What is the role of surgical excision in treating patients with localized atypical lymphoid proliferation, Kimura disease, or angiolymphoid hyperplasia with eosinophilia?

Surgical Excision for Localized Atypical Lymphoid Proliferation, Kimura Disease, and Angiolymphoid Hyperplasia with Eosinophilia

Complete surgical excision is the standard treatment for localized Kimura disease and angiolymphoid hyperplasia with eosinophilia (ALHE), though recurrence rates remain high and adjuvant corticosteroid therapy or steroid-sparing immunomodulatory agents should be considered for maintenance. 1, 2

Distinguishing Between Kimura Disease and ALHE

Before determining surgical approach, accurate histopathological diagnosis is essential as these entities differ significantly:

• Kimura disease presents with large, deep subcutaneous masses predominantly in young Asian males, characterized by lymphoid follicles with germinal centers, intense eosinophilic infiltration, vascular proliferation, and fibrosis 2, 3
• ALHE occurs across all races with smaller, more superficial lesions featuring atypical histiocytoid endothelial cell proliferation, nodular T-cell infiltration with small B-cell clusters, and less organized architecture 3
• Immunohistochemical staining is critical: ALHE shows localized endothelial cell proliferation, while Kimura disease demonstrates invasive lymphoid cell proliferation with potential lymphadenopathy 4
• Incisional or excisional biopsy remains the primary diagnostic method, as no recognized diagnostic criteria currently exist 1

Surgical Management Strategy

For Solitary or Localized Lesions

• Complete surgical excision with negative margins is the treatment of choice for both Kimura disease and ALHE 1, 2
• Wide local excision should be performed to minimize recurrence risk, though recurrences remain common even after complete excision 2
• The surgical specimen must be sent for comprehensive histopathological analysis including immunohistochemical studies of lymphocyte markers, endothelial antigens, and granulocyte proteins 3

Critical Surgical Considerations

• Kimura disease lesions may persist unchanged for years and new lesions frequently develop, requiring long-term surveillance 2
• Superficial lymph nodes and parotid glands may be involved and should be assessed preoperatively 2
• ALHE demonstrates more varied clinical and histopathologic features, requiring individualized surgical planning based on lesion location and depth 3

Adjuvant and Alternative Management

When Surgery Alone Is Insufficient

• Corticosteroid therapy can be combined with surgical excision, particularly for Kimura disease with extensive involvement 1
• Mycophenolate mofetil represents an emerging steroid-sparing maintenance therapy option, particularly for preventing recurrence after surgical excision 1
• Management is shifting toward immunomodulatory therapy for cases with high recurrence risk or multifocal disease 1

For Unresectable or Multifocal Disease

• Systemic corticosteroids or immunosuppressive agents should be considered first-line for extensive disease 5
• Radiotherapy with electrons can be used for localized lesions that are not surgical candidates 5

Post-Surgical Surveillance

• Long-term follow-up is mandatory given the high recurrence rate and tendency for new lesion development 2
• Monitor for peripheral blood eosinophilia, which may indicate disease activity in Kimura disease 2
• Regular clinical examination of the surgical site and regional lymph nodes is essential 2

Key Pitfalls to Avoid

• Do not proceed without definitive histopathological diagnosis: The clinical and histological overlap between Kimura disease and ALHE necessitates thorough pathological examination before finalizing treatment strategy 6, 4
• Do not assume complete cure after excision: Both conditions have high recurrence rates requiring ongoing surveillance 2
• Do not overlook systemic involvement: Kimura disease may involve regional lymph nodes and parotid glands, requiring comprehensive preoperative assessment 2
• Do not dismiss the need for adjuvant therapy: Given high recurrence rates, consider corticosteroids or immunomodulatory maintenance therapy even after complete excision 1