Survival Outcomes and Prognostic Factors in Mycosis Fungoides/Sézary Syndrome: Validation of the Revised International Society for Cutaneous Lymphomas/European Organisation for Research and Treatment of Cancer Staging Proposal

Agar, Nicholas; Wedgeworth, E.; Crichton, Siobhan; Mitchell, Tracey; Cox, Michael C.; Ferreira, Sandra Reis; Robson, Alistair; Calonje, Eduardo; Stefanato, Catherine M.; Wain, E. Mary; Wilkins, Bridget S.; Fields, Paul; Dean, Alan; Webb, Katherine A.; Scarisbrick, Julia; Morris, Stephen; Whittaker, Sean

doi:10.1200/jco.2009.27.7665

Cited by 727 publications

(997 citation statements)

References 40 publications

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“…The incidence of cutaneous T-cell lymphomas (CTCL) has been increasing and is currently 6.4 per million persons, based on Surveillance, Epidemiology, and End Results (SEER) registry data, with the highest incidence rates being reported among males (male:female incidence rate ratio 1.9) and AfricanAmericans (incidence rate ratio 1.5) [3]. While CTCL may occur in children and young adults, this is very uncommon and often associated with histologic variants of MF [5][6][7]. The incidence of CTCL increases significantly with age, with a median age at diagnosis in the mid-50s and a fourfold increase in incidence appreciated in patients over 70 [3,7].…”

Section: Disease Overviewmentioning

confidence: 99%

“…While CTCL may occur in children and young adults, this is very uncommon and often associated with histologic variants of MF [5][6][7]. The incidence of CTCL increases significantly with age, with a median age at diagnosis in the mid-50s and a fourfold increase in incidence appreciated in patients over 70 [3,7].…”

Section: Disease Overviewmentioning

confidence: 99%

“…Patients with patch/plaque stage disease (T1/T2) and architectural preservation of any clinically abnormal lymph nodes are classified as stage IIA. Collectively, patients with stage I-IIA disease have ''limited-stage'' disease, as the overall survival in these patients is measured in decades, with survival in patients with stage IA disease resembling that of normal age-matched controls [7,99,100]. At diagnosis, the majority of MF patients will have limitedstage disease [7].…”

Section: Risk-stratification Stagingmentioning

confidence: 99%

“…Collectively, patients with stage I-IIA disease have ''limited-stage'' disease, as the overall survival in these patients is measured in decades, with survival in patients with stage IA disease resembling that of normal age-matched controls [7,99,100]. At diagnosis, the majority of MF patients will have limitedstage disease [7]. In contrast, patients with tumor stage disease (T3), erythroderma (T4), nodal involvement characterized by partial or complete architectural effacement (N3), visceral metastases (M1), or significant leukemic involvement (B2) have ''advanced-stage'' disease.…”

Section: Risk-stratification Stagingmentioning

confidence: 99%

“…In contrast, patients with tumor stage disease (T3), erythroderma (T4), nodal involvement characterized by partial or complete architectural effacement (N3), visceral metastases (M1), or significant leukemic involvement (B2) have ''advanced-stage'' disease. Detection of a clonal TCR gene rearrangement by PCR, which has been incorporated into the revised ISCL/EORTC node(N) and blood(B) staging classification, is an adverse prognostic factor [7,[185][186][187][188]. Unfortunately, median survivals from 1-5 years are observed in these patients with more extensive disease [7].…”

Section: Risk-stratification Stagingmentioning

confidence: 99%

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Cutaneous T‐cell lymphoma: 2011 update on diagnosis, risk‐stratification, and management

Wilcox

2011

American J Hematol

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Disease overview: Cutaneous T‐cell lymphomas are a heterogenous group of T‐cell lymphoproliferative disorders involving the skin, the majority of which may be classified as Mycosis fungoides (MF) or Sézary syndrome (SS).Diagnosis: The diagnosis of MF or SS requires the integration of clinical and histopathologic data.Risk‐adapted therapy: Tumor, node, metastasis, and blood (TNMB) staging remains the most important prognostic factor in MF/SS and forms the basis for a “risk‐adapted,” multidisciplinary approach to treatment. For patients with disease limited to the skin, expectant management or skin‐directed therapies is preferred, as both disease‐specific and overall survival for these patients is favorable. In contrast, patients with advanced‐stage disease with significant nodal, visceral, or blood involvement are generally approached with biologic‐response modifiers, denileukin diftitox, and histone deacetylase inhibitors before escalating therapy to include systemic, single‐agent chemotherapy. Multiagent chemotherapy may be used for those patients with extensive visceral involvement requiring rapid disease control. In highly‐selected patients with disease refractory to standard treatments, allogeneic stem‐cell transplantation may be considered. Am. J. Hematol., 2011. © 2011 Wiley‐Liss, Inc.

