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ABSTRACT & COMMENTARY
Synopsis: The prognostic factors predictive of survival outcome in patients with extracutaneous mycosis fungoides (MF) and the influence of initial skin involvement on the risk of progression to extracutaneous disease were evaluated in a study of 112 patients with extracutaneous disease at presentation or with progression and 468 patients with initial cutaneous disease only. Prognostic factors important in the cutaneous stage of disease, such as sex, race, age, extent of skin involvement, and peripheral blood Sezary cell involvement, were no longer significant to survival outcome once extracutaneous disease develops. The type and extent of skin involvement were important predictors of risk for developing extracutaneous disease in patients with initial cutaneous disease. Novel approaches to therapy are needed, as patients with extracutaneous MF have a median survival of only 13 months.
Source: de Coninck EC, et al. J Clin Oncol 2001;19:779-784.
Mycosis fungoides (mf) is a cutaneous t-cell lymphoma (CTCL) of CD4+ T-cells with a usual initial presentation in the skin. This disease is rare, with an overall incidence of approximately four in 1,000,000.1 The prognosis for patients with cutaneous disease depends on both the type and extent of cutaneous involvement. The staging for this disease takes into account the importance of different types of skin involvement, with types of skin involvement including suspicious lesions (T0), limited plaques or papules (T1), generalized plaques or papules (T2), tumors (T3), and generalized erythroderma (T4).1 Patients with early stages of disease can have indolent, scaling patches or plaques, while patients with more advanced disease have tumors and generalized erythroderma. Additional aspects of staging include the presence of nodal involvement and the presence of visceral involvement. While several investigators have evaluated prognostic variables for patients with cutaneous MF, similar studies have not been performed for patients with extracutaneous MF.
This study by de Coninck and colleagues evaluated 112 patients with extracutaneous MF either on presentation (n = 37) to the clinic or with progression from stage I-III of disease (n = 75). In addition, another 468 patients seen in the Stanford Mycosis Fungoides Clinic within six months of their initial diagnosis were evaluated in the risk for progression analysis. The median survival from the first treatment for extracutaneous disease was 13 months (range, 8 days-more than 235 months). The survival of patients with extracutaneous MF was evaluated for prognostic groups of importance for patients with cutaneous disease, including age, sex, race, extent of skin involvement, clinical stage, and peripheral blood Sezary involvement. These prognostic factors for cutaneous MF were no longer of prognostic value for patients with extracutaneous MF. The patients received various treatments, and a complete clinical response (CR) was only obtained in 11 patients (10%). However, patients who achieved a CR had a significantly longer median survival than patients with a partial response or patients with no response (1.70 vs 0.91 years, P = 0.047; and 1.70 vs 0.57 years, P = 0.011). No correlation between specific treatment and tumor response could be made.
de Coninck et al also evaluated factors related to risk for progression in 468 patients seen within six months of their initial diagnosis. The important prognostic value of size and extent of disease were again confirmed. No patients who initially presented with T1 disease developed stage IV disease, while this outcome occurred in 2% of patients presenting with T2 disease, 13% of patients presenting with T3 disease, and 25% of patients presenting with T4 disease.
Comment by Mark R. Albertini, MD
Patients with extracutaneous MF have a poor prognosis, and novel treatment strategies are needed for clinical investigation for these patients. In addition, identification of prognostic factors for these patients is important for appropriate interpretation of studies evaluating systemic treatments. While several prognostic variables including age, sex, race, extent of skin involvement, clinical stage, and peripheral blood Sezary involvement are important for cutaneous MF, these factors are not of prognostic value once extracutaneous disease is present. Thus, identification of alternative prognostic markers is of importance. Several investigators have been evaluating the use of PCR to evaluate T-Cell Receptor (TCR) gene rearrangement in cutaneous MF tumors and blood.2 The identification of a clonal T-cell population in cutaneous T-cell infiltrates has been used to help establish the diagnosis of CTCL.2 Identical T-cell clones can also be identified in blood and in cutaneous T-cell infiltrates of MF patients.2 However, determining whether an identical T-cell clone in blood and skin infiltrates is related to advanced disease and a poor prognosis, or corresponds to early circulation of malignant T-cells, will likely require a prospective trial.
The current markers of extent of skin involvement and peripheral blood Sezary cell involvement are of prognostic value in cutaneous MF. However, accurate prognostic factors for patients developing extracutaneous MF are needed. The prognostic value of potential prognostic markers, such as identification of identical T-cell clones in blood and cutaneous infiltrates, should be evaluated in prospective clinical studies.
1. Wilson LD, et al. Cutaneous T-Cell lymphomas. In: DeVita VT, Hellman S, Rosenberg, SA, eds. Cancer: Principles and Practice of Oncology, Vol 2, 5th ed. Philadelphia, Pa: Lippincott Williams & Wilkins; 1997: 2220-2232.
2. Theodorou I, et al. Blood 1995;86:305-310.