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Mesenchymal hamartoma

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https://www.readbyqxmd.com/read/28632950/fibroblastic-connective-tissue-nevus-clinicopathological-and-immunohistochemical-study-of-14-cases
#1
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 labelled as fibroblastic connective tissue nevus (FCTN). METHODS AND RESULTS: Eight patients were male and five 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
#2
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
#3
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
#4
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
#5
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
#6
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
#7
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
#8
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
#9
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
#10
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
#11
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
#12
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
#13
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
https://www.readbyqxmd.com/read/28002331/hepatic-perivascular-epithelioid-cell-tumor-case-report-and-brief-literature-review
#14
REVIEW
Da Tang, Jianmin Wang, Yuepeng Tian, Qiuguo Li, Haixiong Yan, Biao Wang, Li Xiong, Qinglong Li
RATIONAL: Perivascular epithelioid cell tumor (PEComa) is a rare mesenchymal neoplasm which expresses both myogenic and melanocytic markers. PEComas are found in a variety locations in the body, but up to now only approximately 30 cases about hepatic perivascular epithelioid cell tumor are reported in English language worldwide. PATIENT CONCERNS: A 32-year-old woman was admitted in our hospital with intermittent right upper quadrant pain for 1 month and recent (1 day) progressive deterioration...
December 2016: Medicine (Baltimore)
https://www.readbyqxmd.com/read/27994306/a-rare-case-of-hamartoma-chest-wall-following-trauma-in-a-42-year-old-man
#15
Mojtaba Ahmadinejad, Asghar Alie Pour, Peyman Khadem Hosseini, Amir Masoud Hashemian, Koorosh Ahmadi
BACKGROUND: Chest wall mesenchymal hamartoma (CWH) is a distinct and extremely rare tumor-like lesion of the thorax. It usually presents in the neonatal period or in infancy. The common presentation is in the form of a visible chest wall mass with or without respiratory distress. CASE PRESENTATION: A 42-year-old man with a history of chest wall trauma since 5 years ago was admitted with a swelling of the anterior of the chest wall and during this period has grown slowly...
October 2016: Medical Archives
https://www.readbyqxmd.com/read/27976564/nasal-chondromesenchymal-hamartoma-a-rare-pediatric-tumor-case-report
#16
Adnan Ünal, Rauf Oğuzhan Kum, Yonca Avcı, Devrim Tuba Ünal
Nasal chondromesenchymal hamartoma has characteristic clinicopathological features and it is accepted as the upper airway analogue of mesenchymal hamartoma of the chest wall. It is a rare lesion and only 31 cases have been reported in the English literature until 2014. In this article, a 13-year-old nasal chondromesenchymal hamartoma case is presented, which is the first nasal chondromesenchymal hamartoma case from Turkey. Although, nasal chondromesenchymal hamartoma has been accepted as a benign lesion, the possibility of malignant transformation should be kept in mind, and detailed histologic examination should be performed particularly in adult nasal chondromesenchymal hamartoma cases...
2016: Turkish Journal of Pediatrics
https://www.readbyqxmd.com/read/27936889/hamartomas-from-head-to-toe-an-imaging-overview
#17
REVIEW
Francisca Leiter Herrán, Carlos S Restrepo, Daniel I Alvarez Gómez, Thomas Suby-Long, Daniel Ocazionez, Daniel Vargas
Hamartomas are tumours composed of mesenchymal tissues such as cartilage, fat, connective tissue and smooth muscle and can be found in virtually any organ system. These masses commonly develop sporadically, but are also seen in certain syndromes such as tuberous sclerosis or Carney triad. While their imaging appearance varies depending on the organ they arise from, findings are usually unique and a diagnosis can be confidently made. Radiologists must be aware of the clinical and imaging presentations of these lesions with the particular goal of avoiding unnecessary studies or invasive procedures...
March 2017: British Journal of Radiology
https://www.readbyqxmd.com/read/27917338/six-different-extremely-calcified-lesions-of-the-brain-brain-stones
#18
Yurdal Gezercan, Vedat Acik, Gökhan Çavuş, Ali Ihsan Ökten, Emre Bilgin, Hakan Millet, Burak Olmaz
BACKGROUND: This study aimed to extend clinical documentation of cerebral calculi by reporting six cases of cerebral calculi with distinct etiologies and localizations. METHODS: We evaluated the age, sex distribution, presenting symptoms, neurological examination findings, pathology results, and location of the calcifications of six patients with intracranial calcifications. RESULTS: Three of the six patients with brain stones were female (50%), and three were male (50%)...
2016: SpringerPlus
https://www.readbyqxmd.com/read/27888828/nasal-chondromesenchymal-hamartoma-a-rare-nasal-benign-tumor
#19
Hakan Avcı, Şenol Çomoğlu, Erkan Öztürk, Bilge Bilgiç, Ökkeş Erkan Kıyak
Nasal chondromesenchymal hamartoma (NCMH) is a rare nasal benign tumor, which arises from the nasal cavity or paranasal sinuses. In this article, we present a five-year-old male patient with rhabdomyosarcoma unity in remission that emerged with nasal obstruction. Synchronous diagnosis of pediatric tumors such as pleuropulmonary blastoma in the literature is a remarkable finding. We found a mass within the left nasal cavity originating from superior portion of nasal septum, extending to the olfactory cleft and resected all tumor via endoscopic surgical approach...
September 2016: Kulak Burun Boğaz Ihtisas Dergisi: KBB, Journal of Ear, Nose, and Throat
https://www.readbyqxmd.com/read/27833980/mesenchymal-hamartoma-of-the-left-liver-lobe-in-an-18-month-old-female-patient
#20
Emil Radu Iacob, Călin Marius Popoiu, Alexandra Nyiredi, LaurenŢiu Mogoantă, Oana Badea, Eugen Sorin Boia
Mesenchymal hamartoma of the liver (MHL) is a benign and rare hepatic lesion, with an uncertain etiology and a potential for developing into an undifferentiated distant embryonal sarcoma after an incomplete resection. It mainly presents as progressive abdominal distension with normal blood works. Most cases are diagnosed in the first two years of life, with a higher frequency in boys and on the right liver. We report the case of a mesenchymal hamartoma of the left liver in an 18-month-old girl, with a rough evolution and a literature review...
2016: Romanian Journal of Morphology and Embryology, Revue Roumaine de Morphologie et Embryologie
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