Mendonça Gabriel Soares, Artiles Camilla Burla, Malheiros Glícia Campanharo, Amorim Viviane Brandão
Department of Diagnostic Radiology, National Cancer Institute José Alencar Gomes da Silva - INCA, Rio De Janeiro, Brazil.
Department of Diagnostic Radiology, Fleury Group, Rio De Janeiro, Brazil.
Radiol Case Rep. 2021 Mar 10;16(5):1153-1157. doi: 10.1016/j.radcr.2020.12.060. eCollection 2021 May.
Medulloepithelioma is a rare and highly malignant tumor of infancy and early childhood, and classified as a primitive neuroectodermal tumor. Considering that most cases occur in the central nervous system, development in atypical sites associated with secondary comorbidities, such as peritoneal carcinomatosis, becomes an extremely rare association due to its high morbimortality. This study reports a rare case of peripheral medulloepithelioma with peritoneal carcinomatosis in an 11-year-old boy, with a 4-year history of intestinal constipation alternated with fecal incontinence, taken to the emergency room due to increasing abdominal pain and urinary retention. This report aims to contribute to a better understanding of this rare pathology, as well as assist in the establishment of early diagnosis and treatment.
髓上皮瘤是一种罕见的婴幼儿期高度恶性肿瘤，被归类为原始神经外胚层肿瘤。鉴于大多数病例发生在中枢神经系统，在与继发性合并症相关的非典型部位发生，如腹膜癌转移，由于其高病亡率，这种情况极其罕见。本研究报告了一例11岁男孩的外周髓上皮瘤伴腹膜癌转移的罕见病例，该男孩有4年便秘与大便失禁交替的病史，因腹痛加剧和尿潴留被送往急诊室。本报告旨在有助于更好地理解这种罕见病理情况，并协助建立早期诊断和治疗方法。
