Background and Aims

Hepatoblastoma(HB) is the most common pediatric malignant liver tumour. However, none of the present prognostic indicators are targetable or clinically actionable.

This study is to assess the relationship between anti-TERT expression in hepatoblastoma tumour tissue and recurrence.

Methods

Retrospective single centre pilot study of patients treated for hepatoblastoma from January 2010 to December 2018. Patients of HB with preserved tumour specimen were included. Demographic, clinical details, disease characteristics were recorded. Slides were prepared from blocks and stained with anti TERT antibody.

Results

Thirteen patients were included. Mean age of presentation was 26.1months (5-90 months). Staging was PRETEXT-I in 1/13 (7.7%), PRETEXT-II in 8/13 (61.5%), PRETEXT-III in 4/13 (30.8%) and no PRETEXT-IV patients. High Risk disease was observed in 5/13 (38.5%) patients, among them 3/5 had metastatic disease at presentation. One patient had inoperable disease after chemotherapy while remaining twelve patients had undergone resection with clear margins The histological types were embryonal and mixed embryonal/foetal subtype in seven patients, pure fetal in one patient and mixed epithelial and mesenchymal in five. Mean follow up duration was 33.4 months(12-60 months). The 5-year overall survival was 59.3%. Three patients developed recurrence.

Nuclear staining by TERT was positive in four patients. Of this three developed recurrence and expired. Strong correlation (p<0.01) between nuclear staining and recurrence was observed. No significant association between nuclear staining or recurrence with other parameters.

Conclusions

Although complete resection could be achieved in majority of patients with hepatoblastoma, recurrence was seen in 23% with poor outcome. Anti-TERT could be used to identify such patients who are likely to have cancer recurrence requiring aggressive chemoadjuvant therapy, close follow up or in the future, telomerase- targeted therapy. Further studies are needed to validate it.

Background and Aims

Undifferentiated embryonal sarcoma of the liver (UESL) is the third most common liver malignancy of childhood. Intratumoral hemorrhage and rupture of UESL represents a complex clinical scenario for which local control requires deliberate preoperative planning and consideration for advanced neoadjuvant intervention. Interestingly, liver was the only anatomic site for which radiotherapy was not included in the Children’s Oncology Group ARST0332, soft tissue sarcoma unresectable at diagnosis, neoadjuvant treatment algorithm. Here we present our institutional experience with ruptured UESL in children and describe the use of neoadjuvant transarterial radioembolization (TARE) for initially unresectable tumors.

Methods

We reviewed all cases of ruptured UESL treated at our children’s hospital over the past five years. Descriptive analyses were performed to evaluate patient and tumor characteristics. Detailed review of neoadjuvant therapy, operative technique, and adjuvant regimens was performed.

Results

Four patients presented with ruptured UESL during the study period. Ages ranged from four to 17 years at diagnosis. All four tumors emanated from the right liver; one of which had expanded to fill the entire abdomen. Sites of extrahepatic tumor at diagnosis included lung metastasis(1); IVC/RA tumor thrombus(1); diaphragm/pleural effusion(3); peritoneal cavity myxoid tumor/hematoma rupture(3). One patient underwent upfront resection. Of the three who received neoadjuvant chemotherapy, two also received neoadjuvant TARE and one received chemoembolization. Pathologically negative margins were achieved in all four patients. The two patients who received TARE experienced significant tumor regression prior to resection and had dramatic tumor necrosis on pathologic review. All four patients are alive with no evidence of disease at last follow-up (median 36.5 months).

Conclusions

Ruptured UESL is a complicated oncologic situation that requires individualized management. When upfront resection is not feasible, the use of neoadjuvant TARE provides an opportunity to enhance local control and, thus, facilitate long-term survival.

