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[新生儿神经母细胞瘤与产前诊断]

[Neonatal neuroblastoma and prenatal diagnosis].

作者信息

Messina M, Di Maggio G, Garzi A, Molinaro F, Amato G, Ferrara F

机构信息

Dipartimento di Pediatria, Ostetricia e Medicina della Riproduzione, Sezione di Chirurgia Pediatrica, Università di Siena, Siena, Italia.

出版信息

Minerva Pediatr. 2009 Jun;61(3):349-54.

Abstract

The improvement of the imaging has considerably increased the number of cases of neuoroblastoma, diagnosed in preborn age. The authors present a case of neonatal neuroblastoma diagnosed in prenatal age and managed with a multidisciplinary approach. The authors report the case of R.T., born from a scheduled Caesarean delivery. The echographic morphological prenatal diagnosis showed an abdominal mass of 3x2 cm located on the upper side of the kidney, which was not apparently involved. Postnatal ultrasound evaluations confirmed that diagnosis. The diagnostic programme included nuclear magnetic resonance and a renogramm with metaiodobenzylguanidine. These exams confirmed the presence of a mass, probably due to a neuroblastoma. Due to the increasing of the mass, the patient underwent surgical excision of the neoplastic mass. The histological examination confirmed the diagnosis of neuroblastoma Stage I without medullary involvement. During the operation, a medullary biopsy was performed. The FISH exam did not show the amplification of N-myc or a delection of p36 chromosome. For patients younger than 18 months there is no therapeutic gold standard for the treatment of suprarenal masses of neoplastic origin, and the approach is still controversial. The complete excision of the mass should be taken in consideration in presence of an increasing neoformation, and should not include any chemotherapeutical or radiation therapy for stage I, II, IVs (INSS) or L1, MS (INGRSS) neoformations. In conclusion, the effectiveness of a multidisciplinary approach of neonatal neuroblastoma is higher in the early diagnosis and in an accurate staging of the disease, which is fundamental for the favourable prognosis.

摘要

成像技术的改进显著增加了产前诊断出的神经母细胞瘤病例数量。作者介绍了一例产前诊断出的新生儿神经母细胞瘤,并采用多学科方法进行治疗的病例。作者报告了R.T.的病例,其通过计划剖宫产出生。产前超声形态学诊断显示在肾脏上侧有一个3x2厘米的腹部肿块,肾脏显然未受累。产后超声评估证实了该诊断。诊断程序包括核磁共振和间碘苄胍肾图。这些检查证实了肿块的存在,可能是神经母细胞瘤所致。由于肿块增大,患者接受了肿瘤肿块的手术切除。组织学检查证实为I期神经母细胞瘤,无髓质受累。手术期间进行了髓质活检。荧光原位杂交检查未显示N-myc扩增或36号染色体缺失。对于18个月以下的患者,对于起源于肿瘤的肾上腺肿块没有治疗金标准,治疗方法仍存在争议。对于不断增大的新生物,应考虑完整切除肿块,对于I期、II期、IVs期(国际神经母细胞瘤分期系统)或L1期、MS期(国际神经母细胞瘤风险组分期系统)的新生物,不应包括任何化疗或放疗。总之,新生儿神经母细胞瘤多学科方法在疾病的早期诊断和准确分期方面效果更高,这对良好预后至关重要。

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