Related ArticlesMorphologic, Immunohistochemical, and Molecular Distinction Between Fibroepithelioma of Pinkus and "Fenestrated" Basal Cell Carcinoma. Am J Dermatopathol. 2019 Nov 05;: Authors: Russell-Goldman E, Lindeman NI, Laga AC, Hanna J Abstract Fibroepithelioma of Pinkus (FEP) is a rare cutaneous neoplasm with a characteristic fenestrated architecture and a prominent spindle cell stromal component and which invariably pursues an indolent course....
Related ArticlesMixed-type primary germ cell tumor of the mediastinum in a young adult male with a sudden life threatening condition: A case report. Thorac Cancer. 2019 Nov 06;: Authors: Sakane T, Okuda K, Murase T, Watanabe T, Oda R, Tatematsu T, Yokota K, Haneda H, Inagaki H, Nakanishi R Abstract Primary germ cell tumors of the mediastinum are rare neoplasms. Above all, choriocarcinomas are highly aggressive with early haematogenous dissemination....
Related ArticlesCutaneous spindled follicle center cell lymphoma with abundant mucin: A diagnostic pitfall. J Cutan Pathol. 2019 Nov 06;: Authors: Li L, Majerowski J, Sokumbi O Abstract Primary cutaneous follicle center cell lymphoma is the most prevalent type of primary cutaneous B-cell lymphoma and usually portends a favorable prognosis. Typically, the diagnosis can be rendered based on characteristic histologic features and immunohistochemistry profile....
Related Articles[Pseudomyxoma peritonei (PMP) secondary to mucinous carcinoma of the ovary: a case study]. Pan Afr Med J. 2019;33:283 Authors: Belhamidi MS, Zorkani Y, Krimou H, Kaoukabi A, Menfaa M, Sakit F, Choho K Abstract Pseudomyxoma peritonei (PMP), also referred to as gelatinous ascites, is a rare disorder, described for the first time by R. Wyerth in 1884. It is characterized by diffuse peritoneal involvement, composed of mucinous ascites and...
Related ArticlesEpidural cavernous haemangioma during pregnancy: a case report and a literature review. Pan Afr Med J. 2019;33:202 Authors: Bennis A, Hafiane R, Benouhoud J, El Khaoudi A, Ibahioin K, Lakhdar A, Moussaid I, El Youssoufi S, Salmi S Abstract Cavernous haemangiomas are benign vascular malformations that can locate in the central nervous system. The epidural spinal location remains unusual. Pregnancy is known to be a precipitating factor....
Related Articles[Sister Mary Joseph nodule]. Pan Afr Med J. 2019;33:228 Authors: Kettani K, Chahid I Abstract We here report the case of a 6-year old female patient with no pathological history, presenting with diffuse abdominal pain and abdominal distension evolving in the last 2 months. The patient had a deterioration in her general condition. Clinical examination showed distended abdomen, average abundance ascites, venous collateral circulation and...
Related Articles[Management of cerebral radionecrosis: experience in the Department of Neurology at the Mohammed V Military Teaching Hospital]. Pan Afr Med J. 2019;33:188 Authors: Raggabi A, Bourazza A, Lalya I Abstract Brain radionecrosis is a rare but life-threatening complication of external-beam radiotherapy for ENT cancers, in particular of the nasopharynx, and for brain tumors. Very few studies were conducted on this complication in the African...
Related ArticlesProspective development of a patient-reported outcomes instrument for desmoid tumors or aggressive fibromatosis. Cancer. 2019 Nov 06;: Authors: Gounder MM, Maddux L, Paty J, Atkinson TM Abstract BACKGROUND: Desmoid tumors (or aggressive fibromatosis) are locally infiltrative connective-tissue tumors that can arise in any anatomic location; they can be asymptomatic, or they can result in pain, deformity, swelling, and loss of mobility...
Orbital diseases mimicking graves' orbitopathy: a long-standing challenge in differential diagnosis.
Related ArticlesOrbital diseases mimicking graves' orbitopathy: a long-standing challenge in differential diagnosis. J Endocrinol Invest. 2019 Nov 05;: Authors: Marinò M, Ionni I, Lanzolla G, Sframeli A, Latrofa F, Rocchi R, Marcocci C Abstract Graves' orbitopathy (GO) is the most common cause of orbital tissue inflammation, accounting for ~ 60% of all orbital inflammatory conditions in the population aged 21-60 years, and for ~ 40% in the population...
