Tushar Vora

Paediatric cancer stage in population-based cancer registries: the Toronto consensus principles and guidelines.

Population-based cancer registries generate estimates of incidence and survival that are essential for cancer surveillance, research, and control strategies. Although data on cancer stage allow meaningful assessments of changes in cancer incidence and outcomes, stage is not recorded by most population-based cancer registries. The main method of staging adult cancers is the TNM classification. The criteria for staging paediatric cancers, however, vary by diagnosis, have evolved over time, and sometimes vary by cooperative trial group. Consistency in the collection of staging data has therefore been challenging for population-based cancer registries. We assembled key experts and stakeholders (oncologists, cancer registrars, epidemiologists) and used a modified Delphi approach to establish principles for paediatric cancer stage collection. In this Review, we make recommendations on which staging systems should be adopted by population-based cancer registries for the major childhood cancers, including adaptations for low-income countries. Wide adoption of these guidelines in registries will ease international comparative incidence and outcome studies.

Primary non-metastatic Ewing sarcoma of the jaw in children: results of surgical resection and primary reconstruction.

The rarity of Ewing sarcoma (ES) of the jaw coupled with the technical challenge of resection and associated functional and cosmetic impairment has resulted in deficient data on surgical management of these tumors. The purpose of this study is to describe the results of surgical excision and reconstruction of primary non‐metastatic ES of the mandible and maxilla in children.

Alarming prevalence of community-acquired multidrug-resistant organisms colonization in children with cancer and implications for therapy: A prospective study.

BACKGROUND Infection or colonization with multidrug-resistant organisms (MDRO) is associated with high mortality and morbidity. Knowledge of MDRO colonization may help in planning empirical antibiotic approach in neutropenic patients, which is known to improve patient outcomes. While routine cultures are positive and may help direct antibiotic therapy in only up to 15% neutropenic patients, surveillance cultures are positive in more than 90% of cancer patients. AIMS To assess the rate of MDRO carrier status at presentation and rate of conversion to MDRO during the treatment. MATERIALS AND METHODS Rectal swabs of all the outpatients presenting to pediatric oncology unit were sent within 7 days from date of registration from January 2014 to December 2014. Furthermore, stool cultures/rectal swabs of all patients who got directly admitted to the pediatric ward at presentation were sent within 24 h. Repeat rectal swabs were sent again for patients from this cohort when they got readmitted to the ward at least 15 days after last discharge or when clinically indicated. RESULTS Baseline surveillance rectal swabs were sent for 618 patients, which included 528 children with hematological malignancies and 90 children with solid tumors. Forty-five (7.3%) showed no growth. Of the remaining 573, 197 (34.4%) patients were colonized by two organisms and 30 (5.2%) by three organisms. Three hundred and thirty-four (58.4%) showed extended spectrum beta-lactamase (ESBL) Enterobacteriaceae, of which 165 (49.5%) were ESBL sensitive to beta-lactam with beta-lactamase inhibitors combinations and 169 (50.5%) were resistant to combinations. One hundred and sixteen (20.2%) were carbapenem-resistant Enterobacteriaceae (CRE) and 65 (11.4%) had vancomycin-resistant enterococci in baseline cultures. Only 63 (21%) patients were colonized by a sensitive organism in their baseline surveillance cultures. Morbidity (Intensive Care Unit stay) and mortality was higher in patients colonized by MDR organisms. There was a significant correlation between the place of residence and CRE colonization status with the highest rate (60%) of CRE colonization observed in children from East India. The repeat cultures showed the further conversion of sensitive isolates to MDRO in 80% of these children, of which 40% each converted from non-ESBL and non-CRE to ESBL and CRE, respectively. CONCLUSION This is the first study illustrating the alarming high prevalence of community-acquired MDRO colonization, especially CRE, which has grave implications for therapy for children with cancer potentially compromising delivery of aggressive chemotherapy and affecting outcomes. This incidence further increases during the course of treatment. Knowing the baseline colonization also guides us for the planning of chemotherapy as well as antibiotic approach and infection control strategies. Local antibiotics stewardship including education of the healthcare workers as well as national level interventions to prevent antibiotic misuse in the community is critical to minimize this problem.

Nutritional status in survivors of childhood cancer: Experience from Tata Memorial Hospital, Mumbai.

