Primary cutaneous diffuse large B-cell lymphoma, leg type: A report of 4 cases with literature review

Objective: To explore the morbidity, clinical features, metastasis, treatment, and prognostic factors of primary cutaneous diffuse large B-cell lymphoma, leg type (PCDLBCL). Methods: The clinical records of 4 patients treated at the Tianjin Medical University Cancer Hospital from January 1970 to September 2010, including gender, age, site of cutaneous involvement (leg or non-leg, extent of cutaneous involvement, lactate dehydrogenase (LDH, β2-microglobulin (β2-MG), B symptoms, relapse (yes or no), involvement of lymph nodes and organs, treatment and survival were retrospectively reviewed. Results: From January 1970 to September 2010, the incidence of PCLBCL, leg type in Tianjin was 4.8/10 million. Of the 4 patients, 2 were males and 2 were females, aged from 2 to 55 years. The median age was 29 years. The cutaneous nodules or tumors were common clinical features. Of the 4 patients, 3 involved the legs and 1 was non-leg, 3 were localized involvements, and 1 was disseminated involvement. Lymph nodes (1 case) were involved in follow-up visit. Three patients underwent a surgical operation combined with chemotherapy and only 1 underwent only the surgical operation. CHOP or CHOP-like chemotherapy was predominant. The median survival time was only 30.5 months. The mean survival time of the males and females was 25 and 219.5 months, respectively. The mean survival time in terms of the site of cutaneous involvement (leg and non-leg) were 162 and 3 months, respectively. The mean survival times in term of the extent of cutaneous involvement (localized and disseminated) were 158 and 14 months, respectively. The mean survival time in terms of relapse (yes, no) were 30.5 and 214 months, respectively. Conclusion: PCLBCL, leg type is a rare extranodal lymphoma. The clinical manifestations are atypical. The diagnosis of PCLBCL, leg type is confirmed by histopathologic examination. Surgical operation combined with chemotherapy could improve the prognosis, but the prognosis is still poor. The prognosis is probably associated with gender, site of cutaneous involvement, extent of cutaneous involvement, and relapse.