Related ArticlesThe hTERT-VNTR2-2nd alleles are involved in genomic stability in gastrointestinal cancer. Genes Genomics. 2019 Nov 05;: Authors: Kwon JA, Jeong MS, Yoon SL, Mun JY, Kim MH, Yang GE, Park SH, Chung JW, Choi YH, Cha HJ, Leem SH Abstract BACKGROUND: hTERT contains a high density of minisatellites, of which rare alleles of hTERT-VNTR2-2nd have been reported to be associated with prostate cancer. This shows an association between VNTR and...
Related ArticlesPediatric Extraskeletal Ewing Sarcoma Originating in the Heart: A Case Report and Review of the Literature. J Pediatr Hematol Oncol. 2019 Oct 29;: Authors: Buffoni I, Nuri H, Pome' G, Sementa AR, Stagnaro N, Barra S, Manzitti C, Garaventa A Abstract Extraosseous Ewing sarcoma of primary cardiac origin is an extremely rare variety among pediatric cardiac neoplasms. We report a case of extraosseous Ewing sarcoma of primary cardiac origin...
Related ArticlesMutational and Immunophenotypic Profiling of a Series of 8 Tubo-ovarian Carcinosarcomas Revealed a Monoclonal Origin of the Disease. Int J Gynecol Pathol. 2019 Nov 01;: Authors: Trento M, Munari G, Carraro V, Lanza C, Salmaso R, Pizzi S, Santoro L, Chiarelli S, Dal Santo L, Nardelli GB, Saccardi C, Nicoletto O, Baldoni A, Rugge M, Fassan M Abstract Carcinosarcomas are rare, highly aggressive neoplasms composed of a combination of carcinomatous...
Related ArticlesEpithelioid Rhabdomyosarcoma: Report of a Cutaneous Case and Literature Review of a Recently Described Variant of Rhabdomyosarcoma. Am J Dermatopathol. 2019 Oct 29;: Authors: Valério E, Almeida GC, Neotti T, Nascimento AG, Bezerra SM, Costa FD Abstract Rhabdomyosarcoma affects mainly pediatric patients and is currently classified into 4 categories: embryonal, alveolar, pleomorphic, and spindle cell/sclerosing. Epithelioid rhabdomyosarcoma...
Related ArticlesBasomelanocytic Neoplasms: A Report of Two Similar Tumors With Divergent Treatments. Am J Dermatopathol. 2019 Oct 29;: Authors: Ryan MP, Bennett DD, Goodwin BP, Kelly BC Abstract Basomelanocytic neoplasms are tumors consisting of elements of both basal cell carcinoma and melanoma. These tumors are exceedingly rare and present a unique challenge as to how the melanoma component should be classified. Due to the paucity of cases, there are...
Related ArticlesOvarian Intermediate Trophoblastic Tumors: Genotyping Defines a Distinct Category of Nongestational Tumors of Germ Cell Type. Am J Surg Pathol. 2019 Nov 01;: Authors: Xing D, Zhong M, Ye F, O'Malley MT, Li S, Vang R, Ronnett BM Abstract Trophoblastic neoplasms involving the ovary are uncommon and include gestational tumors, which are either metastatic from the uterus or ectopic and nongestational tumors, which include those of germ cell...
Related ArticlesValue assessment of immuno-oncology in the treatment of rare tumors in the era of accelerated conditional approvals. Future Oncol. 2019 Nov 05;: Authors: Lanitis T, Ambavane A, Zheng Y, Schlichting M, Phatak H Abstract Aim: To conduct a value assessment of an immuno-oncology (IO) therapy for a rare cancer and evaluate whether existing frameworks consider challenges associated with valuing IOs for rare cancers. Materials & methods:...
Related ArticlesIncidental finding of bilateral renal and adrenal anastomosing hemangiomas: A rare case report. Urol Case Rep. 2019 Nov;27:100912 Authors: Patel SR, Abimbola O, Bhamber T, Weida C, Roy O Abstract Anastomosing hemangiomas are rare variants of vascular tumors found in adrenal, hepatic, and gastrointestinal tissue. Frequently, renal anastomosing hemangiomas are misdiagnosed on computed tomography (CT) as kidney cancers, resulting in unnecessary...
