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https://www.readbyqxmd.com/read/29033760/colonic-ganglioneuroma-a-rare-finding-during-colorectal-cancer-screening
#1
Emmanuel Ofori, Mel Ona, Daryl Ramai, Tiangui Huang, Philip Xiao, Madhavi Reddy
Ganglioneuromas are very rare clinical entities, and their occurrence in the large bowel lays further emphasis on their rarity. Ganglioneuromas are benign tumors of undifferentiated neural crest cells. Their clinical presentation is mostly asymptomatic, and if any symptoms are present at all, they are usually nonspecific, with excellent prognosis. We report an asymptomatic, 65-year-old male with a solitary ascending colonic polyp found on screening colonoscopy. Histology revealed benign polypoid spindle-cell proliferation as well as S100 reactivity, consistent with ganglioneuroma...
May 2017: Case Reports in Gastroenterology
https://www.readbyqxmd.com/read/29031103/kaposi-s-sarcoma-herpesvirus-induced-endothelial-cell-reprogramming-supports-viral-persistence-and-contributes-to-kaposi-s-sarcoma-tumorigenesis
#2
REVIEW
Silvia Gramolelli, Päivi M Ojala
Kaposi's sarcoma (KS) is an endothelial tumor causally linked to Kaposi's sarcoma herpesvirus (KSHV) infection. At early stages of KS, inflammation and aberrant neoangiogenesis are predominant, while at late stages the disease is characterized by the proliferation of KSHV-infected spindle cells (SC). Since KSHV infection modifies the endothelial cell (EC) identity, the origin of SCs remains elusive. Yet, pieces of evidence indicate the lymphatic origin. KSHV-infected ECs display increased proliferative, angiogenic and migratory capacities which account for KS oncogenesis...
October 11, 2017: Current Opinion in Virology
https://www.readbyqxmd.com/read/29028515/post-biopsy-mri-changes-in-the-size-and-enhancement-of-intramuscular-myxomas-a-report-of-two-cases
#3
Wilbur Wang, Edward Smitaman, Wesley Rubenstein, Tudor Hughes, Brady K Huang
Intramuscular myxomas are benign soft-tissue tumors, characterized by bland spindle-shaped cells and fibroblasts within an abundant mucoid matrix on histologic examination. Classically, these are slowly enlarging masses which may occasionally cause pain, paresthesia, and muscle weakness secondary to mass effect. We present an interesting phenomenon of two histologically confirmed cases of intramuscular myxomas that exhibited size and enhancement changes on follow-up imaging after image-guided biopsy. To our knowledge, this is the first report to describe size and enhancement changes of intramuscular myxomas after biopsy...
September 28, 2017: Clinical Imaging
https://www.readbyqxmd.com/read/29028085/nestin-and-cd146-expression-in-metaplastic-breast-cancer-stem-cell-therapy-in-need-lessons-reported-from-a-male-patient
#4
A Tampakis, E C Tampaki, D Trafalis, A Nonni, K Kontzoglou, E Patsouris, M Kontos, G Kouraklis
OBJECTIVE: Metaplastic breast carcinomas represent a rare subtype of breast cancer exhibiting aggressive clinical features. They appear as highly chemoresistant tumors, therefore showing poor outcome and high rates of local recurrence or distant metastasis. CASE REPORT: A 37-year-old greek man was referred to our hospital for evaluation of a locally advanced, ulcerated, fixed, irregular and hard in consistency mass covering his left breast and chest wall. Further work out with CT and biopsy of the tumor revealed a triple negative metaplastic breast cancer classified as cT4cN3cM1...
