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https://www.readbyqxmd.com/read/28432450/genetic-aberrations-and-molecular-biology-of-skull-base-chordoma-and-chondrosarcoma
#1
Yohei Kitamura, Hikaru Sasaki, Kazunari Yoshida
Chordomas and chondrosarcomas are two major malignant bone neoplasms located at the skull base. These tumors are rarely metastatic, but can be locally invasive and resistant to conventional chemotherapies and radiotherapies. Accordingly, therapeutic approaches for the treatment of these tumors can be difficult. Additionally, their location at the skull base makes them problematic. Although accurate diagnosis of these tumors is important because of their distinct prognoses, distinguishing between these tumor types is difficult due to overlapping radiological and histopathological findings...
April 21, 2017: Brain Tumor Pathology
https://www.readbyqxmd.com/read/28431716/invasive-medullary-type-a-thymoma-with-recurrent-distant-metastases
#2
Maria Cecilia Mengoli, Lucia Longo, Susanna Varini, Giulio Rossi, Filippo Lococo
Thymomas are rare epithelial mediastinal tumors showing a certain propensity for local and intrathoracic recurrences. Otherwise, extrathoracic metastases are very rarely reported and are usually associated with type B thymomas or thymic carcinomas. We present a challenging patient with an invasive (Masaoka-Koga stage IIb) medullary type A thymoma with recurrent extrathoracic metastases (pulmonary and vertebral localizations occurring 2 and 7 years after the initial radical thymectomy, respectively). Despite type A thymoma being considered a low-grade malignancy with a scarce propensity to recurrence, the present case should alert clinicians to the possibility of bone metastasis...
May 2017: Annals of Thoracic Surgery
https://www.readbyqxmd.com/read/28431711/nodular-fasciitis-of-the-posterior-chest-wall-with-bone%C3%A2-invasion-mimicking-a-malignant-neoplasm
#3
Nicholas J Olson, David J Finley, Michael J Tsapakos, Sandra L Wong, Konstantinos Linos
Nodular fasciitis is a self-limiting benign fibroblastic/myofibroblastic proliferation, which typically presents as a rapidly growing mass resembling an aggressive lesion clinically. It can also mimic a sarcoma histologically, hence the frequent characterization as "pseudosarcoma." We describe a case of a 53-year-old man who presented with a posterior chest wall mass that on imaging showed erosion into the adjacent ribs. After resection, the diagnosis of nodular fasciitis was rendered. Bone erosion by nodular fasciitis is extremely rare and can resemble a malignant neoplasm radiologically...
May 2017: Annals of Thoracic Surgery
https://www.readbyqxmd.com/read/28431708/extramedullary-plasmacytoma-arising-from-the-anterior-mediastinum
#4
Kiyoshi Sato, Satoshi Fumimoto, Takehisa Fukada, Yoshio Ichihashi, Kaoru Ochi, Hidetoshi Satomi, Takuya Morita, Nobuharu Hanaoka, Yoshikatsu Okada, Takahiro Katsumata
Plasmacytomas are a localized proliferation of plasma cells in the bone marrow and soft tissue. Extramedullary plasmacytomas are rare and typically solitary plasma cell neoplasms originating from extraosseous organs and tissues. A 31-year-old woman was referred to our hospital with a rapidly growing abnormal shadow on a chest roentgenogram. Chest computed tomography showed that the lesion was located in the anterior mediastinum. She underwent surgery, and the tumor was diagnosed as an extramedullary plasmacytoma...
May 2017: Annals of Thoracic Surgery
https://www.readbyqxmd.com/read/28430954/increased-multinucleated-megakaryocytes-as-an-isolated-finding-in-bone-marrow-%C3%A2-a-rare-finding-and-its-clinical-significance
#5
Majd D Jawad, Ronald S Go, Kaaren K Reichard, Min Shi
Objectives: Multinucleated megakaryocytes are a unique morphologic form of megakaryocytes characterized by multiple, distinctly separated nuclei. We investigated whether increased multinucleated megakaryocytes (≥25%) in otherwise normal-appearing bone marrow were associated with the development of a myelodysplastic syndrome (MDS). Methods: We retrospectively reviewed the medical records and bone marrow biopsy specimens of patients evaluated at our institution from 2011 to 2015 that met all of the following criteria: (1) 25% or more multinucleated megakaryocytes, (2) no other dysplastic features, (3) absence of a myeloid neoplasm, and (4) absence of neoplastic karyotypic abnormalities...