Related ArticlesMature Cystic Teratoma of Douglas' Pouch: A Rare Entity. Cureus. 2019 Aug 29;11(8):e5515 Authors: Sethi P, Purkait S Abstract Mature cystic teratoma is one of the more common ovarian neoplasms; however, teratoma in the pouch of Douglas is extremely rare, and the exact etiology is unknown. Here, we report a rare case of mature cystic teratoma of Douglas' pouch in a 40-year-old woman who had undergone laparotomy. A 15 cm x 15 cm x 10 cm...
Related ArticlesA Case of Histiocytic Sarcoma Arising from Mycosis Fungoides. Case Rep Hematol. 2019;2019:7834728 Authors: Burns EA, Gentille C, Kasparian S, Pingali SR Abstract Histiocytic sarcoma (HS) is an uncommon malignant neoplasm arising from mature histiocytes and most commonly characterized by the immunophenotypic expression of CD68, CD163, or lysozyme. Although rare, HS arising as a second primary malignancy following hematolymphoid neoplasms...
Related ArticlesA Rare Case of Duodenal Adenocarcinoma Presenting as a Subepithelial Lesion in a Patient Undergoing Investigation for Iron Deficiency Anaemia. Case Rep Gastrointest Med. 2019;2019:3434620 Authors: Hu X, Aivazian K, McKenzie C, Wong M, Kaffes A, Saxena P Abstract Introduction: Adenocarcinomas account for approximately 40% of small bowel cancers. They are typically mucosal lesions with distinctive features on endoscopy. We describe a rare...
Related ArticlesTubular apocrine adenoma of the eyelid - A case report and literature review. Saudi J Ophthalmol. 2019 Jul-Sep;33(3):304-307 Authors: Eiger-Moscovich M, Zhang PJL, Lally SE, Shields CL, Eagle RC, Milman T Abstract Tubular apocrine adenoma is a rare benign adnexal neoplasm most commonly identified in the scalp, composed of a dermal proliferation of apocrine tubules in a background of hyalinized stroma. Tubular apocrine adenoma can be a...
Related ArticlesThe prognostic value of pretreatment inflammatory biomarkers in primary angiosarcoma. Cancer Manag Res. 2019;11:7981-7989 Authors: Wang M, Wu S, Tong A, Cui X, Ma X Abstract Background: Recent studies have suggested a significant relationship between inflammatory indexes such as the neutrophil-to-lymphocyte ratio (NLR) and survival outcomes in various cancers. The aim of the present study was to evaluate the prognostic value of 6 pretreatment...
Related ArticlesA Case of Primary Central Nervous System Vasculitis That Worsened Despite Early Corticosteroid Therapy. Brain Tumor Res Treat. 2019 Oct;7(2):164-167 Authors: Yoon SY, Park KS, Park SH, Park JY Abstract Primary central nervous system vasculitis (PCNSV) is rare, and the diagnosis is difficult to make because of its variable radiologic expressions. Early corticosteroid therapy often is effective. Herein we report the case of a 56-year-old...
Related ArticlesAngioleiomyoma in the Orbital Apex: A Case Report. Brain Tumor Res Treat. 2019 Oct;7(2):156-159 Authors: Lee B, Park SJ, Moon JH, Kim SH, Chang JH, Kim SH, Kim EH Abstract A 56-year woman presented eyeball pain and blurred vision. MRI revealed a small well-delineated solid tumor in the apex of right orbit with optic nerve compression. Intraoperatively, the tumor was found very fibrous, hypervascular and adhesive to surrounding structures....
Related ArticlesClinical characteristics of appendiceal diverticular disease. Int J Colorectal Dis. 2019 Nov 04;: Authors: Çakar E, Bayrak S, Çolak Ş, Dal F, Gürbulak B, Bektaş H, Yarıkkaya E, Ferlengez AG Abstract BACKGROUND: Appendiceal diverticular disease (ADD) is a rare pathology which is associated with an increased mortality risk due to rapid perforation and high rates of neoplasm. In our study, we aimed to evaluate the clinical and histopathological...
Related ArticlesWhat is new in epithelioid soft tissue tumors? Virchows Arch. 2019 Nov 04;: Authors: Agaimy A Abstract Epithelioid cell features mimicking carcinomas characterize a variety of histogenetically, phenotypically, and molecularly distinct subsets of mesenchymal neoplasms. In a pathogenetic sense, epithelioid soft tissue tumors basically fall into three main genetic categories: (1) switch/sucrose non-fermenting (SWI/SNF) complex-deficient...
