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Introduction

Lymphoma is a type of neoplasm that affects cells (ie, lymphocytes) that comprise the body’s immune system (ie, B cells, T cells, and natural killer [NK] cells). Hodgkin lymphoma (HL) most commonly affects lymph nodes in the neck or chest cavity; HL also can present in lymph nodes under the arm(s). Classic HL accounts for 95% of all cases; nodular, lymphocyte-predominant HL is more common among younger patients. Non-Hodgkin lymphoma (NHL) consists of three subtypes: B-cell lymphoma (90% of patients), T-cell lymphoma (6%), and NK-cell lymphoma (<1%). Although extremely rare, there are a few reported cases of extranodal B-cell and T-cell lymphoma in the hands and forearm.3

Pathophysiology

  • Some lymphomas are associated with mutations to genes that encode specific proteins (eg, p53 and p16).
  • Viruses, including Epstein-Barr, hepatitis B and C, herpes 6, can play a role in the pathogenesis of lymphoma.
  • In patients being treated for rheumatoid arthritis (RA), if lymphoma occurs, treatment-related immunosuppression may be a factor.

Related Anatomy

  • HL can initiate in the groin, abdomen, or pelvis and may spread to the spleen, liver, bone marrow, or bone. Spreading to other body parts is rare.
  • NHL has been known to involve the stomach, intestines, skin, thyroid gland, and brain.

Incidence and Related Conditions

  • HL accounts for 10% of all lymphomas and <1% of all cancers diagnosed annually in the US; those aged 15–40 years and >55 years are at greatest risk for developing lymphoma.
    • In 2016, 8500 people in the US were diagnosed with HL.
  • NHL is the sixth most common cancer in adults, but is uncommon in children.
    • In 2016, 72,580 people in the US were diagnosed with NHL.
    • Increased rates of NHL in RA, Sjögren syndrome, systemic lupus erythematosus, and possibly celiac disease.
    • NHL of the hand is rare; T-cell lymphoma of the hand is especially uncommon.

Differential Diagnosis

  • Fibromatosis
  • Infection
  • Numerous other tumor and mass types
  • Rheumatoid arthritis
  • Vascular malformation
ICD-10 Codes
  • LYMPHOMA

    Diagnostic Guide Name

    LYMPHOMA

    ICD 10 Diagnosis, Single Code, Left Code, Right Code and Bilateral Code

    DIAGNOSISSINGLE CODE ONLYLEFTRIGHTBILATERAL (If Available)
    LYMPHOMA - HODGKIN'S, REED STERNBERG CELLS PRESENT    
    - HEAD, NECK (UNSPECIFIED)C81.91   
    - AXILLA, UPPER LIMB (UNSPECIFIED)C81.94   
    LYMPHOMA - NON-HODGKIN'S (B-CELL)    
    - HEAD, NECK (UNSPECIFIED)C85.11   
    - AXILLA, UPPER LIMB (UNSPECIFIED)C85.11   

    ICD-10 Reference

    Reproduced from the International statistical classification of diseases and related health problems, 10th revision, Fifth edition, 2016. Geneva, World Health Organization, 2016 https://apps.who.int/iris/handle/10665/246208

