Case Report: A child with acute lymphocytic leukaemia

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Pancreatic neuroendocrine tumor in an individual with Von Hippel Lindau syndrome. A case report and review literature

Von Hippel Lindau (VHL) disease is a heritable cancer syndrome characterized by VHL gene mutation in 3p chromosome. Mutations occur at the exons. Rarely mutations at introns have been reported. It leads to accumulation of Hypoxic ischemic factor α (HIF α) which in turn leads to uncontrolled cellular proliferation. Few reports of pancreatic neuroendocrine tumors (PNETs) are there in the literature. Most of them are associated with a mutation in the exon of VHL gene. Association with intron mutation is not there. Here we are reporting a case of PNET in a patient who has a simultaneous mutation both at the exon and the intron

Acute Philadelphia Chromosome Positive Biphenotypic Leukemia Presenting with Bilateral Orbital Chloroma: A Rare Case Report

Introduction: Chloromas are characteristically formed by the extramedullary soft tissue infiltration by the immature myeloid malignant cells. Such extramedullary masses are most commonly seen in acute myeloid leukemia usually in the M2, M4, M5 subtypes of the AML FAB classification. However, it has been reported to rarely present only in pediatric patients with acute lymphoblastic leukemia.Presentation of the case: We encountered an unusual case of a young male, who presented with proptosis of both eyes followed by fever and fatigue. On evaluation, he was diagnosed to have bilateral orbital chloroma which was due to infiltration by leukemic cells of acute leukemia. Flowcytometry revealed features confirming an acute biphenotypic leukemia. Subsequently, cytogenetic evaluation revealed the leukemic cells to be Philadelphia chromosome positive.Conclusion: To our knowledge, this is the first case of bilateral orbital choloroma due to Philadelphia positive biphenotypic acute lymphoblastic leukemia

Nationwide surveillance for Telmisartan alone or with combination at real world therapy in Indian patients with hypertension (START)

Background: Angiotensin receptor blockers (ARBs) are amongst the most preferred class of antihypertensive as reported at various evidences or guidelines. However, choice amongst ARBs differs between practicing physicians in real-life scenario. This survey aimed to understand the usage preferences of telmisartan therapy alone and in combination for treating hypertension (HT) among practitioners at various clinical settings in real-life scenario in India.Methods: A cross‑sectional survey was conducted with a pre-validated survey questionnaire consisting of 15 questions pertaining to the telmisartan and its combination usage in HT management. Total 498 registered medical practitioners (mostly physicians and cardiologists) had participated in survey. They were approached for seeking their perception, opinions, and prescribing behaviour. Categorical data was summarized by number (n) and percentage (%) in each category. Data were summarised in frequency tables.Results: Key findings from the data analysed were as follows: Around 20-40% of patients been reported to have co-morbid hypertension and diabetes as reported by majority of the physicians. Preferred class of drug in patients with hypertension with diabetes reported to be ARB. Around 90.36% of doctors reported that telmisartan was the most preferred ARB in patients with hypertension associated with high cardiovascular risk. Around 90.76% of doctors reported for their preference for telmisartan in patients with hypertension for 24-hr BP control. Around 82.93% of doctors preferred telmisartan in patients with hypertension and stroke/post-MI status.Conclusions: Indian healthcare practitioners prefer telmisartan as the most preferred ARB either alone or in a combination in patients with hypertension, including those with comorbidities

Head and Neck Lymphomas: Tip of the Iceberg?

ABSTRACT Background: Lymphomas comprise around 5% of all head and neck neoplasms and is the second most common extra nodal non hodgkin's lymphoma (NHL). However there is sporadic data on this entity from the subcontinent and hence we undertook this study. Methodology: This retrospective observational study was conducted at a tertiary care oncology center in India on diagnosed cases of NHL between January 2007 and December 2013. All patients were diagnosed based on histopathology and immunohistochemistry. Staging work up was done in all patients. Patients were considered as primary Head and Neck lymphomas if there was head and neck as the predominant site with or without regional lymph node involvement. Results: A total of 39 patients were studied. The age at presentation ranged from 29 to 78 years. The most common site of presentation was oral cavity (26%; n=10), followed by parotid and thyroid (18% each; n=7), eye (12%, n=5), maxilla (8%; n=3), paranasal sinuses (8%; n-=3) cheek (8%, n=3), and nasal cavity (2%, n=1). 41% (n=16) cases were in stage I, 43% (n=17) in stage II, 3% (n=1) in stage III, and 13% (n=5) were in stage IV. Most common histology was DLBCL (71%; n=28), followed by plasmablastic (10%; n=4), marginal zone (8%, n=3), mantle cell (3%; n=1), follicular lymphomas (5%; n=2), and NK/T cell lymphoma (3%; n=1). Most of the patients were of low risk (67%; n=26), followed by intermediate (23%; n=9), and high risk (10%; n=4). Patients were treated with anthracycline based chemotherapy +/-radiotherapy. In this study, stage I and stage II patients had a better prognosis and overall survival, median OS 28 months and 11 months, respectively. In stage III and IV, it was 7 and 3 months, respectively. According to site, the best median overall survival was seen with parotid (27 m), paranasal sinus (26m), and oral cavity (23 m), followed by thyroid (18 m) nasal cavity (17 m), maxilla (11 m), eye (8 m), and cheek (7 m)

