Abstract:

ALK-negative anaplastic large cell lymphoma (ALCL) is a distinct, provisional entity in the WHO classification. Based on sites of involvement, ALCL is classified as systemic, primary cutaneous, or breast implant-associated ALCL (BIA-ALCL). Since its original description in 1997, 733 cases of BIA-ALCL have been reported. Most cases of BIA-ALCL present as a peri-implant seroma, breast swelling/pain, or peri-implant mass. Only a few cases of cutaneous involvement by BIA-ALCL are known to date. We present a case of a 48-year-old female with a history of breast cancer who underwent nipple-sparing mastectomy with breast implant reconstruction. At five-year post-op, patient complained of a painful, ulcerated papule on left nipple. A punch biopsy revealed a nodular and diffuse infiltrate of atypical cells with perivascular accentuation that demonstrated positivity for GATA-3, CD30, CD4 and CD56. ALK, EBER, CD20, and breast cancer markers were negative. A working diagnosis of a CD30+ T-cell lymphoma was established with the differential including primary cutaneous ALCL versus BIA-ALCL. Initial PET revealed a reactive lymph node in left axilla without other abnormalities. The patient underwent capsulectomy without any evidence of seroma or atypical infiltrate within the peri-capsular skin and lymph nodes. This is a unique case of ALK(-) ALCL confined to the skin in a patient with breast implants. While this could certainly represent a primary cutaneous ALCL, the relationship with breast implants is suspicious for an unusual, extra-capsular manifestation of BIA-ALCL. Increased awareness and recognition of cutaneous manifestations of BIA-ALCL is needed among dermatopathologists in diagnosing atypical breast lesions.

Financial Disclosure:
No current or relevant financial relationships exist.

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Kseniia Malkova, MD