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Dendritic cell sarcoma

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https://www.readbyqxmd.com/read/28929581/gain-of-function-mutation-in-ptpn11-in-histiocytic-sarcomas-of-bernese-mountain-dogs
#1
T Thaiwong, S Sirivisoot, M Takada, V Yuzbasiyan-Gurkan, M Kiupel
Histiocytic sarcoma (HS) is an aggressive malignant neoplasm of dendritic cell origin that is common in certain breeds of dogs. High prevalence of fatal, disseminated HS has been described in Bernese Mountain Dogs (BMDs). Support for genetic predisposition to develop HS has been presented in several studies, but to date, causative genetic events have not been reported. In addition, no driver mutations have been identified in tumours. Recently, E76K gain-of-function mutation in SHP2 encoded by the PTPN11 gene has been described in human histiocytic malignancies...
September 20, 2017: Veterinary and Comparative Oncology
https://www.readbyqxmd.com/read/28928015/dendritic-homeostasis-disruption-in-a-novel-frontotemporal-dementia-mouse-model-expressing-cytoplasmic-fused-in-sarcoma
#2
Gen Shiihashi, Daisuke Ito, Itaru Arai, Yuki Kobayashi, Kanehiro Hayashi, Shintaro Otsuka, Kazunori Nakajima, Michisuke Yuzaki, Shigeyoshi Itohara, Norihiro Suzuki
Cytoplasmic aggregation of fused in sarcoma (FUS) is detected in brain regions affected by amyotrophic lateral sclerosis (ALS) and frontotemporal dementia (FTD), which compose the disease spectrum, FUS proteinopathy. To understand the pathomechanism of ALS-FTD-associated FUS, we examined the behavior and cellular properties of an ALS mouse model overexpressing FUS with nuclear localization signal deletion. Mutant FUS transgenic mice showed hyperactivity, social interactional deficits, and impaired fear memory retrieval, all of which are compatible with FTD phenotypes...
September 9, 2017: EBioMedicine
https://www.readbyqxmd.com/read/28926357/first-in-human-treatment-with-a-dendritic-cell-targeting-lentiviral-vector-expressing-ny-eso-1-lv305-induces-deep-durable-response-in-refractory-metastatic-synovial-sarcoma-patient
#3
Seth M Pollack, Hailing Lu, Sacha Gnjatic, Neeta Somaiah, Ryan B O'Malley, Robin L Jones, Frank J Hsu, Jan Ter Meulen
Effective induction of antitumor T cells is a pivotal goal of cancer immunotherapy. To this end, lentiviral vectors (LV) are uniquely poised to directly prime CD8 T-cell responses via transduction of dendritic cells in vivo and have shown promise as active cancer therapeutics in preclinical tumor models. However, until now, significant barriers related to production and regulation have prevented their widespread use in the clinic. We developed LV305, a dendritic cell-targeting, integration-deficient, replication incompetent LV from the ZVex platform, encoding the full-length cancer-testis antigen NY-ESO-1...
September 18, 2017: Journal of Immunotherapy
https://www.readbyqxmd.com/read/28901492/interactions-between-hematopoietic-progenitor-kinase-1-and-its-adaptor-proteins-review
#4
Qing Zhang, Shu Ding, Huilin Zhang
Hematopoietic progenitor kinase 1 (HPK1), also known as mitogen‑activated protein kinase kinase kinase kinase 1 is a serine/threonine protein kinase. It is involved in various cellular events, including mitogen‑activated protein kinase signaling, nuclear factor‑κB signaling, cytokine signaling, cellular proliferation and apoptosis, T cell receptor/B cell receptor signaling and T/B/dendritic cell‑mediated immune responses. Therefore, HPK1 has variety of roles in immunity, and is associated with the pathogenesis of autoimmune diseases, cancer, and the inflammatory response...
September 13, 2017: Molecular Medicine Reports
https://www.readbyqxmd.com/read/28891906/first-in-human-treatment-with-a-dendritic-cell-targeting-lentiviral-vector-expressing-ny-eso-1-lv305-induces-deep-durable-response-in-refractory-metastatic-synovial-sarcoma-patient
#5
Seth M Pollack, Hailing Lu, Sacha Gnjatic, Neeta Somaiah, Ryan B O'Malley, Robin L Jones, Frank J Hsu, Jan Ter Meulen
Effective induction of antitumor T cells is a pivotal goal of cancer immunotherapy. To this end, lentiviral vectors (LV) are uniquely poised to directly prime CD8 T-cell responses via transduction of dendritic cells in vivo and have shown promise as active cancer therapeutics in preclinical tumor models. However, until now, significant barriers related to production and regulation have prevented their widespread use in the clinic. We developed LV305, a dendritic cell-targeting, integration-deficient, replication incompetent LV from the ZVex platform, encoding the full-length cancer-testis antigen NY-ESO-1...
