Outcomes after diagnosis of mycosis fungoides and Sézary syndrome before 30 years of age: A population-based study

Weiyun Z. Ai, Theresa H Keegan, David J. Press, Juan Yang, Laura B. Pincus, Youn H. Kim, Ellen T. Chang

Research output: Contribution to journalArticle

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Abstract

IMPORTANCE: Mycosis fungoides and Sézary syndrome (MF/SS) are rare in children and young adults, and thus the incidence and outcomes in this patient population are not well studied. OBJECTIVE: To assess the incidence and outcomes of MF/SS in patients diagnosed before 30 years of age. DESIGN, SETTING, AND PARTICIPANTS: Retrospective study of 2 population-based cancer registries - the California Cancer Registry (n = 204) and 9 US cancer registries of the Surveillance, Epidemiology, and End Results program (SEER 9; n = 195) - for patients diagnosed with MF/SS before 30 years of age. MAIN OUTCOMES AND MEASURES Overall survivalwas calculated by the Kaplan-Meier method. The risk of a second cancer was assessed by calculating the standard incidence ratio (SIR) comparing observed cancer incidence in patients with MF/SS with the expected incidence in the age-, sex-, and race-standardized general population. RESULTS: The incidence of MF/SS is rare before 30 years of age, with an incidence rate of 0.05 per 100 000 persons per year before age 20 years and 0.12 per 100 000 persons per year between ages 20 and 29 years in the California Cancer Registry. At 10 years, patients with MF/SS had an overall survival of 94.3% (95% CI, 89.6%-97.2%) in the California Cancer Registry and 88.9% (95% CI, 82.4%-93.2%) in SEER 9. In SEER 9, there was a significant excess risk of all types of second cancers combined (SIR, 3.40; 95% CI, 1.55-6.45), particularly lymphoma (SIR, 12.86; 95% CI, 2.65-37.59) and melanoma (SIR, 9.31; 95% CI, 8.75-33.62). In the California Cancer Registry, the SIR for risk of all types of second cancers was similar to that in SEER 9 (SIR, 3.45; 95% CI, 0.94-8.83), although not statistically significant. CONCLUSIONS AND RELEVANCE: Young patients with MF/SS have a favorable outcome, despite a strong suggestion of an increased risk of second primary cancers. Prolonged follow-up is warranted to definitively assess their risk of developing second cancers in a lifetime.

Original languageEnglish (US)
Pages (from-to)709-715
Number of pages7
JournalJAMA Dermatology
Volume150
Issue number7
DOIs
StatePublished - 2014
Externally publishedYes

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Mycosis Fungoides
Incidence
Second Primary Neoplasms
Population
Registries
Neoplasms
SEER Program
Young Adult
Melanoma
Lymphoma
Retrospective Studies

ASJC Scopus subject areas

  • Dermatology

Cite this

Outcomes after diagnosis of mycosis fungoides and Sézary syndrome before 30 years of age : A population-based study. / Ai, Weiyun Z.; Keegan, Theresa H; Press, David J.; Yang, Juan; Pincus, Laura B.; Kim, Youn H.; Chang, Ellen T.

In: JAMA Dermatology, Vol. 150, No. 7, 2014, p. 709-715.

Research output: Contribution to journalArticle

Ai, Weiyun Z. ; Keegan, Theresa H ; Press, David J. ; Yang, Juan ; Pincus, Laura B. ; Kim, Youn H. ; Chang, Ellen T. / Outcomes after diagnosis of mycosis fungoides and Sézary syndrome before 30 years of age : A population-based study. In: JAMA Dermatology. 2014 ; Vol. 150, No. 7. pp. 709-715.
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abstract = "IMPORTANCE: Mycosis fungoides and S{\'e}zary syndrome (MF/SS) are rare in children and young adults, and thus the incidence and outcomes in this patient population are not well studied. OBJECTIVE: To assess the incidence and outcomes of MF/SS in patients diagnosed before 30 years of age. DESIGN, SETTING, AND PARTICIPANTS: Retrospective study of 2 population-based cancer registries - the California Cancer Registry (n = 204) and 9 US cancer registries of the Surveillance, Epidemiology, and End Results program (SEER 9; n = 195) - for patients diagnosed with MF/SS before 30 years of age. MAIN OUTCOMES AND MEASURES Overall survivalwas calculated by the Kaplan-Meier method. The risk of a second cancer was assessed by calculating the standard incidence ratio (SIR) comparing observed cancer incidence in patients with MF/SS with the expected incidence in the age-, sex-, and race-standardized general population. RESULTS: The incidence of MF/SS is rare before 30 years of age, with an incidence rate of 0.05 per 100 000 persons per year before age 20 years and 0.12 per 100 000 persons per year between ages 20 and 29 years in the California Cancer Registry. At 10 years, patients with MF/SS had an overall survival of 94.3{\%} (95{\%} CI, 89.6{\%}-97.2{\%}) in the California Cancer Registry and 88.9{\%} (95{\%} CI, 82.4{\%}-93.2{\%}) in SEER 9. In SEER 9, there was a significant excess risk of all types of second cancers combined (SIR, 3.40; 95{\%} CI, 1.55-6.45), particularly lymphoma (SIR, 12.86; 95{\%} CI, 2.65-37.59) and melanoma (SIR, 9.31; 95{\%} CI, 8.75-33.62). In the California Cancer Registry, the SIR for risk of all types of second cancers was similar to that in SEER 9 (SIR, 3.45; 95{\%} CI, 0.94-8.83), although not statistically significant. CONCLUSIONS AND RELEVANCE: Young patients with MF/SS have a favorable outcome, despite a strong suggestion of an increased risk of second primary cancers. Prolonged follow-up is warranted to definitively assess their risk of developing second cancers in a lifetime.",
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T1 - Outcomes after diagnosis of mycosis fungoides and Sézary syndrome before 30 years of age

