Teaching Case

A 65-year-old woman presented with a diffusely swollen and red-coloured right breast for four months. A week following presenting to clinic, the patient began to experience increased focal sensitivity in the right breast and axilla. Additionally, she complained of pain in her right upper arm. The patient did not have any other clinically relevant history other than bilateral silicone prosthesis implanted more than 20 years prior. No abnormalities were found at the last populational screening for breast cancer eight years prior.

First, a bilateral mammography was performed, which showed side size reduction of the prosthesis surrounded by a dens rim on the right, compared to the mammography 8 years prior. Ultrasound demonstrated multiple extensive masses and a minimal amount of fluid surrounding the prosthesis. There was one suspect axillary lymph node. In the area of complaints in her right upper arm no abnormalities were found on ultrasound.

MRI showed multiple nodular masses with rim enhancement and without diffusion restriction both cranially and caudally of the prosthesis. One slightly enlarged lymph node was observed in the right axilla.

Multiple 14G histological biopsies were taken of the mass and fine needle aspiration (FNA) of the lymph node was taken. No FNA was taken of the fluid.

PA biopsy CD30 staining  of the mass was positive for large cell anaplastic T-cell lymphoma (ALK immunohistochemistry negative).

PA cytology lymph node showed maligned cells.

FDG PET-CT showed infiltrated fat and multiple masses with diffuse FDG accumulation surrounding the prosthesis and extending intramuscularly towards the right shoulder, upper arm and the elbow. One axillary lymph node showed increased FDG uptake, confirming metastatic spread.

Background

Earlier case reports (no. 17436, 16769 and 12537) published on EURORAD already showed that breast implant-associated anaplastic large cell T-cell lymphoma (BIA-ALCL) is an exceptionally rare ALK-negative T-cell lymphoma.[1] Pathohistological BIA-ALCL is considered an immune system cancer. It is hypothesized that a bacterial biofilm elicits an inflammatory immune response resulting in malignant transformation of cells surrounding the textured implants.[2] BIA-ALCL is divided in a peri-implant effusion-type (late-onset effusion >1 year after implant, 85% of the cases), or less common a peri-implant mass-type (palpable mass, infiltration, lymph nodes and/or effusion, 15% of the cases). Locally advanced or metastatic disease as in this case is seldomly seen.[2,3,4]

Clinical Perspective

Nearly all BIA-ALCL cases develop a fibrous capsule around the textured breast implants, and usually presents with unilateral swelling, mostly due to effusion.[3] Axillary lymphadenopathy is described in up to 15% of cases, and 10% to 20% of the patients present with a breast mass invading and extending the capsule around the implant. Cutaneous lesions and B symptoms are far less common.[5,6] The peri-implant effusion-type has a median overall survival rate up to 93% at 3 years and of 89% at 5 years.[7] The peri-implant mass-type has a significantly worse prognosis with a disease-related mortality as high as 40% in 2 years.[8]

Imaging Perspective

Evaluation should always include ultrasound. To evaluate the peri-implant effusion type, ultrasound has a better sensitivity and specificity for evaluations of fluid collections compared to MRI, CT and PET.[9] The peri-implant mass-type has preference for MRI and PET to evaluate local infiltration, mass forming, chest wall involvement, systemic spread to regional or distant lymph nodes and is essential in establishing if disease is already disseminated.[1] Mammography is not considered an acceptable imaging modality.[10] Although imaging can give a highly likely diagnosis, histological biopsy of a mass and fine-needle aspiration of fluid (and lymph nodes) is crucial for diagnosis and a positive CD30 staining is required to confirm the diagnosis.

Outcome

Currently, there is no standard treatment. The peri-implant effusion type is in most cases curable with surgery (total capsulectomy) and optional adjuvant radiation and/or systemic chemotherapy. The peri-implant mass-type (advanced, widespread) BIA-ALCL is rare and there is no standard of care. Surgical resection (total capsulectomy including excision of necessary lymph nodes) and combination chemotherapy treatment, following the same treatment principles of systemic ALK-negative ALCL, are most suitable.[1,6,11]

Take-Home Message / Teaching Points

(Infiltrating) BIA-ALCL is an extremely rare subtype of non-Hodgkin T-cell lymphoma. A proper workup and expert multidisciplinary consultation are essential for appropriate diagnosis and therapy.

Written informed patient consent for publication has been obtained.