Turton 2021 - JPRAS - BIA-ALCL

Summary

Breast implant-associated anaplastic large cell lymphoma (BIA-ALCL) is a rare type of lymphoma that can develop in the scar tissue surrounding breast implants. It usually presents 8-10 years after implant surgery with swelling, pain, or lumps in the breast.

Diagnosis requires aspiration of implant fluid or biopsy of capsular tissue. Cells are examined microscopically and tested for markers like CD30 positivity and ALK negativity.

Treatment involves surgical removal of the implant and surrounding capsule. This is usually curative for early stage disease confined to the capsule.

More advanced BIA-ALCL may require systemic chemotherapy, monoclonal antibody therapy, radiation, or stem cell transplant. The CHOP regimen is commonly used. Brentuximab vedotin was recently approved for frontline treatment.

Most patients have an excellent prognosis with early diagnosis and treatment. However, some cases have progressed rapidly leading to poor outcomes.

These guidelines aim to raise awareness and promote early recognition, diagnosis, and management of BIA-ALCL to improve outcomes. Key recommendations include referral to specialist centers, multi-disciplinary input, and en-bloc capsulectomy.

Diagnosing BIA-ALCL:

Algorithm involves clinical exam, imaging, and cytology/biopsy to evaluate peri-implant effusion or masses.

Imaging: Ultrasound is initial test of choice to assess effusion. Mammography if over 40yrs. MRI for further evaluation or surgical planning. PET/CT for staging.

Cytology: Drain entire effusion volume. Check for abnormal/atypical lymphocytes. If suspicious, do immunohistochemistry for confirmation.

Biopsy: Core biopsy of any capsular masses. Multiple sections of capsule needed to detect microscopic disease.

Send samples to specialist center and hematopathologist for analysis.

Managing BIA-ALCL:

Refer to specialist center. Discuss in MDT. Report to regulators.

Early stage 1-2A: Surgical excision with en-bloc capsulectomy is first-line treatment. Ensures diagnosis and local control.

Advanced stage 2B-4: Systemic chemo/immunotherapy needed. CHOP regimen commonly used. Brentuximab vedotin recently approved for frontline use.

Autologous stem cell transplant can be considered for consolidation after systemic therapy.

Radiation is recommended if margins positive after surgery or incomplete excision. 24-36 Gy doses proposed.

Bilateral capsulectomy often done for contralateral staging. Can delay reconstruction initially.

Follow up clinically for minimum 2 years. Routine imaging surveillance not recommended.

Overall, guidelines promote a systematic approach to ensure early diagnosis, appropriate specialist input, surgical excision, and systemic therapy if needed to improve outcomes.