Petit T, de Lagausie P, El Ghoneimi A, Garel C, Aigrain Y
Department of Paediatric Surgery, Robert Debré University Hospital, Paris, France.
Eur J Pediatr Surg. 2001 Dec;11(6):411-4. doi: 10.1055/s-2001-19720.
Cystic adrenal neuroblastoma (NB) is highly unusual. We report two cases of cystic NB, detected antenatally and emphasize postnatal strategy management. Case 1: a right cystic mass was detected in a foetus in the 34th week of pregnancy, and checked as the same in the 36th week. Postnatal ultrasonography confirmed the presence of a growing adrenal cystic mass. MRI and MIBG scintigraphy suggested an isolated adrenal tumour, without catecholamine secretion. Surgical resection was decided upon and pathological examination confirmed the diagnosis of cystic NB. Follow-up found a recurrence at 10 months and the patient underwent excision after chemotherapy. Histology confirmed the metastatic origin from NB. Case 2: a right adrenal cystic mass was detected during pregnancy, with no regression of tumour size on postnatal US. MRI, MIBG scintigraphy, and catechol plasma and urinalysis led to the conclusion of an isolated non-secreting lesion. A right adrenalectomy was performed at 1 month. Pathologic examination diagnosed a cystic NB. We would suggest serial US examinations and early removal of any cystic mass with characteristic US signs (thick complex wall) or without regression on one month follow-up. Prenatal diagnosis may provide the best chances for neonatal excision and good prognosis.
肾上腺囊性神经母细胞瘤(NB)极为罕见。我们报告两例产前检测出的肾上腺囊性NB病例,并着重介绍产后的治疗策略。病例1:一名胎儿在妊娠34周时被检测出右侧有一个囊性肿块,36周时复查结果相同。产后超声检查证实存在一个不断增大的肾上腺囊性肿块。磁共振成像(MRI)和间碘苄胍(MIBG)闪烁扫描显示为孤立性肾上腺肿瘤,无儿茶酚胺分泌。决定进行手术切除,病理检查确诊为肾上腺囊性NB。随访发现10个月时复发,患者在化疗后接受了切除手术。组织学检查证实转移源于NB。病例2:孕期检测出右侧肾上腺囊性肿块,产后超声检查显示肿瘤大小未缩小。MRI、MIBG闪烁扫描以及儿茶酚血浆和尿液分析得出结论,这是一个孤立的无分泌功能的病变。患儿1个月时进行了右侧肾上腺切除术。病理检查诊断为肾上腺囊性NB。我们建议进行系列超声检查,对于任何具有特征性超声表现(厚而复杂的壁)或随访1个月无缩小的囊性肿块应尽早切除。产前诊断可能为新生儿切除手术及良好预后提供最佳机会。