Madanur Mansoor Ahmed, Battula Narendra, Davenport Mark, Dhawan Anil, Rela Mohamed
Kings College Hospital, Denmark Hill, London, SE5 9RS, UK.
Pediatr Surg Int. 2007 Jun;23(6):609-11. doi: 10.1007/s00383-006-1827-0. Epub 2006 Oct 26.
Hepatoblastoma (HB) is a rare germ cell tumour of childhood usually presenting with progressive abdominal distention. However, presentation as acute abdomen is a rare occurrence and is secondary to spontaneous rupture. This presentation carries high mortality. To our knowledge, six cases of ruptured hepatoblastoma have previously been reported, although the long-term outcome has not been clear. We report a case of ruptured HB who was managed by initial control of haemorrhage by laparotomy followed by chemotherapy with high-risk hepatoblastoma protocol as per SIOPEL 2 (cisplatin, carboplatin and doxorubicin) and a staged hepatectomy 5 months later. Patient is currently disease free at 6-year follow-up. Staged hepatectomy after initial control of haemorrhage does not preclude a curative resection.
肝母细胞瘤(HB)是一种罕见的儿童生殖细胞肿瘤,通常表现为进行性腹胀。然而,以急腹症形式出现的情况较为罕见,多继发于自发性破裂。这种表现具有很高的死亡率。据我们所知,此前已有6例肝母细胞瘤破裂的病例报道,但长期预后尚不清楚。我们报告了1例肝母细胞瘤破裂的病例,该病例最初通过剖腹手术控制出血,随后按照SIOPEL 2(顺铂、卡铂和阿霉素)的高危肝母细胞瘤方案进行化疗,并在5个月后进行分期肝切除术。患者在6年随访时目前无疾病。在最初控制出血后进行分期肝切除术并不排除根治性切除。