

4. Systemic Therapies

For more advanced or disseminated cases, systemic treatments may be necessary. These can include:

  • Chemotherapy: Regimens may involve combinations of agents such as cyclophosphamide, doxorubicin, vincristine, and prednisone (CHOP).
  • Monoclonal Antibodies: Agents like rituximab, which targets CD20 on B-cells, can be used alone or in combination with chemotherapy.
  • Targeted Therapies: Newer agents, such as copanlisib (Aliqopa) and obinutuzumab (Gazyva), may be considered based on the specific characteristics of the lymphoma and patient health.

5. Stem Cell Transplantation

In cases of refractory or relapsed CFCL, autologous stem cell transplantation may be an option. This approach is typically reserved for younger patients with good performance status and significant disease burden.

6. Clinical Trials

Patients may also consider enrolling in clinical trials, which can provide access to novel therapies and treatment strategies that are not yet widely available. These trials often explore new drugs, combinations, or treatment modalities.

Conclusion

The management of cutaneous follicle center lymphoma, particularly when it involves the lymph nodes of the inguinal region and lower limbs, requires a tailored approach based on individual patient factors and disease characteristics. Treatment options range from observation and topical therapies for localized disease to systemic therapies and potential stem cell transplantation for more advanced cases. Ongoing research and clinical trials continue to evolve the treatment landscape, offering hope for improved outcomes in patients with this condition. For personalized treatment recommendations, consultation with a hematologist or oncologist specializing in lymphomas is essential.

Clinical Information

Cutaneous follicle center lymphoma (CFCL), classified under ICD-10 code C82.65, is a subtype of non-Hodgkin lymphoma that primarily affects the skin and can involve lymph nodes, particularly in the inguinal region and lower limbs. Understanding the clinical presentation, signs, symptoms, and patient characteristics associated with this condition is crucial for accurate diagnosis and management.

Clinical Presentation

Overview of Cutaneous Follicle Center Lymphoma

CFCL is characterized by the proliferation of follicle center (germinal center) B-cells, which can lead to localized skin lesions and lymphadenopathy. This lymphoma typically presents in a more indolent form compared to other aggressive lymphomas, allowing for a range of clinical manifestations.

Common Signs and Symptoms

  1. Skin Lesions:
    - Patients often present with solitary or multiple nodules, plaques, or tumors on the skin, particularly in sun-exposed areas. These lesions may be erythematous, violaceous, or skin-colored and can vary in size.
    - Lesions may be asymptomatic or associated with mild pruritus (itching) but are generally not painful.

  2. Lymphadenopathy:
    - Involvement of lymph nodes, especially in the inguinal region, is common. Patients may notice swelling or enlargement of lymph nodes in the groin or lower limbs.
    - Lymph nodes may be firm and non-tender, reflecting the indolent nature of the disease.

  3. Systemic Symptoms:
    - While CFCL is often localized, some patients may experience systemic symptoms such as fatigue, weight loss, or night sweats, although these are less common in early stages.

Patient Characteristics

  • Demographics: CFCL typically affects adults, with a higher incidence in individuals over the age of 50. There is no significant gender predilection, although some studies suggest a slight male predominance.
  • Risk Factors:
  • Patients with a history of immunosuppression (e.g., due to HIV, organ transplantation, or autoimmune diseases) may be at increased risk for developing CFCL.
  • Chronic skin conditions or previous skin cancers may also contribute to the risk.

Diagnosis and Evaluation

Diagnosis of CFCL involves a combination of clinical evaluation, imaging studies, and histopathological examination. Key steps include:

  1. Clinical Examination: A thorough physical examination to assess skin lesions and lymphadenopathy.
  2. Biopsy: Skin biopsy of the lesions is essential for histological confirmation, revealing characteristic follicle center cell proliferation.
  3. Imaging Studies: Imaging, such as ultrasound or CT scans, may be utilized to evaluate lymph node involvement and assess the extent of the disease.

Conclusion

Cutaneous follicle center lymphoma, particularly affecting the lymph nodes of the inguinal region and lower limbs, presents with distinctive skin lesions and potential lymphadenopathy. Understanding the clinical signs, symptoms, and patient demographics is vital for healthcare providers to ensure timely diagnosis and appropriate management. Regular follow-up and monitoring are essential, especially in patients with risk factors for progression or systemic involvement.

