Casos Clínicos: Paciente de 15 años, sexo femenino con Micosis Fungoide.

Buenas noches Doctor Juan Macaluso.

Le escribe la Dra Yuranis Tirado residente de Oncología Pediátrica, envío imágenes de paciente de 15 años con cuadro clínico de 2 años de evolución manejada con múltiples medidas convencionales por servicio de dermatología e infectología; posterior a un tiempo se envía muestra a patología quienes reportan diagnóstico de Micosis  Fungoide, que aunque la literatura expresa ser unos de los tipos de linfomas cutaneos de células T más frecuentes, en 2 años ha sido el primero que observo y quise compartirles.

Actualmente paciente en espera de estudio inmunohistoquímico.

 

   

Dra. Yuranis Tirado.

Maracaibo. Venezuela.

Opinión: Muy interesante el caso Yuranis por varios motivos. Siempre tuve el concepto de que micosis fungoide (MF), era una enfermedad poco frecuente y de adultos. Cuando leí tu caso me fui a actualizar información en UpToDate y encontré que la incidencia de MF en Europa y los Estados Unidos es de aproximadamente seis casos por millón por año, lo que representa alrededor del 4 por ciento de todos los casos de linfoma no Hodgkin. Por otro lado, la edad pico de presentación es de 55 a 60 años, con una proporción hombre:mujer de 2:1. La enfermedad es más común en poblaciones negras. Aunque la MF es una enfermedad principalmente de pacientes mayores, se puede observar en pacientes menores de 35 años con hallazgos clínicos y evolución similares (39-41).

 

Referencias

39) Crowley JJ, Nikko A, Varghese A, et al. Mycosis fungoides in young patients: clinical characteristics and outcome. J Am Acad Dermatol 1998; 38:696.

40) Quaglino P, Zaccagna A, Verrone A, et al. Mycosis fungoides in patients under 20 years of age: report of 7 cases, review of the literature and study of the clinical course. Dermatology 1999; 199:8.

41) Pope E, Weitzman S, Ngan B, et al. Mycosis fungoides in the pediatric population: report from an international Childhood Registry of Cutaneous Lymphoma. J Cutan Med Surg 2010; 14:1.

 

ABSTRACTS

Abstracts for References 39-41 of 'Clinical manifestations, pathologic features, and diagnosis of mycosis fungoides'

39

PubMed

TI

Mycosis fungoides in young patients: clinical characteristics and outcome.

AU

Crowley JJ, Nikko A, Varghese A, Hoppe RT, Kim YH

SO

J Am Acad Dermatol. 1998;38(5 Pt 1):696.

 

BACKGROUNDMycosis fungoides (MF) can begin as early as the first decade of life. Few studies have reviewed MF in younger patients and none has been large enough to assess prognosis and outcome.

 

OBJECTIVEWe reviewed the clinical characteristics, prognosis, factors related to disease progression, and therapy in patients with MF younger than 35 years of age.

 

METHODSFifty-eight patients were entered into this retrospective cohort analysis.

 

RESULTS Significantly fewer patients with MF who are younger than 35 years presented with erythroderma (T4) and more with limited patch/plaque (T1) disease than older patients. Duration of skin disease before diagnosis of MF did not vary between the two groups. The long-term survival of younger patients with MF is significantly decreased when compared with a race-, age-, and sex-matched control population (p<0.001). Disease-specific survivals (DSS) of younger and older patients are similar, but young patients show a slight but significantly better overall DSS (p<0.02). However, DSS comparison of generalized patch/plaque (T2) and tumor stage (T3) patients with MF showed no significant difference between young and old patients (p=0.47, p=0.59). Patient age was not a significant predictor of survival when controlled for T-stage. Sixteen of 58 young patients with MF have died, 13 because of MF (22%), compared with 138 of 500 older patients (28%) who died as a result of MF. All younger patients with MF who progressed had at least T2 disease at presentation. Fifty of 56 young patients with MF and T1-T3 disease were treated initially with total skin electron beam or topical nitrogen mustard. The response to therapy was similar in younger and older patients with MF.

 

CONCLUSIONT1 disease is more common and T4 disease is unusual in young patients with MF compared with an older population of patients with MF. Young patients with T1 disease, all of whom were treated with either topical nitrogen mustard or total skin electron beam therapy, or both therapies, showed no disease progression. Overall, young patients with MF showed slightly better DSS, but this was because of differences in stage distribution.

 

AD

Department of Dermatology, Stanford University School of Medicine, California, USA.

PMID

9591813

40

PubMed

TI

Mycosis fungoides in patients under 20 years of age: report of 7 cases, review of the literature and study of the clinical course.

AU

Quaglino P, Zaccagna A, Verrone A, Dardano F, Bernengo MG

SO

Dermatology. 1999;199(1):8.

 

BACKGROUNDMycosis 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.

 

AIMTo ascertain if early-onset MF represents a heterogeneous group of cutaneous T cell lymphomas.

 

MATERIALS AND METHODSClinical, 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.

 

CONCLUSIONSNo statistically significant differences were found in the disease course between early- and adult-onset MF.

 

AD

Section of Dermatology, Department of Medical and Surgical Specialties, University of Turin, Italy.

PMID

10449950

41

PubMed

TI

Mycosis fungoides in the pediatric population: report from an international Childhood Registry of Cutaneous Lymphoma.

AU

Pope E, Weitzman S, Ngan B, Walsh S, Morel K, Williams J, Stein S, Garzon M, Knobler E, Lieber C, Turchan K, Wargon O, Tsuchiya A

SO

J Cutan Med Surg. 2010 Jan;14(1):1-6.

 

BACKGROUND/OBJECTIVESThere are limited data on the clinical presentation and progression of pediatric cutaneous lymphoma. This study focuses on the clinical characteristics of pediatric patients with mycosis fungoides (MF).

 

MATERIALS AND METHODS This descriptive study presents clinical characteristics of 22 pediatric patients with MF, enrolled in the international Childhood Registry for Cutaneous Lymphomas (CRCL).

 

RESULTS The mean ages at onset and at diagnosis were 7.5 (SD 3.8 years) years and 9.9 (SD 3.4) years, respectively. The most common MF presentation was patch stage (68%), followed by hypopigmentation (59%) and plaque stage disease (50%). Epidermotropism and lymphocytic atypia were the most common pathologic features, found in 89% and 85%, respectively. Cerebriform nuclei were noted in 42%, and Pautrier microabscesses were seen in 16% of cases. A cytotoxic pattern was more commonly seen (67% vs 33%), and clonality was detected in 21% (3 of 14) of patients. All patients presented with early-stage disease and received skin-directed therapy (topical steroids, 73%; light therapy, 54%; or combination therapy, 35%).

 

CONCLUSIONS Pediatric patients with MF present in the first decade of life, with early-stage disease and unusual forms such as hypopigmented variant. Further patient enrollment will provide information regarding natural history, treatment response, and overall prognosis of pediatric cutaneous T-cell lymphoma (CTCL).

 

AD

Section of Dermatology, The Hospital for Sick Children, Toronto, ON, Canada. Elena.pope@sickkids.ca

PMID

20128983