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Mesenchymal hamartoma

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https://www.readbyqxmd.com/read/28801289/-lkb1-regulates-epithelial-mesenchymal-transition-in-peutz-jeghers-hamartoma-and-intestinal-epithelial-cells
#1
Chao Zhong, Liang Peng, Ran Li, Jing Chen, Xin-Qi Chen, Di Zeng, Xiao-Ping Xu, Zhi-Qing Wang, Chu-di Chen, Ya-Dong Wang, Ai-Min Li, Si-de Liu, Bao-Ping Wu
OBJECTIVE: To investigate the molecular mechanism by which LKB1 regulates epithelial-mesenchymal transition (EMT) in Peutz-Jeghers hamartoma and intestinal epithelial cells. METHODS: Immunohistochemistry was used to detect gene expression of LKB1, E-cadherin, and vimentin in 20 hamartoma tissues and 10 normal intestinal tissues, and collagen fiber deposition was analyzed using Masson trichrome staining. Normal intestinal epithelial NCM460 cells were transfected with LKB1 shRNA plasmid or negative control via lentiviral vectors, and the role of LKB1 in cell polarization and migration were determined using CCK8 and Transwell assays...
August 20, 2017: Nan Fang Yi Ke da Xue Xue Bao, Journal of Southern Medical University
https://www.readbyqxmd.com/read/28767567/a-4-and-a-half-years-old-boy-with-mesenchymal-hamartomas-in-the-left-lateral-lobe-of-the-liver-a-case-report-and-literature-review
#2
Wei Liao, Binhao Zhang, Wei Zhang, Lin Chen, Wanguang Zhang, Bixiang Zhang, Xiaoping Chen
RATIONALE: Mesenchymal hamartomas of the liver is one type of rare liver tumor. PATIENT CONCERNS: Mesenchymal hamartomas of the liver (MHL) is rarely reported in the left lobe of the liver in children who are more than 2 years old. It is difficult to distinguish it from liver lesions such as hepatoblastoma in children, and hepatocellular carcinoma and focal nodular hyperplasia in adults. In addition, it is hard to correctly diagnose it without pathological examination...
August 2017: Medicine (Baltimore)
https://www.readbyqxmd.com/read/28766950/imaging-features-of-rare-mesenychmal-liver-tumors-beyond-hemangiomas
#3
Rajesh Thampy, Khaled M Elsayes, Christine O Menias, Perry J Pickhardt, Hyunseon C Kang, Sandeep Deshmukh, Kareem Ahmed, Brinda Rao Korivi
Tumors arising from mesenchymal tissue components such as vascular, fibrous and adipose tissue can manifest in the liver. Although histopathology is often necessary for definitive diagnosis, many of these lesions exhibit characteristic imaging features. The radiologist plays an important role in suggesting the diagnosis, which can direct appropriate immunohistochemical staining at histology. The aim of this review is to present clinical and imaging findings of a spectrum of mesenchymal liver tumors such as hemangioma, epithelioid hemangioendothelioma, lipoma, PEComa, angiosarcoma, inflammatory myofibroblastic tumor, solitary fibrous tumor, leiomyoma, leiomyosarcoma, Kaposi sarcoma, mesenchymal hamartoma, undifferentiated embryonal sarcoma, rhabdomyosarcoma and hepatic metastases...
August 2, 2017: British Journal of Radiology
https://www.readbyqxmd.com/read/28754078/3d-simulation-assisted-resection-of-giant-hepatic-mesenchymal-hamartoma-in-children
#4
Jing Zhao, Xian-Jun Zhou, Cheng-Zhan Zhu, Ying Wu, Bin Wei, Gang Zhang, Xi-Wei Hao, Hong Zhang, Zhong Jiang, Qian Dong
OBJECTIVE: To evaluate the usefulness of Hisense Computer Assisted Surgery System (Hisense CAS) in pre-operative surgical planning and intra-operative navigation for resection of pediatric giant hepatic mesenchymal hamartoma (HMH). METHODS: Five children with HMH underwent hepatectomy in our hospital. Pre-operative abdominal enhanced CT was performed for diagnosis and treatment planning. Using CT DICOM files, three-dimensional reconstruction was performed in three cases for operation planning and intra-operative navigation, with SID carrying out precise liver resection during the operation with Hisense CAS...
