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Mariella G Filbin, Dominik Sturm
Gliomas are the most common primary central nervous system (CNS) neoplasms in children and adolescents and are thought to arise from their glial progenitors or stem cells. Although the exact cells of origin for most pediatric gliomas remain to be identified, our current understanding is that specific cell populations during CNS development are susceptible to particular oncogenic events during certain time windows and thus give rise to pediatric gliomas with distinct histological, molecular, and clinical features...
February 2018: Seminars in Neurology
Scott S Short, Brian T Bucher, Douglas C Barnhart, Nadia Van Der Watt, Sarah Zobell, Ashley Allen, Michael D Rollins
PURPOSE: We sought to examine the short-term outcomes following single-stage repair of rectoperineal and rectovestibular fistulae in infants and identify risk factors for wound complication. METHODS: Patients with a rectoperineal or rectovestibular fistula treated with a single-stage repair beyond the neonatal period (>30days of age) at a pediatric colorectal center (2011-2016) were reviewed. RESULTS: 36 patients with a rectoperineal and 7 patients with a rectovestibular fistula were repaired using the Posterior Sagittal Anorectoplasty (PSARP) approach...
February 12, 2018: Journal of Pediatric Surgery
Hans Michael Kvasnicka, Jürgen Thiele, Carlos E Bueso-Ramos, William Sun, Jorge Cortes, Hagop M Kantarjian, Srdan Verstovsek
BACKGROUND: Myelofibrosis (MF) is a life-shortening complication of myeloproliferative neoplasms associated with ineffective hematopoiesis, splenomegaly, and progressive bone marrow (BM) fibrosis. The oral Janus kinase (JAK) 1/JAK2 inhibitor ruxolitinib has been shown to improve splenomegaly, symptom burden, and overall survival in patients with intermediate-2 or high-risk MF compared with placebo or best available therapy (BAT). METHODS: The effects of ruxolitinib therapy for up to 66 months on BM morphology in 68 patients with advanced MF with variable BM fibrosis grade were compared with those in 192 matching patients treated with BAT...
March 15, 2018: Journal of Hematology & Oncology
Efrat L Amitay, Agne Krilaviciute, Hermann Brenner
Colorectal cancer (CRC) is a leading cause of cancer morbidity and mortality. Dysbiosis in the gut microbiota may be associated with CRC. This systematic review focuses on differences in gut microbial community between people diagnosed with CRC or adenoma and healthy individuals using fecal samples, emphasizing non-invasive fecal microbiome models for CRC early diagnosis. Nineteen studies were identified in a systematic literature search of Pubmed, Web of Science and ScienceDirect. Several bacteria were reported to differ in abundance between CRC and adenoma cases and healthy controls, with Fusobacterium the most common...
March 15, 2018: Gut Microbes
Giuliano Tosello, Maria Regina Torloni, Bruna S Mota, Teresa Neeman, Rachel Riera
BACKGROUND: Metastatic breast cancer is not a curable disease, but women with metastatic disease are living longer. Surgery to remove the primary tumour is associated with an increased survival in other types of metastatic cancer. Breast surgery is not standard treatment for metastatic disease, however several recent retrospective studies have suggested that breast surgery could increase the women's survival. These studies have methodological limitations including selection bias. A systematic review mapping all randomised controlled trials addressing the benefits and potential harms of breast surgery is ideal to answer this question...
March 15, 2018: Cochrane Database of Systematic Reviews
Ozgur Mete, Sylvia L Asa, Thomas J Giordano, Mauro Papotti, Hironobu Sasano, Marco Volante
Careful morphological evaluation forms the basis of the workup of an adrenal cortical neoplasm. However, the adoption of immunohistochemical biomarkers has added tremendous value to enhance diagnostic accuracy. The authors provide a brief review of immunohistochemical biomarkers that have been used in the confirmation of adrenal cortical origin and in the detection of the source of functional adrenal cortical proliferations, as well as diagnostic, predictive, and prognostic biomarkers of adrenal cortical carcinoma...
March 14, 2018: Endocrine Pathology
Alexandra Okihiro, Rachana Hasija, Lillia Fung, Bonnie Cameron, Brian M Feldman, Ronald Laxer, Rayfel Schneider, Earl Silverman, Lynn Spiegel, Rae S M Yeung, Shirley M L Tse
BACKGROUND: Anti-TNF (Tumor necrosis factor) therapy is effective in treating pediatric patients with refractory rheumatic disease. There is however a concern that anti-TNF usage may increase the risk of malignancy. Reports on specific types of malignancy in this patient population have been emerging over the past decade, but there is a need for additional malignancy reports, as these events are rare. Therefore, a retrospective chart review was performed on the biologic database of pediatric rheumatology patients at The Hospital for Sick Children (SickKids) from 1997 to 2013 for neoplasms, patient demographic information and rheumatologic treatment course...
