Advantages and implications of high dose rate (HDR) total skin electron irradiation (TSEI) for the management of Mycosis Fungoides. Indian experience

BackgroundMycosis Fungoides (MF) is an indolent lymphoproliferative disorder affecting dermis caused by abnormal proliferation of CD4+ T-cells. Radiation therapy is the most effective modality of treatment for MF which offers cure in limited stage disease and desirable palliation in advance stage disease. Treating entire skin having many curved surfaces and folds with radiation is the real challenge for the radiation oncologist. Many techniques, dose schedules and modifications in total skin electron irradiation (TSEI) have been tried since 1950s. TSEI treatment is a very time consuming, inconvenient and physically challenging to both patient as well as oncologist.AimAt our center TSEI was performed since 1983 with conventional linear accelerator where the treatment time was prolonged beyond two hours, which was very difficult or the patient, oncologist, technical officer and eating away the machine time hampering the treatment of other patients. From 1998 we shifted to high dose rate (HDR) mode, in order to bring down the treatment time of a single patient every day from two and half hour to 15[[ce:hsp sp="0.25"/]]min. The reduction of treatment time increases patient compliance and at the same time saved machine time.Materials and methodsBetween 1998 and 2003, eleven pathological diagnosed MF patients were treated using HDR TSEI. All the patients were male between 40 and 70 years of age, who had the history of having the disease for 7–22 months. Four patients had T2 and seven patients had T3 stage disease with more than 90% skin surface involvement. TSEI was performed with 4[[ce:hsp sp="0.25"/]]MeV electrons with a daily fraction size of 120[[ce:hsp sp="0.25"/]]cGy to a total dose of 36[[ce:hsp sp="0.25"/]]Gy. At the end of 36[[ce:hsp sp="0.25"/]]Gy, boost dose of 10[[ce:hsp sp="0.25"/]]Gy was delivered to self shielding regions like sole, scalp and perineum. Considering the treatment related toxicities and consequent treatment interruptions, in the first seven patients, the last four patients were treated using similar HDR TSEI technique with modified treatment schedule, where the treatment was given on an alternate day basis following 2nd week of initiation of treatment.ResultsThe patients were followed over a period of 144 months with a median of 72 months. Nine patients are alive without any evidence of disease, one patient relapsed and one died due to progression of disease. The most common radiation related morbidities are erythema, skin blisters, various degree of desquamations, swelling of joints (specially small joints) etc. which are controlled by treatment interruptions and conservative measures. By modifying the treatment schedule, the incidence of toxicity as well as treatment interruptions were brought down.ConclusionsWe can conclude that HDR-TSEI is an excellent and safe therapeutic modality for the patients with MF both curative as well as palliative without any added toxicity profile, provided patient positioning is done properly

Pierwotny śródczaszkowy bazaloidalny rak płaskonabłonkowy

Primary intracranial squamous cell carcinoma is extremely rare, with most cases arising from malignant transformation of dysembryogenetic lesions such as epidermoid and dermoid cysts. Intracranial squamous cell neoplasm arising de novo is even rarer and has been reported in only four patients to date. We herein describe a case of primary intracranial squamous cell carcinoma arising de novo in the right frontal lobe in a 35-year-old woman treated with a combination of surgery and postoperative conformal radiation. We have also shed light on the biology and the therapeutic options of this enigmatic tumour.Pierwotny śródczaszkowy rak płaskonabłonkowy jest wyjątkową rzadkością i w większości przypadków rozwija się w wyniku zezłośliwienia zmian o charakterze dysembriogenetycznym, np. torbieli naskórkowej lub skórzastej. Śródczaszkowy rak płaskonabłonkowy powstały de novo jest jeszcze rzadszy – dotąd opisano 4 takie przypadki. W niniejszej pracy przedstawiono przypadek nowotworu powstałego de novo w prawym płacie czołowym u 35-letniej chorej, którą z tego powodu poddano leczeniu chirurgicznemu i pooperacyjnej radioterapii konformalnej. Podano również informacje na temat biologii i możliwości leczenia tego zagadkowego guza

The global burden of adolescent and young adult cancer in 2019 : a systematic analysis for the Global Burden of Disease Study 2019

