Department of Medical Oncology, Fox Chase Cancer Center, PA 19111, USA.
Leuk Lymphoma. 2010 Jan;51(1):89-94. doi: 10.3109/10428190903349670.
Pediatric nodal marginal zone lymphoma (NMZL) is described as a separate variant of NMZL in the most recent WHO classification of tumors of hematologic and lymphoid tissues. It has distinctive morphology and clinical presentation and stands out as an indolent disease with remarkably better overall prognosis compared to classic NMZL. Here we report two adult patients with NMZL with clinical and morphologic features consistent with pediatric NMZL (pNMZL) and review available literature describing the clinical and histologic presentation of pNMZL. Two men, ages 44 and 18 years, each presented with localized cervical lymphadenopathy, both demonstrated florid proliferation of the marginal zone and disruption of reactive germinal centers, progressive transformation of germinal centers-like morphologic features typical for pNMZL and clonal disease with immunophenotype consistent with NMZL. This is the first report of pNMZL in a middle-aged person. Distinct histologic features and characteristic benign clinical course will help to distinguish this rare variant from other NMZL in the adults. Clinically, recognition is important to understand the true incidence of this rare form in the adult population and to avoid unnecessary overtreatment of this indolent form.
儿科结外边缘区淋巴瘤（NMZL）在最近的世界卫生组织（WHO）血液和淋巴组织肿瘤分类中被描述为 NMZL 的一个独立变异型。它具有独特的形态学和临床表现，与经典 NMZL 相比，表现为一种惰性疾病，总体预后明显更好。在这里，我们报告了两例具有符合儿科 NMZL（pNMZL）临床和形态学特征的成人 NMZL 患者，并回顾了描述 pNMZL 临床和组织学表现的可用文献。两名男性，年龄分别为 44 岁和 18 岁，均表现为局限性颈淋巴结病，均表现为边缘区的明显增生和反应性生发中心的破坏、生发中心样形态特征的进行性转化，这些特征是 pNMZL 的典型特征，且克隆性疾病具有与 NMZL 一致的免疫表型。这是首例中年人群中出现 pNMZL 的报道。独特的组织学特征和特征性的良性临床病程将有助于将这种罕见变异型与成人中的其他 NMZL 区分开来。临床上，认识到这种罕见形式在成人中的真实发病率很重要，以避免对这种惰性形式进行不必要的过度治疗。