BACKGROUND Survivors of childhood cancer are at increased risk for several cardiometabolic complications. Obesity/overweight and metabolic syndrome have been widely reported in Western literature, but data from India are lacking. AIMS To perform an objective assessment of nutritional status in a cohort of childhood cancer survivors (CCSs) and to find risk factors for extremes in nutritional status. SETTINGS AND DESIGN The study was a retrospective chart review of CCSs who attended the late effects clinic of a referral pediatric oncology center over the period of 1 year. MATERIALS AND METHODS An objective assessment of nutritional status was done, and results were analyzed in two groups: Adult survivors (present age <18 years) and child and adolescent survivors (CASs) (<18 years). The data were then analyzed for possible risk factors. RESULTS Six hundred and forty-eight survivors were included in the study; of these, 471 were <18 years at follow-up, and 177 were 18 years or older. The prevalence of obesity, overweight, normal, and undernutrition was 2.6%, 10.8%, 62.7%, and 28.8% (CASs) and 0%, 8.5%, 62.7%, and 28.8% (adult survivors), respectively. Factors predictive of overweight/obesity were an initial diagnosis of acute lymphoblastic leukemia, or brain tumor and follow-up duration of >20 years or current age >30 years in adult survivors. CONCLUSIONS The prevalence of obesity/overweight is lower in our cohort when compared to Western literature. It remains to be clarified whether this reflects the underlying undernutrition in our country, or whether our cohort of survivors is indeed distinct from their Western counterparts. Comparison with age/sex-matched normal controls and baseline parameters would yield more meaningful results.

Prognostic factors in primary nonmetastatic Ewing sarcoma of the rib in children and young adults

BACKGROUND The rarity of Ewing sarcoma of rib has resulted in paucity of data, particularly on the prognostic factors and pattern of relapses. We analyzed the recurrences in patients with primary nonmetastatic Ewing sarcoma of the rib and examined prognostic factors of poor outcome. METHODS From January 2004 to January 2011, 37 patients were treated. After induction chemotherapy, complete (from costal cartilage to vertebra) or partial excision of involved rib with or without adjacent ribs was performed. Postoperative radiotherapy was administered for positive margins, poor response to chemotherapy, and large primary tumors with significant soft tissue component at presentation. RESULTS Disease relapsed in 16 patients: at the local site (n = 5), both local and distant (n = 2), and distant site only (n = 9). The projected 5-year cause-specific, relapse-free survival and local control were 50%, 44%, and 72%. Poor response to chemotherapy (>5% residual tumor) and resection of adjacent lung parenchyma (a surrogate for tumor extension) were adverse prognostic factors for relapse-free survival in multivariate analysis. CONCLUSION Relapses occurred more often at distant sites and had a poor outcome. In this study, poor histologic response to chemotherapy (P = .04) and the infiltration of adjacent lung parenchyma (P = .01) are adverse prognostic factors.

Diagnosis and Management of Rhabdomyosarcoma in Children and Adolescents: ICMR Consensus Document

Rhabdomyosarcoma (RMS) is a highly malignant tumor which is thought to originate from the pluripotent mesenchyme. It is the most common soft-tissue sarcoma of childhood. This review article summarizes the recent and older published literature and gives an overview of management of RMS in children. RMS can arise in a wide variety of primary sites, some of which are associated with specific patterns of local invasion, regional lymph nodal spread, therapeutic response and long term outcome, hence requiring physicians to be familiar with site-specific staging and treatment details. Most common primary sites include the head and neck region, genitourinary tract, and extremities. Prognosis for children and adolescents with RMS has recently improved substantially, especially for patients with local or locally extensive disease because of the development of multi-modal therapy incorporating surgery, dose-intensive combination chemotherapy, and radiation therapy. Despite aggressive approaches the outcome for patients who present with metastatic disease remains unsatisfactory. Clinical trials are ongoing to reduce toxicity and improve outcomes of such patients; newer agents in combination are being investigated.

Epidemiology of blood stream infections in pediatric patients at a Tertiary Care Cancer Centre

BACKGROUND Blood stream infections (BSI) are among the most common causes of preventable deaths in children with cancer in a developing country. Knowledge of its etiology as well as antibiotic sensitivity is essential not only for planning antimicrobial policy, but also the larger infection prevention and control measures. AIMS To describe the etiology and sensitivity of BSI in the pediatric oncology unit at a tertiary cancer center. MATERIALS AND METHODS All the samples representative of BSI sent from pediatric oncology unit during the period of January to December, 2013 were included in the study, and analyzed for microbiological spectrum with their antibiotic sensitivity. RESULTS A total of 4198 samples were representative of BSI. The overall cultures positivity rate was 6.97% with higher positivity rate (10.28%) from central lines. Of the positive cultures, 208 (70.9%) were Gram-negative bacilli (GNB), 71 (24.2%) were Gram-positive organisms, and 14 (4.7%) were Candida species. Lactose fermenting Enterobacteriaceae i.e., Escherichia coli (28.4%), Klebsiella pneumoniae (22.1%), and Enterobacter (4.8%) accounted for 55.3% of all GNB. Pseudomonas accounted for 53 (25.5%) and Acinetobacter 19 (9.1%) of GNB. Among Gram-positive isolates, staphylococci were the most frequent (47.8%), followed by Streptococcus pneumoniae 17 (23.9%), beta-hemolytic streptococci 11 (15.5%), and enterococci 9 (12.68%). Of GNB, 45.7% were pan-sensitive, 24% extended spectrum beta-lactamase (ESBL) producers, 27% were resistant to carbapenems, and 3.4% resistant to colistin. Pseudomonas was most sensitive, and Klebsiella was least sensitive of GNB. Of the staphylococcal isolates, 41.67% were methicillin-resistant Staphylococcus aureus (MRSA) and 10% of Coagulase Negative Stapylococci (CONS) were methicillin. CONCLUSION A high degree of ESBL producers and carbapenem-resistant Enterobacteriaceae is concerning; with emerging resistance to colistin, raising the fear of a return to the preantibiotic era. An urgent intervention including creating awareness and establishment of robust infection control and antibiotic stewardship program is the most important need of the hour.