December 2017: Computer Assisted Surgery (Abingdon, England)
https://www.readbyqxmd.com/read/28751104/myofibroblastic-fibroblastic-and-myoid-lesions-of-the-breast
#5
REVIEW
Gregor Krings, Patrick McIntire, Sandra J Shin
Myofibroblastic, fibroblastic and/or myoid lesions are rare in the breast but comprise the majority of mammary mesenchymal spindle cell lesions. Whereas most have similar features to their counterparts at extramammary sites, pseudoangiomatous stromal hyperplasia is considered a breast-specific myofibroblastic proliferation on the same spectrum as myofibroblastoma. Other lesions with myofibroblastic/fibroblastic differentiation include fibromatosis and nodular fasciitis, as well as more aggressive tumors such as the rarely reported myofibrosarcoma, inflammatory myofibroblastic tumor and fibrosarcoma...
May 28, 2017: Seminars in Diagnostic Pathology
https://www.readbyqxmd.com/read/28695825/tsc2-disruption-in-mesenchymal-progenitors-results-in-tumors-with-vascular-anomalies-overexpressing-lgals3
#6
Peter J Klover, Rajesh L Thangapazham, Jiro Kato, Ji-An Wang, Stasia A Anderson, Victoria Hoffmann, Wendy K Steagall, Shaowei Li, Elizabeth McCart, Neera Nathan, Joshua D Bernstock, Matthew D Wilkerson, Clifton L Dalgard, Joel Moss, Thomas N Darling
Increased mTORC1 signaling from TSC1/TSC2 inactivation is found in cancer and causes tuberous sclerosis complex (TSC). The role of mesenchymal-derived cells in TSC tumorigenesis was investigated through disruption of Tsc2 in craniofacial and limb bud mesenchymal progenitors. Tsc2cKO(Prrx1-cre) mice had shortened lifespans and extensive hamartomas containing abnormal tortuous, dilated vessels prominent in the forelimbs. Abnormalities were blocked by the mTORC1 inhibitor sirolimus. A Tsc2/mTORC1 expression signature identified in Tsc2-deficient fibroblasts was also increased in bladder cancers with TSC1/TSC2 mutations in the TCGA database...
July 11, 2017: ELife
https://www.readbyqxmd.com/read/28660814/perianal-rhabdomyomatous-mesenchymal-hamartoma
#7
K Sampat, E Cheesman, S Siminas
A rhabdomyomatous mesenchymal hamartoma (RMH) is a rare benign soft tissue tumour found in the face and neck region of children. A three-month-old male infant was referred to our unit with a polypoid anal lesion that had been present from birth. Histopathological examination of the excised sample showed haphazard arrangement of mature striated skeletal muscle in among nerves and blood vessels, and immunohistochemistry confirmed the lesion to be a RMH. There was no evidence of recurrence during the follow-up period...
July 2017: Annals of the Royal College of Surgeons of England
https://www.readbyqxmd.com/read/28632950/fibroblastic-connective-tissue-nevus-clinicopathological-and-immunohistochemical-study-of-14-cases
#8
Ilaria Pennacchia, Heinz Kutzner, Dmitry V Kazakov, Thomas Mentzel
BACKGROUND: We present herein a series of 14 lesions showing overlapping features with the newly defined benign cutaneous mesenchymal neoplasm labeled as fibroblastic connective tissue nevus (FCTN). METHODS AND RESULTS: Total of 8 patients were male and 5 were female, ranging in age from 1 to 56 years. Lesions appeared as isolated nodules or plaques on the trunk (7 cases), the limbs (4 cases) and the neck (2 cases). Histologically, all cases were composed of bundles of bland spindle cells of fibroblastic/myofibroblastic lineage irregularly branching within the reticular dermis and along fibrous septa in the subcutis...
June 20, 2017: Journal of Cutaneous Pathology
https://www.readbyqxmd.com/read/28580399/does-pamidronate-enhance-the-osteogenesis-in-mesenchymal-stem-cells-derived-from-fibrous-hamartoma-in-congenital-pseudarthrosis-of-the-tibia
#9
Vrisha Madhuri, Smitha Elizabeth Mathew, Karthikeyan Rajagopal, Sowmya Ramesh, B Antonisamy
Neurofibromatosis type 1 (NF1) is a commonly occurring genetic disorder in children. Mutation in the NF1 gene has its implication in poor osteoblastic capabilities. We hypothesised that pamidronate will enhance the osteoblastic potential of the mesenchymal stem cells (MSCs) derived from lipofibromatosis tissue of children with congenital pseudarthrosis tibia (CPT) associated with NF1. In this study, bone marrow MSCs (BM MSCs) and CPT MSCs were obtained from three patients undergoing salvage surgeries/bone grafting (healthy controls) and those undergoing excision of the hamartoma and corrective surgeries respectively...
