Cuccia Vicente, Alderete Daniel
Department of Pediatric Neurosurgery, Hospital Nacional de Pediatría Prof Dr Juan P Garrahan, Combate de los Pozos 1881, 1245 Buenos Aires, Argentina.
Childs Nerv Syst. 2010 Aug;26(8):1043-9. doi: 10.1007/s00381-010-1120-3. Epub 2010 Mar 11.
Intracranial germ cell tumors (GCT) arise from embryonal rests of germinal cells. The aim of this report is to analyze a small group of GCT located simultaneously in the suprasellar and pineal regions without seeding either between both tumors or to other places. We named this group as suprasellar/pineal bifocal germ cell tumors (SPBT).
A retrospective review of a series of 25 GCT showed a) 16 cases of unifocal non-disseminated pineal or sellar GCT, b) one case of unifocal disseminated pineal GCT, c) three cases with suprasellar and pineal double tumors with dissemination, and d) five cases with SPBT. The analysis is focused on the latter group.
The series includes four pure germinomas and one germinal non-germinoma. MRI and endoscopic exploration were necessary to define SPBT. Endocrine, ocular, and increased intracranial pressure syndromes were identified and related to the size of the tumors. Chemotherapy and radiotherapy were performed in all SPBT. Radical or partial resection of SPBT offered no benefits over biopsy. Prognosis for bifocal groups was similar to unifocal tumors of the same histological type. Complete remission without recurrence and mortality were achieved in all cases.
SPBT seem to be an entity defined by a) one tumor in the suprasellar and another in the pineal region, b) GCT with predominance of PG, but not exclusively, and c) MRI and endoscopy without any dissemination. The presence of two tumors does not indicate dissemination; SPBT were non-disseminated but focal tumors, and spinal radiotherapy was not necessary.
颅内生殖细胞肿瘤(GCT)起源于生殖细胞的胚胎残余。本报告的目的是分析一小群同时位于鞍上和松果体区域的GCT,且肿瘤之间或其他部位均无播散。我们将这一组命名为鞍上/松果体双灶性生殖细胞肿瘤(SPBT)。
对一系列25例GCT进行回顾性分析,结果显示:a)16例单灶性非播散性松果体或鞍区GCT;b)1例单灶性播散性松果体GCT;c)3例鞍上和松果体双肿瘤伴播散;d)5例SPBT。分析重点为后一组。
该系列包括4例纯生殖细胞瘤和1例生殖细胞非生殖细胞瘤。MRI和内镜探查对于定义SPBT很有必要。识别出内分泌、眼部和颅内压升高综合征,并与肿瘤大小相关。所有SPBT均进行了化疗和放疗。SPBT的根治性或部分切除与活检相比并无益处。双灶性组的预后与相同组织学类型的单灶性肿瘤相似。所有病例均实现完全缓解且无复发和死亡。
SPBT似乎是一种由以下特征定义的实体:a)一个肿瘤位于鞍上,另一个位于松果体区域;b)以生殖细胞瘤为主但并非唯一的GCT;c)MRI和内镜检查显示无任何播散。两个肿瘤的存在并不表明播散;SPBT是无播散的局灶性肿瘤,无需进行脊髓放疗。