Braune C, Fangmann J, Scheumann G F, Klempnauer J
Orthopädische Universitätsklinik und Poliklinik, Friedrichsheim Frankfurt/Main.
Zentralbl Chir. 2001 Apr;126(4):318-21; discussion 322. doi: 10.1055/s-2001-14738.
The fibrolamellar karzinoma of the liver (FLC) as an uncommon variant of the hepatocellular karzinoma (HCC) is an indolent growing tumor. In its prior manifestation the FLC occurs at the adolescence and young adult stage. Early stage diagnosis and aggressive surgical treatment achieve better long-term results than usual resection of the HCC. Usually the FLC is, caused by its inconspicuous clinical appearance, diagnosed at a stage too advanced for effective surgical treatment. Especially the young patient's age and the remaining therapeutic options for palliative or curative treatment postulate a difficult decision for the surgeon. When a subtotal hepatectomy cannot be performed, total hepatectomy with liver transplantation is a valuable option. Palliative treatment protocols include systemic chemotherapy, ethanol instillation and chemoembolisation. We report the case of a 21-year-old male patient who presented with a recurrent intrahepatic FLC, peritoneal karzinomatosis confined to the right lower abdomen including gastric, splenic, diaphragmatic and colon transversum metastasis 14 months after primary surgery. We selected this patient as a reasonable candidate for an extended resection in trying to offer the optimal therapeutic modality. Thus we performed a right hemihepatectomy, near complete resection of the right diaphragm, total gastrectomy with lymphadenectomy including en bloc resection of spleen, colon transversum, omentum majus and peritonectomy of the paravesical region. Furthermore hyperthermic intraperitoneal chemotherapy was carried out the next day. The patient's postoperative course remained uncomplicated with fast recovery. Presently, 6 months after surgery, the patient has no evidence of recurrence.
肝纤维板层癌(FLC)作为肝细胞癌(HCC)的一种罕见变体,是一种生长缓慢的肿瘤。FLC先前表现为在青少年和年轻成人阶段发病。早期诊断和积极的手术治疗比常规的HCC切除术能取得更好的长期效果。通常由于其不明显的临床表现,FLC在诊断时已处于无法进行有效手术治疗的晚期阶段。特别是年轻患者的年龄以及姑息或根治性治疗的剩余选择,给外科医生带来了艰难的决策。当无法进行肝次全切除术时,肝移植全肝切除术是一种有价值的选择。姑息治疗方案包括全身化疗、乙醇注射和化疗栓塞。我们报告一例21岁男性患者,其在初次手术后14个月出现复发性肝内FLC,腹膜癌转移局限于右下腹,包括胃、脾、膈和横结肠转移。我们选择该患者作为扩大切除术的合理候选者,试图提供最佳治疗方式。因此,我们进行了右半肝切除术、近乎完全切除右膈、全胃切除术及淋巴结清扫术,包括整块切除脾脏、横结肠、大网膜和膀胱旁区域的腹膜切除术。此外,术后第二天进行了腹腔内热化疗。患者术后恢复顺利,无并发症。目前,术后6个月,患者无复发迹象。