November 1, 2016: American Journal of Clinical Pathology
https://www.readbyqxmd.com/read/28430293/three-cases-of-ectopic-sphenoid-sinus-pituitary-adenoma
#6
Ernest Jan Bobeff, Karol Wiśniewski, Wielisław Papierz, Ludomir Stefańczyk, Dariusz Jan Jaskólski
<i><b>Introduction</b>: Ectopic sphenoid sinus pituitary adenoma is a rare tumour originating from embryologic remnants of Rathke's pouch. Although it is considered a clinically benign neoplasm, necrosis is encountered in 25% of cases and it can invade adjacent bone structures. <b>Aims</b>: To establish clinical, radiological and histopathological features of ectopic sphenoid sinus pituitary adenoma. <b>Material and methods: </b> Analysis of three cases: two females and one man, aged 61-70...
2017: Folia Neuropathologica
https://www.readbyqxmd.com/read/28429277/primary-ewing-sarcoma-primitive-neuroectodermal-tumor-of-the-kidney-a-clinicopathologic-study-of-23-cases
#7
Paari Murugan, Priya Rao, Pheroze Tamboli, Bogdan Czerniak, Charles C Guo
Primary Ewing sarcoma / primitive neuroectodermal tumor (ES) of the kidney is a rare neoplasm with limited clinicopathologic data. We report 23 such cases with no history of ES elsewhere in the body. The patients included 13 male and 10 female, aged 8-70 years (mean, 31 years). The average tumor size was 11.7 cm (range, 5-20 cm). Microscopic analysis showed predominantly lobular growth (n = 14), with focal papillary (n = 3), alveolar (n = 1), and hemangiopericytoma-like (n = 1) patterns. Several tumors (n = 11) exhibited robust mitotic activity (>10 mitoses/10 high-power fields)...
April 20, 2017: Pathology Oncology Research: POR
https://www.readbyqxmd.com/read/28428103/characteristics-and-clinical-implications-of-reactive-germinal-centers-in-the-bone-marrow
#8
Rose Lou Marie C Agbay, L Jeffrey Medeiros, Joseph D Khoury, Alireza Salem, Carlos E Bueso-Ramos, Sanam Loghavi
Reactive germinal centers (GCs) in the bone marrow (BM) have been described in patients with autoimmune disorders, infections, malignancies and following certain drug therapies, or as an isolated finding without obvious underlying disease. In this study, we describe the clinical conditions in which reactive GCs occur in BM samples, and their topography and accompanying laboratory and ancillary findings in the setting of a cancer center. We identified 32 BM specimens with reactive GCs with an estimated frequency of <0...
April 17, 2017: Human Pathology
https://www.readbyqxmd.com/read/28427520/lymphoblastic-lymphoma
#9
REVIEW
Sergio Cortelazzo, Andrés Ferreri, Dieter Hoelzer, Maurilio Ponzoni
Lymphoblastic lymphoma (LBL) is a neoplasm of immature B cells committed to the B-(B-LBL) or T-cell lineage (T-LBL) that accounts for approximately 2% of all lymphomas. Although histological features are usually sufficient to distinguish lymphoblastic from mature B- or T-cell neoplasms, of greater importance for diagnosis is the characterization of immunophenotype by flow cytometry. LBL occurs more commonly in children than in adults, mostly in males. A bone marrow involvement <25% (or 20% according to WHO) formally distinguishes LBL from ALL...
May 2017: Critical Reviews in Oncology/hematology
https://www.readbyqxmd.com/read/28423984/congenital-amelanotic-melanoma-in-a-crossbred-heifer-calf
#10
Christine M Winslow, Jason Wood, John N Gilliam, Melanie A Breshears
A large, pedunculated cutaneous mass protruding from the left flank fold and an enlarged left prefemoral lymph node were found on examination of a 3-d-old crossbred Aberdeen Angus heifer. The calf was asymptomatic aside from peripheral lymphadenopathy, and the mass, along with the left prefemoral lymph node, was surgically excised. Histologic examination of the mass and the lymph node revealed a homogeneous population of neoplastic cells that stained positively with immunohistochemical stains S100 and melan A, supporting a diagnosis of congenital amelanotic melanoma with nodal metastasis...