Related ArticlesFurther delineation of neuropsychiatric findings in Tatton-Brown-Rahman syndrome due to disease-causing variants in DNMT3A: seven new patients. Eur J Hum Genet. 2019 Nov 04;: Authors: Tenorio J, Alarcón P, Arias P, Dapía I, García-Miñaur S, Palomares Bralo M, Campistol J, Climent S, Valenzuela I, Ramos S, Monseny AM, Grondona FL, Botet J, Serrano M, Solís M, Santos-Simarro F, Álvarez S, Teixidó-Tura G, Fernández Jaén A, Gordo G, Bardón Rivera MB, Nevado...
Related ArticlesCutaneous soft tissue tumors: diagnostically disorienting epithelioid tumors that are not epithelial, and other perplexing mesenchymal lesions. Mod Pathol. 2019 Nov 04;: Authors: Carter CS, Patel RM Abstract Cutaneous soft tissue tumors with epithelioid features present a diagnostic challenge given that many entities in this category are rare, and they show morphologic overlap with significantly more common cutaneous epithelial and melanocytic...
Related ArticlesPET-CT for Evaluating Breast Cancer Yields Incidental Finding in the Lung. Radiol Technol. 2019 Nov;91(2):120-125 Authors: Perez R, Montane G, Gluskin JS, Nanni G Abstract BACKGROUND: A woman aged 75 years presented with a palpable left axillary lymph node. Mammography showed a spiculated mass in the left breast, demonstrated by biopsy to be triple-negative breast cancer. A positron emission tomography-computed tomography (PET-CT) scan...
Related ArticlesDelivering genome sequencing in clinical practice: an interview study with healthcare professionals involved in the 100 000 Genomes Project. BMJ Open. 2019 Nov 03;9(11):e029699 Authors: Sanderson SC, Hill M, Patch C, Searle B, Lewis C, Chitty LS Abstract OBJECTIVES: Genome sequencing is poised to be incorporated into clinical care for diagnoses of rare diseases and some cancers in many parts of the world. Healthcare professionals are...
Related ArticlesMetastatic pheochromocytoma and paraganglioma: Management of endocrine manifestations, surgery and ablative procedures, and systemic therapies. Best Pract Res Clin Endocrinol Metab. 2019 Oct 24;:101354 Authors: Jasim S, Jimenez C Abstract Metastatic pheochromocytomas and paragangliomas (MPPGs) are rare neuroendocrine tumors. Most patients present with advanced disease that is associated with manifestations of catecholamine release. Surgical...
Related ArticlesPD-L1 Expression and Tumor-Infiltrating Lymphocytes in Thymic Epithelial Neoplasms. J Clin Med. 2019 Nov 01;8(11): Authors: Higuchi R, Goto T, Hirotsu Y, Nakagomi T, Yokoyama Y, Otake S, Amemiya K, Oyama T, Omata M Abstract Thymic epithelial tumors (TETs) are rare malignant mediastinal tumors that are difficult to diagnose and treat. The programmed death 1 (PD-1) receptor and its ligand (PD-L1) are expressed in various malignant tumors...
Related ArticlesHistone Deacetylases and their Inhibitors in Cancer Epigenetics. Diseases. 2019 Nov 01;7(4): Authors: Hassell KN Abstract Histone deacetylases (HDAC) and histone deacetylase inhibitors (HDACi) have greatly impacted the war on cancer. Their role in epigenetics has significantly altered the development of anticancer drugs used to treat the most rare, persistent forms of cancer. During transcription, HDAC and HDACi are used to regulate the...
Related ArticlesAdenosquamous carcinoma development as a recurrence of squamous cell carcinoma in the oral floor: A case report. Medicine (Baltimore). 2019 Oct;98(43):e17688 Authors: Eguchi T, Basugi A, Kanai I, Miyata Y, Suzuki T, Hamada Y Abstract RATIONALE: Oral adenosquamous carcinoma (ASC) is rare and its origins are controversial. We here present a patient with oral ASC that developed after surgery for oral squamous cell carcinoma (SCC). ...
Related ArticlesMerkel cell carcinoma in Taiwan: A series of 24 cases and literature review. Medicine (Baltimore). 2019 Oct;98(42):e17538 Authors: Chang JW, Chang YY, Huang YL, Lo YF, Ho TY, Huang YT, Chen HW, Yeh CN, Wu CE Abstract Merkel cell carcinoma (MCC) is a rare but aggressive neuroendocrine carcinoma of the skin. The available reports of MCC in Asia are limited; in this study, we report the largest series of MCC in Taiwan to date.The series...
