We report a case of malignant peritoneal mesothelioma (MPM) in a 31-year-old male with history of cerebral palsy, hydrocephalus, and ventriculoperitoneal shunt (VPS) placed since infancy. He presented with fever, abdominal pain and distension. Computed tomography scan revealed a thick-walled rim-enhancing fluid collection, interpreted as pseudocyst. Intraoperatively, diffuse nodular peritoneal thickening with adhesions was demonstrated. The resection specimen consisted of multiple membranous fragments displaying firm nodules. Microscopic examination revealed a tumefactive malignant-appearing epithelioid proliferation involving the peritoneum, focally invading the underlying fat. Immunohistochemically, the tumor cells expressed keratin AE1/AE3, CK7, CK5/6, Calretinin, WT1 and D2-40, and were negative for CEA and MOC31. The findings were consistent with MPM, epithelioid type. The patient's condition continued to decline with increasing abdominal distension during the month following the original diagnosis. While atypical mesothelial hyperplasia has been described in association with long standing VPS, well-documented cases of MPM have not been previously reported in such context.

中文翻译：

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腹膜-腹膜分流设置的恶性腹膜间皮瘤：一种新颖的临床表现。

我们报告了一名31岁男性恶性腹膜间皮瘤（MPM）的病例，自婴儿以来就患有脑瘫，脑积水和脑室腹膜分流（VPS）。他表现出发烧，腹痛和腹胀。计算机断层扫描显示有一个厚壁的边缘增强液体集合，被解释为假性囊肿。术中证实弥漫性结节性腹膜增厚并伴有粘连。切除标本由多个显示坚硬结节的膜碎片组成。显微镜检查显示，肿瘤累及上皮样上皮样增生，累及腹膜，局部侵犯基础脂肪。免疫组织化学分析，肿瘤细胞表达角蛋白AE1 / AE3，CK7，CK5 / 6，Calretinin，WT1和D2-40，CEA和MOC31阴性。该发现与MPM，上皮样类型一致。最初诊断后的一个月内，患者的状况随着腹胀的增加而持续下降。虽然已经描述了不典型的间皮增生与长期存在的VPS相关联，但以前尚未在这种情况下报道过有据可查的MPM病例。