What is Anaplastic Large Cell Lymphoma (ALCL) associated with breast implant?

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SECPRE Guideline on Anaplastic Large Cell Lymphoma (ALCL) associated with breast implantation

What is Anaplastic Large Cell Lymphoma (ALCL) associated with breast implant?
What is Anaplastic Large Cell Lymphoma (ALCL) associated with breast implantation?

Anaplastic large cell lymphoma (ALCL) is a T-cell lymphoma composed of large lymphoid cells. The cells have a CD30+ marker. Most cases are ALK+ (anaplastic large cell lymphoma kinase protein), although ALK- cases are also included in this category. They account for approximately 3% of all adult non-Hodgkin’s lymphomas. The ALK+ type is more frequent in children under 30 years of age and predominates in males. The ALK-negative form is more frequent in the elderly, with no difference between sexes.

Anaplastic giant cell lymphoma (ALCL) has a low prevalence so its diagnosis is complicated and can be confused with other alterations, which is why all patients with breast implants in the past and in the future should know the diagnosis of suspicion.

Forms of presentation of LACG in breast prostheses:

  • The most frequent form of presentation is a sudden increase in volume in one breast due to the development of a seroma (accumulation of clear fluid), usually late (beyond the first year after implantation).
  • Other less frequent forms of presentation are ulceration, the appearance of a nodule, diffuse inflammation, pain or localized dermatological alterations.

The first step to be taken is to request a breast ultrasound including the study of the adjacent lymph nodes, indicating to the radiologist that in the presence of lichen he/she will be obliged to perform an ultrasound-guided puncture. If the ultrasound cannot assess the cause of the increase in volume, a magnetic resonance imaging (MRI) will be requested. If with ultrasound the fluid is accompanied by a mass and/or lymphadenopathy (swollen lymph nodes) and/or skin lesion, a selective biopsy of the lesion in the operating room with sampling of the periprosthetic capsule should be considered.

The material extracted by the puncture should be sent to an anatomopathologist experienced in hematologic disorders for culture, cytology and flow cytometry, always specifying the suspected diagnosis so that CD30 and ALK markers can be analyzed.

After the first assessment and having a positive diagnosis, with the study of the fluid or tissue, the patient should be referred to a specialized unit, composed of plastic surgery, oncology/hematology and pathological anatomy, proceeding to the second step in their assessment.

This second step consists of an extension study by means of a PET-CT scan and a hematologic evaluation with mandatory assessment by bone marrow puncture, to rule out other forms of ALCL that have a more aggressive course.

Once we have the imaging test and if the involvement is limited to the capsule, it will be appropriate to remove the breast implants bilaterally with complete capsulectomy (complete removal of the periprosthetic capsule), referring the tissue and fluid present to study.

If there is involvement outside the breast, it will be necessary to proceed to the removal of the prosthesis with complete capsulectomy in both breasts and excision with oncologic margins of the masses or lesions that are present, as well as the affected lymph nodes. The study of the sentinel lymph node has no diagnostic value.

Once the extension study and excision have been performed, the presentation will be differentiated:

  • Intracapsular involvement (only lichen) or localized disease, proceeding to reviews by hematologists/oncologists every 6 months with PET-CT for 2-4 years according to protocol. No additional treatment is required.
  • Extramammary involvement, chemotherapy treatment should be complemented according to individual assessment. Radiotherapy has not been shown to have therapeutic validity. It will be evaluated in cases of incomplete excision.

We recommend reconstruction with autologous technique or lipofilling. It is mandatory to REPORT the case to the AEMPS (Spanish Medicines Agency).

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