Successful management of multi-focal hepatic infantile hemangioendothelioma using TACE/surgery followed by maintenance metronomic therapy.

Infantile hepatic hemangioendothelioma (IHE) is a rare angiogenic tumour with diverse clinical presentations and varied course ranging from spontaneous regression to life-threatening complications. The authors report a 2-year boy who presented with respiratory distress and was identified as a case of inoperable multi-focal hepatic IHE. He showed a transient response to trans-arterial-chemo-embolisation and liver resection but had progressive disease despite chemotherapy (prednisolone/vicristine/ifosfamide/cisplatin). Thereafter, he was successfully managed with metronomic therapy using cyclophosphamide and tamoxifen.

Management of Wilms Tumor: ICMR Consensus Document

Wilms tumor (WT) is the most common renal tumor of childhood. Although multidisciplinary care including surgery, chemotherapy and radiotherapy have greatly improved the survival rates in WT, there is a scope for further improvement in India and other resource-poor settings. In resource-limited settings, the majority of patients present with large tumors, which may either be unresectable or risky to resect; making preoperative chemotherapy followed by delayed surgery the preferred approach. Histology and staging are used for risk stratification. The imaging procedure of choice is Contrast Enhanced CT scan (CECT) of thorax/ abdomen and pelvis, which is to be done at presentation, as well as for re-evaluation. Surgery is the cornerstone of treatment in WT and Radical Nephroureterectomy and Lymph node sampling is the procedure of choice, to be performed at week 5 in Non Metastatic WT and week 7 in Metastatic WT. WT is an extremely chemosensitive and radiosensitive tumor. Preoperative chemotherapy for Non Metastatic WT consists of 4 wk of Vincristine /Actinomycin and 6 wk of Vincristine /Actinomycin/ Adriamycin for Metastatic WT, with post-operative chemotherapy depending on stage and histology. Radiation therapy is recommended mainly in Stage III and Stage IV WT, with other indications given in the text. Other recommendations, such as treatment of WT in special situations and for supportive care are also detailed in the text.

Adaptive radiation therapy for pediatric head and neck malignancies: Dosimetric implications

PURPOSE This study was designed to evaluate the role of adaptive radiation therapy for children receiving curative radiation therapy to the head and neck region. METHODS AND MATERIALS Ten children receiving definitive, conformal radiation therapy to the head and neck region were prospectively evaluated for anatomic changes during the course of radiation therapy. Images were acquired midway through the number of planned radiation therapy fractions during the planned course of radiation therapy. Body contours, target volumes, and organs at risk were redrawn on the new set of images. Two sets of additional treatment plans were generated: (1) a nonoptimized plan (plan 2), that is, an overlay of the original plan (plan 1) on the new set of contours, and (2) an optimized plan (plan 3) with the new set of contours. These 3 sets of plans were then compared for dosimetric differences. RESULTS Five children had nasopharynx carcinoma, whereas the other 5 had embryonal rhabdomyosarcoma. The average reduction in gross tumor volume was 40% (mean volume, 41.87 mL; P = .005). The average changes in right and left parotid volumes were 2.72 and 1.92 mL, respectively. With nonoptimized plans, the average increase in maximum dose to the spinal cord was 15% (79.99%-94.99%; P = .013). With reoptimization, the maximum dose to the spinal cord decreased from 94.99% to 85.26% (mean difference, -9.73%; P = .02). Average D99 for the planning target volume (dose received by 99% of the target volume) was 88.66% and 86.89% with the original and reoptimized plans, respectively (P = .50). For the entire group, the mean conformation number index with nonoptimized plans was reduced from 0.734 to 0.628 (P = .013). This improved with reoptimization (P = .114). The homogeneity index improved with reoptimization from a mean value of 0.113 to 0.098 (P = 0.28). For nonoptimized plans, the average integral dose increased from 74.66 to 76.27 L-Gy (P = .486) compared with the original plans. Reoptimization resulted in a 5% average reduction in the integral dose, from 76.27 to 72.28 L-Gy (P = .007). CONCLUSIONS This study demonstrates the usefulness of adaptive radiation therapy for children receiving radiation therapy to the head and neck region.