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.
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Terminology
In BIA-ALCL, the peri-implant fluid is referred to as an effusion rather than a 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 1000 women with breast implants 8,10. Less than 1000 cases have been reported (c. 2019) 11. The vast majority of cases are associated with textured breast implants 8,10.
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
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 8,10,13.
While late onset effusion is very strongly associated with malignancy and should be investigated, the incidence of malignancy in late onset effusion was shown in a meta-analysis to be ~1.2% 14.
Pathology
BIA-ALCL is a T-cell non-Hodgkin lymphoma grouped with other anaplastic large cell lymphomas in the WHO classification of haematolymphoid tumors (5th edition) 12.
Two subtypes are recognized based on morphology 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.
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 BIA-ALCL 3,11. It typically demonstrates a fluid collection between the breast implant and the capsule, and 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-ALCL 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 microbiology 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
Practical points
5-10 mL of peri-implant fluid can be considered normal in an asymptomatic patient 11
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