Primary Cutaneous CD30+ Anaplastic Large Cell Lymphoma: Analysis of the Stanford Series Reveals Two Clinical Subsets of Patients.

Primary cutaneous CD30+ anaplastic large cell lymphoma (PCALCL) represents the malignant end of the spectrum of CD30+ lymphoproliferative disorders. The management of PCALCL is markedly different than that of its primary nodal counterpart. The basis of this difference is due to the less aggressive nature of PCALCL. There is no evidence that chemotherapy is necessary or superior to the excellent results of local therapies such as radiation and/or excision for unilesional or local disease. Even patients with generalized disease have done well in some series. Here we review the characteristics and management of 40 cases of this rare cutaneous lymphoma. We have excluded Lymphomatoid Papulosis (LyP), the benign counterpart of PCALCL. In our series PCALCL was defined as CD30+ ALCL with disease limited to the skin as defined by a negative CT scan. Bone marrow biopsy was not necessary although it was always negative when done. Unilesional, local and extensive regional patients were generally treated with radiotherapy and/or excision, while chemotherapy and biologics were reserved for extensive regional and multifocal patients. The study included 30 men and 10 women with a mean age of 57.5 years (range 24–86). The estimated Kaplan-Meier disease specific survival (DSS) at 4 years for the entire group is 75.6%, with a median follow-up of 27.5 months (1–213). Twenty-six patients presented with unilesional or local disease, while there were 8 multifocal and 6 extensive regional patients (defined as 2 or more lesions beyond a 15 cm2 area apart but limited to 1 or 2 contiguous body regions). The unilesional/local patients had an estimated 4 year DSS of 93%. All but 2 of these patients are alive. The estimated Kaplan-Meier 4 year DSS of patients with either multifocal or extensive regional disease is 30%. Estimated 4 year Kaplan-Meir survival of PCALCL by extent of skin involvement unilesional/local 93% multifocal/extensive regional 30% P-value<0.001 Seven deaths occurred in this series of which 6 were either directly or indirectly attributable to PCALCL. Of the deaths 3 were among the 6 patients with extensive regional disease and 2 were among the 8 patients with multifocal disease. Furthermore, 4 multifocal and 2 extensive regional patients have active disease at the time of last follow-up. Further followup will determine their eventual outcome. The 2 patients with unilesional disease who died, both developed rapid progression to extensive regional disease. Although most patients with PCALCL present with local disease and do quite well with local therapy, it appears that some PCALCL patients with multifocal disease, extensive regional disease, and rapidly progressive unilesional disease behave as high grade lymphomas refractory to standard chemotherapy.