April 1, 2017: Journal of Veterinary Diagnostic Investigation
https://www.readbyqxmd.com/read/28422729/the-relevance-of-a-low-jak2v617f-allele-burden-in-clinical-practice-a-monocentric-study
#11
Margherita Perricone, Nicola Polverelli, Giovanni Martinelli, Lucia Catani, Emanuela Ottaviani, Elisa Zuffa, Eugenia Franchini, Arbana Dizdari, Dorian Forte, Elena Sabattini, Michele Cavo, Nicola Vianelli, Francesca Palandri
Since low JAK2V617F allele burden (AB) has been detected also in healthy subjects, its clinical interpretation may be challenging in patients with chronic myeloproliferative neoplasms (MPNs). We tested 1087 subjects for JAK2V617F mutation on suspicion of hematological malignancy. Only 497 (45.7%) patients were positive. Here we present clinical and laboratory parameters of a cohort of 35/497 patients with an AB ≤ 3%.Overall, 22/35 (62.9%) received a WHO-defined diagnosis of MPN and in 14/35 cases (40%) diagnosis was supported by bone marrow (BM) histology (''Histology-based'' diagnosis)...
March 31, 2017: Oncotarget
https://www.readbyqxmd.com/read/28421271/-hereditary-bone-tumors
#12
D Baumhoer
Hereditary bone tumors are rare and result from mutations affecting cell cycle regulation (e.g. retinoblastoma syndrome/RB1 and Li-Fraumeni syndrome/TP53, Gardner syndrome/APC), energy metabolism (enchondromatosis/IDH1/2), complex signaling cascades (multiple hereditary exostoses/EXT1/2) and DNA integrity (Rothmund-Thomson/RECQL4, Werner/WRN and Bloom syndromes/BLM). The majority of syndromes are incompletely understood and can lead to multiple benign tumors, of which some might undergo secondary malignant transformation over time (enchondromatosis: enchondromas, multiple hereditary exostoses: osteochondromas, Gardner syndrome: osteomas) or bone sarcomas, primarily osteosarcomas as primary (Li-Fraumeni, Rothmund-Thomson, Werner and Bloom syndromes) or secondary manifestation (retinoblastoma syndrome) of the disease...
April 18, 2017: Der Pathologe
https://www.readbyqxmd.com/read/28418282/immunohistochemical-detection-of-hairy-cell-leukemia-in-paraffin-sections-the-role-of-pax5-and-cd103-double-staining-to-improve-specificity-and-sensitivity
#13
Igor B Rozenvald, Maurice D Richardson, Lenessa Brock, Russell L Maiese
CONTEXT: - In hematopathology practice, abnormal expression of CD103 on B cells is detected by flow cytometry in hairy cell leukemia (HCL) and, in combination with other phenotypic and morphologic findings, provides diagnostic specificity and sensitivity. Immunostaining on paraffin sections makes it possible to perform immunophenotyping in situ. However, normal bone marrow contains CD103-positive cells, which are not B cells, making it difficult to be certain about low-level involvement by HCL...
April 18, 2017: Archives of Pathology & Laboratory Medicine
https://www.readbyqxmd.com/read/28415158/clinicopathological-study-of-18-cases-of-inflammatory-myofibroblastic-tumors-with-reference-to-alk-1-expression-5-year-experience-in-a-tertiary-care-center
#14
Ramesh Babu Telugu, Anne Jennifer Prabhu, Nobin Babu Kalappurayil, John Mathai, Birla Roy Gnanamuthu, Marie Therese Manipadam
Background: Inflammatory myofibroblastic tumor is a histopathologically distinctive neoplasm of children and young adults. According to World Health Organization (WHO) classification, inflammatory myofibroblastic tumor is an intermediate-grade tumor, with potential for recurrence and rare metastasis. There are no definite histopathologic, molecular or cytogenetic features to predict malignant transformation, recurrence or metastasis. Materials and Methods: A 5-year retrospective study of histopathologically diagnosed inflammatory myofibroblastic tumors of various anatomic sites was conducted to correlate anaplastic lymphoma kinase-1 (ALK-1) expression with histological atypia, multicentric origin of tumor, recurrence and metastasis...
April 17, 2017: Journal of Pathology and Translational Medicine
https://www.readbyqxmd.com/read/28414089/juvenile-myelomonocytic-leukemia-with-prominent-cd141-myeloid-dendritic-cell-differentiation
#15
Howard Meyerson, Amad Awadallah, Georgetta Blidaru, Ebenezer Osei, June Schlegelmilch, Rachel Egler, Rolla Abu-Arja, Hilda Ding
Myeloid malignancies showing CD141(+) myeloid dendritic cell (MDC) differentiation have not been documented. Here, we describe a patient with juvenile myelomonocytic leukemia (JMML) in which a prominent CD141(+) cell population was identified most consistent with CD141(+) MDCs based on phenotypic similarity with normal CD141(+) MDCs. Molecular studies demonstrated a KRAS mutation. The findings from the spleen and bone marrow are described. This is the first well-documented demonstration of CD141(+) MDC differentiation of a hematopoietic neoplasm...
