Choque-Velasquez Joham, Colasanti Roberto, Resendiz-Nieves Julio, Jahromi Behnam Rezai, Tynninen Olli, Collan Juhani, Niemelä Mika, Hernesniemi Juha
Department of Neurosurgery, Helsinki University Hospital, University of Helsinki, Helsinki, Finland.
Department of Neurosurgery, Umberto I General Hospital, Università Politecnica delle Marche, Ancona, Italy; Department of Neurosurgery, Ospedali Riuniti Marche Nord, Pesaro, Italy.
World Neurosurg. 2018 Sep;117:144-152. doi: 10.1016/j.wneu.2018.06.020. Epub 2018 Jun 12.
Papillary tumor of the pineal region (PTPR) is a rare grade II-III pineal lesion with peculiar histological and immunohistochemical features. These tumors mostly occur in adults, only rarely in children, with 19 cases reported up to now.
We present a 3-year-old boy who underwent reoperation for a recurrent PTPR (grade II). Gross total resection of the lesion through an occipital interhemispheric approach with the patient in a sitting position was followed by adjuvant radiotherapy and chemotherapy. Histological examination revealed tumor progression (grade III) and an MIB-1 proliferation index >25%. The patient continues to do well with no evidence of recurrence more than 3 years following surgery. A comprehensive literature review regarding the PTPR, including the current management in children, is reported.
PTPRs are extremely rare in children, and immunohistochemistry is needed to differentiate them from other pineal tumors. These tumors show a high rate of recurrence, and a multidisciplinary management approach (microsurgical resection followed by radiotherapy and/or chemotherapy) can help achieve a favorable outcome.
松果体区乳头状瘤（PTPR）是一种罕见的II - III级松果体病变，具有独特的组织学和免疫组化特征。这些肿瘤大多发生于成人，儿童罕见，目前报道的病例有19例。
我们报告一名3岁男孩，因复发性PTPR（II级）接受再次手术。采用枕部经半球间入路，患者取坐位，将病变进行了全切除，随后进行辅助放疗和化疗。组织学检查显示肿瘤进展（III级），MIB - 1增殖指数>25%。术后3年多患者情况良好，无复发迹象。本文报道了关于PTPR的全面文献综述，包括目前儿童患者的治疗方法。
PTPR在儿童中极为罕见，需要通过免疫组化将其与其他松果体肿瘤区分开来。这些肿瘤复发率高，多学科管理方法（显微手术切除后进行放疗和/或化疗）有助于取得良好疗效。
