Cutaneous B-cell lymphoblastic lymphoma in children: A rare diagnosis - 16/12/11
, Etty Laloum-Grynberg, MD a, Géraldine Jeudy, MD b, Marie-Hélène Aubriot-Lorton, MD b, Pierre Vabres, MD, PhD b, Yves de Prost, MD, PhD a, Hélène Pacquement, MD c, Nicole Brousse, MD, PhD a, Sylvie Fraitag, MD a, Christine Bodemer, MD, PhD a
Reprint requests: Olivia Boccara, MD, Service de Dermatologie Hôpital Necker-Enfants Malades, Université de Paris V René Descartes, 149, rue de Sèvres, 75743 Paris Cedex 15, France, EU.Abstract |
Background |
Lymphoblastic lymphoma (LBL) is a rare malignant neoplasm usually occurring in the mediastinum of children and adolescents. The B-cell immunophenotype of LBL (B-LBL) accounts for less than 20% of all cases and may involve extramediastinal areas, such as the skin. Although highly aggressive, LBL is potentially curable if diagnosed early.
Objective |
We sought to describe the clinical and histopathologic features of B-LBL in children presenting with cutaneous lesions, and to highlight the specific features of this rare and serious disease.
Methods |
Seven children with a confirmed diagnosis of cutaneous B-LBL were identified by retrospective chart review. The clinical and histopathologic features were documented, analyzed, and compared with cases previously published in the literature.
Results |
Six children developed nodules on the head, and one child presented with lesions on the back and abdomen. Histopathology showed a diffuse dermal and subcutaneous monomorphous infiltrate made up of atypical cells with an immature B-cell phenotype. The average duration of the lesions before diagnosis was 3.2 months. A staging workup revealed extracutaneous disease in 5 patients, including bone-marrow involvement in 4 children.
Limitations |
This was a retrospective study with a small number of patients.
Conclusion |
The cutaneous lesions of B-LBL typically manifest as rapidly growing erythematous firm nodules located on the head. Awareness of these clinical features is important for the diagnosis to be reached rapidly and treatment started without delay.
Le texte complet de cet article est disponible en PDF.Key words : chemotherapy, children, cutaneous nodule, hematology, lymphoblastic, lymphoma, pediatrics
Abbreviations used : ALL, B-LBL, LBL
Plan
| Funding sources: None. |
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| Conflicts of interest: None declared. |
Vol 66 - N° 1
P. 51-57 - janvier 2012 Retour au numéro
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