April 13, 2017: Human Pathology
https://www.readbyqxmd.com/read/28413392/t-cell-lymphoma-in-a-patient-with-neurofibromatosis-type-1-and-aids
#16
Izana Junqueira de Castro, Esther Botelho Soares da Silva, Talita Rezende Dos Santos, Amanda Barroso de Freitas, Inara Junqueira de Castro, Alessandra Santos Portela, Marilza Campos de Magalhães, Karina Lebeis Pires, Guilherme Almeida Rosa da Silva, Marcelo Costa Velho Mendes de Azevedo
Neurofibromatosis type 1 (NF1) and AIDS are risk factors for the development of malignant neoplasms, including hematological malignancies, such as non-Hodgkin lymphoma. NF1 is an autosomal dominant disease that primarily manifests as café-au-lait spots, dermal neurofibromas, axillary and/or inguinal ephelides or freckles, plexiform neurofibromas, Lisch nodules, and bone deformities. In this report, we present a 38-year-old female patient with NF1 from childhood and AIDS who developed peripheral T-cell lymphoma with good response to chemotherapeutic treatment...
January 2017: Case Reports in Oncology
https://www.readbyqxmd.com/read/28410203/exploring-the-key-genes-and-pathways-in-enchondromas-using-a-gene-expression-microarray
#17
Zhongju Shi, Hengxing Zhou, Bin Pan, Lu Lu, Yi Kang, Lu Liu, Zhijian Wei, Shiqing Feng
Enchondromas are the most common primary benign osseous neoplasms that occur in the medullary bone; they can undergo malignant transformation into chondrosarcoma. However, enchondromas are always undetected in patients, and the molecular mechanism is unclear. To identify key genes and pathways associated with the occurrence and development of enchondromas, we downloaded the gene expression dataset GSE22855 and obtained the differentially expressed genes (DEGs) by analyzing high-throughput gene expression in enchondromas...
March 30, 2017: Oncotarget
https://www.readbyqxmd.com/read/28409040/a-case-of-blastic-plasmacytoid-dendritic-cell-neoplasm-extensively-studied-by-flow-cytometry-and-immunohistochemistry
#18
Martina Pennisi, Clara Cesana, Micol Giulia Cittone, Laura Bandiera, Barbara Scarpati, Valentina Mancini, Silvia Soriani, Silvio Veronese, Mauro Truini, Silvano Rossini, Roberto Cairoli
Blastic plasmacytoid dendritic cell neoplasm (BPDCN) is a rare hematologic malignancy with aggressive clinical course and poor prognosis. Diagnosis is based on detection of CD4(+) CD56(+), CD123(high), TCL-1(+), and blood dendritic cell antigen-2/CD303(+) blasts, together with the absence of lineage specific antigens on tumour cells. In this report we present a case of BPDCN presenting with extramedullary and bone marrow involvement, extensively studied by flow cytometry and immunohistochemistry, who achieved complete remission after acute lymphoblastic leukemia like chemotherapy and allogeneic hematopoietic stem cell transplantation...
2017: Case Reports in Hematology
https://www.readbyqxmd.com/read/28407783/exosomal-transfer-of-tumor-associated-macrophage-derived-mir-21-confers-cisplatin-resistance-in-gastric-cancer-cells
#19
Peiming Zheng, Lei Chen, Xiangliang Yuan, Qin Luo, Yi Liu, Guohua Xie, Yanhui Ma, Lisong Shen
BACKGROUND: Cisplatin-based chemotherapy is frequently used to treat advanced gastric cancer (GC). However, the resistance often occurs with the mechanisms being not well understood. Recently, emerging evidence indicates that tumor-associated macrophages (TAMs) play an important role in chemoresistance of cancer. As the important mediators in intercellular communications, exosomes secreted by host cells mediate the exchange of genetic materials and proteins to be involved in tumor aggressiveness...
April 13, 2017: Journal of Experimental & Clinical Cancer Research: CR
https://www.readbyqxmd.com/read/28407623/fine-needle-aspiration-of-langerhans-cell-histiocytosis-a-cytopathologic-study-of-37-cases
#20
Jen-Fan Hang, Momin T Siddiqui, Syed Z Ali
OBJECTIVE: Langerhans cell histiocytosis (LCH) is an uncommon neoplasm of dendritic cells and predominantly occurs in children and young adults. The study aims to evaluate cytopathologic features and current diagnostic concepts in a large series of LCH on fine needle aspiration (FNA). STUDY DESIGN: We retrospectively searched the pathology archives of The Johns Hopkins Hospital (JHH) and Emory University Hospital (EUH) to identify all FNA cases diagnosed as LCH in a period of 21 years...
April 14, 2017: Acta Cytologica
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