Emergency Hepatic Resection a Life Saving Procedure in Ruptured Hepatoblastoma: Case Report

Excerpt

Hepatoblastoma is the most common primary liver tumor in children, accounting for just over 1 % of pediatric cancers with most cases occurring prior to 5 years [1]. The incidence of this tumor has not changed since last decade with average male: female ratio of 2:1 [2]. The etiology is unknown, but it has been associated with Beckwith-Weidemann syndrome [3], familial adenomatosis polypi [4], and low birth weight [5]. These tumors present with an asymptomatic abdominal mass palpated either by a parent or paediatrician. Abdominal pain, weight loss, anorexia, nausea, and vomiting may be present, particularly in advanced disease. Imaging is an important aspect of diagnosis of these patients, which includes an ultrasonography of abdomen and a contrast enhanced CT scan. The management is multimodal, involves medical oncologists, surgical oncologists, paediatricians and specialist nursing team. The primary treatment is surgical resection: however, chemotherapy plays an important role by increasing the number of tumors that are resectable. Serum Alpha Feto-Protein (AFP) is a sensitive biological marker of the disease. There is a correlation between AFP and extent of disease for all stages, and the rate of decline in AFP with treatment is prognostic. Low AFP levels are associated with anaplastic histology and poor outcome [6]. Tumor size, number of involved lobes, regional lymph node involvement, and distant metastases determine stage [7]. The prognosis for patients with resectable tumors is fairly good; however, the outcome for those with nonresectable or recurrent disease is poor. Surgical resections done can be anatomical or non-anatomical resections. Resections as much as trisegmentectomies with Intraoperative trans-arterial embolisation have been done in patients with adequate hepatic reserve for ruptured hepatoblastomas [8]. In very few occasions hepatoblastomas have presented with spontaneous rupture with hemoperitoneum, we are presenting one such case. …