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产前检测出的肾上腺肿块的临床特征及手术结果

Clinical features and surgical outcome of a suprarenal mass detected before birth.

作者信息

Moon Suk-Bae, Shin Hyun-Baik, Seo Jeong-Meen, Lee Suk-Koo

机构信息

Division of Pediatric Surgery, Department of Surgery, Samsung Medical Center, Sungkyunkwan University School of Medicine, #50 Ilwon-dong, Gangnam-gu, Seoul, 135-710, Korea.

出版信息

Pediatr Surg Int. 2010 Mar;26(3):241-6. doi: 10.1007/s00383-009-2531-7. Epub 2009 Nov 12.

Abstract

PURPOSE

With the widespread use of the obstetrical ultrasound, identification of a fetal suprarenal mass has become more common. Most of these masses prove to be congenital neuroblastomas (CNB), but the diagnosis is often confused with other benign lesions and the postnatal management remains to be controversial.

METHODS

The medical records of 18 patients that underwent primary surgical excision for an antenatally detected suprarenal mass, between January 1995 and April 2009, were reviewed. The clinical, radiological, surgical, and pathological data were collected. Staging evaluation was performed after histological confirmation of the CNB.

RESULTS

There were 13 cases of CNB, 1 adrenal cyst, 2 adrenal hemorrhages, and 2 pulmonary sequestrations. The differential diagnosis was impossible before surgery. Most of the CNBs were stage I (N = 11), with 1 stage IV and 1 stage IV-S. Four patients (3 stage I and 1 stage IV-S) had more than one copy of N-myc gene. The stage I patients were cured by surgery alone, and stage IV patients underwent nine cycles of adjuvant chemotherapy and currently have no evidence of disease. The five benign lesions were cured with excision alone. There were no postoperative complications.

CONCLUSION

For early diagnosis and treatment, surgical excision should be considered as the primary therapy for an antenatally detected suprarenal mass. The surgery can be safely performed during the neonatal period and provides a cure in most cases.

摘要

目的

随着产科超声的广泛应用,胎儿肾上腺肿块的识别变得更为常见。这些肿块大多被证实为先天性神经母细胞瘤(CNB),但其诊断常与其他良性病变相混淆,且产后管理仍存在争议。

方法

回顾了1995年1月至2009年4月期间18例因产前检测到的肾上腺肿块而接受初次手术切除的患者的病历。收集了临床、放射学、手术和病理数据。在CNB组织学确诊后进行分期评估。

结果

有13例CNB、1例肾上腺囊肿、2例肾上腺出血和2例肺隔离症。术前无法进行鉴别诊断。大多数CNB为I期(N = 11),1例为IV期,1例为IV-S期。4例患者(3例I期和1例IV-S期)有多个N-myc基因拷贝。I期患者仅通过手术治愈,IV期患者接受了9个周期的辅助化疗,目前无疾病证据。5例良性病变仅通过切除治愈。无术后并发症。

结论

为了早期诊断和治疗,手术切除应被视为产前检测到的肾上腺肿块的主要治疗方法。该手术可在新生儿期安全进行,且在大多数情况下可治愈。

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