Primary cutaneous anaplastic large cell lymphoma (C-ALCL) is a rare form of cutaneous T-cell lymphoma characterized clinically by a solitary nodule or tumor that ulcerates, and histopathologically by CD30+ T-cell lymphoproliferation.

This condition typically presents during late adulthood with a median age of 60 at the time of diagnosis and may be associated with underlying immunosuppression. Males are more commonly affected. It demonstrates a chronic, indolent, intermittently recurring clinical course. C-ALCL and lymphomatoid papulosis (LYP) constitute a spectrum of primary cutaneous CD30+ lymphoproliferative disorders, with C-ALCL at one end, the even more indolent LYP at the other, and borderline cases in between.

C-ALCL typically presents as one or a localized cluster of a few rapidly growing nodule(s) with or without secondary ulceration, most commonly on the head, neck, or extremities. Multifocal cutaneous involvement occurs in around one-fifth of cases. Approximately 10%-15% of individuals will experience extracutaneous involvement (most often to the regional lymph node).

Overall, prognosis is excellent with a 5-year survival rate above 90% for patients with involvement limited to the skin. Extensive limb disease, with multifocal lesions on one limb or contiguous body regions, may be associated with a poorer prognosis and thus may require more aggressive treatment. Disease on the head and neck may have a worse prognosis as well.

Breast implant-associated anaplastic large cell lymphoma (BIA-ALCL) can develop in the scar capsule of an implant. The risk of developing this cancer is low, and it is more frequently seen in patients with textured breast implants. It occurs most commonly 7-10 years after implantation. Development of seroma more than 1 year after implantation may be a risk factor for BIA-ALCL development. The majority of cases remain localized and are treatable with implant removal. However, recurrences and systemic dissemination have been seen.

Related topic: non-Hodgkin lymphoma