October 2017: Journal of Immunotherapy
https://www.readbyqxmd.com/read/28768873/human-herpesvirus-8-infects-and-replicates-in-langerhans-cells-and-interstitial-dermal-dendritic-cells-and-impairs-their-function
#6
Giovanna Rappocciolo, Mariel Jais, Paolo A Piazza, Diana C DeLucia, Frank J Jenkins, Charles R Rinaldo
The predominant types of dendritic cells (DC) in the skin and mucosa are Langerhans cells (LC) and interstitial dermal DC (iDDC). LC and iDDC process cutaneous antigens and migrate out of the skin and mucosa to the draining lymph nodes to present antigens to T and B cells. Because of the strategic location of LC and iDDC and the ability of these cells to capture and process pathogens, we hypothesized that they could be infected with human herpesvirus 8 (HHV-8; Kaposi's sarcoma-associated herpesvirus) and have an important role in the development of Kaposi's sarcoma (KS)...
August 2, 2017: Journal of Virology
https://www.readbyqxmd.com/read/28767565/small-intestine-follicular-dendritic-cell-sarcoma-with-liver-metastasis-a-case-report
#7
Yun-Chen Chang, Ivy Yenwen Chau, Yi-Chen Yeh, Gar-Yang Chau
RATIONALE: Follicular dendritic cell sarcoma (FDCS) is a rare neoplasia composed of spindle or oval cells with follicular dendritic cell differentiation, usually occurring in lymphoid tissue. In this report, we present a case of FDCS of the small intestine with liver metastasis. PATIENT CONCERNS: A 19-year-old female presented with recent onset of left upper abdominal pain. Abdominal computed tomography scan showed a large tumor mass in the liver lateral segment with compression to the pancreas upper part, and a smaller mass in the terminal ileum, respectively...
August 2017: Medicine (Baltimore)
https://www.readbyqxmd.com/read/28755918/indeterminate-dendritic-cell-tumor-in-thoracic-spine-a-case-report-and-review-of-literature
#8
Sze Kiat Tan, Lee Onn Chieng, Karthik Madhavan, Andrew Rosenberg, Ian Cote
Indeterminate dendritic cell tumor (IDCT) is an extremely rare hematological disorder with poorly understood pathogenesis. Occasionally encountered by hematologists, unusual presentations of IDCT have never been reported in the spine literature. The authors report a case of a 51-year-old man who presented with progressively worsening axial thoracic back pain radiating to his sides for three months. MRI revealed a large 3-cm enhancing mass at the T9 vertebral body with an exophytic component causing significant canal stenosis...
July 26, 2017: World Neurosurgery
https://www.readbyqxmd.com/read/28752676/fine-needle-aspiration-findings-of-a-rare-hematopoietic-neoplasm-presenting-as-obstructive-jaundice
#9
Jessica Tracht, Ali M Ahmed, Frida Rosenblum Donath
A 51-year-old female who presented with obstructive jaundice was found to have masses in the pancreatic head and tail as well as suspicious liver and periaortic masses on imaging. Aspiration cytology of the pancreatic tail mass showed abundant large single cells with vacuolated eosinophilic cytoplasm, marked nuclear pleomorphism, large bizarre irregular nuclei, binucleation, and prominent nucleoli. Numerous cells also showed intracytoplasmic black to brown pigmentation. A cell block was obtained and extensive immunohistochemical staining was performed...
July 27, 2017: Diagnostic Cytopathology
https://www.readbyqxmd.com/read/28748971/follicular-dendritic-cell-sarcoma-of-the-head-and-neck-literature-review-and-report-of-the-tonsil-occurrence-in-a-ugandan-patient
#10
I Pecorella, T R Okello, G Ciardi, E Ochola, M D Ogwang
We report a case of follicular dendritic cell sarcoma (FDCS) in a 60-year-old Ugandan female who presented with a 6-year history of a progressive left sided tonsillary mass. General systemic examination was unrevealing and the patient underwent left tonsillectomy. She was subsequently lost to follow-up. Grossly, the mass measured 6 cm in diameter and had a mottled appearance due to tissue microhaemorrhages. Markers specific for follicular dendritic cell differentiation (CD21, CD35 and CD23), p53 and EGFR were expressed on immunohistochemical analysis...
