Atypical teratoid rhabdoid tumor of the central nervous system: Case series from a regional Tertiary Care Cancer Centre in South India.

BACKGROUND: Atypical teratoid rhabdoid tumors (AT/RT) constitute a rare group of pediatric brain tumors. AIM: To study the clinical, histopathological, and immunohistochemical (IHC) profile, management and outcome of children with AT/RT of the central nervous system who presented between the years 2007 and 2015 in a regional tertiary care center in South India. MATERIALS AND METHODS: This was a retrospective study. Demographic and clinical data were obtained from the clinical case files. Archived slides and tissue blocks were retrieved. All cases had hematoxylin and eosin stained sections. IHC was available in all the cases. RESULTS: There were eight cases with the mean age of presentation being 4 years (range: 4 months to 15 years) and with slight male predominance (male:female = 1.66:1). Most of the presenting complaints were due to raised intra-cranial tension. The median duration of symptoms was 0.75 months. About 62.5% of the tumors were infratentorial in location. The tumors were heterogeneous showing variable expression of cytokeratin, epithelial membrane antigen, glial fibrillary acid protein, and synaptophysin. Loss of integrase interactor-1 expression was demonstrated in seven cases in which it was done. Multimodal treatment comprising surgical resection, radiotherapy and chemotherapy was tailored based on location of tumor, resectability and patient's age. The median overall survival was 2.5 months (range: 1.5-30 months). CONCLUSION: Awareness of this tumor is important as it portends a poor outcome in most patients, in spite of multi-modal treatment. Several new molecules which aim to prolong survival and improve quality of life are being developed to combat this enigmatic tumor.

Bloodstream infections in febrile neutropenic patients at a tertiary cancer institute in South India: A timeline of clinical and microbial trends through the years.

INTRODUCTION: Febrile neutropenia (FN) is an oncological emergency. The choice of empiric therapy depends on the locally prevalent pathogens and their sensitivities, the sites of infection, and cost. The Infectious Diseases Society of America guidelines are being followed for the management of FN in India. METHODS: This is a prospective observational study conducted at a tertiary care cancer centre from September 2012 to September 2014. OBJECTIVES: The objectives of this study were as follows: (1) To review the pattern of microbial flora, susceptibility pattern, and important clinical variables among bloodstream infections in febrile neutropenic patients with solid tumors and hematological malignancies. (2) As per the institutional protocol to periodically review the antibiotic policy and susceptibility pattern, and compare the findings with an earlier study done in our institute in 2010. This was a prospective study conducted from September 2012 to September 2014. RESULTS: About 379 episodes of FN were documented among 300 patients. About 887 blood cultures were drawn. Of these, 137 (15%) isolates were cultured. Isolates having identical antibiograms obtained from a single patient during the same hospitalization were considered as one. Hence, 128 isolates were analyzed. About 74 (58%) cultures yielded Gram-negative bacilli, 51 (40%) were positive for Gram-positive cocci, and 3 (2%) grew fungi. Among Gram-negative organisms, Escherichia coli followed by Acinetobacter baumannii and Klebsiella pneumoniae accounted for 78% of the isolates. Among Gram-positive cocci, Staphylococcus species accounted for 84% of the isolates. We have noted a changing trend in the antibiotic sensitivity pattern over the years. Following the switch in empirical antibiotics, based on the results of the study done in 2010 (when the empirical antibiotics were ceftazidime + amikacin), the sensitivity to cefoperazone-sulbactam has plunged from about 80% to 60%%. Similar reduction in susceptibility was noted for piperacillin-tazobactam, imipenem, and meropenem. On the contrary, there was a marked increase in sensitivity to ceftazidime (50-76%). Based on these results, we have reverted to ceftazidime + amikacin as the empirical antibiotics. CONCLUSION: Every institute must have a regular revision of antibiotic policy based on periodic assessment of the clinical and microbiological profile in FN. This will combat antibiotic resistance.

Cancer-associated thrombotic microangiopathy.

Cancer-associated thrombotic microangiopathy refers to a group of disorders characterised by microvascular thrombosis, thrombocytopenia, and ischaemic end-organ damage. Haemolytic uraemic syndrome and thrombotic thrombocytopenic purpura are the two major subtypes. It can be a manifestation of the malignancy itself or a complication of its therapy. The addition of several new drugs to the therapeutic armamentarium of cancer has brought to light several novel causative factors of this hitherto uncommon complication. This review covers the aetiology, pathogenesis, clinical manifestations, complications, and the management of cancer-associated thrombotic microangiopathy. Careful review of the patient's medical records coupled with the correlation of clinical findings and laboratory reports can help clinch the diagnosis and institute appropriate treatment on time.

Acute Coronary Syndrome Manifesting as an Adverse Effect of All-trans-Retinoic Acid in Acute Promyelocytic Leukemia: A Case Report with Review of the Literature and a Spotlight on Management.

Background. Acute promyelocytic leukemia is characterized by t(15;17). This leads to the formation of PML/RARα which blocks the differentiation of blasts at the stage of promyelocytes. This is reversed by all-trans-retinoic acid (ATRA), a vitamin A derivative. Acute myocardial ischemia is a rare side effect of ATRA. Case Report. We report a case of acute coronary syndrome manifesting as an adverse effect of ATRA in a lady with APL who had no other risk factors for cardiovascular disease. Conclusions. We emphasize the need for high index of suspicion for the diagnosis of this entity. In the light of this case, the rare instances of ATRA associated acute myocardial ischemia recorded in the literature and the options available for treatment of acute promyelocytic leukemia sans ATRA have been reviewed.

Outcomes, cost comparison, and patient satisfaction during long-term central venous access in cancer patients: Experience from a Tertiary Care Cancer Institute in South India.

INTRODUCTION: Prolonged treatment, frequent administration of chemotherapy, antibiotics and blood products in cancer patients requires long term venous access. Central venous catheters (CVC) inserted into the subclavian vein or internal jugular vein, peripherally inserted central venous catheters (PICC) and chemoport (CP) are the commonly used central venous access devices (CVAD). AIM: This study was envisaged to review our experience of CVADs over a one year period and analyze the outcome with regard to catheter life, reasons for removal, complications, patient satisfaction and cost comparison between the CVAD types in the Indian setting. SETTINGS AND DESIGN: This was a prospective, observational study carried out in a tertiary care cancer institute. MATERIALS AND METHODS: 180 CVADs placed in patients with hematological malignancies and solid tumors from January 2014 to December 2014 were included. STATISTICAL ANALYSIS USED: Data was analyzed using descriptive statistics, Mann Whitney U test. P <0.05 was taken as statistically significant. RESULTS: 180 CVADs were placed in 160 patients. The median catheter indwelling period was 76 days (16 days to 313 days) for CVC, 59 days (20days - 313 days) for PICC and 137 days (70 days - 258 days) for CP. 66 out of 160 patients developed complications (41.2%). 108 complication events were noted in 66 patients. There were 40 episodes of CRBSI. Out of the 68 mechanical complications, 37 were encountered during insertion of the CVAD and 31 were during the catheter indwelling period. Out of 160 patients, 138 (86.25%) were satisfied with the CVAD. The cost incurred for CVC/PICC (INR 4,480) was lower than that for CP (INR 24,150) and it was statistically significant (P < 0.0001). Our patients were highly satisfied with the CVAD. CONCLUSION: Use of CVC and PICC is a safe, reliable and cost saving way of administration of chemotherapy in developing countries. The incidence of complications and catheter loss was acceptable. Our patients were highly satisfied with the CVAD.