National Comprehensive Cancer Network
This is a quick summary of the guideline without analysis or commentary. For more information, go directly to the guideline by clicking the link in the reference.
February 27, 2019
The guidelines on breast implant–associated anaplastic large cell lymphoma (BIA-ALCL) were released in January 2019 by the NCCN.
Diagnosis and Workup
The most common presentation of BIA-ALCL is a large spontaneous periprosthetic fluid collection occurring at least 1 year and on average 7 to 10 years following cosmetic or reconstructive implantation of a textured-surface breast implant.
Less commonly described presentations (< 5% of cases) include skin rash, fevers, and capsular contracture. Patients with a large fluid collection may have fluid levels around an implant.
Initial workup of an enlarged breast should include ultrasound evaluation for fluid collection, breast masses, and enlarged regional lymph nodes. If ultrasound findings are equivocal, magnetic resonance imaging is recommended for further characterization.
Fine needle aspiration is the optimal method to sample a periprosthetic fluid collection. Ultrasound guidance may aid in implant displacement and protection during aspiration. As much fluid as possible (minimum 50 mL) should be collected.
If a suspicious mass is visualized, tissue biopsy and evaluation is required. Specimens should be sent for cell morphology by cytology, CD30 immunohistochemistry, and flow cytometry for evaluation, quantification, and characterization of T cells within the specimen.
Additional biomarkers that may be required to establish the diagnosis and exclude other malignancies include CD2, CD3, CD4, CD5, CD7, CD8, CD45, and anaplastic lymphoma kinase (ALK) expression.
Scant or rare CD30 positive lymphocytes with normal morphology is considered a normal finding; treat as benign seroma.
Once the diagnosis of BIA-ALCL has been established, consultation with a multidisciplinary team including oncologists, pathologists, surgical oncologists, and plastic surgeons is strongly encouraged.
Suggested laboratory testing includes a complete blood count with differential, comprehensive metabolic panel, lactate dehydrogenase, and hepatitis B testing (if adjuvant chemotherapy is being considered). Bone marrow biopsy is suggested when systemic ALCL is strongly suspected (eg, patients with aggressive local invasion or lymph node metastasis).
A preoperative positron emission tomography computed tomography (PET/CT) scan is optimal for demonstrating associated capsular masses and chest wall involvement and will serve as a “roadmap” for surgical excision.
En bloc resection—with removal of the implant, the surrounding fibrous capsule, and any associated capsule mass—is the recommended goal of surgery.
Surgical specimens should be oriented and inked to allow for the anatomic location of the diseases. This is important for tumor site surveillance and in cases of recurrence requiring re-excision.
At present, there is no clear role for radical mastectomy or sentinel lymph node biopsy.
Disease localized to the capsule may be treated with surgery alone in most cases if complete surgical excision is possible.
Local or involved site radiation therapy with 24 to 36 Gray (Gy) is suggested for patients with local residual disease, positive margins, or unresectable disease with chest wall invasion.
Systemic therapy is warranted in patients with Lugano stage II-IV or MD Anderson Cancer Center stage IIB-IV disease. Either a standard approach for systemic ALCL (eg, combination anthracycline-based
chemotherapy [CHOP, daEPOCH]) or (preferred) a combination with brentuximab vedotin is recommended.
Patients who have a complete treatment response can be monitored with history and physical every 3 to 6 months for 2 years and then as clinically indicated. The role of routine radiographic surveillance is unclear, but either a chest/abdominal/pelvic CT scan with contrast or PET scan could be considered every 6 months for 2 years, then only as clinically indicated.