March 14, 2018: Pediatric Rheumatology Online Journal
L Tagliaferri, C Casà, G Macchia, A Pesce, G Garganese, B Gui, G Perotti, S Gentileschi, F Inzani, R Autorino, S Cammelli, A G Morganti, V Valentini, M A Gambacorta
OBJECTIVE/PURPOSE: Extramammary Paget disease (EMPD) is a rare neoplasm of the skin generally affecting the anogenital area. Because of the low-frequency of the disease, no specific guidelines about the treatment strategy are available. Surgery is the recommended therapy for resectable and localized disease, but several other local treatments have been reported such as radiotherapy (RT). Most articles report small retrospective studies, referring to patients treated decades ago with large heterogeneity in terms of RT dose and technique...
March 17, 2018: International Journal of Gynecological Cancer
Uayporn Kaosombatwattana, Yoshiki Hirooka, Hiroki Kawashima, Eizaburo Ohno, Takuya Ishikawa, Hiroki Suhara, Hidemi Goto
Endoscopic ultrasound is increasingly being used for evaluation of pancreatic diseases and pancreatic tumors. Among various pancreatic cystic lesions, cystic degeneration of pancreatic neuroendocrine neoplasm is of the challenge in making diagnosis. Although unique characteristic of each type of pancreatic cystic lesions has been proposed abundantly, typical morphology of cystic degeneration of pancreatic neuroendocrine neoplasm is still unclear. We, herein, reported a case of 66-year-old woman who was incidentally found to have a cystic lesion in the tail of pancreas upon screening transabdominal ultrasonography...
March 13, 2018: Clinical Journal of Gastroenterology
Dominic Cheong, Seoung Yoon Rho, Ji Hong Kim, Chang Moo Kang, Woo Jung Lee
Renal cell carcinoma is the most common primary renal neoplasm in adults. Although renal cell carcinoma is known to spread to unusual sites, the ampulla of Vater is considered a rare site for metastasis. Here we present a case of renal cell carcinoma metastasized to the ampulla of Vater along with literature review. A 62-year-old Korean male had a history of hypertension and right-sided renal cell carcinoma diagnosed in September 2004, for which he underwent right radical nephrectomy in October 2004. The patient eventually underwent laparoscopic pylorus-preserving total pancreaticoduodenectomy in January 2017...
February 2018: Annals of Hepato-Biliary-Pancreatic Surgery
Federica Camela, Marcella Gallucci, Emanuela di Palmo, Salvatore Cazzato, Mario Lima, Giampaolo Ricci, Andrea Pession
The inflammatory myofibroblastic tumor (IMT) is a rare lesion of unclear etiology and variable clinical course, consisting of a proliferation of fibroblasts and myofibroblasts, mixed with inflammatory cells. Synonyms of IMT are inflammatory pseudotumor and plasma cell granuloma reflecting the alleged inflammatory nature attributed to this lesion, even though this heterogeneity in the disease denomination is probably involved in a dispersion of the literature data. Among primary pulmonary neoplasms, it represents the most frequent endobronchial tumor of childhood and beyond the lung it has been described mainly in the bladder, mediastinum and mesentery...
2018: Frontiers in Pediatrics
Carine Ghassan Richa, Khadija Jamal Saad, Georges Habib Halabi, Elie Mekhael Gharios, Fadi Louis Nasr, Marie Tanios Merheb
The objective of this study is to report three cases of paraneoplastic or ectopic Cushing syndrome, which is a rare phenomenon of the adrenocorticotropic hormone (ACTH)-dependent Cushing syndrome. Three cases are reported in respect of clinical presentation, diagnosis and treatment in addition to relevant literature review. The results showed that ectopic ACTH secretion can be associated with different types of neoplasm most common of which are bronchial carcinoid tumors, which are slow-growing, well-differentiated neoplasms with a favorable prognosis and small-cell lung cancer, which are poorly differentiated tumors with a poor outcome...
2018: Endocrinology, Diabetes & Metabolism Case Reports
Jennifer M Kolb, Pamela Argiriadi, Karen Lee, Xiaoyu Liu, Emilia Bagiella, Aimee L Lucas, Michelle Kang Kim, Nikhil A Kumta, Satish Nagula, Umut Sarpel, Christopher J DiMaio
BACKGROUND & AIMS: For patients with branch duct intraductal papillary mucinous neoplasms (BD-IPMNs, cysts), it is a challenge to identify those at high risk for malignant lesions. We sought to identify factors associated with development of pancreatic cancer, focusing on neoplasm growth rate. METHODS: We performed a retrospective study of 189 patients with BD-IPMNs who underwent at least 2 contrast-enhanced cross-sectional imaging studies, 1 year or more apart, at a tertiary referral center from January 2003 through 2013...
March 10, 2018: Clinical Gastroenterology and Hepatology
Przemysław Janas, Iwona Kucybała, Małgorzata Radoń-Pokracka, Hubert Huras
Telocytes are emerging cell population localized in the stroma of numerous organs, characterized by a distinctive morphology - small cell body with very long, slender prolongations, termed telopodes. Those cells can be found in the whole female reproductive system: in the vagina, uterus, oviducts and ovaries, mammary glands and also in the placenta. In our review, we aim at complete and transparent revision of the current knowledge of telocytes' localization and function, enriched by the analysis of the possible future direction of development of their clinical applications...