Related Articles[Anesthesia management of cervical chordoma resection: A case report]. Beijing Da Xue Xue Bao Yi Xue Ban. 2019 Oct 18;51(5):981-983 Authors: Han YZ, Jing FY, Xu M, Guo XY Abstract Chordoma is a slow-growing, locally invasive, lowgrade malignant tumor with a prevalence of one in 100 000, accounting for 1%-4% of all malignant bone tumors. At present, it is considered that chordoma originates from ectopic embryonic chordal tissue and can...
Related Articles[Endoscopic removal of middle ear haemangioma and review of the literature]. Orv Hetil. 2019 Oct;160(41):1639-1643 Authors: Hirschberg A, Pozsgay E, Erős M, Liktor B Abstract The endoscopic middle ear surgery as a new technique has been introduced during the past few years in our country and it is available in only a few hospitals. Beyond the lack of external incision, endoscopic transcanal approach provides wide field of view to previously...
Related ArticlesMolecular targeted therapy of glioblastoma. Cancer Treat Rev. 2019 Nov;80:101896 Authors: Le Rhun E, Preusser M, Roth P, Reardon DA, van den Bent M, Wen P, Reifenberger G, Weller M Abstract Glioblastomas are intrinsic brain tumors thought to originate from neuroglial stem or progenitor cells. More than 90% of glioblastomas are isocitrate dehydrogenase (IDH)-wildtype tumors. Incidence increases with age, males are more often affected....
Related Articles[Oral manifestations of KID syndrome: rare clinical case]. Stomatologiia (Mosk). 2019;98(4):93-95 Authors: Korolenkova MV, Dmitrieva NA, Babichenko II, Gusova YV, Poberezhnaya AA Abstract The paper presents a rare clinical case of an infant with KID (Keratitis, Ichthyosis, Deafness) syndrome (about 100 patients reported so far) admitted for histological verification of oral mucosa lesions. Disease pathogenesis defines inadequate reparation...
Related Articles[A rare observation of amyloidosis of the larynx simulating a tumor]. Vestn Otorinolaringol. 2019;84(3):65-67 Authors: Popadyuk VI, Novozhilova EN, Fedotov AP, Chernolev AI, Korshunova IA, Olyshanskaya OV, Bitsaeva AV Abstract Presented a clinical case of a rare localization amyloidosis of the larynx, simulating a tumor. Considered the features of diagnosis and treatment of this disease.PMID: 31486431 [PubMed - indexed for MEDLINE]
Related Articles[Primary tumours and pseudotumors of the hand in adults. Epidemiological analysis of cases, management and evolution]. Acta Ortop Mex. 2019 Mar-Apr;33(2):81-87 Authors: Velázquez-Rueda ML, Hernández-Méndez-Villamil E, Mendoza-Muñoz M, Rivas-Montero JA, Espinosa-Gutiérrez A Abstract INTRODUCTION: Tumors in the hand are rare, and malignant tumors in the hand are peculiar; However, primary pseudotumoral lesions of the hand, such as ganglion,...
Related Articles[Ectomesenchymal chondromyxoid tumor of the tongue, a rare and benign lesion]. Orv Hetil. 2019 Aug;160(33):1319-1323 Authors: Mészáros B, Vasas B, Paczona R Abstract Ectomesenchymal chondromyxoid tumor is a rare benign intraoral soft tissue neoplasm. Till date, 93 cases have been reported in literature. Clinically, it presents as a slowly growing, painless, firm, submucosal swelling mainly occurring on the anterior two thirds of the dorsum...
Related ArticlesPost-tuberculosis Pneumocytoma. J Coll Physicians Surg Pak. 2019 Jun;29(6):580-581 Authors: Kupeli M, Nomenoglu H Abstract Pneumocytoma is a rare benign tumor of the lung that usually manifests as a solitary pulmonary nodule. A 69-year lady, who had history of tuberculosis treatment 20 years ago, admitted to the hospital. A round mass was seen on chest radiography. Thorax computed tomography was reported as a round lesion in the left...
Related ArticlesMetastatic Carcinoma of Endometrium from Primary Ovarian Serous Carcinoma Mimicking a Primary Uterine Tumour. J Coll Physicians Surg Pak. 2019 Jun;29(6):577-579 Authors: Xu M, Zhou F, Huang L Abstract A 44-year female was admitted to the hospital, complaining of vaginal bleeding. Ultrasound imaging revealed two masses in the pelvic cavity, measuring 6.6 x 3.4 x 1.8 cm and 8.2 x 4.7 x 3.7 cm in size. After surgical debulking of the tumours,...
