Primary cutaneous anaplastic large cell lymphoma
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
C86.6 – Primary cutaneous CD30-positive T-cell proliferations
397352006 – Primary cutaneous anaplastic large cell lymphoma
Differential Diagnosis & Pitfalls
- Mycosis fungoides (MF) with large cell transformation – Transformed MF may present with large CD30+ T cells. While histopathologic and immunochemical findings may be similar, clinical features and disease outcome are helpful for distinguishing the two entities. Transformed MF typically involves the trunk with multifocal lesions, is more common in patients older than 60, and exhibits a more aggressive course with early progression and no spontaneous regression.
- Systemic anaplastic large cell lymphoma with cutaneous involvement
- Lymphomatoid papulosis
- B-cell lymphoma
- Leukemia cutis
- Lichen planus
- Cutaneous metastatic carcinoma
- Cutaneous metastatic melanoma