December 2016: Bone Reports
https://www.readbyqxmd.com/read/28560148/mesenchymal-cystic-hamartoma-of-the-lung
#10
Adebayo Ayodeji Fasanya, Yousef Hattab, Ami Patel, Mark Lega
Mesenchymal cystic hamartoma, although first reported as early as 1980s, remains a very rare lung disease. There have been less than 20 cases reported to date. Mesenchymal cystic hamartoma usually has an indolent course, but it could potentially result in morbidity and mortality. Biopsy is needed to confirm the diagnosis because it is essential to rule out other possibilities including malignancy.
2017: Respiratory Medicine Case Reports
https://www.readbyqxmd.com/read/28549884/glomus-tumors-of-the-upper-limb-single-center-retrospective-study-of-clinical-and-functional-outcomes
#11
K Hufschmidt, R Foissac, O Camuzard, N Cardot-Leccia, B Chignon-Sicard, T Balaguer
Glomus tumors are rare and benign hamartomas, arising from neuro-myo-arterial proliferation and deriving from mesenchymal origin. As they have a long-term impact on the individual's quality of life, the primary complaint is unbearable pain. The aim of this study was to assess the clinical and functional outcomes of their surgical treatment, and to review their clinical, radiological and therapeutic features. We performed a retrospective study over a 16-year period including 31 patients with an upper limb glomus tumor...
May 23, 2017: Hand Surgery and Rehabilitation
https://www.readbyqxmd.com/read/28543313/congenital-atrophic-plaque-fibroblastic-connective-tissue-nevus
#12
Luis Jesús Allemant Ortiz, Ximena Calderón-Castrat, Alex Orellana Cortez, Beatriz Ingar Carbone, Angel Santos-Briz
Fibroblastic connective tissue nevus (FCTN) is a rare, benign, dermal mesenchymal hamartoma that affects children. We report a 15-year-old boy with a congenital FCTN and describe the clinical, dermatoscopic, and histopathologic features.
May 23, 2017: Pediatric Dermatology
https://www.readbyqxmd.com/read/28468559/pediatric-mesenchymal-hamartomas-of-the-liver-can-show-both-foregut-and-hindgut-phenotype
#13
Hao Wu, William Ferguson, Eumenia Castro, Milton Finegold, Kalyani Patel
Cystic epithelium in mesenchymal hamartoma (MH) is typically biliary type. Mucinous differentiation of the epithelium and increased hepatocellular component may pose a diagnostic challenge. We studied MH in 7 children (6 M, 1 F; age 4 months to 8 years, median 1 year). Resected tumors varied from 3.0 to 17.0 cm. All tumors showed biliary epithelium in the cystic component with strong and diffuse reactivity for CK7 and CK19. Expression of CK20 and CDX2 was additionally seen in 2 tumors, rare/focal in 1, and diffuse with mucinous differentiation in the other...
January 1, 2017: Pediatric and Developmental Pathology
https://www.readbyqxmd.com/read/28419575/prepubertal-vulvar-fibroma-a-rare-entity-little-known-to-dermatologists-report-of-two-cases
#14
S Nguyen, S Fraitag, N Cardot-Leccia, S Lagrange, C Trastour, T Passeron, S Croce, J M Coindre, J P Lacour, H Montaudié
Prepubertal vulvar tumors are rare and may represent mesenchymal tumors, hamartomas, or vascular malformations. Their presentation may be similar but they do not have the same prognosis and management. We report 2 cases of labium majus hypertrophy corresponding to prepubertal vulvar fibromas. This article is protected by copyright. All rights reserved.
April 17, 2017: Journal of the European Academy of Dermatology and Venereology: JEADV
https://www.readbyqxmd.com/read/28391267/a-clinical-and-radiological-approach-to-the-management-of-benign-mesenchymal-sinonasal-tumors
#15
Lukas Anschuetz, Melanie Buchwalder, Matthias Dettmer, Marco D Caversaccio, Franca Wagner
PURPOSE: Benign mesenchymal sinonasal neoplasms (BMSN) are rare and histologically heterogeneous. Differential diagnosis, appropriate management, and outcome are still a matter of debate. The aim of this study is to provide evidence for further refinement of assessment and treatment in the future. PROCEDURES: We retrospectively reviewed data on 93 patients with neuroradiologically verified BMSN treated at our university reference center during the past 22 years...
