What is the recommended treatment approach for a patient with an abdominal wall mass diagnosed as Hodgkin lymphoma nodular sclerosis?

Treatment of Abdominal Wall Mass in Hodgkin Lymphoma Nodular Sclerosis

For a patient with an abdominal wall mass diagnosed as Hodgkin lymphoma nodular sclerosis, treatment depends entirely on accurate staging and risk stratification, with combined modality therapy (chemotherapy followed by radiotherapy) being the standard approach for most presentations. 1

Immediate Diagnostic Requirements

Before initiating treatment, complete staging workup is mandatory 1, 2:

• Contrast-enhanced CT scan of neck, chest, and abdomen to determine extent of disease 3, 1
• PET-CT for accurate initial staging when available 3, 1
• Bone marrow biopsy if clinically indicated 1
• Laboratory assessment including complete blood count, ESR, liver and kidney function 3
• Assessment for B symptoms (fever >38°C, drenching night sweats, unexplained weight loss >10% in 6 months) as these are critical prognostic factors 3, 1

The abdominal wall involvement represents extranodal disease, which influences staging and risk stratification 3.

Treatment Based on Stage and Risk Factors

Early-Stage Favorable Disease (Stage I-II, No Unfavorable Features)

Standard treatment is 2 cycles of ABVD (doxorubicin, bleomycin, vinblastine, dacarbazine) followed by 30 Gy involved-field radiotherapy 1:

• ABVD remains the backbone of therapy for early-stage disease 3, 1, 4
• The abdominal wall mass would receive radiation as part of involved-site radiotherapy 3, 1
• This approach balances efficacy with reduced long-term toxicity 1

Early-Stage Unfavorable Disease (Stage I-II with Risk Factors)

Standard treatment is 4 cycles of ABVD followed by 30 Gy involved-field radiotherapy 1:

Unfavorable features include 3:

• Age ≥50 years
• ESR ≥50 (without B symptoms) or ≥30 (with B symptoms)
• ≥4 nodal areas involved
• Large mediastinal mass (>1/3 maximum chest diameter)
• Extranodal extension (such as abdominal wall involvement)

For patients under 60 years eligible for intensive treatment, 2 cycles of BEACOPPescalated followed by 2 cycles of ABVD and 30 Gy involved-field radiotherapy may provide superior outcomes 1, though BEACOPP carries significantly higher toxicity 3.

Advanced-Stage Disease (Stage III-IV)

Standard treatment is 6-8 cycles of ABVD chemotherapy 3, 1:

• Radiotherapy is limited to residual masses >2.5 cm after chemotherapy completion 3
• For patients under 60 years, 8 cycles of BEACOPPescalated followed by radiation to residual disease >1.5 cm is an alternative 3, 1
• BEACOPP should NOT be given to patients >60 years due to excessive toxicity 3

Response-Adapted Treatment Strategy

PET-CT assessment after 2-4 cycles is essential for treatment adaptation 3, 1:

• Deauville score 1-3 (PET-negative): Continue planned therapy 3, 1
• Deauville score 4-5 (PET-positive): Consider treatment intensification or clinical trial enrollment 3, 1
• This approach allows de-escalation in good responders and intensification in poor responders 3, 4

Special Considerations for Abdominal Wall Involvement

The abdominal wall mass represents extranodal disease, which has specific implications 3:

• Ensure adequate radiation field planning to cover the abdominal wall mass with appropriate margins 3
• 36 Gy to initially involved areas is recommended in some protocols for stage I-II disease below the diaphragm 3
• The mass should be measured and documented for response assessment 3

Critical Treatment Modifications

Age-Related Adjustments

Patients >60 years should receive ABVD-based chemotherapy rather than BEACOPP 3:

• Bleomycin should be discontinued after the second cycle in older patients due to increased pulmonary toxicity risk 3
• Modified ABVD without bleomycin is appropriate for patients with compromised organ function 2

Fertility Preservation

Fertility preservation counseling and interventions should be offered to young patients prior to treatment initiation 1, as both ABVD and radiation can affect reproductive function.

Relapsed or Refractory Disease

If the patient fails initial therapy 3, 1:

• High-dose chemotherapy followed by autologous stem cell transplantation (ASCT) is the standard of care 3, 4, 5
• Salvage regimens (DHAP, IGEV, or ICE) are used to reduce tumor burden prior to ASCT 3
• Brentuximab vedotin consolidation following ASCT is recommended for patients with poor-risk factors 3
• Achieving PET-negativity should be the goal of salvage therapy 3

Common Pitfalls to Avoid

• Do not omit staging PET-CT if available, as it significantly impacts treatment decisions 3, 1
• Do not use BEACOPP in patients >60 years due to unacceptable toxicity 3
• Do not continue bleomycin in patients developing pulmonary symptoms (dyspnea, cough, hypoxia) 3
• Do not treat without confirming nodular sclerosis subtype through adequate biopsy with immunophenotyping 1
• Do not delay fertility preservation discussions until after treatment has started 1

Monitoring During Treatment

Interim PET-CT after 2-4 cycles is mandatory to assess response and guide treatment continuation 3, 1:

• Physical examination and laboratory monitoring before each cycle 3
• Cardiac assessment if using anthracyclines, especially with pre-existing cardiac risk factors 2
• Pulmonary function monitoring if bleomycin is used 3