Approximate Synonyms

ICD-10 code C82.65 refers specifically to cutaneous follicle center lymphoma located in the inguinal region and lower limb. This type of lymphoma is a subtype of non-Hodgkin lymphoma (NHL) and is characterized by the proliferation of B-cells in the skin. Below are alternative names and related terms associated with this condition:

Alternative Names

  1. Primary Cutaneous Follicle Center Lymphoma: This term emphasizes that the lymphoma originates in the skin rather than spreading from another site.
  2. Cutaneous B-cell Lymphoma: A broader term that includes various types of B-cell lymphomas that manifest in the skin, including follicle center lymphoma.
  3. Follicular Lymphoma (Cutaneous Variant): While follicular lymphoma typically refers to nodal involvement, this term can be used to describe its cutaneous manifestations.
  1. Non-Hodgkin Lymphoma (NHL): A general category of lymphomas that includes various subtypes, including follicle center lymphoma.
  2. B-cell Lymphoma: Refers to lymphomas that arise from B-cells, which are a type of white blood cell involved in the immune response.
  3. Lymphadenopathy: This term describes the enlargement of lymph nodes, which can occur in cases of lymphoma, including C82.65.
  4. Skin Lymphoma: A general term for lymphomas that primarily affect the skin, encompassing various subtypes, including cutaneous follicle center lymphoma.
  5. Inguinal Lymphadenopathy: Specifically refers to the enlargement of lymph nodes in the inguinal region, which may be associated with cutaneous follicle center lymphoma.

Clinical Context

Cutaneous follicle center lymphoma is often indolent, meaning it tends to grow slowly, and may present with skin lesions that can be mistaken for other dermatological conditions. Understanding the terminology and related terms is crucial for accurate diagnosis, treatment planning, and coding in medical records.

In summary, the ICD-10 code C82.65 is associated with a specific type of lymphoma that has various alternative names and related terms, reflecting its characteristics and clinical implications. If you need further details or specific aspects of this condition, feel free to ask!

Related Information

Description

  • Proliferation of B-lymphocytes
  • Arises from follicle center germinal center
  • Indolent growth pattern
  • Primarily manifests in skin
  • Can involve lymph nodes and organs
  • Skin lesions appear as plaques or nodules
  • Lymphadenopathy occurs in inguinal region and lower limbs
  • Pruritus may occur in affected areas

Diagnostic Criteria

  • Skin lesions appear as nodules or plaques
  • Lymph nodes enlarged in inguinal region
  • Follicle center cell morphology seen on biopsy
  • Infiltration pattern is follicular or diffuse
  • CD10, BCL6, and BCL2 positive on immunostain
  • CD19 and CD20 expressed by neoplastic cells
  • Exclusion of other lymphomas and skin conditions
  • Ann Arbor staging system used for disease extent

Treatment Guidelines

  • Watchful waiting for asymptomatic early-stage CFCL
  • Topical corticosteroids to reduce inflammation
  • Chemotherapy creams applied directly to lesions
  • Immunomodulators stimulate immune response against lymphoma cells
  • Localized radiation therapy targets specific areas
  • Systemic chemotherapy regimens like CHOP or R-CHOP
  • Monoclonal antibodies target CD20 on B-cells
  • Targeted therapies based on lymphoma characteristics
  • Autologous stem cell transplantation for refractory cases
  • Enrollment in clinical trials for novel treatments

Clinical Information

  • Localized skin lesions appear as nodules or plaques
  • Lymphadenopathy occurs in inguinal region and lower limbs
  • Systemic symptoms include fatigue and weight loss
  • Patients typically over age 50 with no gender predilection
  • Immunosuppression increases risk of developing CFCL
  • Chronic skin conditions or previous skin cancers contribute to risk

Approximate Synonyms

  • Primary Cutaneous Follicle Center Lymphoma
  • Cutaneous B-cell Lymphoma
  • Follicular Lymphoma (Cutaneous Variant)
  • Non-Hodgkin Lymphoma (NHL)
  • B-cell Lymphoma
  • Lymphadenopathy
  • Skin Lymphoma
  • Inguinal Lymphadenopathy

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