Mycosis fungoides beginning in childhood and adolescence

Koch, Susan E.; Zackheim, Herschel S.; Williams, Mary L.; Fletcher, Van; LeBoit, Philip E.

doi:10.1016/s0190-9622(87)70238-2

Cited by 79 publications

(66 citation statements)

References 14 publications

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“…Forty-nine patients (30 males and 19 females; ratio: 1.58) were included in the early-onset group, of whom 42 were identified by a literature review [3, 4, 5, 6, 7, 8, 9, 10, 11, 12, 13, 14, 15, 16, 17, 18, 19, 20, 21]. The median age of histological diagnosis was 14 years (range: 2–19).…”

Section: Resultsmentioning

confidence: 99%

“…5 and one reported by Zackheim et al [20]). The immunophenotype of the lymphoid infiltrate [6, 7, 8, 9, 11, 12, 13, 14, 15, 16, 17, 18, 21]was CD2+CD3+CD4+CD5+CD7–CD8– in the majority of cases. A loss of T cell lineage markers was found in 4 cases [7, 11, 15], whereas in 3 patients half or more of the skin-infiltrating lymphocytes lacked CD4 expression [8, 11, 12].…”

Section: Resultsmentioning

confidence: 99%

“…Two groups of patients were compared: (1) MF patients diagnosed in our Clinic from January 1975 to August 1997, in whom the first histological diagnosis was performed after 20 years of age (252 cases; median age at diagnosis: 61 years, range: 24–91) and (2) the cases of early-onset MF (first histological diagnosis before 20 years of age) reported in the literature from January 1975 to August 1997 [3, 4, 5, 6, 7, 8, 9, 10, 11, 12, 13, 14, 15, 16, 17, 18, 19, 20, 21]plus the 7 herein reported. SS cases reported in the literature [26, 27]with histological diagnosis before 20 years of age were not included in this study.…”

Section: Methodsmentioning

confidence: 99%

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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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Section: Resultsmentioning

confidence: 99%

Section: Resultsmentioning

confidence: 99%

Section: Methodsmentioning

confidence: 99%

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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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“…Although CTCL is primarily a disease of adulthood and occurs with the highest incidence in middle-aged men, its onset in childhood, adolescence and early adulthood with a preponderance in males has been reported [3, 4, 5, 6, 7, 8, 9]. Up to 6% of all mycosis fungoides cases [6, 10]are estimated to occur in childhood.…”

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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“…Although CTCL in general and MF in particular are usually observed in middle to late adulthood and are rarely seen before the age of 30 [18], there has been increased recognition that MF (including Sézary syndrome) may arise in children and adolescents, with younger adults reporting onset of symptoms during adolescence [22, 23, 24, 25, 26, 27]. …”

Section: Incidencementioning

confidence: 99%

Mycosis fungoides: Review of Epidemiological Observations

Suárez-Varela¹,

González²,

Vila

et al. 2000

Dermatology

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Background: Mycosis fungoides (MF) is a chronic cutaneous T-cell lymphoma characterized by small cells with cerebriform nuclei that usually express a mature peripheral T-helper cell (CD4+) immunophenotype. Its evolution is typically quite slow, with years between the first manifestations and development of advanced stages of disease. Objective: The purpose of the present paper is to contribute to the material about MF already present in the literature. The review articles that have appeared to date fundamentally address the morphological characteristics, diagnostic criteria and treatment of the disease; in contrast, the present study centers on the evolution of the incidence of MF and on the knowledge of the possible risk factors implicated in its development. Methods: Review of published papers about MF epidemiology. Results: The evidence suggests that the incidence is increasing, but this may be artifactual due to improved diagnostic techniques. The risk of MF is limited to gender and race, being higher in males and in blacks. Survival is highly stage dependent, but 90% of patients survive 15 years with only 10% of cutaneous involvement. Few risk factors have been identified, but several studies have found an association with industrial exposure, particularly to oils. Conclusion: MF is a rare disease and its risk factors have not been studied in any great detail. A European case-control study in progress will substantially increase the evidence available and progress towards identifying a prevention strategy.

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Mycosis fungoides beginning in childhood and adolescence

Cited by 79 publications

References 14 publications

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

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

Mycosis fungoides with Mucinosis follicularis in Childhood

Mycosis fungoides: Review of Epidemiological Observations

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