Necker-Enfants-Malades Hospital, Dermatology Department and Pathology Department, Assistance Publique Hôpitaux de Paris, University René Descartes Paris V, Paris, France.
J Am Acad Dermatol. 2012 Jan;66(1):51-7. doi: 10.1016/j.jaad.2010.10.040. Epub 2011 Jul 13.
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.
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.
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.
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.
This was a retrospective study with a small number of patients.
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.
淋巴母细胞淋巴瘤（LBL）是一种罕见的恶性肿瘤，通常发生在儿童和青少年的纵隔。LBL 的 B 细胞免疫表型（B-LBL）占所有病例的不到 20%，可能涉及纵隔外区域，如皮肤。尽管 LBL 具有高度侵袭性，但如果早期诊断，是可以治愈的。
我们旨在描述表现为皮肤病变的儿童 B-LBL 的临床和组织病理学特征，并强调这种罕见且严重疾病的具体特征。
通过回顾性病历审查，确定了 7 例经证实的皮肤 B-LBL 患儿。记录了临床和组织病理学特征，并与文献中先前报道的病例进行了分析和比较。
6 例患儿头部出现结节，1 例患儿背部和腹部出现病变。组织病理学显示弥漫性真皮和皮下单相浸润，由具有不成熟 B 细胞表型的异型细胞组成。诊断前病变的平均持续时间为 3.2 个月。分期检查显示 5 例患儿有皮肤外疾病，其中 4 例患儿骨髓受累。
这是一项回顾性研究，患者数量较少。
B-LBL 的皮肤病变通常表现为位于头部的快速生长的红斑性坚实结节。了解这些临床特征对于快速诊断和及时开始治疗非常重要。
