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 .
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 . Less than 1000 cases have been reported (September 2019) . The vast majority of cases are associated with textured breast implants .
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 .
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 .
Pathology
BIA-ALCL is a T-cell non-Hodgkin lymphoma with two subtypes :
- 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 . 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)
- factors that have been shown not to alter risk include
- indication: primary augmentation vs reconstruction
- type: saline vs silicone
- location: retroglandular vs retropectoral
Staging
It can be staged using the TNM system :
- 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 .
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 . Nodal disease (axillary, supraclavicular, mediastinal, and/or internal mammary groups) may rarely be involved . Ultrasound is the first-line modality followed by MRI; mammography demonstrates non-specific findings .
Ultrasound
Ultrasound has high sensitivity (84%) for BI-ALCL . 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 . Peri-implant breast parenchymal inflammation may also be seen .
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 .
Nuclear medicine
PET/CT is not able to distinguish between benign and malignant peri-implant effusions due to the low cellularity of BIA-ACLC .
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 . At least 50 mL of fluid should be aspirated for MC&S and cytology .
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 . Patients with the peri-implant effusion subtype have a better prognosis than those with peri-implant mass subtype or advanced disease .
History and etymology
The first case of BIA-ALCL was reported in 1997 by Keech and Creech . The association with breast implants was suggested in 2008 by de Jong et al and Roden et al .
Differential diagnosis
Practical points
- 5-10 mL of peri-implant fluid can be considered normal in an asymptomatic patient