Breast implant-associated anaplastic large cell lymphoma

Breast implant-associated anaplastic large cell lymphoma (BIA-ALCL) is a rare form of T-cell primary breast lymphoma that has primarily been associated with textured breast implants. 

Terminology

In BIA-ALCL, the peri-implant fluid is referred to as an effusion rather than seroma, as the latter is acellular ¹¹.  

Epidemiology

The entity is rare, with a reported prevalence of between 0.3 in 100,000 to 1 in 1,000 women with breast implants ^8,10. Less than 1000 cases have been reported (September 2019) ¹¹. The vast majority of cases are associated with textured breast implants ^8,10.

Clinical presentation

Patients may complain of breast swelling, pain, or asymmetry. Clinical breast examination usually reveals a fluid collection (effusion) or mass. Two-thirds of patients present with a late-onset effusion (>1 year from the surgery) and one-third present with a mass ¹⁰.

The time of onset is at least a year following breast augmentation surgery. The average time of presentation is 8-10 years after implant placement ^8,10.

Pathology

BIA-ALCL is a T-cell non-Hodgkin lymphoma with two subtypes ^10,11:

• peri-implant effusion
• peri-implant mass

Etiology

The exact etiology remains unclear, however, it is widely thought to be multifactorial in nature, due to a combination of chronic inflammation, implant texture and a subclinical infective pathology related to the formation of a biofilm ¹⁰. The end result is thought to be the malignant transformation of T-cells, which become anaplastic lymphoma kinase (ALK) negative and CD30 positive. 

Risk factors

• textured implant surface (vs smooth implant surface) ¹⁰
• factors that have been shown not to alter risk include ¹⁰
  • indication: primary augmentation vs reconstruction
  • type: saline vs silicone
  • location: retroglandular vs retropectoral

Staging

It can be staged using the TNM system ¹⁰:

• stage 1: confined to the external fibrous capsule
• stage 2: extracapsular mass (locally advanced disease)
• stage 3: regional and distant metastases

The Lugano classification and Deauville scale have not been validated for BIA-ALCL ¹¹.

Radiographic features

Patients most commonly manifest with a peri-implant effusion (range between 50-1000 mL) only, while less commonly they present with a breast mass +/- effusion ^8,10. Nodal disease (axillary, supraclavicular, mediastinal, and/or internal mammary groups) may rarely be involved ¹⁰. Ultrasound is the first-line modality followed by MRI; mammography demonstrates non-specific findings ¹¹.

Ultrasound

Ultrasound has high sensitivity (84%) for BI-ALCL ^3,11. Sonography typically demonstrates a fluid collection between the breast implant and the capsule; septa are often seen. If a mass is present it is typically solid, hypoechoic and well-circumscribed but without hypervascularity; complex cystic masses have also been reported ¹¹. Peri-implant breast parenchymal inflammation may also be seen ¹¹.

MRI

BIA-ALCL related effusions and masses may be appreciated on MRI. Capsular enhancement has also been reported in a small number of cases as has evidence of implant rupture ^ref.

Nuclear medicine

PET/CT is not able to distinguish between benign and malignant peri-implant effusions due to the low cellularity of BIA-ACLC ¹¹.

Treatment and prognosis

On initial workup, tissue sampling should be undertaken, including aspiration of the effusion and/or fine-needle aspiration or core needle biopsy of the mass if present ⁸. At least 50 mL of fluid should be aspirated for MC&S and cytology ¹¹.

Management typically involves a complete en-bloc capsulectomy and exploration of the prosthesis with patients subsequently receiving some form of chemotherapy and/or radiotherapy depending on the extent of disease ⁹. Patients with the peri-implant effusion subtype have a better prognosis than those with peri-implant mass subtype or advanced disease ¹¹.

History and etymology

The first case of BIA-ALCL was reported in 1997 by Keech and Creech ⁶. The association with breast implants was suggested in 2008 by de Jong et al and Roden et al ¹¹.

Differential diagnosis

• seroma
• liquefied hematoma
• primary breast cancer

Practical points

• 5-10 mL of peri-implant fluid can be considered normal in an asymptomatic patient ¹¹