Background and Aims

The complex process of diagnosing, staging and treating Liver Tumours in Children is unique in a resource-limited setting. A previous review in South Africa found a low tumour resection rate due to advanced disease. This study intends to explore recent changes in interventions, outcomes and prognostic factors of paediatric liver tumours in Johannesburg, South Africa, in order to improve decision-making and survival. The study aims to describe the experiences and outcomes of paediatric liver tumours at Chris Hani Baragwanath Academic Hospital (CHBAH).

Methods

A retrospective review of all patients aged below 16 years, with benign or malignant liver tumours, from 1^st January 2005 to 31^st December 2017.

Results

Twenty Nine children presented with primary hepatic tumours, 16 (55%) were female; 13 (45%) were male, and the median age was 3 years. Biopsy was done in all cases, there were 15 hepatoblastomas (52%), five hepatocellular carcinomas (17%), three undifferentiated embryonal sarcomas (10%), 2 hamartomas, 2 vascular tumours, and 2 rhabdomyosarcomas. Three cases were PRETEXT stage 1 (11%), 15 were stage 2 (55%), four stage 3 (15%), and four stage 4 (15%). Seven children (23%) had metastasis, four (13%) to the lungs, and three (10%) to the bone. Sixteen cases had surgery, 8 right hepatectomies, 5 extended right hepatectomies, 1 left sectionectomy, and 2 emergencies for rupture. Six cases had inoperable tumours, four demised before surgery, and two defaulted. Overall survival was 48% (14), 38% died (11), and 14%(4) were lost to follow-up. Ten of 15 hepatoblastomas survived (67%), two of three Embryonal Sarcoma’s (66%), one of two hamartoma’s (50%), and one of two vascular tumour’s (50%). Hepatocellular carcinomas and rhabdomyosarcomas both had 0% survival rates.

Conclusions

There was a poor overall survival of 48%, influenced by the large number of inoperable tumours, distant metastases, unfavourable histology, patients demising before surgery or defaulting treatment, necessitating an earlier diagnosis and expedited management.

Background and Aims

Pancreatic neoplasms are very rare in children. There are few large pediatric series in the literature, so multicentric studies are needed in order to add knowledge to the topic. The Latin American Pediatric Surgical Oncology Collaborative Group was recently created and chose to address this topic.

Methods

Retrospective review of clinical and surgical features of pediatric patients with pancreatic tumors cases diagnosed at 7 Latin American hospitals, between 2006 and 2018. Participating centers were invited by local pediatric surgical societies, and local ethical were approved

Results

Forty-nine patients were included, 59% were female. The median age at operation was152 months. Most patients presented with abdominal pain and abdominal mass. Two had associated syndromes, one with Wolf-Parkinson and another with congenital hydrocephaly. Magnetic resonance imaging and computed tomographic scan were used in 87% and 38%, respectively. Four patients were metastatic (2 pancreatoblastoma with liver metastasis, 1 neuroendocrine tumor with lymph node metastasis and 1 desmoplasic tumor with liver metastasis), two patients had a metastatic tumor at the pancreas (Ewing’s and synovial sarcoma). Thirty-two patients underwent surgery (Whipple procedure in 16, distal pancreatectomy in 8 cases, tumorectomy in 7). The most common histology was solid pseudopapillary neoplasm (SPN) (n = 26), followed by pancreatoblastoma (n= 5), neuroendocrine tumor (n = 4), lymphoma (n = 3) and others (myofibrobastic tumor, desmoplasic tumor, insulinoma, indiferencied carcinoma, Ewing’s sarcoma, synovial sarcoma). Thirty-eight patients (84.4 %) are currently alive and disease free at a median follow-up of months. All SPN patients are alive. Five patients died: 2 pancreatoblastoma, 1 neuroendocrine tumor,1 lymphoma, and 1 sarcoma.

Conclusions

We report the first multicentric Latin American collaboration on pediatric surgical oncology, focused on pancreatic tumors. Patients with SPN have an excellent outcome, suggesting that limited surgical resection may be appropriate for these patients. Other malignant pancreatic tumors are more aggressive.