Clinical Presentation Photos and Related Diagrams
Lymphoma
  • Lymphoma - Note swelling (arrow) in area of first extensor muscles
    Lymphoma - Note swelling (arrow) in area of first extensor muscles
  • Lymphoma - Note swelling (arrow) in anterior medial elbow
    Lymphoma - Note swelling (arrow) in anterior medial elbow
Symptoms
Swelling of the hand and wrist with blisters and sometimes ulcerations
Nodular plaques, small nodules and/or enlarging mass, but not necessarily discrete borders
Enlarged lymph nodes in the neck or underarms
No constitutional symptoms like fatigue, fever, night sweats, or weight loss with hand lesions but positive symptoms in arm lesions
No tenderness or pain associated with lymphoma masses
Positive Tests, Exams or Signs
Work-up Options
Images (X-Ray, MRI, etc.)
  • Lymphoma - Imaging study with mass in anterior medial elbow
    Lymphoma - Imaging study with mass in anterior medial elbow
Treatment Options
Conservative
  • In elderly patients with no systemic symptoms, radiotherapy may be sufficient for T-cell lymphoma.
  • For B-cell lymphoma with malignancy suggested by PET, a sample regimen is allopurinol, an anti-CD20 monoclonal antibody, followed by chemotherapy.
  • Therapy for the hands may conclude with external beam radiation.
Operative
  • Nonoperative treatment is usually appropriate for lymphomas; however, extranodal lymphoma may require surgical treatment for diagnosis and to decrease local symptoms.  
Treatment Photos and Diagrams
  • Lymphoma - Extensor tendons (arrow) with EPB and AbPL muscle bellies(double arrows) invaded by lymphomatous tissue
    Lymphoma - Extensor tendons (arrow) with EPB and AbPL muscle bellies(double arrows) invaded by lymphomatous tissue
CPT Codes for Treatment Options

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CPT Code References

The American Medical Association (AMA) and Hand Surgery Resource, LLC have entered into a royalty free agreement which allows Hand Surgery Resource to provide our users with 75 commonly used hand surgery related CPT Codes for educational promises. For procedures associated with this Diagnostic Guide the CPT Codes are provided above. Reference materials for these codes is provided below. If the CPT Codes for the for the procedures associated with this Diagnostic Guide are not listed, then Hand Surgery Resource recommends using the references below to identify the proper CPT Codes.

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CPT 2021 Professional Edition: Spiralbound

Complications
  • Most treatment risk is associated with radiation and chemotherapy.
Outcomes
  • For T-cell lymphoma of the hand, there may be considerable reduction in hand size and healing of dorsal blisters.
  • For B-cell lymphoma of the hand, a return to baseline hand function before the development of lymphoma is possible, with no recurrence at 5.5 years post treatment.
Key Educational Points
  • In a patient with RA and an enlarging mass in the hand, NHL should be considered.
  • There are reports of patients with RA in whom the NHL regressed when methotrexate was stopped.
  • Most cases of NHL in RA do not arise in the vicinity of involved joints.
  • Lymphadenopathy is usual in B-cell lymphoma of the hand, but may be absent.
  • MRI and incisional biopsy are advisable in the setting of undiagnosed hand tumor.
References

New Articles

  1. Gordon MJ, Peterson BA, Skubitz KM. Lymphoma of the hands in a patient with rheumatoid arthritis: case report. J Hand Surg Am 2014;39(4):728-31.
  2. Kung TA, Smith LB, Chung KC. Atypical presentation of isolated peripheral t-cell lymphoma in the hand: case report. J Hand Surg Am 2014;39(4):732-6.

Reviews

  1. Fino P, Spagnoli AM, Ruggieri M, et al. Bilateral hand squamous-cells carcinoma in patient affected with non-Hodgkin lymphoma. Case report and literature review. G Chir 2015;36(4):172-82. PMID: 26712073
  2. Sickinger MT, von Tresckow B, Kobe C, et al. PET-adapted omission of radiotherapy in early stage Hodgkin lymphoma-a systematic review and meta-analysis. Crit Rev Oncol Hematol 2016;101:86-92. PMID: 26971064

Classics

  1. Kerin R. The hand in metastatic disease. J Hand Surg Am 1987;12(1):77-83. PMID: 3543107
  2. Schultz RJ, Kearns RJ. Tumors in the hand. J Hand Surg Am 1983;8(5):803-6. PMID: 6630962
  3. Ellstein J, Xeller C, Fromowitz F, Elias JM, Saletan S, Hurst LC.  Soft Tissue T-Cell Lymphoma of the Forearm: A Case Report.  J Hand Surg 1984;9A(#3):346-350.
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