促纤维化婴儿星形细胞瘤和神经节胶质瘤(DIA/DIG)通常表现为大尺寸,大的囊性成分,大型硬脑膜植入物,大型船只的包裹,18个月内的临床表现,癫痫发作发生率高,总体预后良好,即使肿瘤手术在第一次手术时可能非常具有挑战性。
我们回顾性回顾了2008年至2019年间接受手术治疗的被诊断患有促纤维增生性婴儿肿瘤的患者的临床和放射学数据。
该系列包括12名患者。手术时的中位年龄为91天。平均肿瘤体积为212cm3。囊性成分主要在0至295cm3之间。5例术前明显活动性脑积水。八名患者(66.6%)接受了全部或小计切除,其中三人(25%)接受了部分切除,1例患者(8.3%)接受活检。一名患者因严重低血压在术后24小时内死亡,作为术中大量失血的结果。总的来说,第一次手术后,有7例(58.3%)患者对肿瘤进行了再次手术:4例患者进行了两次手术;3例患者进行了3次手术。两名患者进行了远程定位并接受了化疗。在最后的随访中,7例患者无瘤,2患有稳定的疾病,和2患有进行性疾病(软脑膜播种)。
促增生性婴儿肿瘤是罕见的巨大新生儿肿瘤。完全去除是治疗的目标,但即使不能完全切除,预后仍然良好。如果肿瘤进展或残留肿瘤引起癫痫,重新操作是第一种选择,化疗保留给不可切除或传播的病例,结果不一,while,到目前为止,放射治疗仍然不起作用。
Desmoplastic infantile astrocytomas and gangliogliomas (DIA/DIG) usually present with a large size, large cystic component, large dural implant, encasement of big vessels, clinical presentation within 18 months of life, high incidence of seizures and overall good prognosis, even if tumour surgery can be very challenging at first procedure.
We retrospectively reviewed clinical and radiological data of patients diagnosed with desmoplastic infantile tumours who were surgically treated between 2008 and 2019.
The series included 12 patients. The median age at surgery was 91 days. The average tumour volume was 212 cm3. Cystic components were predominant ranging from 0 to 295 cm3. Active hydrocephalus was pre-operatively evident in 5 cases. Eight patients (66.6%) received total or subtotal removal, three of them (25%) underwent partial removal, and one patient (8.3%) received a biopsy. One patient died within 24 h after surgery due to severe hypotension, as a consequence of significant intraoperative blood loss. Overall, seven (58.3%) patients were reoperated on the tumour after the first procedure: 4 patients were operated twice; 3 patients were operated 3 times. Two patients presented remote localizations and underwent chemotherapy. At last follow-up, 7 patients were tumour-free, 2 are alive with stable disease, and 2 are alive with progressive disease (leptomeningeal seeding).
Desmoplastic infantile tumours are rare giant neonatal tumours. Total removal is the goal of treatment, but prognosis remains good even if total removal is not achieved. In case of tumour progression or epilepsy from residual tumour, reoperation is the first option, with chemotherapy reserved to unresectable or disseminated cases with mixed results, while, to date, radiotherapy still plays no role.