Multifocal Desmoplastic Infantile Ganglioglioma/Astrocytoma (DIA/DIG): An Institutional Series Report and a Clinical Summary of This Rare Tumor

Frontiers in Oncology

Jian Cheng¹  Qiguang Wang¹  Wenke Liu¹  Qiang Li¹  Xuhui Hui¹  Yan Ju¹  Si Zhang¹  Jinli Meng³  Lin Sun⁴ 

[1] 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;Huaxi MR Research Center (HMRRC), Functional and Molecular Imaging Key Laboratory of Sichuan Province, Department of Radiology, West China Hospital, Sichuan University, Chengdu, China;Medical Insurance Office, West China Hospital, Sichuan University, Chengdu, China;School of Computer Science and Engineering, University of Electronic Science and Technology of China, Chengdu, China;

关键词: Multiple DIA/DIGs;    treatment;    outcome;    infant;    clinical feature;   

DOI  :  10.3389/fonc.2021.608129

来源: DOAJ

【 摘 要 】

AimMultifocal 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.Patients and MethodsWe 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.ResultsDIA/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.ConclusionsMultifocal 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.

【 授权许可】

Unknown