Cutaneous B-cell lymphoblastic lymphoma in children: A rare diagnosis Olivia Boccara, MD, a Etty Laloum-Grynberg, MD, a G eraldine Jeudy, MD, b Marie-H el ene Aubriot-Lorton, MD, b Pierre Vabres, MD, PhD, b Yves de Prost, MD, PhD, a H el ene Pacquement, MD, c Nicole Brousse, MD, PhD, a Sylvie Fraitag, MD, a and Christine Bodemer, MD, PhD a Paris and Dijon, France 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. ( J Am Acad Dermatol 2012;66:51-7.) Key words: chemotherapy; children; cutaneous nodule; hematology; lymphoblastic; lymphoma; pediatrics. B - and T-cell non-Hodgkin lymphomas repre- sent about 4% to 6% of malignant neoplasms in children. 1 Skin involvement in pediatric non-Hodgkin lymphoma is rare. 2 It manifests as either primary cutaneous lymphoma or with symp- toms secondary to primary extracutaneous dis- ease. 3,4 In a study by Wright et al, 5 only 15 (5%) of 293 cases presented with skin or soft-tissue involve- ment. After Burkitt lymphoma, lymphoblastic lymphoma (LBL) is the most commonly observed non-Hodgkin lymphoma in children. LBL and acute lymphoblastic leukemia (ALL) are the two extremes of a disease spectrum characterized by a malignant proliferation of immature T or B cells. The most From the Necker-Enfants-Malades Hospital, Dermatology Depart- ment and Pathology Department, Assistance Publique H^ opitaux de Paris, University Ren e Descartes Paris V a ; Dijon Hospital, Dermatology Department, Pathology and Pediatric Oncology, University of Dijon b ; and Curie Institute, Paris. c Funding sources: None. Conflicts of interest: None declared. Accepted for publication October 1, 2010. Reprint requests: Olivia Boccara, MD, Service de Dermatologie H^ opital Necker-Enfants Malades, Universit e de Paris V Ren e Descartes, 149, rue de S evres, 75743 Paris Cedex 15, France, EU. E-mail: [email protected]. Published online July 11, 2011. 0190-9622/$36.00 ª 2010 by the American Academy of Dermatology, Inc. doi:10.1016/j.jaad.2010.10.040 Abbreviations used: ALL: acute lymphoblastic leukemia B-LBL: B-cell immunophenotype of lymphoblas- tic lymphoma LBL: lymphoblastic lymphoma 51
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