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新生儿间叶性肝错构瘤;病例报告

Mesenchymal liver hamartoma in a newborn; case report.

作者信息

Balmer B, Le Coultre C, Feldges A, Hanimann B

机构信息

Kinderchirurgische Klinik, Ostschweizerisches Kinderspital, St. Gallen, Suisse.

出版信息

Eur J Pediatr Surg. 1996 Oct;6(5):303-5. doi: 10.1055/s-2008-1071003.

DOI:10.1055/s-2008-1071003
PMID:8933137
Abstract

A newborn boy had a severe, unexplained apnoea. On clinical examination a palpable abdominal mass was found extending over the right hemiabdomen. Further investigations by Ultrasound, CT scan and MRI revealed a liver tumor (7 x 5 x 9 cm). Histology from an open liver biopsy confirmed the diagnosis of a benign mesenchymal hamartoma. Operative resection of the tumor was performed when the patient was 2 1/2 months old. Postoperative management and clinical follow-up for twelve months were without complications. Primary liver tumors are rarely found in children. About half of them are malignant and associated with high mortality rates (e.g.hepatoblastoma). The most common benign liver tumors are mesenchymal hemangiomas and hamartomas. In this group of tumors prognosis has improved mainly due to progress in imaging techniques and progress in liver surgery during recent years.

摘要

一名男婴出生后出现严重的不明原因呼吸暂停。临床检查发现可触及腹部肿块,延伸至右侧半腹。超声、CT扫描和MRI进一步检查显示肝脏有一个肿瘤(7×5×9厘米)。开放肝脏活检的组织学检查确诊为良性间叶性错构瘤。患儿2个半月大时进行了肿瘤手术切除。术后十二个月的管理和临床随访均无并发症。儿童原发性肝肿瘤很少见。其中约一半是恶性的,且死亡率很高(如肝母细胞瘤)。最常见的良性肝肿瘤是间叶性血管瘤和错构瘤。在这组肿瘤中,近年来由于成像技术的进步和肝脏手术的进展,预后有所改善。

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