Related Articles[Differential diagnosis of lateral cystic lesions in the knee joint]. Orv Hetil. 2019 Apr;160(15):593-599 Authors: Szuper K, Giyab O, Than P Abstract Soft tissues detected on the lateral side of the knee joint may have differential diagnostic difficulties. Although most of these are benign, mostly cystic in appearance, they may raise the clinical suspicion of tumors due to their increased growth. The authors describe two rare soft tissue...
Related ArticlesLeukoencephalopathy, Intracranial Calcifications, Cysts, and SNORD118 Mutation (Labrune Syndrome) with Obstructive Hydrocephalus. World Neurosurg. 2019 05;125:271-272 Authors: Shtaya A, Elmslie F, Crow Y, Hettige S Abstract Labrune syndrome is a neurologic disorder that manifests as a progressive cerebral degeneration characterized by a radiologic triad of cerebral white matter disease (leukoencephalopathy), intracranial calcification,...
Related ArticlesRosette-Forming Glioneuronal Tumor in Opticochiasmatic Region-Novel Entity in New Location. World Neurosurg. 2019 05;125:253-256 Authors: Sekar A, Rudrappa S, Gopal S, Ghosal N, Rai A Abstract BACKGROUND: Rosette-forming glioneuronal tumour [RGNT] is a relatively rare entity first identified as a separate entity in 2002. We are reporting the second case of RGNT in the opticochiasmatic region. CASE DESCRIPTION: We report a case...
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Related ArticlesEosinophilic Granuloma of the Cervical Spine in Adults: A Review. World Neurosurg. 2019 05;125:301-311 Authors: Prasad GL, Divya S Abstract OBJECTIVE: Spinal eosinophilic granulomas (EGs) are uncommon tumors, constituting <1% of all bone tumors. They are mostly seen in the pediatric age group, whereas adult onset is rare. The cervical spine is an infrequent location for EG. The literature is sparse regarding the clinical and management...
Related ArticlesPulmonary nodules in a patient with Giant Cell Arteritis. Pulmonology. 2019 Mar - Apr;25(2):109-113 Authors: Pinho Dos Santos D, Fonseca JP, Romão V, Capelo J, Carragoso A Abstract Giant Cell Arteritis (GCA) is a common systemic vasculitis that involves medium and large arteries, most frequently extracranial branches of the carotid artery. Prompt diagnosis and treatment is essential to prevent devastating complications, such as blindness,...
Related ArticlesA registry-based analysis of survival outcomes in mast cell leukemia. Leuk Res. 2019 03;78:24-28 Authors: Budnik J, Milano MT Abstract INTRODUCTION: Mast cell leukemia (MCL) is rare and carries a poor prognosis. No standard-of-care has been established. No USA registry-based analyses have examined clinical correlates of overall survival (OS) in MCL patients, thus we aimed to do this using the Surveillance, Epidemiology, and End Results...
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Related ArticlesUnusual etiology of bilateral acute visual impairment: Optochiasmatic cavernoma haemorrhage. Am J Emerg Med. 2019 03;37(3):564.e1-564.e4 Authors: Ajhoun Y, Ismail A, Nisrin L, Yasmine CR, Yassine M, Reda K, Oubaaz A Abstract Optochiasmatic cavernoma haemorrhage is unusual etiology of bilateral acute visual impairment. This vascular hamartoma is extremely rare with a prevalence rate of 0.4-0.9% of the general population. They are frequently...
Related ArticlesMalignant peripheral nerve sheath tumor: Transformation in a patient with neurofibromatosis type 2. Pediatr Blood Cancer. 2019 02;66(2):e27520 Authors: Agresta L, Salloum R, Hummel TR, Ratner N, Mangano FT, Fuller C, McMasters RL, Pater L, Jones BV, Szabo S, Pressey JG Abstract Malignant peripheral nerve sheath tumor (MPNST) is a rare soft-tissue sarcoma with an unfavorable prognosis and limited therapeutic options. MPNSTs can be sporadic,...
