Merli Laura, Mussini Charlotte, Gabor Flaviu, Branchereau Sophie, Martelli Hélène, Pariente Danièle, Guérin Florent
Unit of Hepato-Biliary and Transplant Surgery, Department of Surgery and Transplantation Centre, Bambino Gesù Childrens Hospital, Rome, Italy.
Department of Pathology, Bicêtre Hospital, Le Kremlin-Bicêtre, France.
Eur J Pediatr Surg. 2015 Feb;25(1):132-7. doi: 10.1055/s-0034-1387937. Epub 2014 Sep 26.
Undifferentiated sarcoma of the liver (USL) is the third commonest malignant liver tumor in children. The aim of our study is to evaluate the outcome of this rare entity according to the quality of the surgical resection and the compliance to the European pediatric soft tissue sarcoma group guidelines.
We conducted a monocentric review of patients referred to our department with a definitive pathologic diagnosis of USL between 1997 and 2013. We looked at the diagnosis and management pitfalls, surgical technique, and outcomes. Results are expressed as median (range).
There were 13 patients (M/F=7:6=1.1). Age at presentation was 8 years (range, 11 months-16 years). Of the 13 patients, 10 patients (69%) presented with abdominal pain, 5 (38%) with tumoral bleeding, and 2 (15%) with peritoneal rupture. All lesions were unique, nonmetastatic, and heterogeneous with cystic components measuring 14 (6-19) cm. Six (46%) patients had an upfront surgery: five because of wrong clinical diagnosis (three query mesenchymal hamartoma, one spontaneous peritoneal bleeding, and one cystic lymphangioma), and one because of rapid enlargement of the mass. Seven (54%) patients (including one with tumoral bleeding) received neoadjuvant chemotherapy, and had their tumor diameter decreasing by 40% (range, 0-60%). Final surgery consisted of seven right hepatectomies; one right extended hepatectomy; three mesohepatectomies; two left hepatectomies. There were three incomplete resection in the upfront surgery group versus none in the neoadjuvant chemotherapy group. The degree of tumor necrosis after chemotherapy ranged from 95 to 100%. Surgical complications included the following: liver transplantation (LT) following a Budd-Chiari syndrome after a mesohepatectomy, one biliary ducts injury treated by Roux-en-Y loop. All patients received the postoperative chemotherapy according to the European protocol. One of seven patients (14%) with neoadjuvant chemotherapy underwent radiotherapy for rupture at diagnosis versus three of six patients (50%) with upfront surgery: one for rupture at diagnosis and two for rupture during upfront surgery. One patient (17%) with upfront surgery had local recurrence at 2 years after initial surgery, and is in second complete remission 1 year after a redo surgery. All patients are alive at a median 34 months (range, 5-134) follow-up.
USL presents with painful mixed cystic and solid liver mass. If misdiagnosed and mistreated (enucleation or unroofing), the usual good outcome of this malignancy could be impaired. Preoperative chemotherapy is recommended.
肝未分化肉瘤（USL）是儿童中第三常见的恶性肝脏肿瘤。我们研究的目的是根据手术切除质量和对欧洲儿科软组织肉瘤组指南的依从性来评估这种罕见实体的治疗结果。
我们对1997年至2013年间转诊至我科并最终病理诊断为USL的患者进行了单中心回顾性研究。我们观察了诊断和治疗中的陷阱、手术技术及结果。结果以中位数（范围）表示。
共13例患者（男/女 = 7:6 = 1.1）。就诊时年龄为8岁（范围11个月至16岁）。13例患者中，10例（69%）表现为腹痛，5例（38%）表现为肿瘤出血，2例（15%）表现为腹膜破裂。所有病变均为单发、无转移且不均质，伴有大小为14（6 - 19）cm的囊性成分。6例（46%）患者接受了一期手术：5例是因为临床诊断错误（3例怀疑间叶性错构瘤，1例自发性腹膜出血，1例囊性淋巴管瘤），1例是因为肿块迅速增大。7例（54%）患者（包括1例肿瘤出血患者）接受了新辅助化疗，肿瘤直径缩小了40%（范围0 - 60%）。最终手术包括7例右半肝切除术；1例右扩大肝切除术；3例中肝切除术；2例左半肝切除术。一期手术组有3例切除不完全，而新辅助化疗组无。化疗后肿瘤坏死程度为95%至100%。手术并发症包括：中肝切除术后布加综合征行肝移植，1例胆管损伤行Roux-en-Y吻合术治疗。所有患者均根据欧洲方案接受了术后化疗。接受新辅助化疗的7例患者中有1例（14%）因诊断时破裂接受了放疗，而一期手术的6例患者中有3例（50%）：1例因诊断时破裂，2例因一期手术期间破裂。1例一期手术患者在初次手术后2年出现局部复发，再次手术后1年处于第二次完全缓解期。所有患者在中位随访34个月（范围5 - 134个月）时均存活。
USL表现为伴有疼痛的肝脏混合性囊性和实性肿块。如果误诊和误治（摘除或开窗），这种恶性肿瘤通常良好的治疗结果可能会受到损害。建议术前化疗。