June 2017: Pathologica
https://www.readbyqxmd.com/read/28695297/histiocytic-and-dendritic-cell-neoplasms-what-have-we-learnt-by-studying-67-cases
#11
Fabio Facchetti, Stefano Aldo Pileri, Luisa Lorenzi, Valentina Tabanelli, Lisa Rimsza, Stefania Pittaluga, Stephan Dirnhofer, Christiane Copie-Bergman, Laurence de Leval, Andreas Rosenwald, Andrew Wotherspoon, Falko Fend
Tumors derived from histiocytic and dendritic cells encompass a large and heterogeneous group of neoplastic and reactive conditions, and their diagnosis is challenging both for pathologists and clinicians. Diagnosis is based on morphological and phenotypical findings, but hybrid features are not uncommon. Furthermore, recent studies uncovered the molecular mechanisms driving some of these tumors, improving diagnostic adequacy, and providing the basis for effective therapeutic breakthroughs.Sixty-seven cases were submitted to the accessory cell and histiocytic neoplasms session at the European Association of Haematopathology/Society for Hematopathology workshop 2016 held in Basel, Switzerland...
July 10, 2017: Virchows Archiv: An International Journal of Pathology
https://www.readbyqxmd.com/read/28653223/multi-targeted-therapy-of-everolimus-in-kaposi-s-sarcoma-associated-herpes-virus-infected-primary-effusion-lymphoma
#12
Suchitra Mohanty, Amit Kumar, Piyanki Das, Sushil Kumar Sahu, Tathagata Choudhuri
Kaposi's sarcoma associated herpes virus (KSHV) infected primary effusion lymphoma (PEL) is a rare aggressive form of non-Hodgkin's lymphoma of B cells. KSHV latent and lytic antigens modulate several host cellular signalling pathways especially mammalian target of rapamycin (mTOR), STAT-3 and nuclear factor-kappa B (NF-κB) for rapid tumor progression and immune evasion. Current chemotherapeutic strategies are becoming ineffective as they kill only dividing cells and inefficient to target molecular pathways crucial for active virus replication and its survival...
June 26, 2017: Apoptosis: An International Journal on Programmed Cell Death
https://www.readbyqxmd.com/read/28653001/follicular-dendritic-cell-sarcoma-of-the-liver-with-metachronous-small-bowel-and-splenic-metastases-a-case-report-and-literature-review
#13
Ken Min Chin, Woon Yang Ho, Kiat Hon Tony Lim, Yaw Fui Alexander Chung, Ser Yee Lee
Follicular dendritic cell sarcoma (FDCS) of the liver is an extremely rare disease, accounting for a mere 0.4% of all soft tissue sarcomas. FDCS most commonly involves lymph nodes but also affects extranodal sites such as the gastrointestinal system, oral cavity, liver, spleen and pancreas, albeit less commonly. It is widely considered a low-to-intermediate grade malignancy. We report a case of FDCS with metachronous involvement of the liver, small intestines and spleen, its imaging, histological findings and its management...
June 2017: Hepatobiliary Surgery and Nutrition
https://www.readbyqxmd.com/read/28645296/myd88-dependent-pro-interleukin-1%C3%AE-induction-in-dendritic-cells-exposed-to-food-grade-synthetic-amorphous-silica
#14
Hans Christian Winkler, Julian Kornprobst, Peter Wick, Lea Maria von Moos, Ioannis Trantakis, Elisabeth Maria Schraner, Barbara Bathke, Hubertus Hochrein, Mark Suter, Hanspeter Naegeli
BACKGROUND: Dendritic cells (DCs) are specialized first-line sensors of foreign materials invading the organism. These sentinel cells rely on pattern recognition receptors such as Nod-like or Toll-like receptors (TLRs) to launch immune reactions against pathogens, but also to mediate tolerance to self-antigens and, in the intestinal milieu, to nutrients and commensals. Since inappropriate DC activation contributes to inflammatory diseases and immunopathologies, a key question in the evaluation of orally ingested nanomaterials is whether their contact with DCs in the intestinal mucosa disrupts this delicate homeostatic balance between pathogen defense and tolerance...
June 23, 2017: Particle and Fibre Toxicology
https://www.readbyqxmd.com/read/28643685/-disseminated-interdigitating-dendritic-cell-sarcoma
#15
Ignacio M Santarelli, Mariano Veltri, Diego J Manzella, María Alejandra Avagnina, Pablo M Pereyra, Hernán C Chavín
A 70 year-old woman was admitted to our hospital with a 3-month history of abdominal pain, weight loss and night sweats. On physical examination, she presented with a 5 cm diameter abdominal mass extended from epigastrium to the left flank, and at least three bilateral supraclavicular adenopathies. A disseminated interdigitating dendritic cell sarcoma was diagnosed through a biopsy of the abdominal mass. After that, a CHOP regime (cyclophosphamide, doxorubicin, vincristine and prednisone) was iniciated. She died after completion of the first cycle of treatment, six months after diagnosis...
