[Primary cutaneous anaplastic large cell lymphoma: clinical presentation, therapy and prognosis study of 10 cases].

Objective: To explore the clinical presentation, therapy and prognosis study of primary cutaneous anaplastic large cell lymphoma (PCALCL).
Methods: We reviewed and analyzed ten cases of PCALCL receiving treatment at our hospital from January 1999 to January 2009.
Results: There were 8 males and 2 females with a median age of 48 years old (range: 22 - 69). There were single subcutaneous nodule (n = 7) and multiple nodules (n = 3). And the lesions could be found on head and neck (n = 5), trunk (n = 3) and all over body (n = 2). The lesions appeared red, solid and stable subcutaneous nodules. Partial lesions had a spontaneous regression and new nodules appeared at the same or different sites. Two patients had lymphadenopathy and one had bone involvement with anaplastic lymphoma kinase (ALK) positive and high cell proliferation ratio index (ki-67 > 80%). Seven cases with single lesion received surgical excision plus radiotherapy, chemotherapy or radiochemotherapy, one case recurred, six cases survived without disease. Three cases with multiple lesions received systemic chemotherapy mainly in combination with radiotherapy or biotherapy, two cases recurred and one case survived without disease. The median follow-up was 44 months (range: 9 - 95), progression free survival 89% and overall survival 100%.
Conclusion: PCALCL is found more commonly in males. Visceral and lymph node involvement are rare. The patients with single lesion have a longer disease-free survival than those with multiple lesions after surgical excision in combination with chemotherapy or radiotherapy. Multiple lesions can not be cured.