T2 - A population-based study

AU - Ai, Weiyun Z.

AU - Keegan, Theresa H

AU - Press, David J.

AU - Yang, Juan

AU - Pincus, Laura B.

AU - Kim, Youn H.

AU - Chang, Ellen T.

PY - 2014

Y1 - 2014

N2 - IMPORTANCE: Mycosis fungoides and Sézary syndrome (MF/SS) are rare in children and young adults, and thus the incidence and outcomes in this patient population are not well studied. OBJECTIVE: To assess the incidence and outcomes of MF/SS in patients diagnosed before 30 years of age. DESIGN, SETTING, AND PARTICIPANTS: Retrospective study of 2 population-based cancer registries - the California Cancer Registry (n = 204) and 9 US cancer registries of the Surveillance, Epidemiology, and End Results program (SEER 9; n = 195) - for patients diagnosed with MF/SS before 30 years of age. MAIN OUTCOMES AND MEASURES Overall survivalwas calculated by the Kaplan-Meier method. The risk of a second cancer was assessed by calculating the standard incidence ratio (SIR) comparing observed cancer incidence in patients with MF/SS with the expected incidence in the age-, sex-, and race-standardized general population. RESULTS: The incidence of MF/SS is rare before 30 years of age, with an incidence rate of 0.05 per 100 000 persons per year before age 20 years and 0.12 per 100 000 persons per year between ages 20 and 29 years in the California Cancer Registry. At 10 years, patients with MF/SS had an overall survival of 94.3% (95% CI, 89.6%-97.2%) in the California Cancer Registry and 88.9% (95% CI, 82.4%-93.2%) in SEER 9. In SEER 9, there was a significant excess risk of all types of second cancers combined (SIR, 3.40; 95% CI, 1.55-6.45), particularly lymphoma (SIR, 12.86; 95% CI, 2.65-37.59) and melanoma (SIR, 9.31; 95% CI, 8.75-33.62). In the California Cancer Registry, the SIR for risk of all types of second cancers was similar to that in SEER 9 (SIR, 3.45; 95% CI, 0.94-8.83), although not statistically significant. CONCLUSIONS AND RELEVANCE: Young patients with MF/SS have a favorable outcome, despite a strong suggestion of an increased risk of second primary cancers. Prolonged follow-up is warranted to definitively assess their risk of developing second cancers in a lifetime.

AB - IMPORTANCE: Mycosis fungoides and Sézary syndrome (MF/SS) are rare in children and young adults, and thus the incidence and outcomes in this patient population are not well studied. OBJECTIVE: To assess the incidence and outcomes of MF/SS in patients diagnosed before 30 years of age. DESIGN, SETTING, AND PARTICIPANTS: Retrospective study of 2 population-based cancer registries - the California Cancer Registry (n = 204) and 9 US cancer registries of the Surveillance, Epidemiology, and End Results program (SEER 9; n = 195) - for patients diagnosed with MF/SS before 30 years of age. MAIN OUTCOMES AND MEASURES Overall survivalwas calculated by the Kaplan-Meier method. The risk of a second cancer was assessed by calculating the standard incidence ratio (SIR) comparing observed cancer incidence in patients with MF/SS with the expected incidence in the age-, sex-, and race-standardized general population. RESULTS: The incidence of MF/SS is rare before 30 years of age, with an incidence rate of 0.05 per 100 000 persons per year before age 20 years and 0.12 per 100 000 persons per year between ages 20 and 29 years in the California Cancer Registry. At 10 years, patients with MF/SS had an overall survival of 94.3% (95% CI, 89.6%-97.2%) in the California Cancer Registry and 88.9% (95% CI, 82.4%-93.2%) in SEER 9. In SEER 9, there was a significant excess risk of all types of second cancers combined (SIR, 3.40; 95% CI, 1.55-6.45), particularly lymphoma (SIR, 12.86; 95% CI, 2.65-37.59) and melanoma (SIR, 9.31; 95% CI, 8.75-33.62). In the California Cancer Registry, the SIR for risk of all types of second cancers was similar to that in SEER 9 (SIR, 3.45; 95% CI, 0.94-8.83), although not statistically significant. CONCLUSIONS AND RELEVANCE: Young patients with MF/SS have a favorable outcome, despite a strong suggestion of an increased risk of second primary cancers. Prolonged follow-up is warranted to definitively assess their risk of developing second cancers in a lifetime.

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