Clinical characteristics, differential diagnosis, and treatment outcome of subcutaneous panniculitis-like T-cell lymphoma: a literature review of published Japanese cases.

BACKGROUND: Subcutaneous panniculitis-like T-cell lymphoma (SPTCL) is a rare subtype of non-Hodgkin lymphoma that shows phenotypic features of cytotoxic T cells and preferentially involves subcutaneous tissue. SPTCL is believed to show an indolent clinical course, unless patients develop haemophagocytic syndrome. Various reported therapies include corticosteroids, immunosuppressive drugs, and chemotherapies. The use of chemotherapy as a first-line treatment remains controversial, and treatment approaches for SPTCL have not been established yet.

OBJECTIVES: To investigate the clinicopathological features, treatment modalities, and outcomes of Japanese patients with SPTCL.

MATERIALS & METHODS: We performed a literature review of Japanese cases of SPTCL.

RESULTS: Twenty-two cases have been reported in the English and Japanese literature. Six cases were excluded due to a lack of sufficient clinical and immunohistological data, and treatment modalities and outcome were available in 16 cases. Clinical characteristics of the Japanese cases were generally similar to those of Western countries. Approximately half of the Japanese patients were treated initially with corticosteroids. Among them, more than half subsequently received chemotherapies due to a lack of response or recurrence of the disease. Overall, chemotherapy was used for approximately 80% of reported cases as a primary or secondary therapy.

CONCLUSION: Our findings suggest that corticosteroids may be beneficial for some SPTCL patients, but most patients required chemotherapy during the course of their disease. Further investigations are needed to both establish appropriate treatment strategies for SPTCL and clarify predictive factors in order to identify patients who may benefit from corticosteroid therapy as a primary treatment.