BACKGROUND: Mycosis fungoides (MF) is rare in young patients. Its clinical behavior is still uncertain, as some reports have suggested that it has a more aggressive course than does the adult-onset type. AIM: To ascertain if early-onset MF represents a heterogeneous group of cutaneous T cell lymphomas. MATERIALS AND METHODS: Clinical, immunohistopathological and follow-up data of early-onset (<20 years of age) MF cases reported in the literature (n = 42) plus 7 described herein were compared with those of a cohort of adult-onset MF patients (n = 252) diagnosed at our institution since 1975. RESULTS: The majority of the 49 early-onset MF patients had patch-plaque stage disease at diagnosis. Ten had hypopigmented lesions. The predominant phenotype was CD3+ CD4+CD7-CD8-. Seven patients had a stage progression, 6 with extracutaneous involvement. Five- and 10-year survival rates were 93 and 74%, respectively. CONCLUSIONS: No statistically significant differences were found in the disease course between early- and adult-onset MF.
Mycosis fungoides in patients under 20 years of age: report of 7 cases, review of the literature and study of the clinical course.
QUAGLINO, Pietro
First
;BERNENGO, Maria Grazia
1999-01-01
Abstract
BACKGROUND: Mycosis fungoides (MF) is rare in young patients. Its clinical behavior is still uncertain, as some reports have suggested that it has a more aggressive course than does the adult-onset type. AIM: To ascertain if early-onset MF represents a heterogeneous group of cutaneous T cell lymphomas. MATERIALS AND METHODS: Clinical, immunohistopathological and follow-up data of early-onset (<20 years of age) MF cases reported in the literature (n = 42) plus 7 described herein were compared with those of a cohort of adult-onset MF patients (n = 252) diagnosed at our institution since 1975. RESULTS: The majority of the 49 early-onset MF patients had patch-plaque stage disease at diagnosis. Ten had hypopigmented lesions. The predominant phenotype was CD3+ CD4+CD7-CD8-. Seven patients had a stage progression, 6 with extracutaneous involvement. Five- and 10-year survival rates were 93 and 74%, respectively. CONCLUSIONS: No statistically significant differences were found in the disease course between early- and adult-onset MF.
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