Wang Qiguang, Meng Jinli, Cheng Jian, Zhang Si, Hui Xuhui, Li Qiang, Liu Wenke, Ju Yan, Sun Lin
Department of Neurosurgery, West China Hospital, Sichuan University, Chengdu, China.
Department of Radiology, Hospital of Chengdu Office of People's Government of Tibetan Autonomous Region (Hospital C.T), Chengdu, China.
Front Oncol. 2022 May 9;11:608129. doi: 10.3389/fonc.2021.608129. eCollection 2021.
Multifocal desmoplastic infantile ganglioglioma/astrocytoma (DIA/DIG) has rarely been reported. Here, two cases have been presented, reviewing the literature and proposed treatment algorithms for this rare tumor.
We report two patients diagnosed with multifocal DIA/DIGs in West China Hospital. In addition, a literature review was performed, in October 2019, on case reports of DIA/DIGs with multifocal lesions. The clinical and radiological features, treatment, and outcome of this rare disease were discussed.
DIA/DIGs with multifocal locations were rare, and only thirteen cases (including ours) had been reported. This series included 8 males and 5 females with a mean age of 31.4 ± 45.7 months (range, 3-144 months). The supratentorial hemisphere, suprasellar region, posterior cranial fossa, and spinal cord were frequently involved. Ten patients (76.9%) received surgical resection for the symptomatic lesions and three patients (23.1%) underwent biopsy. Seven patients received chemotherapy postoperatively. Six individuals had tumor recurrences during the follow-up period, while three patients had tumors that spontaneously regressed. Finally, two patients died of tumor progression and one patient died of respiratory insufficiency and hypothalamic dysfunction.
Multifocal DIA/DIGs have more aggressive clinical behavior and poor outcome despite benign histology. DIA/DIGs should be included in the differential diagnosis of multifocal brain tumors in children. The mainstay of treatment is surgical resection; adjuvant treatment with chemotherapeutic drugs is unknown and requires additional research.
多灶性促纤维增生性婴儿型神经节胶质瘤/星形细胞瘤（DIA/DIG）鲜有报道。本文报告2例病例，并复习文献，提出针对这种罕见肿瘤的治疗方案。
我们报告了两名在华西医院被诊断为多灶性DIA/DIG的患者。此外，于2019年10月对多灶性病变的DIA/DIG病例报告进行了文献复习。讨论了这种罕见疾病的临床和影像学特征、治疗方法及预后。
多灶性DIA/DIG罕见，仅报道了13例（包括我们的病例）。该系列包括8名男性和5名女性，平均年龄为31.4±45.7个月（范围3 - 144个月）。幕上半球、鞍上区、后颅窝和脊髓常受累。10例患者（76.9%）因有症状的病变接受了手术切除，3例患者（23.1%）接受了活检。7例患者术后接受了化疗。6例患者在随访期间肿瘤复发，3例患者的肿瘤自发消退。最后，2例患者死于肿瘤进展，1例患者死于呼吸功能不全和下丘脑功能障碍。
多灶性DIA/DIG尽管组织学为良性，但具有更具侵袭性的临床行为和较差的预后。DIA/DIG应纳入儿童多灶性脑肿瘤的鉴别诊断。治疗的主要方法是手术切除；化疗药物的辅助治疗尚不清楚，需要进一步研究。