September 2017: European Review for Medical and Pharmacological Sciences
https://www.readbyqxmd.com/read/29026362/inflammatory-myofibroblastic-tumor-of-the-breast-mimicking-malignancy-in-an-elderly-male
#5
Fouzia Siraj, Manveen Kaur, Varsha Dalal, Jain Sonam
BACKGROUND: Inflammatory myofibroblastic tumor (IMT) is a rare, distinctive lesion composed of a proliferation of myofibroblastic spindle cells accompanied by an inflammatory infiltrate. It was first described in the lung, but its occurrence at various extrapulmonary sites has also been reported. The literature mentions only a handful of cases of IMT in the breast and only 1 case in the male breast. We report the second case of IMT in the male breast. CASE REPORT: A 60-year-old male presented with a large, lobulated lump in the left breast that had progressively increased in size during the past year...
2017: Ochsner Journal
https://www.readbyqxmd.com/read/28992816/a-theoretical-model-of-the-wnt-signaling-pathway-in-the-epithelial-mesenchymal-transition
#6
Kelsey Gasior, Marlene Hauck, Alyson Wilson, Sudin Bhattacharya
BACKGROUND: Following the formation of a primary carcinoma, neoplastic cells metastasize by undergoing the epithelial mesenchymal transition (EMT), which is triggered by cues from inflammatory and stromal cells in the microenvironment. EMT allows epithelial cells to lose their highly adhesive nature and instead adopt the spindle-like appearance, as well as the invasive and migratory behavior, of mesenchymal cells. We hypothesize that a bistable switch between the epithelial and mesenchymal phenotypes governs EMT, allowing the cell to maintain its mesenchymal phenotype even after it leaves the primary tumor microenvironment and EMT-inducing extracellular signal...
October 10, 2017: Theoretical Biology & Medical Modelling
https://www.readbyqxmd.com/read/28991373/novel-enriched-pathways-in-superficial-malignant-peripheral-nerve-sheath-tumors-mpnst-and-spindle-desmoplastic-melanomas-sdm
#7
George Jour, Nicole K Andeen, Rami Al -Rohil, Phyu P Aung, Meenakshi Mehrotra, Dzifa Duose, Benjamin Hoch, Zolt Argenyi, Rajyalakshmi Luthra, Ignacio I Wistuba, Victor G Prieto
Superficial malignant peripheral nerve sheath tumor (MPNST) is a rare, soft tissue neoplasm that shares morphological features and some molecular events with spindle and desmoplastic melanoma (SDM). Herein we sought to identify molecular targets for therapy using targeted RNA/DNA sequencing and gene expression of key immunological players. DNA and RNA from formalin-fixed, paraffin-embedded (FFPE) tissue were extracted and processed. Massive high-throughput deep parallel sequencing was performed using the Oncomine comprehensive panel enabling detection of relevant SNVs, CNVs, gene fusions, and indels from 143 unique genes on the Ion torrent sequencer for clinical trial research programs...
October 9, 2017: Journal of Pathology
https://www.readbyqxmd.com/read/28991228/deletion-of-menin-in-craniofacial-osteogenic-cells-in-mice-elicits-development-of-mandibular-ossifying-fibroma
#8
S Lee, P Liu, R Teinturier, J Jakob, M Tschaffon, A Tasdogan, R Wittig, S Hoeller, D Baumhoer, L Frappart, S Vettorazzi, P Bertolino, C Zhang, J Tuckermann
Ossifying fibroma (OF) is a rare benign tumor of the craniofacial bones that can reach considerable and disfiguring dimensions if left untreated. Although the clinicopathological characteristics of OF are well established, the underlying etiology has remained largely unknown. Our work indicates that Men1-a tumor suppressor gene responsible of Multiple endocrine neoplasia type 1-is critical for OF formation and shows that mice with targeted disruption of Men1 in osteoblasts (Men1(Runx2Cre)) develop multifocal OF in the mandible with a 100% penetrance...
