Mycosis fungoides with onset before 20 years of age

Zackheim, Herschel S.; McCalmont, Timothy H.; Deanovic, Frank W.; Odom, Richard B.

doi:10.1016/s0190-9622(97)70243-3

Cited by 94 publications

(92 citation statements)

References 18 publications

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“…2,8 In the current study, 8 of 34 (24%) patients had hypopigmented lesions compared with a similar incidence of 5 of 24 (21%) patients in the juvenile series reported by Zackheim et al 2 In a small series (n ϭ 9) of patients with MF reported by Tan et al, 10 8 had purely hypopigmented lesions. This subset is most commonly reported in patients with skin types IV-V. 12 In the current study, of the eight patients with hypopigmented lesions, only one was Caucasian, four were Asian, and …”

Section: Clinical Featuressupporting

confidence: 81%

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Outcome in 34 patients with juvenile‐onset mycosis fungoides

Wain

Orchard

Whittaker

et al. 2003

Cancer

136

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New methodology for the stereoselective synthesis of trisubstituted olefins is presented. The use of ortho‐diphenylphosphanyl benzoate (o‐DPPB) as a directing leaving group for copper‐mediated allylic substitution with Grignard reagents allowed for the stereoselective construction of a wide range of E olefins, without the need for an adjacent electron‐withdrawing group. Our modular three‐step approach toward trisubstituted alkenes commenced with geminal α‐methylene aldehydes. Addition of an organometallic reagent and introduction of the o‐DPPB group by esterification was followed by the o‐DPPB‐directed copper‐mediated allylic substitution with a Grignard reagent to furnish stereodefined trisubstituted olefins. Additionally, incorporation of a stereocenter from the chiral pool allowed the preparation of an enantiomerically pure olefin that bore three alkyl substituents in high E/Z selectivity.

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Section: Clinical Featuressupporting

confidence: 81%

“…6 -11 This delay in diagnosis might introduce bias, which could account for the apparently poor prognosis of young patients with MF in the literature. Therefore, in our study and in the study conducted by Zackheim et al, 2 all patients with MF presenting in childhood were included, regardless of when the diagnosis was proven by biopsy.…”

Section: Discussionmentioning

confidence: 99%

Outcome in 34 patients with juvenile‐onset mycosis fungoides

Wain

Orchard

Whittaker

et al. 2003

Cancer

136

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“…Up to 6% of all mycosis fungoides cases [6, 10]are estimated to occur in childhood. This estimate might be too low, because physicians often hesitate or delay performing skin biopsies in children [11].…”

Section: Discussionmentioning

confidence: 99%

“…These findings suggest both a poor prognosis in young patients and the necessity of aggressive treatment [9, 11]. On the other hand, other reports have revealed no specific characteristic prognosis of juvenile mycosis fungoides and suggest the use of nonaggressive therapies such as PUVA [5, 10, 12, 13]. These contradictions might be caused by the lack of a clear histopathologic classification of the CTCL cases.…”

Section: Discussionmentioning

confidence: 99%

Mycosis fungoides with Mucinosis follicularis in Childhood

et al. 1999

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Mycosis fungoides is a cutaneous T-cell lymphoma (CTCL) usually observed in mid to late adulthood. It occurs only rarely during childhood. Follicular mucinosis is a chronic dermatosis involving the sebaceous glands and outer root sheaths. It is normally differentiated into a juvenile benign form and an adult form possibly associated with mycosis fungoides. We report a 12-year-old boy who presented with an 8-month history of erythematous mucinous plaques on the scalp. Three months later, he developed erythematous patches and plaques on his whole body, accompanied by cervical lymphadenopathy. A biopsy showed follicular mucinosis and epidermotropism of the lymphocytic infiltrate. Immunophenotyping and a PCR clonality test were consistent with CTCL. The patient received PUVA treatment and local steroids, resulting in partial remission. Mycosis fungoides should be considered in the differential diagnosis of chronic, scaling dermatoses in childhood. Moreover, follicular mucinosis in childhood can be associated with mycosis fungoides.

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“…In fact, some authors suggest that early-onset MF/SS is more likely to be associated with extracutaneous involvement [7, 12, 21]as well as with the development of high-grade T cell lymphomas [12]or other non-T-cell lymphomas, such as Hodgkin’s disease [12]. On the other hand, other studies [15, 20]suggest that early-onset MF/SS is not more aggressive than those cases with a later onset.…”

Section: Introductionmentioning

confidence: 99%

Mycosis fungoides in Patients under 20 Years of Age: Report of 7 Cases, Review of the Literature and Study of the Clinical Course

et al. 1999

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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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Mycosis fungoides with onset before 20 years of age

Cited by 94 publications

References 18 publications

Outcome in 34 patients with juvenile‐onset mycosis fungoides

Outcome in 34 patients with juvenile‐onset mycosis fungoides

Mycosis fungoides with Mucinosis follicularis in Childhood

Mycosis fungoides in Patients under 20 Years of Age: Report of 7 Cases, Review of the Literature and Study of the Clinical Course

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