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Section: Disease Overviewmentioning

confidence: 99%

Section: Disease Overviewmentioning

confidence: 99%

Section: Risk-stratification Stagingmentioning

confidence: 99%

Section: Risk-stratification Stagingmentioning

confidence: 99%

Section: Risk-stratification Stagingmentioning

confidence: 99%

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Cutaneous T‐cell lymphoma: 2011 update on diagnosis, risk‐stratification, and management

Wilcox

2011

American J Hematol

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Hypopigmented Patches on the Skin

Juhas

English²

2013

JAMA

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Clinical Staging and Prognostic Factors in Folliculotropic Mycosis Fungoides

et al. 2016

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IMPORTANCE Large case series suggest that patients with folliculotropic mycosis fungoides (FMF) have a worse prognosis than patients with classic mycosis fungoides (MF). However, recent studies described a subgroup of patients with FMF with a more favorable prognosis. Distinction between indolent and aggressive FMF may have important therapeutic consequences but is hampered by the inability of the current tumor-node-metastasis-blood (TNMB) staging system to classify patients with FMF in a clinically meaningful way. OBJECTIVE To differentiate between indolent and aggressive FMF using clinicopathological criteria and to define prognostic factors in patients with FMF. DESIGN, SETTING, AND PARTICIPANTS In this prospective cohort study, we followed 203 patients with FMF, included in the Dutch Cutaneous Lymphoma Registry between October 1985 and May 2014 at a tertiary referral center hosting the Dutch Cutaneous Lymphoma Registry. Overall, 220 patients with FMF had been registered, but 17 patients with incomplete follow-up data or a history of classic MF were excluded. MAIN OUTCOMES AND MEASURES Main outcomes included clinical and histological characteristics, disease progression, and survival. Prognostic factors were investigated using Cox proportional hazard regression analysis. Distinction between early plaque-stage FMF and advanced plaque-stage FMF was made by a blinded review of skin biopsy specimens from patients presenting with plaques. RESULTS In a cohort of 147 men and 56 women (median [range] age, 59 [15-93] years), patients with histologically early plaque-stage FMF had a very similar overall survival (OS) rate to patients with only patches and/or follicular papules (10-year OS, 71% vs 80%), while the survival rate of patients with histologically advanced plaque-stage FMF was almost identical to that of patients presenting with tumors (10-year OS, 25% vs 27%). Subsequently, 3 clinical subgroups with significantly different survival data were distinguished: early skin-limited FMF (group A; n = 84; 5-year and 10-year OS, 92% and 72%); advanced skin-limited FMF (group B; n = 102; 5-year and 10-year OS, 55% and 28%); and FMF presenting with extracutaneous disease (group C; n = 17; 5-year and 10-year OS, 23% and 2%). Age at diagnosis, large cell transformation and secondary bacterial infection were independent risk factors for disease progression and/or poor survival. CONCLUSIONS AND RELEVANCE The results of this study provide useful criteria to differentiate between indolent and aggressive FMF and confirm the existence of a subgroup of FMF with a favorable prognosis.

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Survival Outcomes and Prognostic Factors in Mycosis Fungoides/Sézary Syndrome: Validation of the Revised International Society for Cutaneous Lymphomas/European Organisation for Research and Treatment of Cancer Staging Proposal

Abstract: This study has validated the recently proposed ISCL/EORTC staging system and identified new prognostic factors.

Cited by 727 publications

References 40 publications

Cutaneous T‐cell lymphoma: 2011 update on diagnosis, risk‐stratification, and management

Cutaneous T‐cell lymphoma: 2011 update on diagnosis, risk‐stratification, and management

Hypopigmented Patches on the Skin

Clinical Staging and Prognostic Factors in Folliculotropic Mycosis Fungoides

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