October 9, 2017: Oncogene
https://www.readbyqxmd.com/read/28984298/polypoid-fibroadipose-tumors-of-the-esophagus-giant-fibrovascular-polyp-or-liposarcoma-a-clinicopathological-and-molecular-cytogenetic-study-of-13-cases
#9
Rondell P Graham, Saba Yasir, Karen J Fritchie, Michelle D Reid, Patricia T Greipp, Andrew L Folpe
Giant fibrovascular polyp of the esophagus is a descriptive diagnostic term intended to encompass rare, large, polypoid esophageal masses composed of fibroadipose tissue. Despite sometimes dramatic clinical presentations, they have historically been considered to represent reactive, non-neoplastic proliferations. Recently, however, a small number of reports have described well-differentiated liposarcomas of the esophagus, mimicking giant fibrovascular polyps. In order to clarify the relationship between esophageal liposarcoma and giant fibrovascular polyp, we retrieved esophageal cases coded as 'giant fibrovascular polyp,' 'lipoma' and 'liposarcoma' from our archives and re-examined their clinicopathologic features and MDM2 amplification status...
October 6, 2017: Modern Pathology: An Official Journal of the United States and Canadian Academy of Pathology, Inc
https://www.readbyqxmd.com/read/28982917/gastric-spindle-cell-neuroendocrine-tumor-mimicking-gastrointestinal-stromal-tumor-unique-morphology-and-diagnostic-pitfall
#10
Alexandria A Lee, Nishant Poddar, Muhammad B Hammami, Jula Veerapong, Dengfeng Cao, Jin-Ping Lai
Gastric neuroendocrine tumors (GNETs) with spindle cell morphology are extremely rare. We present a case of a 49-year-old female patient with a history of systemic lupus erythematosus, Sjogren's syndrome, and gastroesophageal reflux disease. She was initially thought to have a spindle cell gastrointestinal stromal tumor per histological studies of the fundic polypectomy samples. Immunohistochemically, the tumor cells were negative for CD117, and CD34, but positive for chromogranin, synaptophysin, and CD56 with a 6% Ki-67 index, consistent with a spindle cell-type well differentiated neuroendocrine tumor, World Health Organization (WHO) Grade 2...
October 2017: Anticancer Research
https://www.readbyqxmd.com/read/28982854/clinicopathological-characteristics-of-ovarian-sclerosing-stromal-tumor-with-an-emphasis-on-tfe3-overexpression
#11
Cheol Keun Park, Hyun-Soo Kim
A sclerosing stromal tumor is a very rare benign sex cord-stromal tumor of the ovary. Because its clinical presentation and imaging findings are similar to those of borderline or malignant epithelial tumors and other sex cord-stromal tumors, accurate preoperative clinical diagnosis can be difficult. The aim of this study was to analyze the clinicopathological characteristics of SSTs and examine the immunohistochemical expression TFE3, which has not been studied in SSTs. Our study cohort consisted of 9 patients diagnosed as having SST; the median age was 36 years...
October 2017: Anticancer Research
https://www.readbyqxmd.com/read/28982039/whorling-sclerosing-meningioma-a-review-on-the-histological-features-of-a-rare-tumor-including-an-illustrative-case
#12
REVIEW
İlhan Elmaci, Meric Adil Altinoz, Aydin Sav, Fatih Han Bolükbaşı, Mustafa Önöz, Özdil Başkan, Ramazan Sari
Whorling-Sclerosing variant of meningioma (WSM) is a very rare variant of meningioma and only 28 cases were previously reported in the English medical literature. The term "whorling" describes different morphological features including psammoma bodies formed by precipitated calcium or by layered whorling sheets of tumor cells in meningothelial meningiomas. In WSM, the sclerosing structures are formed by typical paucicellular or acellular collagen whorls which form the majority of tumor volume. Hence, diagnosis of these tumors is based more often on morphological and histochemical features rather than on immunohistochemical findings...