Related ArticlesBone marrow transplant and pediatric multiple myeloma. Pediatr Blood Cancer. 2019 02;66(2):e27528 Authors: Davidow KA, Walter AW, Kolb EA Abstract Multiple myeloma is a malignant plasma cell disorder that is rare in the pediatric population, with only approximately 0.3% of cases diagnosed before the age of 30. In this report, we present two patients diagnosed with multiple myeloma between the ages of 12 and 16. Their respective treatment...
Related ArticlesThe incidence and survival of pancreatic cancer by histology, including rare subtypes: a nation-wide cancer registry-based study from Taiwan. Cancer Med. 2018 11;7(11):5775-5788 Authors: Chang JS, Chen LT, Shan YS, Chu PY, Tsai CR, Tsai HJ Abstract Studies have indicated a significant rise in the incidence of pancreatic adenocarcinoma. However, the epidemiology of other rare histologic subtypes of pancreatic cancer is not well understood....
Related ArticlesIs hepatocellular carcinoma the same disease in children and adults? Comparison of histology, molecular background, and treatment in pediatric and adult patients. Pediatr Blood Cancer. 2019 02;66(2):e27475 Authors: Weeda VB, Aronson DC, Verheij J, Lamers WH Abstract Pediatric hepatocellular carcinoma (HCC) is rare, resulting in scattered knowledge of tumor biology and molecular background. Thus far, the variant in children has been treated...
Related ArticlesMultiple Recurrent Cardiac Myxomas With Protein Kinase A Regulatory Subunit 1α Gene Mutation. Ann Thorac Surg. 2019 02;107(2):e83-e85 Authors: Kong X, Zhou M, Tu X, Wang J, Yao Y Abstract Multiple recurrent cardiac myxomas are quite rare in clinical practice. A young male patient had four myxoma occurrences, twice in the left atrium and twice in the left ventricle. He had no medical history of cardiovascular diseases, familial cardiac...
Related ArticlesA porcine model of neurofibromatosis type 1 that mimics the human disease. JCI Insight. 2018 06 21;3(12): Authors: White KA, Swier VJ, Cain JT, Kohlmeyer JL, Meyerholz DK, Tanas MR, Uthoff J, Hammond E, Li H, Rohret FA, Goeken A, Chan CH, Leidinger MR, Umesalma S, Wallace MR, Dodd RD, Panzer K, Tang AH, Darbro BW, Moutal A, Cai S, Li W, Bellampalli SS, Khanna R, Rogers CS, Sieren JC, Quelle DE, Weimer JM Abstract Loss of the NF1 tumor...
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Hirst et al. claim that proteins ejected directly from mitochondrial membranes in our study are degraded, are incorrectly assigned, lack lipids, and show discrepancies with "native states" mostly obtained in detergent micelles. Here, we add further evidence in full support of our assignments and show that all complexes are either ejected intact or in known intermediate states, with core subunit interactions maintained. None are degraded or rearranged.
Adult stem cells are essential for tissue homeostasis. In skeletal muscle, muscle stem cells (MuSCs) reside in a quiescent state, but little is known about the mechanisms that control homeostatic turnover. Here we show that, in mice, the variation in MuSC activation rate among different muscles (for example, limb versus diaphragm muscles) is determined by the levels of the transcription factor Pax3. We further show that Pax3 levels are controlled by alternative polyadenylation of its transcript,...
Activating mutations in PIK3CA are frequent in human breast cancer, and phosphoinositide 3-kinase alpha (PI3Kα) inhibitors have been approved for therapy. To characterize determinants of sensitivity to these agents, we analyzed PIK3CA-mutant cancer genomes and observed the presence of multiple PIK3CA mutations in 12 to 15% of breast cancers and other tumor types, most of which (95%) are double mutations. Double PIK3CA mutations are in cis on the same allele and result in increased PI3K activity,...
In general, mixed cations and anions containing formamidinium (FA), methylammonium (MA), caesium, iodine, and bromine ions are used to stabilize the black α-phase of the FA-based lead triiodide (FAPbI3) in perovskite solar cells. However, additives such as MA, caesium, and bromine widen its bandgap and reduce the thermal stability. We stabilized the α-FAPbI3 phase by doping with methylenediammonium dichloride (MDACl2) and achieved a certified short-circuit current density of between 26.1 and 26.7...
The dominance of the major crops that feed humans and their livestock arose from agricultural revolutions that increased productivity and adapted plants to large-scale farming practices. Two hormone systems that universally control flowering and plant architecture, florigen and gibberellin, were the source of multiple revolutions that modified reproductive transitions and proportional growth among plant parts. Although step changes based on serendipitous mutations in these hormone systems laid the...
