Histopathologic Staging at Initial Diagnosis of Mycosis Fungoides and the Sézary Syndrome

Abstract

A staging system based on histopathologic evaluation of skin, lymph nodes, blood, and visceral sites provides more comprehensive prognostic information than clinical evaluation of skin disease and adenopathy. Patients may be divided at initial presentation into three prognostic groups: good-risk patients, who have plaque-only skin disease without lymph node, blood, or visceral involvement (median survival, greater than 12 years); intermediate-risk patients, who have cutaneous tumors, erythroderma, or plaque disease with node or blood involvement but no visceral disease or node effacement (median survival, 5 years); and poor-risk patients, who have visceral involvement or node effacement (median survival, 2.5 years).

Revisions to the staging and classification of mycosis fungoides and Sézary syndrome: a proposal of the International Society for Cutaneous Lymphomas (ISCL) and the cutaneous lymphoma task force of the European Organization of Research and Treatment of Cancer (EORTC)

“…24,77 But what constitutes "visceral disease" has not been well defined. It is generally asymptomatic and therefore prone to be underdiagnosed, especially in those with more advanced skin involvement.…”

“…The definition and differentiation of patch versus plaque 23 versus tumor lesions 24,25 is more than of just semantic importance because both prognosis and choice of and response to treatment are linked to these different designations. Currently, the distinction between patch and thin plaque lesions and between thick plaque and tumor lesions in MF is quite subjective 4,26 (personal communication, E. Vonderheid, ISCL Workshop San Francisco, 1999).…”

“…However, the prognostic importance of lesser degrees of involvement as defined by light microscopy alone is less clear. 24,59,61,62 Some investigators advocate that the LN 3 rating should be considered equivalent to LN 4 for staging purposes. 13 Although in the context of MF and SS, the survival of patients with an NCI/VA LN 3 node rating is worse than patients with LN 0-2 , 24,62 it must be remembered that findings comparable with LN 3 have been found in nodes from patients with benign disorders 56,60,63 and that the survival of patients with LN 4 is worse than for those with LN 3 .…”

“…24 In 3 studies that have reported on the use of Southern blot technique, which has a 5% detection threshold to show clonal T cells in lymph nodes, 62,64,65 none of the lymph nodes classified histologically as uninvolved (NCI/VA LN 0-1 or Dutch grade 1) had evidence of a clone. However, 13% of LN 2 nodes, 83% of LN 3 nodes, and 100% of patients with LN 4 nodes had clonal rearrangements in one of the studies.…”

“…14,24,26,36,37,52,53 In the MFCG classification, "N" represented only peripheral lymph nodes. Although there was no size designation for "abnormal" nodes, this was not problematic as all patients, even those without palpable nodes, were to have node biopsies as part of the staging evaluation since each N rating for staging was based on both clinical and histopathologic findings.…”