Objective  To explore the clinical presentation and therapy of primary cutaneous anaplastic large cell lymphoma (PCALCL).

  Methods  We reviewed and analyzed six cases of PCALCL who were treated at our hospital from January 2009 to December 2012.

  Results  The subjects were three males and three females with a median age of 54 years old (range: 38 years to 60 years). Three patients had a single subcutaneous nodule and three had multiple nodules. The subcutaneous lesions appeared red, solid, and stable nodules. Some patients developed pruritus around the lesions, or developed more nodules, which coalesced with other nodules. Some of the lesions exhibited partial spontaneous regression and new nodules appeared at the same site or at different sites. Finally, the lesions changed color from red to brown. The surfaces of some lesions developed ulcerations. Only 1 case had superficial lymph node metastasis. Three cases with single lesions received surgical excision plus radiotherapy, chemotherapy, or radiochemotherapy; all three cases survived and achieved complete remission. The three cases with multiple lesions mainly received systemic chemotherapy, with two of the cases recurring and one surviving with complete remission. The median follow-up was 24 months (range: 11 months to 35 months), with a progression free survival of 66.7% and an overall survival of 100%.

  Conclusion  Surgical excision or radiotherapy provides satisfactory control in patients with single lesions. Chemotherapy may be prescribed to patients with multiple lesions or with extracutaneous metastasis.