Global Retinoblastoma Presentation and Analysis by National Income Level.

Importance: Early diagnosis of retinoblastoma, the most common intraocular cancer, can save both a child's life and vision. However, anecdotal evidence suggests that many children across the world are diagnosed late. To our knowledge, the clinical presentation of retinoblastoma has never been assessed on a global scale. Objectives: To report the retinoblastoma stage at diagnosis in patients across the world during a single year, to investigate associations between clinical variables and national income level, and to investigate risk factors for advanced disease at diagnosis. Design, Setting, and Participants: A total of 278 retinoblastoma treatment centers were recruited from June 2017 through December 2018 to participate in a cross-sectional analysis of treatment-naive patients with retinoblastoma who were diagnosed in 2017. Main Outcomes and Measures: Age at presentation, proportion of familial history of retinoblastoma, and tumor stage and metastasis. Results: The cohort included 4351 new patients from 153 countries; the median age at diagnosis was 30.5 (interquartile range, 18.3-45.9) months, and 1976 patients (45.4%) were female. Most patients (n = 3685 [84.7%]) were from low- and middle-income countries (LMICs). Globally, the most common indication for referral was leukocoria (n = 2638 [62.8%]), followed by strabismus (n = 429 [10.2%]) and proptosis (n = 309 [7.4%]). Patients from high-income countries (HICs) were diagnosed at a median age of 14.1 months, with 656 of 666 (98.5%) patients having intraocular retinoblastoma and 2 (0.3%) having metastasis. Patients from low-income countries were diagnosed at a median age of 30.5 months, with 256 of 521 (49.1%) having extraocular retinoblastoma and 94 of 498 (18.9%) having metastasis. Lower national income level was associated with older presentation age, higher proportion of locally advanced disease and distant metastasis, and smaller proportion of familial history of retinoblastoma. Advanced disease at diagnosis was more common in LMICs even after adjusting for age (odds ratio for low-income countries vs upper-middle-income countries and HICs, 17.92 [95% CI, 12.94-24.80], and for lower-middle-income countries vs upper-middle-income countries and HICs, 5.74 [95% CI, 4.30-7.68]). Conclusions and Relevance: This study is estimated to have included more than half of all new retinoblastoma cases worldwide in 2017. Children from LMICs, where the main global retinoblastoma burden lies, presented at an older age with more advanced disease and demonstrated a smaller proportion of familial history of retinoblastoma, likely because many do not reach a childbearing age. Given that retinoblastoma is curable, these data are concerning and mandate intervention at national and international levels. Further studies are needed to investigate factors, other than age at presentation, that may be associated with advanced disease in LMICs

The synthesis and acid-catalyzed rearrangement of the derivatives of 1-methyltricyclo[4400[superscript 2,6]]decan-3-one

M.S.Drury S. Caine, II

Advances in penile cancer management.

Penile cancer is a rare disease that may cause devastating physical and psychological effects on patients due to the disease itself and/or the associated treatments. As with many other cancer types, significant efforts have been made in penile cancer to minimize invasiveness and morbidity of therapeutic approaches, while aiming to conserve organ function and optimize disease control. This updated review focuses on penile cancer management data published in the last few years.Journal ArticleReviewSCOPUS: re.jinfo:eu-repo/semantics/publishe

Management of metastatic HER2-positive breast cancer progression after adjuvant trastuzumab therapy - current evidence and future trends.

Trastuzumab is now considered the standard of care for the adjuvant treatment of human epidermal growth factor receptor-2 (HER2)-positive breast cancer patients, yet a sizable number of HER2-positive patients do not benefit from this treatment. For patients who progress on or after completion of adjuvant trastuzumab therapy, the standard of care is uncertain. Newer tyrosine kinase inhibitors and monoclonal antibodies are being evaluated in clinical trials for optimisation of treatment in this group. The interplay of HER2 and hormonal signalling pathways is being manipulated to see if response rates can be bettered. In this reveiw we explain the present role of the new molecules with a case scenario. The new anti-HER2 drugs have the potential to change clinical practise of targeting HER2 in the future. Better understanding of HER2 functions and interactions of different signalling pathways will be essential for optimal targetted therapies.Journal ArticleResearch Support, Non-U.S. Gov'tReviewSCOPUS: cp.jinfo:eu-repo/semantics/publishe

Novel therapeutics in breast cancer-Looking to the future

Breast cancer treatment has evolved dramatically in the last few years. Despite the benefit of anthracyclines, taxanes and trastuzumab for patients with metastatic and early breast cancer, the challenges of de novo and acquired resistance are still present. With advances in the molecular characterization of breast cancer, patient selection and individualization of treatment has taken on singular importance. Three main types of breast cancer have been reported to date: (a) HER-2 positive; (b) basal-like; and (c) luminal breast cancer. A large number of new agents now target different receptors and signalling pathways that sustain cancer survival and proliferation. In this review we highlight the novel molecules currently being tested in clinical trials that have or will have the potential to change our daily clinical practice; in particular, we focus on molecules used in the treatment of HER-2 positive and basal-like breast cancer patients. © 2009 Elsevier Ltd. All rights reserved.SCOPUS: re.jinfo:eu-repo/semantics/publishe

Intracranial extramedullary hematopoiesis masquerading as progressive metastasis in a child with stage 4 neuroblastoma: Utility of sulfur colloid scan

Extramedullary hematopoiesis is production of blood cells outside bone marrow which occurs in a variety of disorders including myelofibrosis. We present a 15 month old child with stage 4 neuroblastoma with secondary myelofibrosis which resulted in intracranial extramedullary hematopoiesis, posing a diagnostic and therapeutic dilemma. This was resolved with a sulfur colloid isotope scan which confirmed extramedullary hematopoiesis and not a progressing metastasis. This helped us continue therapy with curative intent. To the best of our knowledge we did not come across any report of neuroblastoma with myelofibrosis and intracranial extramedullary hematopoiesis mimicking progression of metastasis