September 28, 2017: Clinical Neurology and Neurosurgery
https://www.readbyqxmd.com/read/28981730/establishment-and-proteomic-characterization-of-ncc-lms1-c1-a-novel-cell-line-of-primary-leiomyosarcoma-of-the-bone
#13
Marimu Sakumoto, Mami Takahashi, Rieko Oyama, Yoko Takai, Fusako Kito, Kumiko Shiozawa, Zhiwei Qiao, Akihiko Yoshida, Makoto Endo, Akira Kawai, Tadashi Kondo
Background: Leiomyosarcoma (LMS) is one of most aggressive mesenchymal malignancies that differentiate towards smooth muscle. The clinical outcome of LMS patients is poor; as such, there is an urgent need for novel therapeutic approaches. Experimental models such as patient-derived cell lines are invaluable tools for pre-clinical studies. In the present study, we established a stable cell line from the tumor tissue of a patient with a primary LMS of the bone. Despite the urgent need for novel therapeutic strategies in LMS, there are only a few LMS cell lines available in public cell banks, none of which are primary to the bone...
October 1, 2017: Japanese Journal of Clinical Oncology
https://www.readbyqxmd.com/read/28981153/pleomorphic-fibroma-of-the-skin-with-mdm2-immunoreactivity-a-potential-diagnostic-pitfall
#14
Mehrnoosh Tashakori, Jason Pimentel, Brooke E Howitt, Jessica Sanchez, Susan Michalowski, Dhananjay Chitale, Adrian H Ormsby, Sean R Williamson
Pleomorphic fibroma is a rare benign cutaneous neoplasm characterized by spindle-shaped cells and multinucleated giant cells scattered throughout collagenous stroma. These morphologic features can lead to diagnostic confusion, including atypical lipomatous tumor as one consideration. In contrast to atypical lipomatous tumor, previous studies have found pleomorphic fibroma to be negative for MDM2 immunohistochemical staining and MDM2 gene amplification. Here, we present a case of pleomorphic fibroma of skin with nuclear MDM2 immunoreactivity in the absence of MDM2 gene amplification, underscoring the superiority of fluorescence in situ hybridization as a diagnostic test in this differential diagnosis...
October 5, 2017: Journal of Cutaneous Pathology
https://www.readbyqxmd.com/read/28979029/lower-extremity-desmoplastic-malignant-melanoma-in-egypt
#15
Reham William Doss, Sahar Daoud, Alshimaa Mohamed Abbas Mostafa, Sara Abd Elrahman Mohammed
Desmoplastic melanoma (DM) is a type of spindle cell melanoma characterized by the absence of pigment. The clinical diagnosis of DM represents a challenge for the practitioner and the pathologists because it can mimic benign or malignant skin tumors and even inflammatory skin disorders. We here discuss a case of a patient presented with multiple nodular lesions of the lower extremity following electrocautary to a lesion in her sole which was misdiagnosed as planter wart. Our clinical diagnosis was Kaposi sarcoma, hypertrophic lichen, or extensive verruca vulgaris...
September 2017: Indian Journal of Dermatology
https://www.readbyqxmd.com/read/28976138/leiomyosarcoma-of-maxilla-a-case-report-with-review-of-literature
#16
Renuka Gupta, Madhusudan Astekar, Ramakant Dandriyal, Manjunath Bs
One challenging feature of head and neck pathology is that a dizzying array of spindle cell lesions occurs here which ranges all the way from reactive, very aggressive forms to malignant lesions. Leiomyosarcoma is one such malignant tumour of mesenchymal origin exhibiting smooth muscle differentiation; presenting generally nonspecific signs and symptoms. Here we present a case of leiomyosarcoma in a 21 year old female patient associated with single reddish pink swelling present in the posterior right maxillary tuberosity region with moderate facial asymmetry...
