Is breast conservative surgery safe in patients with scleroderma?

Breast Conservative Surgery in Patients with Scleroderma

Breast conservative surgery is generally contraindicated in patients with scleroderma due to poor tolerance of radiation therapy, which can lead to severe breast fibrosis, retraction, and pain requiring subsequent mastectomy. 1, 2

Contraindications to Breast Conservation Therapy

Absolute Contraindications

• Pregnancy (first and second trimester) 1
• Two or more primary tumors in separate quadrants of the breast 1
• Diffuse malignant-appearing microcalcifications 1
• History of prior therapeutic irradiation to the breast region 1
• Persistent positive margins after reasonable surgical attempts 1

Relative Contraindications

• Collagen vascular disease, particularly scleroderma and active lupus erythematosus 1
  • Most radiation oncologists consider scleroderma an absolute contraindication to breast conservation therapy 1
  • Patients with collagen vascular disease tolerate irradiation poorly 1, 3

Evidence Against Breast Conservation in Scleroderma

• Case reports document extremely poor cosmetic outcomes and severe complications in scleroderma patients receiving breast radiation therapy 3
• Patients with scleroderma who undergo radiation therapy experience:
  • Severe breast fibrosis 2, 3
  • Breast retraction and hardening 3
  • Significant pain 3
  • Progression of systemic scleroderma symptoms 3
  • These complications can occur as early as 1-4 months post-radiation therapy 3

Pathophysiological Mechanism

• Scleroderma is a chronic systemic disease characterized by:
  • Severe inflammation 3
  • Autoimmune components 3
  • Excessive collagen deposition in tissues 2, 3
• Radiation therapy appears to exacerbate these processes, leading to:
  • Accelerated fibrosis within the radiation field 3, 4
  • Potential systemic progression of the collagen vascular disease 3

Radiation-Induced Scleroderma

• Even in patients without pre-existing scleroderma, radiation can induce localized scleroderma in approximately 1 in 500 breast cancer patients 4
• Radiation-induced scleroderma typically presents with:
  • Erythema and violaceous discoloration 4
  • Induration and retraction of breast tissue 4
  • Progressive pigmentation 4
  • Symptoms contained entirely within the radiation treatment field 4

Alternative Treatment Approaches

• Mastectomy should be considered the primary surgical approach for breast cancer patients with scleroderma 2, 3
• For patients with localized scleroderma of the breast requiring reconstruction:
  • Tissue expansion has been successfully used 5
  • This approach may help avoid additional scarring and functional loss associated with autologous tissue transfer 5

Clinical Decision Algorithm

1. Confirm diagnosis of scleroderma 2, 3
2. Evaluate extent and activity of scleroderma 2, 3
3. Consider mastectomy as primary surgical approach 2, 3
4. If breast reconstruction is desired, consider tissue expansion techniques 5
5. Avoid radiation therapy due to high risk of severe complications 2, 3

Common Pitfalls and Caveats

• Failing to recognize scleroderma as a contraindication to breast conservation therapy 1, 2
• Underestimating the severity of radiation-induced complications in scleroderma patients 3
• Not distinguishing between different collagen vascular diseases (rheumatoid arthritis is not a contraindication, while scleroderma is) 1
• Confusing radiation-induced scleroderma with cellulitis or recurrent breast cancer 4