Mascarin M, De Bernardi B, Brisigotti M, Cuneo G P, Conte M, Taccone A, Tamaro P, Boldrini R
Clinica Pediatrica, Istituto Burlo Garofalo, Trieste, Italia.
Pediatr Med Chir. 1991 Jul-Aug;13(4):359-63.
Since there is little information regarding the possible prognostic significance of tumor rupture in localized neuroblastoma, we have analyzed the clinical courses of 163 children registered from 1979-1990 in 12 italian pediatric oncology Centers participating in the Neuroblastoma Cooperative Group of the A.I.E.O.P. (Italian Association for Paediatric Haematology-Oncology). Ten instances (6%) of tumor rupture were described. Ruptures occurred preoperatively in one child, during the operation in 9; among these 9, two were provoked by the surgeon to allow radical tumor excision, 7 were accidental. Of these 10 children, 7 relapsed at 3-25 months (median, 8 months) from diagnosis. Relapses were local in 5 children (2 of the 5 died), disseminated in one (who died), local + disseminated in one (presently alive with disease). Two local relapses were followed by bony or haematologic spread at 4 and 8 months, respectively. Of the 7 children who relapsed, 2 are alive in complete remission at 29, 100 months, respectively; two are alive with disease at 3 and 65 months, 3 died at 8, 15 and 24 months, respectively. We conclude that rupture of a localized neuroblastoma is a factor predisposing to relapse and may compromise the chance of cure. The surgeon should be aware of the risks connected with this complication and make any effort to avoid it.
由于关于局限性神经母细胞瘤中肿瘤破裂可能的预后意义的信息很少，我们分析了1979年至1990年在参与意大利儿科血液学 - 肿瘤学协会（A.I.E.O.P.）神经母细胞瘤合作组的12个意大利儿科肿瘤中心登记的163名儿童的临床病程。共描述了10例（6%）肿瘤破裂情况。1例破裂发生在术前，9例发生在手术期间；在这9例中，2例是外科医生为了彻底切除肿瘤而引发的，7例是意外发生的。在这10名儿童中，7例在诊断后3至25个月（中位数为8个月）复发。5名儿童出现局部复发（其中5名中有2名死亡），1名出现播散性复发（死亡），1名出现局部 + 播散性复发（目前带病存活）。2例局部复发分别在4个月和8个月后出现骨或血液学转移。在7例复发的儿童中，2例分别在29个月和100个月时处于完全缓解状态存活；2例分别在3个月和65个月时带病存活，3例分别在8个月、15个月和24个月时死亡。我们得出结论，局限性神经母细胞瘤破裂是复发的一个易感因素，可能会影响治愈机会。外科医生应意识到与这种并发症相关的风险，并尽一切努力避免它。