March 13, 2018: Advances in Clinical and Experimental Medicine: Official Organ Wroclaw Medical University
B Raone, A Patrizi, C Gurioli, A Gazzola, G M Ravaioli
BACKGROUND: Narrowband-ultraviolet B (NB-UVB) is widely used for the treatment of several dermatological diseases. A cutaneous carcinogenic effect has been hypothesized, but not proved. METHODS: We retrospectively reviewed the data of patients treated with NB-UVB between January 1998 and December 2013 at the Dermatology Unit of our University Hospital, to evaluate the cutaneous carcinogenic risk of NB-UVB. RESULTS: 375 patients were included, each receiving a mean follow-up of 6...
March 13, 2018: Photodermatology, Photoimmunology & Photomedicine
Ignacia Braga-Tanaka, Satoshi Tanaka, Atsushi Kohda, Daisaku Takai, Shingo Nakamura, Tetsuya Ono, Kimio Tanaka, Jun-Ichiro Komura
PURPOSE: This review summarizes the results of experiments conducted in the Institute for Environmental Sciences for the past 21 years, focusing on the biological effects of long-term low dose-rate radiation exposure on mice. Mice were chronically exposed to gamma rays at dose-rates of 0.05, 1 or 20 mGy/day for 400 days to total doses of 20, 400 or 8000 mGy, respectively. The dose rates 0.05 mGy/day is comparable to the dose limit for radiation workers. The parameters examined were lifespan, neoplasm incidence, anti-neoplasm immunity, body weight, chromosome aberration(s), gene mutation(s), alterations in mRNA and protein levels, and trans-generational effects...
March 13, 2018: International Journal of Radiation Biology
Helena Claerhout, Sophie Van Aelst, Celine Melis, Thomas Tousseyn, Olivier Gheysens, Peter Vandenberghe, Daan Dierickx, Nancy Boeckx
OBJECTIVE: Diagnosing myeloid sarcoma remains challenging and we aimed to provide clinicopathological features to facilitate diagnosis. METHOD: Clinicopathological data from 41 patients with de novo and 31 with secondary myeloid sarcoma were reviewed. RESULTS: Most de novo cases presented with isolated myeloid sarcoma (n=19) or myeloid sarcoma with concurrent acute myeloid leukemia (n=15). Most secondary cases presented after acute myeloid leukemia (n=11), myeloproliferative neoplasm (n=9) or myelodysplastic syndrome (n=8)...
March 12, 2018: European Journal of Haematology
Safa Alshaikh, Zainab Harb, Eman Aljufairi, S Ali Almahari
Background: Fine-needle aspiration cytology (FNAC) of thyroid is the initial screening test for thyroid nodules. The Bethesda system classifies thyroid FNAC into six categories. Each category is linked to a malignancy risk and has a recommended clinical management. The aim of this study is to analyze the thyroid cytology smears by Bethesda system and to correlate the diagnosis of cytopathology with histopathology, whenever surgery was done. Materials and Methods: This study presents our experience with the Bethesda system in 681 thyroid FNAs from 632 patients in the period between January 2013 and December 2016...
2018: CytoJournal
Zhong-Ling Wei, Cai-Ming Pan, Yi-Zhi Jiang, Yan-Dai, Lai-Quan Huanguang, Dong-Ping Huang
High-dose chemotherapy combined with autologous hematopoietic stem-cell transplantation (ASCT) is the first-line treatment for multiple myeloma. Yet, some patients will relapse. Testicular plasmacytoma which rarely happens can be isolated or associated with progressive multiple myeloma. Here, we report a case of multiple myeloma (MM) undergoing ASCT when the patient obtained complete remission. He developed painless right testicular swelling after nearly 3 years since the ASCT. After radical orchiectomy, histopathology showed diffuse abnormal plasma cells infiltration of the testicular tissue...
January 2018: Annals of Clinical and Laboratory Science
Vincent P Groot, Sameer S Thakker, Georgios Gemenetzis, Michaël Noë, Ammar A Javed, Richard A Burkhart, Behnoud B Noveiry, John L Cameron, Matthew J Weiss, Christopher J VandenBussche, Elliot K Fishman, Ralph H Hruban, Christopher L Wolfgang, Anne Marie Lennon, Jin He
BACKGROUND: Lymphoepithelial cysts (LECs) are rare pancreatic cystic lesions. Since LECs are benign, preoperative diagnosis is important to differentiate from a cystic neoplasm and avoid unnecessary surgery. The aim of this study was to identify clinical, radiographic and cytopathologic features associated with LECs. METHODS: A retrospective review was performed of patients diagnosed with LEC between 1995 and 2017 at our hospital. Clinicopathologic and radiographic imaging features were documented...
March 9, 2018: HPB: the Official Journal of the International Hepato Pancreato Biliary Association
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