September 2017: Journal of Experimental Therapeutics & Oncology
https://www.readbyqxmd.com/read/28975018/comprehensive-genomic-profiling-of-a-rare-thyroid-follicular-dendritic-cell-sarcoma
#17
Jaime I Davila, Jason S Starr, Steven Attia, Chen Wang, Ryan A Knudson, Brian M Necela, Vivekananda Sarangi, Zhifu Sun, Yingxue Ren, John D Casler, David M Menke, Gavin R Oliver, Richard W Joseph, John A Copland, Alexander S Parker, Jean-Pierre A Kocher, E Aubrey Thompson, Robert C Smallridge, Yan W Asmann
We previously reported an extremely rare case of follicular dendritic cell sarcoma (FDCS) presented as a thyroid mass. Given the rarity of this disease, there are no personalized and molecularly targeted treatment options due to the lack of knowledge in the genomic makeup of the tumor. A 44-year-old white woman was diagnosed with an extranodal FDCS in thyroid. The patient underwent a total thyroidectomy, central compartment dissection, parathyroid re-implantation, and adjuvant radiation therapy. Tumor DNA sequencing of 236 genes by FoundationOne panel found truncating mutations in PTEN and missense mutations in RET and TP53...
July 3, 2017: Rare Tumors
https://www.readbyqxmd.com/read/28970958/plexiform-angiomyxoid-myofibroblastic-tumor-of-the-stomach-a-rare-case
#18
Su Mi Kim, Ji Yeong An, Min-Gew Choi, Jun Ho Lee, Tae Sung Sohn, Kyung-Mee Kim, Sung Kim, Jae Moon Bae
Plexiform angiomyxoid myofibroblastic tumor (PAMT) of the stomach is a very rare mesenchymal tumor of the gastrointestinal tract. We report a case of asymptomatic gastric PAMT that was pathologically confirmed after surgical resection. The tumor had a multinodular plexiform growth pattern, bland-looking spindle cells, and an Alcian blue-positive myxoid stromal matrix rich in small blood vessels. Immunohistochemistry analysis revealed that the tumor cells of the PAMT were positive for smooth muscle actin (SMA) and negative for c-kit, CD34, S-100 protein, epithelial membrane antigen (EMA), and desmin...
September 2017: Journal of Gastric Cancer
https://www.readbyqxmd.com/read/28969015/depdc1-is-required-for-cell-cycle-progression-and-motility-in-nasopharyngeal-carcinoma
#19
Xuefei Feng, Chundong Zhang, Ling Zhu, Lian Zhang, Hongxia Li, Longxia He, Yan Mi, Yitao Wang, Jiang Zhu, Youquan Bu
DEP domain containing 1 (DEPDC1) is a newly identified cancer-related and cell cycle related gene and has been demonstrated as a novel therapeutic target for bladder cancer. However, the functional involvement and therapeutic potential of DEPDC1 in nasopharyngeal carcinoma (NPC) remains unclear. Our results showed that DEPDC1 was overexpressed at both mRNA and protein levels in NPC tissues compared with normal or non-tumor tissues. The siRNA-mediated DEPDC1 depletion resulted in significant inhibition of proliferation and delay in cell cycle progression in both NPC cell lines, CNE-1 and HNE-1...
September 8, 2017: Oncotarget
https://www.readbyqxmd.com/read/28968297/solitary-fibrous-tumor-of-the-vulva-report-of-2-cases-including-a-de-novo-dedifferentiated-solitary-fibrous-tumor-diagnosed-after-molecular-demonstration-of-nab2-stat6-gene-fusion
#20
Juan C Tardío, Isidro Machado, Isabel Alemany, M Victoria López-Soto, M Gema Nieto, Antonio Llombart-Bosch
Solitary fibrous tumor (SFT) is a neoplasm of fibroblastic lineage that has been documented in almost every anatomic location. Vulval SFT is very rare with only 10 cases reported to date. We present 2 additional SFTs located in the vulva, in adult women of 59 and 25 yr of age. The first showed a classic morphology and immunophenotype with uniform and strong STAT6 nuclear expression. The other one was a spindle-cell de novo dedifferentiated SFT with heterogeneous nuclear and cytoplasmic STAT6 staining, which could only be correctly diagnosed after molecular analysis with demonstration of a NAB2-STAT6 gene fusion...
September 29, 2017: International Journal of Gynecological Pathology
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