Epigenetic modulators as therapeutic targets in prostate cancer

Prostate cancer is one of the most common non-cutaneous malignancies among men worldwide. Epigenetic aberrations, including changes in DNA methylation patterns and/or histone modifications, are key drivers of prostate carcinogenesis. These epigenetic defects might be due to deregulated function and/or expression of the epigenetic machinery, affecting the expression of several important genes. Remarkably, epigenetic modifications are reversible and numerous compounds that target the epigenetic enzymes and regulatory proteins were reported to be effective in cancer growth control. In fact, some of these drugs are already being tested in clinical trials. This review discusses the most important epigenetic alterations in prostate cancer, highlighting the role of epigenetic modulating compounds in pre-clinical and clinical trials as potential therapeutic agents for prostate cancer management.info:eu-repo/semantics/publishedVersio

Chemotherapy in adult soft tissue sarcoma

Soft tissue sarcomas (STSs) are rare and histologically diverse neoplasms. Recent results of various meta-analyses and development of newer drugs have changed the medical management of soft tissue sarcoma. This review gives an outline of chemotherapy and the newer targeted therapies for the same. We have carried out an extensive search in PubMed, Medline for almost all relevant articles concerning chemotherapy of soft tissue sarcoma. The available data from the literature is mainly composed of the most recent reviews, meta-analyses, phase II, and randomized phase III trials published in various peer reviewed journals and various international conferences. The role of neoadjuvant and adjuvant chemotherapy has been found to be controversial. The recent meta-analysis for adjuvant therapy in STSs has shown an increase in the overall survival with combination of ifosfamide and adriamycin. In locally advanced and metastatic STSs, single agent adriamycin remains the basic standard of medication. The combination of ifosfamide and adriamycin may also be used for rapid symptom relief and in patients planned for curative resection for metastases. Newer combinations of docetaxel and gemcitabine appear promising in selected subgroups, especially in leiomyosarcoma and malignant fibrous histiocytoma. Some recent developments include the European Union\u2032s approval of trabectedin for advanced STSs patients who had progressed on adriamycin and ifosfamide therapy. The future of mTOR inhibitors, insulin like growth factor receptor inhibitors and anti-angiogenic drugs appear quite promising. Newer methodologies such as, Bayesian adaptive randomization and inclusion of newer end points like progression-free rate, time of progression rate, and tumor growth rate will improve the results of sarcoma trials. At the end of each section we have also presented recommendations from FNx01European Society of Medical Oncology and FNx08National Comprehensive Cancer Network guidelines v.1.2009 for better correlation with the present literature

Chemotherapy in adult soft tissue sarcoma

Soft tissue sarcomas (STSs) are rare and histologically diverse neoplasms. Recent results of various meta-analyses and development of newer drugs have changed the medical management of soft tissue sarcoma. This review gives an outline of chemotherapy and the newer targeted therapies for the same. We have carried out an extensive search in PubMed, Medline for almost all relevant articles concerning chemotherapy of soft tissue sarcoma. The available data from the literature is mainly composed of the most recent reviews, meta-analyses, phase II, and randomized phase III trials published in various peer reviewed journals and various international conferences. The role of neoadjuvant and adjuvant chemotherapy has been found to be controversial. The recent meta-analysis for adjuvant therapy in STSs has shown an increase in the overall survival with combination of ifosfamide and adriamycin. In locally advanced and metastatic STSs, single agent adriamycin remains the basic standard of medication. The combination of ifosfamide and adriamycin may also be used for rapid symptom relief and in patients planned for curative resection for metastases. Newer combinations of docetaxel and gemcitabine appear promising in selected subgroups, especially in leiomyosarcoma and malignant fibrous histiocytoma. Some recent developments include the European Union′s approval of trabectedin for advanced STSs patients who had progressed on adriamycin and ifosfamide therapy. The future of mTOR inhibitors, insulin like growth factor receptor inhibitors and anti-angiogenic drugs appear quite promising. Newer methodologies such as, Bayesian adaptive randomization and inclusion of newer end points like progression-free rate, time of progression rate, and tumor growth rate will improve the results of sarcoma trials. At the end of each section we have also presented recommendations from FNx01European Society of Medical Oncology and FNx08National Comprehensive Cancer Network guidelines v.1.2009 for better correlation with the present literature

Cancer notification in India

In many developed countries, notification of cancer cases is compulsory. Developing countries including India accounts for more than half of new cancer cases in the world, however notification of cancer is not yet mandatory. The primary purpose of notification is to effect prevention and control and better utilization of resources. It is also a valuable source for incidence, prevalence, mortality and morbidity of the disease. Notification of cancer will lead to improved awareness of common etiologic agents, better understanding of common preventable causes and better utilization of health resources with better monitoring and evaluation of the effectiveness of health programs such as cancer screening and cancer treatment programs, which ultimately might improve survival. Notification of cancer can be done by the doctor or the hospital. Akin to the integrated disease surveillance project where more than 90% of the districts report weekly data through E-mail/portal, notification of cancer can be implemented if it is incorporated into the National Program for Prevention and Control of Cancer, Diabetes, Cardiovascular diseases and Stroke scheme. The need of the hour is cancer notification in India