Background In estimating the global burden of cancer, adolescents and young adults with cancer are often overlooked, despite being a distinct subgroup with unique epidemiology, clinical care needs, and societal impact. Comprehensive estimates of the global cancer burden in adolescents and young adults (aged 15-39 years) are lacking. To address this gap, we analysed results from the Global Burden of Diseases, Injuries, and Risk Factors Study (GBD) 2019, with a focus on the outcome of disability-adjusted life-years (DALYs), to inform global cancer control measures in adolescents and young adults. Methods Using the GBD 2019 methodology, international mortality data were collected from vital registration systems, verbal autopsies, and population-based cancer registry inputs modelled with mortality-to-incidence ratios (MIRs). Incidence was computed with mortality estimates and corresponding MIRs. Prevalence estimates were calculated using modelled survival and multiplied by disability weights to obtain years lived with disability (YLDs). Years of life lost (YLLs) were calculated as age-specific cancer deaths multiplied by the standard life expectancy at the age of death. The main outcome was DALYs (the sum of YLLs and YLDs). Estimates were presented globally and by Socio-demographic Index (SDI) quintiles (countries ranked and divided into five equal SDI groups), and all estimates were presented with corresponding 95% uncertainty intervals (UIs). For this analysis, we used the age range of 15-39 years to define adolescents and young adults. Findings There were 1.19 million (95% UI 1.11-1.28) incident cancer cases and 396 000 (370 000-425 000) deaths due to cancer among people aged 15-39 years worldwide in 2019. The highest age-standardised incidence rates occurred in high SDI (59.6 [54.5-65.7] per 100 000 person-years) and high-middle SDI countries (53.2 [48.8-57.9] per 100 000 person-years), while the highest age-standardised mortality rates were in low-middle SDI (14.2 [12.9-15.6] per 100 000 person-years) and middle SDI (13.6 [12.6-14.8] per 100 000 person-years) countries. In 2019, adolescent and young adult cancers contributed 23.5 million (21.9-25.2) DALYs to the global burden of disease, of which 2.7% (1.9-3.6) came from YLDs and 97.3% (96.4-98.1) from YLLs. Cancer was the fourth leading cause of death and tenth leading cause of DALYs in adolescents and young adults globally. Interpretation Adolescent and young adult cancers contributed substantially to the overall adolescent and young adult disease burden globally in 2019. These results provide new insights into the distribution and magnitude of the adolescent and young adult cancer burden around the world. With notable differences observed across SDI settings, these estimates can inform global and country-level cancer control efforts. Copyright (C) 2021 The Author(s). Published by Elsevier Ltd.Peer reviewe

Global burden of 369 diseases and injuries in 204 countries and territories, 1990–2019: a systematic analysis for the Global Burden of Disease Study 2019

Background: In an era of shifting global agendas and expanded emphasis on non-communicable diseases and injuries along with communicable diseases, sound evidence on trends by cause at the national level is essential. The Global Burden of Diseases, Injuries, and Risk Factors Study (GBD) provides a systematic scientific assessment of published, publicly available, and contributed data on incidence, prevalence, and mortality for a mutually exclusive and collectively exhaustive list of diseases and injuries. Methods: GBD estimates incidence, prevalence, mortality, years of life lost (YLLs), years lived with disability (YLDs), and disability-adjusted life-years (DALYs) due to 369 diseases and injuries, for two sexes, and for 204 countries and territories. Input data were extracted from censuses, household surveys, civil registration and vital statistics, disease registries, health service use, air pollution monitors, satellite imaging, disease notifications, and other sources. Cause-specific death rates and cause fractions were calculated using the Cause of Death Ensemble model and spatiotemporal Gaussian process regression. Cause-specific deaths were adjusted to match the total all-cause deaths calculated as part of the GBD population, fertility, and mortality estimates. Deaths were multiplied by standard life expectancy at each age to calculate YLLs. A Bayesian meta-regression modelling tool, DisMod-MR 2.1, was used to ensure consistency between incidence, prevalence, remission, excess mortality, and cause-specific mortality for most causes. Prevalence estimates were multiplied by disability weights for mutually exclusive sequelae of diseases and injuries to calculate YLDs. We considered results in the context of the Socio-demographic Index (SDI), a composite indicator of income per capita, years of schooling, and fertility rate in females younger than 25 years. Uncertainty intervals (UIs) were generated for every metric using the 25th and 975th ordered 1000 draw values of the posterior distribution. Findings: Global health has steadily improved over the past 30 years as measured by age-standardised DALY rates. After taking into account population growth and ageing, the absolute number of DALYs has remained stable. Since 2010, the pace of decline in global age-standardised DALY rates has accelerated in age groups younger than 50 years compared with the 1990–2010 time period, with the greatest annualised rate of decline occurring in the 0–9-year age group. Six infectious diseases were among the top ten causes of DALYs in children younger than 10 years in 2019: lower respiratory infections (ranked second), diarrhoeal diseases (third), malaria (fifth), meningitis (sixth), whooping cough (ninth), and sexually transmitted infections (which, in this age group, is fully accounted for by congenital syphilis; ranked tenth). In adolescents aged 10–24 years, three injury causes were among the top causes of DALYs: road injuries (ranked first), self-harm (third), and interpersonal violence (fifth). Five of the causes that were in the top ten for ages 10–24 years were also in the top ten in the 25–49-year age group: road injuries (ranked first), HIV/AIDS (second), low back pain (fourth), headache disorders (fifth), and depressive disorders (sixth). In 2019, ischaemic heart disease and stroke were the top-ranked causes of DALYs in both the 50–74-year and 75-years-and-older age groups. Since 1990, there has been a marked shift towards a greater proportion of burden due to YLDs from non-communicable diseases and injuries. In 2019, there were 11 countries where non-communicable disease and injury YLDs constituted more than half of all disease burden. Decreases in age-standardised DALY rates have accelerated over the past decade in countries at the lower end of the SDI range, while improvements have started to stagnate or even reverse in countries with higher SDI. Interpretation: As disability becomes an increasingly large component of disease burden and a larger component of health expenditure, greater research and developm nt investment is needed to identify new, more effective intervention strategies. With a rapidly ageing global population, the demands on health services to deal with disabling outcomes, which increase with age, will require policy makers to anticipate these changes. The mix of universal and more geographically specific influences on health reinforces the need for regular reporting on population health in detail and by underlying cause to help decision makers to identify success stories of disease control to emulate, as well as opportunities to improve. Funding: Bill & Melinda Gates Foundation. © 2020 The Author(s). Published by Elsevier Ltd. This is an Open Access article under the CC BY 4.0 licens