Spin-orbit interactions lead to distinctive functionalities in photonic systems. They exploit the analogy between the quantum mechanical description of a complex electronic spin-orbit system and synthetic Hamiltonians derived for the propagation of electromagnetic waves in dedicated spatial structures. We realize an artificial Rashba-Dresselhaus spin-orbit interaction in a liquid crystal–filled optical cavity. Three-dimensional tomography in energy-momentum space enabled us to directly evidence the...
First-order relationships between organic matter content and mineral surface area have been widely reported and are implicated in stabilization and long-term preservation of organic matter. However, the nature and stability of organomineral interactions and their connection with mineralogical composition have remained uncertain. In this study, we find that continentally derived organic matter of pedogenic origin is stripped from smectite mineral surfaces upon discharge, dispersal, and sedimentation...
Recent research not only confirms the existence of substantial psychological variation around the globe but also highlights the peculiarity of many Western populations. We propose that part of this variation can be traced back to the action and diffusion of the Western Church, the branch of Christianity that evolved into the Roman Catholic Church. Specifically, we propose that the Western Church’s transformation of European kinship, by promoting small, nuclear households, weak family ties, and residential...
Ancient Rome was the capital of an empire of ~70 million inhabitants, but little is known about the genetics of ancient Romans. Here we present 127 genomes from 29 archaeological sites in and around Rome, spanning the past 12,000 years. We observe two major prehistoric ancestry transitions: one with the introduction of farming and another prior to the Iron Age. By the founding of Rome, the genetic composition of the region approximated that of modern Mediterranean populations. During the Imperial...
NOx lifetime relates nonlinearly to its own concentration; therefore, by observing how NOx lifetime changes with changes in its concentration, inferences can be made about the dominant chemistry occurring in an urban plume. We used satellite observations of NO2 from a new high-resolution product to show that NOx lifetime in approximately 30 North American cities has changed between 2005 and 2014 in a manner consistent with our understanding of NOx chemistry.
Revealing and understanding the mechanisms behind social inequality in prehistoric societies is a major challenge. By combining genome-wide data, isotopic evidence, and anthropological and archaeological data, we have gone beyond the dominating supraregional approaches in archaeogenetics to shed light on the complexity of social status, inheritance rules, and mobility during the Bronze Age. We applied a deep microregional approach and analyzed genome-wide data of 104 human individuals deriving from...
During the epoch of reionization, neutral gas in the early Universe was ionized by hard ultraviolet radiation emitted by young stars in the first galaxies. To do so, ionizing ultraviolet photons must escape from the host galaxy. We present Hubble Space Telescope observations of the gravitationally lensed post-reionization galaxy PSZ1-ARC G311.6602–18.4624 (nicknamed the "Sunburst Arc"), revealing bright, multiply imaged ionizing photon escape from a compact star-forming region through a narrow channel...
Atom interferometers are powerful tools for both measurements in fundamental physics and inertial sensing applications. Their performance, however, has been limited by the available interrogation time of freely falling atoms in a gravitational field. By suspending the spatially separated atomic wave packets in a lattice formed by the mode of an optical cavity, we realize an interrogation time of 20 seconds. Our approach allows gravitational potentials to be measured by holding, rather than dropping,...
Stereoselective polymerization of chiral or prochiral monomers is a powerful method to produce high-performance stereoregular crystalline polymeric materials. However, for monomers with two stereogenic centers, it is generally necessary to separate diastereomers before polymerization, resulting in substantial material loss and added energy cost associated with the separation and purification process. Here we report a diastereoselective polymerization methodology enabled by catalysts that directly...
Nonribosomal peptide synthetases (NRPSs) are biosynthetic enzymes that synthesize natural product therapeutics using a modular synthetic logic, whereby each module adds one aminoacyl substrate to the nascent peptide. We have determined five x-ray crystal structures of large constructs of the NRPS linear gramicidin synthetase, including a structure of a full core dimodule in conformations organized for the condensation reaction and intermodular peptidyl substrate delivery. The structures reveal differences...
Chorev et al. (Reports, 16 November 2018, p. 829) describe mass spectrometry on mitochondrial membrane proteins ionized directly from their native environment. However, the assignments made to measured masses are incorrect or inconclusive and lack experimental validation. The proteins are not in their "native" condition: They have been stripped of tightly bound lipids, and the complexes are fragmented or in physiologically irrelevant oligomeric states.
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