Prognostic impact of pre-referral tumor resection in unilateral Wilms tumor: A single-institute experience from a lower middle-income country.

INTRODUCTION: The objectives of this study were to evaluate the prognostic impact of pre-referral surgical resection of Wilms tumor (WT) performed at non-oncology centers, and to strategize an improved care plan for this very curable pediatric tumor. METHODS: In this study conducted in a large pediatric cancer center in Pakistan, we retrospectively reviewed the electronic medical records (EMR) of 149 patients with unilateral WT from September 2008 to August 2017. Based on treatment approach, patients were categorized into two groups: (i) pre-referral tumor resection (PTR: n = 75), and (ii) post-neoadjuvant chemo nephrectomy (PCN: n = 74). RESULTS: The proportion of metastatic disease in PTR and PCN groups was 33.3% and 35.1%, respectively. In the PTR subset, median time to admission after PTR was 5 weeks (mean 11, SEM 2.8, range: 2-202) weeks, with 53.3% (n = 40) presenting more than 4 weeks after PTR. Twenty patients had no cross-sectional imaging prior to PTR and underwent surgery after abdominal ultrasound only. On baseline imaging at our center, 58.7% (n = 44) of the PTR group had radiologically evaluable disease (four metastases only, 19 local residual tumor only, 21 both localized tumor and visible metastases). Disease staging was uncertain in 23 patients because of no or inadequate histology specimens and/or lymph node sampling in patients with no evaluable disease. Statistically significant differences were recorded for the two subsets regarding tumor volume, extent and nodularity, renal vein and renal sinus involvement, lymph node status, tumor rupture and histopathologic features, and tumor stage, with a 10-year event-free survival (EFS) for PCN and PTR of 74.3% and 50.7%, respectively (p < .001). In the PTR group, EFS for those presenting within 4 weeks and later was 91.4% versus 15.0%, respectively (p < .0001). CONCLUSION: Suboptimal pre-referral surgical intervention results in poor survival outcomes in unilateral WT. Our findings highlight the need for a comprehensive action plan for educating healthcare professionals engaged in WT diagnosis and referral process. PCN in a multidisciplinary team approach can reduce surgical morbidity and seems to be a better strategy to improve the survival rates in low-resource settings.

Prophylactic Antibiotics for Reducing Central Line-associated Blood Stream Infection in Children with Totally Implantable Venous Access Devices.

OBJECTIVE: To analyse the need of prophylactic antibiotic before the insertion of totally implantable venous access devices (TIVADs) in terms of preventing central line-associated blood stream infection (CLABSI) in early postoperative period in pediatric oncology patients. STUDY DESIGN: A cohort study. PLACE AND DURATION OF STUDY: Shaukat Khanum Memorial Cancer Hospital and Research Centre, Lahore from January 2005 to June 2016. METHODOLOGY: A total of 645 consecutive children with malignancy, who were implanted with TIVAD, were included in the study. The data were collected retrospectively and divided into two groups on the basis of prophylactic antibiotic administration (Group A) received the antibiotic; and Group B did not receive the antibiotic. Both the groups were compared in terms of positive central blood cultures in the absence of any other clinical source of infection during the early postoperative period of 30 days. RESULTS: The overall infection rate was 12.54% (35 out of 279) in Group B (did not receive prophylactic antibiotic) and 11.68% (41 out of 351) in Group A (received prophylactic antibiotic) without any significant difference (p = 0.741). CONCLUSION: There was no advantage of the use of prophylactic antibiotic before TIVAD insertion in preventing early postoperative CLABSI.

Short term surgical outcomes of Wilms tumour from a single institute.

Wilms tumour is the commonest solid tumour of childhood in Pakistan. In developed world the long-term outcome of Wilms tumour have significantly improved reaching up to 98% long term survival. We have no National data of prevalence, treatment and survival of this condition in Pakistan. Surgical treatment also varies in various parts of the country. We have studied our patients presenting with a diagnosis of Wilms tumour to our institute from Jan 2014 to April 2016. A total of 42 patients were operated for Wilms tumour. The most common symptoms were abdominal mass (75%), pain (28%) and fever (19%). A total of 48 tumour resections were performed, 45 total nephrectomies and 3 had nephron sparing surgery (NSS). Mean operative time was 225±78.7 minutes. Mean blood loss was 165±223.5ml. Mean size of the tumour was 102±48.4mm and mean weight of the tumour was 433±400.7gm. Ninety percent patients had a favourable histology. Mean high dependency unit (HDU) stay was 1.16±1.2 day and mean hospital stay was 6.89±3.47 days. Complications were observed in 8 patients. Surgery remains a major part of treatment for Wilms tumour.

Clinical profile, treatment and survival outcomes of peadiatric germ cell tumours: A Pakistani perspective.

Germ Cell Tumours (GCTs) are rare tumours. Generally 80% are benign and 20% malignant with a bimodal age distribution. The retrospective study was conducted at Shaukat Khanum Cancer Hospital, Lahore, Pakistan, and comprised all paediatric patients below 18 years of age who received treatment for histology-proven GCT from 2006 to 2014. Of the 207 patients, 98(42.3%) were males and 109(52.7%) were females. The most common GCT was yolk sac tumour in 90(43.5%) children followed by mixed GCT in 40(19.3%) and dysgerminoma in 34(16.4%). Gonads were most commonly involved in 165(79.7%) patients with metastasis in 24(11.6%) at presentation and recurrence in 26(12.5%) patients. Overall, 133(64.3%) patients are well and followed up at regular intervals and 55(26.5%) have been lost to follow-up with an expected overall 5-year median survival of 45%. Despite the distinct clinical profile of paediatric GCT, survival can be improved by early diagnosis, regimented treatment according to set guidelines, protocols and by improving follow-up.

Endoscopic cystogastrostomy: minimally invasive approach for pancreatic pseudocyst.

Pancreatic pseudocysts in children are not uncommon. Non-resolving pseudocysts often require surgical intervention. Endoscopic cystogastrostomy is a minimally invasive procedure which is recommended for this condition. We report a large pancreatic pseudocyst in a 4-year old child, which developed following therapy with PEG-Asparaginase for acute lymphoblastic leukemia. It was managed with minimally invasive procedure.

Separation of xiphi-omphalo-ischiopagus tetrapus twins with favorable internal anatomy.

Conjoined twinning is a rare occurrence, and ischiopagus variety is even more rare. So far, only 20 cases have been reported. The incidence is 1 in 500,000. Because of its rarity and variable anatomy, no definite line of treatment can be adapted. Time of operation and mode of treatment have to be tailored according to the local circumstances and organ sharing in each individual pair. The twins in this report were xi phi-omphalo-ischiopagus tetrapus conjoined twins. Although several internal organs were fused and shared, they were complete mirror image sets for each twin. In this case report, details of anatomy and more importantly, preoperative investigations, preparations, and organizational aspects that were designed to suit local conditions are described. Operative details, postoperative care, and short and long-term progress also has been highlighted.