Cutaneous B‐cell lymphoma: Pathological spectrum and clinical outcome in 51 consecutive patients

Abstract

The clinical behavior and optimal treatment of patients presenting with skin infiltration by B-cell lymphoma have not been established. To clarify this we assessed the clinical and laboratory features of 51 patients presenting with cutaneous infiltration by B-cell lymphoma. Follow-up data was available for 46 patients with a median age of 68 years (range 16-89 years) and a median follow-up of 32.5 months (range 5-123 months). Thirty-three of 51 (65%) patients had diffuse large B-cell lymphoma (DLBCL), and 15/51 (29%) had marginal zone lymphoma (MZL). The remaining 3 patients had follicular lymphoma, CLL, and post-transplant lymphoproliferative disease. Of the 33 patients with DLBCL, follow-up was available in 29; 24/29 (83%) had primary cutaneous disease, which was unifocal in 17/24 (71%). Following treatment, 8/24 (33%) of the primary cases relapsed. Of the 8 who relapsed, 7 had received local forms of treatment only. Follow-up data was available in 14/15 patients with MZL. 11/14 (79%) had primary cutaneous disease, which was unifocal in 8 (73%). Following treatment, 4 of these cases relapsed (36%); all had received local therapy only. It is evident from this study that a significant proportion (~20%) of patients who present with cutaneous infiltration by B-cell lymphoma have systemic disease. Staging is therefore mandatory in these patients. Approximately 1/3 patients with primary cutaneous DLBCL or MZL ultimately relapse, and relapse rates appear higher in thosepatients receiving local therapy only. Systemic or combined modality therapy may therefore be the most appropriate treatment at presentation. This should be assessed prospectively in randomized trials. Am.

European Organization for Research and Treatment of Cancer and International Society for Cutaneous Lymphoma consensus recommendations for the management of cutaneous B-cell lymphomas

“…Multiagent chemotherapy, mostly cyclophosphamide, doxorubicin (Adriamycin), oncovin (Vincristine), prednisone (CHOP), was administered to 33 PCMZL patients reported in the literature, again mostly patients with multifocal skin lesions. 21,25,30,40 CR rate was 85% and a relapse rate of 57% was found.…”

“…There was a total number of 14 patients with a PCMZL in these studies, all with multifocal skin lesions. 23,25,31,39 The response rate was 100% with a CR in 9 patients (64%) and a partial remission in the other 5 patients. Of the 9 patients with a CR, 3 patients showed relapses (33%), one of whom also with extracutaneous localizations.…”

“…[21][22][23][24][25][26][27][28][29][30] In nearly all cases (130 of 132; 99%), a CR after initial therapy was reached. Sixty patients (46%) showed one or more relapses, which were mostly confined to the skin.…”

“…In the literature, 75 patients treated with surgical excision have been reported. 21,25,[29][30][31][32] A CR was reached in all but one case. 30 However, 32 patients (43%) developed new skin lesions.…”

Clinical implication of genome-wide profiling in diffuse large B-cell lymphoma and other subtypes of B-cell lymphoma

“…PCFCCL demonstrated more localized disease with histological features showing a spectrum of centrocytes and centroblasts in diffuse or follicular patterns. [59] PCFCCL showed more favorable prognosis (five-year survival of 95%) than PCLBL-leg (five-year survival of 50%). GEP of PCLBCL demonstrated the distinction between these two subtypes of neoplasm at molecular levels.…”