İncecik Faruk, Altunbaşak Şakir, Hergüner M Özlem, Bayram İbrahim, Küpeli Serhan, Demirbilek Hüseyin
Divisions of 1Pediatric Neurology, Department of Pediatrics, Faculty of Medicine, Çukurova University, Adana, Turkey.
Turk J Pediatr. 2013 May-Jun;55(3):266-70.
Children with neurofibromatosis type 1 (NF1) are predisposed to malignancies such as brain tumors, leukemia, and pheochromocytomas. The aim of this study was to evaluate malignancy in patients with NF1. We studied 120 patients with NF1 in this study. Demographic data from these patients were retrospectively reviewed. We found 20 malignancies in 19 patients in our study. Ten children with NF1 had optic glioma. Four children had solid central nervous system tumors (3 pilocytic astrocytomas, 1 glioblastoma multiforme). Three patients had myeloid malignancies (1 juvenile myelomonocytic leukemia, 1 acute myeloid leukemia, 1 acute lymphocytic leukemia). Hodgkin lymphoma, T-cell lymphoblastic lymphoma, and malignant triton tumor were found in one patient each. Patients with NF1 are predisposed to both benign and malignant tumors of neurogenic and non-neurogenic origin. Therefore, systematic medical follow-up in patients with NF1 is important.
1型神经纤维瘤病（NF1）患儿易患恶性肿瘤，如脑肿瘤、白血病和嗜铬细胞瘤。本研究的目的是评估NF1患者的恶性肿瘤情况。我们在本研究中对120例NF1患者进行了研究。对这些患者的人口统计学数据进行了回顾性分析。我们在研究中发现19例患者中有20处恶性肿瘤。10例NF1患儿患有视神经胶质瘤。4例患儿患有实体性中枢神经系统肿瘤（3例毛细胞型星形细胞瘤，1例多形性胶质母细胞瘤）。3例患者患有髓系恶性肿瘤（1例青少年粒单核细胞白血病，1例急性髓系白血病，1例急性淋巴细胞白血病）。1例患者分别患有霍奇金淋巴瘤、T细胞淋巴母细胞淋巴瘤和恶性蝾螈瘤。NF1患者易患神经源性和非神经源性的良性和恶性肿瘤。因此，对NF1患者进行系统的医学随访很重要。