2017: Medicina
https://www.readbyqxmd.com/read/28641661/-clinical-and-pathologic-features-of-myeloid-sarcoma
#16
Ya-Jun Jiang, Hong-Xia Wang, Wan-Chuan Zhuang, Hao Chen, Chang Zhang, Xiu-Mei Li, Gui-Hua Zhu, Yao He
OBJECTIVE: To explore the clinicopathologic features, differential diagnosis and therapy of myeloid sarcoma. METHODS: The clinical data including clinical manifestations, laboratorial tests, histopathologicical examination, immunohistochemistry and clinical prognosis of 10 patients with myeloid sarcoma were analyzed retrospectively. Among 10 patients, 5 male and 5 female, aged 23 to 71 years old (median = 36 years). RESULTS: 2 cases of myeloid sarcoma were secondary from chronic myeloid leukemia, and 1 cases of myeloid sarcoma occurred after the allogeneic hematopoietic stem cell transplantation due to acute myeloid leukemia, and the others lacked the anamnesis of malignancies...
June 2017: Zhongguo Shi Yan Xue Ye Xue za Zhi
https://www.readbyqxmd.com/read/28626593/langerhans-cell-sarcoma-a-case-report-demonstrating-morphological-and-immunophenotypical-variability-within-a-single-lesion
#17
Rasika Singh, Charles Edward Keen, Christopher Stone, Patrick Sarsfield
Langerhans cells are antigen presenting dendritic cells and tumours arising from these are rare. The tumours arising from these dendritic cells are divided into two categories according to a WHO classification: Langerhans cell histiocytosis and Langerhans cell sarcoma. It is the degree of atypia and clinical aggressiveness that distinguishes the two subtypes. Langerhans cell sarcoma (LCS) is a neoplastic proliferation of Langerhans cells which can occur in skin, bone marrow, lymph nodes, spleen, liver, and lung...
2017: Case Reports in Pathology
https://www.readbyqxmd.com/read/28621320/genomic-analysis-of-follicular-dendritic-cell-sarcoma-by-molecular-inversion-probe-array-reveals-tumor-suppressor-driven-biology
#18
Erica F Andersen, Christian N Paxton, Dennis P O'Malley, Abner Louissaint, Jason L Hornick, Gabriel K Griffin, Yuri Fedoriw, Young S Kim, Lawrence M Weiss, Sherrie L Perkins, Sarah T South
Follicular dendritic cell sarcoma is a rare malignant neoplasm of dendritic cell origin that is currently poorly characterized by genetic studies. To investigate whether recurrent genomic alterations may underlie the biology of follicular dendritic cell sarcoma and to identify potential contributory regions and genes, molecular inversion probe array analysis was performed on 14 independent formalin-fixed, paraffin-embedded samples. Abnormal genomic profiles were observed in 11 out of 14 (79%) cases. The majority showed extensive genomic complexity that was predominantly represented by hemizygous losses affecting multiple chromosomes...
September 2017: Modern Pathology: An Official Journal of the United States and Canadian Academy of Pathology, Inc
https://www.readbyqxmd.com/read/28611161/follicular-dendritic-sarcoma-masquerading-as-fibrosing-mediastinitis
#19
Shashank Reddy Cingam, Majd Al Shaarani, Amol Takalkar, Prakash Peddi
Fibrosing mediastinitis (FM) is a rare disorder resulting from abnormal immunological-mediated fibro-proliferative reaction in the mediastinum. Here, we describe a case of a 46-year-old female with an incidentally found 11×9 cm posterior mediastinal mass. Multiple biopsies of this unresectable, 18-fluorodeoxyglucose avid mass revealed marked fibrosis without any evidence of malignancy, suggesting idiopathic fibrosing mediastinitis as our initial diagnosis. Multiple interventions including a trial of steroids, fluconazole, and azathioprine to target fibrosing mediastinitis were not successful...
June 13, 2017: BMJ Case Reports
https://www.readbyqxmd.com/read/28554574/p16-expression-in-follicular-dendritic-cell-sarcoma-a-potential-mimicker-of-hpv-related-oropharyngeal-squamous-cell-carcinoma
#20
Lingxin Zhang, Chen Yang, James S Lewis, Samir K El-Mofty, Rebecca D Chernock
Follicular dendritic cell sarcoma is a rare mesenchymal neoplasm that most commonly occurs in cervical lymph nodes. It has histologic and clinical overlap with the much more common p16-positive human papillomavirus (HPV)-related squamous cell carcinoma of the oropharynx which characteristically has nonkeratinizing morphology and often presents as an isolated neck mass. Not surprisingly, follicular dendritic cell sarcomas are commonly misdiagnosed as squamous cell carcinoma. Immunohistochemistry is helpful in separating the two entities...
May 26, 2017: Human Pathology
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