Renu Madan

and 9 more

Background: Series on radiotherapy (RT) practice in pediatric malignancies are limited in India as only few centers practice pediatric RT, particularly under anesthesia. We aimed to study the clinical profile of pediatric cancer patients treated with RT and to assess various aspects of pediatric RT under anaesthesia. Procedure: The data was prospectively maintained in Microsoft Excel spreadsheets. Pediatric cancer patients aged 0–14 years, registered in the department between February 1, 2019, and July 30, 2021were analyzed. Results: A total of 193 pediatric cancer patients (non central nervous system) received radiation during above mentioned period. Median age at presentation was 5.2 years (range, 9 months to 14 years) with a male to female ratio of 1.8:1. Maximum population was in 0-4 years of age group (52.8%) followed by 5-9 years (29.5%) and ≥ 10 years (17.6%). Most common sites for RT included bone and soft tissue tumors (BST), retinoblastoma, wilms tumor, neuroblastoma, and hematological malignancies. Overall, BST constituted the most common malignancy among all age groups, followed by renal tumors in the 0-4 years and hematological malignancies in 5-14 years. One hundred and seventy-nine (92.7%) patients received RT with curative intent, while 14 (7.3%) patients received palliative RT. Thirty patients needed anaesthesia for RT. Thirty-four patients required RT interruption due to toxicities with a median gap of 7 days. Conclusions: RT is an important aspect of multi-disciplinary care in pediatric cancers. Establishing burden of pediatric cancer patients in RT department may help in resource development and prioritisation.

Radhika Srinivasan

and 5 more

Objective:  Anaplastic Lymphoma Kinase ( ALK) gene gain-of-function point mutation leading to its overexpression has recently been identified and targeted in neuroblastoma (NB). We evaluated ALK gene mutation and its protein expression in cases of NB on fine needle aspiration biopsy (FNAB). Material and Methods: FNAB diagnosed cases of NB (n=56) were evaluated with cell blocks for MYCN amplification and ALK protein expression by Fluorescence in-situ hybridization and immunocytochemistry respectively. MGG stained smears (n=22) were used for Next generation sequencing (NGS) analysis using the Cancer Hotspot panel (version2) on Ion Torrent S5 platform. Staging and risk assignment as per International Neuroblastoma Risk Group (INRG) was performed and managed. All the parameters were correlated with overall survival. Results: ALK protein showed cytoplasmic expression in 65% of cases and did not correlate with MYCN amplification (p=0.35), INRG groups (p=0.52), and overall survival (p=0.2); however, ALK+ poorly differentiated NB showed better prognosis (p=0.02). ALK negativity was associated with poor outcome by Cox proportional hazard model (hazard ratio=2.36). ALK gene, exon 23 missense mutations (F1174L) were seen in 2/21 cases with an allele frequency of 8% and 54%. Both these cases showed ALK protein expression and died of disease within 1 and 17 months respectively. A novel IDH1 exon 4 mutation was also detected. Conclusion:  ALK expression is a promising prognostic as well as a predictive marker in advanced NB along with traditional prognostic parameters. FNAB smears are suitable for NGS and ALK gene mutation confers a poor prognosis.