Sarcomatoid squamous cell carcinoma of uterine cervix: Pathology, imaging, and treatment

Sarcomatoid squamous cell carcinoma of the cervix is a rare tumor. Only 16 cases have so far been reported in literature. We report here one such tumor occurring in a 54-year-old postmenopausal woman. Our case report describes the clinical, pathological, and PET scan characteristics of this tumor. The patient was treated with concurrent chemoradiotherapy and is disease free at 6-months follow-up

Medullomyoblastoma treated with craniospinal radiation and adjuvant chemotherapy: Report of 4 cases and review of the literature

Medullomyoblastoma (MMB) is a rare primitive neuro-epithelial tumor and seen in mainly pediatric age group1. There have been about 50 cases reported so far in the literature. In this report, we describe the clinical features and treatment of 4 cases of MMB

Short course palliative radiotherapy in the management of choroidal metastasis: An effective technique since ages

AbstractPurposeUveal tract is the most common site of intra-ocular metastasis. Overall, the reported prevalence of clinically evident uveal metastases in patients with cancer ranges from 2% to 9%, with the majority of the cases being due to breast cancer. We aimed at evaluating the role of palliative radiotherapy in the management of choroidal metastasis from carcinoma breast.Materials and methodsWe describe the clinico-pathologic features, treatment and outcome of ten patients of carcinoma breast who presented to the ophthalmology department at our institution with ocular symptoms attributable to choroidal metastasis.ResultsNine of the patients were female while one was male. All of them presented with painless progressive diminution of vision. Median duration of symptoms was 2.25months. Five patients had associated lung metastasis while bone and brain metastases were seen in three and two patients respectively. All of them received palliative radiotherapy (RT) to the involved eye (or eye+brain) by 3D-CRT (n=7), or 2 Dimensional technique (n=2) or electron therapy (n=1). Doses prescribed were 30Gy/10#/2weeks (n=8); 20Gy/5 #/1week (n=2). Simultaneously they received hormonal therapy (n=6) or systemic chemotherapy (n=3). After a median follow up of 18months seven patients had complete resolution and two patients had partial resolution of the metastases.ConclusionShort course palliative radiation therapy is an effective modality for the management of choroidal metastasis in patients of carcinoma breast. In the current report it led to formidable local control with acceptable radiation induced toxicity

Malignant and borderline phyllodes tumor of breast treated with a multi-modality approach in a tertiary cancer care centre in North India

Background: Phyllodes tumor (PT) of the breast can be categorized into benign, borderline and malignant subgroups depending on various histopathological factors. Although malignant PTs may be indolent and controlled by local excision, they frequently show local and distant relapses. Literature reveals local recurrence to be the predominant pattern of failure and thus emphasizes the importance of adjuvant radiation in these tumors. The role of systemic chemotherapy has remained doubtful. Materials and Methods: We have analyzed details of all patients of PT (n = 33) treated with adjuvant multi-modality approach in our institute since 1994–2009. The demographic data, treatment details, recurrence patterns and salvage treatment options were documented. Results: All patients received adjuvant radiation. Seven patients received adjuvant chemotherapy. The mean survival of the entire cohort was 150.618 months. There was a trend for better overall survival with borderline grade (193.6 vs. 160.2 months; P = 0.08, log rank). The disease free survival (DFS) favored borderline grade (193.6 months vs. 82.9 months for high grade; P = 0.02, log rank). The DFS was significantly better in tumors having negative margins on postoperative histopathological examination (DFS rate at 5 years being 100% vs. 69.2% for positive or close margins; P = 0.015). The mode of surgery did not have any impact on survival. Conclusion: Adjuvant Radiation should be discussed taking into account surgical margins, size and various pathological factors of the primary. Adjuvant radiation may be utilized in high risk patients to enhance loco-regional control. Systemic chemotherapy is an option, worth exploring, in cases of systemic failure