2017: ORL; Journal for Oto-rhino-laryngology and its related Specialties
https://www.readbyqxmd.com/read/28298740/hepatoblastoma-with-solid-and-multicystic-aspect-mimicking-a-mesenchymal-hamartoma-imaging-and-anatomopathologic-findings
#16
Pedro Vinícius Staziaki, Bernardo Corrêa de Almeida Teixeira, Bruno Mauricio Pedrazzani, Elizabeth Schneider Gugelmin, Mauricio Zapparolli
No abstract text is available yet for this article.
January 2017: Radiologia Brasileira
https://www.readbyqxmd.com/read/28144858/a-case-of-adult-undifferentiated-embryonal-sarcoma-of-the-liver-successfully-treated-with-right-trisectionectomy-a-case-report
#17
Akiko Mori, Koji Fukase, Kunihiro Masuda, Naoaki Sakata, Masamichi Mizuma, Hideo Ohtsuka, Takanori Morikawa, Kei Nakagawa, Hiroki Hayashi, Fuyuhiko Motoi, Takeshi Naitoh, Keigo Murakami, Michiaki Unno
BACKGROUND: Undifferentiated embryonal sarcoma of the liver (UESL) is a rare malignant mesenchymal tumor that usually occurs in children and is rarely diagnosed in adults. CASE PRESENTATION: Here, we describe the case of a 65-year-old woman who presented with a huge cystic lesion in the liver. Laboratory studies performed on admission showed modest inflammation, poor nutrition, and elevated levels of total bilirubin, alkaline phosphatase, and γ-glutamyl transferase...
December 2017: Surgical Case Reports
https://www.readbyqxmd.com/read/28109716/primary-mesenchymal-liver-tumors-of-childhood
#18
Omer Saeed, Romil Saxena
Primary mesenchymal tumors of the liver represent a significant proportion of liver tumors among neonates, infants and young children. They may be discovered incidentally or present with an alarmingly large, rapidly growing abdominal mass. One third of pediatric liver tumors are benign with hemangioma and mesenchymal hamartoma being the commonest in that order. Infantile hemangioendothelioma and mesenchymal hamartoma represent the commonest liver tumors among neonates and infants. Mesenchymal hamartoma may even present in utero...
March 2017: Seminars in Diagnostic Pathology
https://www.readbyqxmd.com/read/28070642/distinct-expression-profile-of-stem-cell-markers-lgr5-and-lgr6-in-basaloid-skin-tumors
#19
Bo Gun Jang, Cheol Lee, Hye Sung Kim, Myung Soo Shin, Min Seok Cheon, Jae Wang Kim, Woo Ho Kim
Mammalian epidermis, which is composed of hair follicles, sebaceous glands, and interfollicular epidermis, is maintained by discrete stem cells. In vivo lineage tracing demonstrated that murine LGR5 cells are mainly responsible for hair follicle regeneration whereas LGR6 cells generate sebaceous glands and interfollicular epidermis. However, little is known about their expression in the human skin tumors. In this study, we investigated the expression profile of LGR5 and LGR6 in a variety of human skin tumors including basaloid tumors with follicular differentiation (94 basal cell carcinomas, 18 trichoepitheliomas, 3 basaloid follicular hamartomas, and 12 pilomatricomas) and tumors with ductal differentiation (7 eccrine poromas, 8 hidradenomas, and 5 spiradenomas)...
March 2017: Virchows Archiv: An International Journal of Pathology
https://www.readbyqxmd.com/read/28059097/fibrous-hamartoma-of-infancy-a-clinicopathologic-study-of-145-cases-including-2-with-sarcomatous-features
#20
Alyaa Al-Ibraheemi, Anthony Martinez, Sharon W Weiss, Harry P Kozakewich, Antonio R Perez-Atayde, Henry Tran, David M Parham, William R Sukov, Karen J Fritchie, Andrew L Folpe
Fibrous hamartoma of infancy is a rare soft tissue lesion of infants and young children with characteristic triphasic morphology, which typically occurs in the axilla and less commonly in other locations. We reviewed 145 cases of fibrous hamartoma of infancy from our consultation archives. Cases occurred in 106 males and 39 females (mean age-15 months; range-birth to 14 years), and involved both typical sites (eg, axilla/back/upper arm) (n=69) and unusual locations (n=76). Six were congenital. The tumors presented as subcutaneous masses and ranged from 0...
April 2017: Modern Pathology: An Official Journal of the United States and Canadian Academy of Pathology, Inc
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