Drut R, Jones M C
Department of Pathology, Hospital de Niños, La Plata, Argentina.
Pediatr Pathol. 1988;8(3):331-9. doi: 10.3109/15513818809042976.
We present a male newborn (weight 4000 g) who died at age 12 days with a clinical history of persistent hypoglycemia and polycythemia. Clinical examination disclosed somatic hemihypertrophy (left side), a large umbilical hernia, macroglossia, and an intraabdominal tumor, consistent with the diagnosis of Beckwith-Wiedemann syndrome (EMG syndrome) and hemihypertrophy. Necropsy findings included visceromegaly (left kidney and adrenal), cytomegaly of the fetal cortex and nodular arrangement of both adrenals, diffuse nesidioblastosis and islet cell hyperplasia of the pancreas, and persistent glomerulogenesis. The tumor was a cystic pancreatoblastoma attached to the anterior surface of the pancreas. Three other examples of this association, congenital pancreatoblastoma and Beckwith-Wiedemann syndrome, all in males, are on record in the literature, indicating a strong relationship between both conditions.
我们报告一例男性新生儿（体重4000克），该患儿于12日龄时死亡，临床病史为持续性低血糖和红细胞增多症。临床检查发现躯体半侧肥大（左侧）、巨大脐疝、巨舌症以及腹腔内肿瘤，符合贝克威思-维德曼综合征（EMG综合征）和半侧肥大的诊断。尸检结果包括内脏肿大（左肾和肾上腺）、胎儿皮质细胞肿大以及双侧肾上腺呈结节状排列、胰腺弥漫性胰岛细胞增殖症和胰岛细胞增生，以及持续性肾小球生成。肿瘤为附着于胰腺前表面的囊性胰腺母细胞瘤。文献中记载了另外三例这种关联病例，即先天性胰腺母细胞瘤与贝克威思-维德曼综合征，均为男性，表明这两种病症之间存在密切关系。
