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Mesenchymal hamartoma

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https://www.readbyqxmd.com/read/28144858/a-case-of-adult-undifferentiated-embryonal-sarcoma-of-the-liver-successfully-treated-with-right-trisectionectomy-a-case-report
#1
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
#2
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...
December 23, 2016: Seminars in Diagnostic Pathology
https://www.readbyqxmd.com/read/28070642/distinct-expression-profile-of-stem-cell-markers-lgr5-and-lgr6-in-basaloid-skin-tumors
#3
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)...
January 9, 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
#4
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...
January 6, 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
#5
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
#6
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
#7
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
#8
Francisca Leiter Herrán, Carlos S Restrepo, Daniel I Alvarez Gómez, Thomas Suby-Long, Daniel Ocazionez, Daniel Vargas
Hamartomas are tumors 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 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...
December 12, 2016: British Journal of Radiology
https://www.readbyqxmd.com/read/27917338/six-different-extremely-calcified-lesions-of-the-brain-brain-stones
#9
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
#10
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
#11
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
https://www.readbyqxmd.com/read/27721599/expression-of-ck14-and-vimentin-in-adenomatoid-odontogenic-tumor-and-dentigerous-cyst
#12
Muniswamappa Sudhakara, S Puranik Rudrayya, Srineevas S Vanaki, RamanPreet Kaur Bhullar, M S Shivakumar, Mahadevi Hosur
BACKGROUND: Origin of adenomatoid odontogenic tumor (AOT) has long been a controversy, and the issue of it being a neoplasm or hamartoma was a subject of debate for a long time. Earlier it was grouped under a mixed group of odontogenic tumors considering the varying degrees of inductive changes. Recently, the WHO classification states that the presence of hard tissue within AOT was not due to induction but was rather a metaplastically produced mineralization and hence the tumor was reclassified under a group of tumors arising from odontogenic epithelium...
September 2016: Journal of Oral and Maxillofacial Pathology: JOMFP
https://www.readbyqxmd.com/read/27691780/torsion-of-a-mesenchymal-hamartoma-of-the-liver-in-a-1-year-old-boy-case-report
#13
Marko Bašković, Maja Đinkić, Božidar Župančić, Jasminka Stepan, Lucija Čizmić
We report a case of a one-year-old boy who was referred to our clinic suspected of having acute abdomen. On physical examination, the abdomen was soft, diffusely tender with weak peristalsis. Ultrasonography and MRI of the right hemiabdomen demonstrated a well-defined, solid, expansive formation with slightly lobulated contours and an interspersed inhomogeneous structure with overall dimensions of 59 × 45 × 50 mm. After midline laparotomy was performed, a cystic tumor was found, twisted around a pedicle which was arising from the falciform ligament and it measured 5-6 cm in diameter...
October 3, 2016: Acta Chirurgica Belgica
https://www.readbyqxmd.com/read/27684838/uncommon-liver-tumors-case-report-and-literature-review
#14
REVIEW
Chia-Hung Wu, Nai-Chi Chiu, Yi-Chen Yeh, Yu Kuo, Sz-Shian Yu, Ching-Yao Weng, Chien-An Liu, Yi-Hong Chou, Yi-You Chiou
BACKGROUND: Beside hepatocellular carcinoma, metastasis, and cholangiocarcinoma, the imaging findings of other relatively uncommon hepatic lesions are less discussed in the literature. Imaging diagnosis of these lesions is a daily challenge. In this article, we review the imaging characteristics of these neoplasms. METHODS: From January 2003 to December 2014, 4746 patients underwent liver biopsy or hepatic surgical resection in our hospital. We reviewed the pathological database retrospectively...
September 2016: Medicine (Baltimore)
https://www.readbyqxmd.com/read/27631514/egfr-exon-20-insertion-duplication-mutations-characterize-fibrous-hamartoma-of-infancy
#15
Jason Y Park, Cynthia Cohen, Dania Lopez, Erica Ramos, Jennifer Wagenfuehr, Dinesh Rakheja
Fibrous hamartoma of infancy (FHI) is a benign mesenchymal tumor histologically characterized by a mixture of intersecting fascicles of fibroblasts/myofibroblasts in collagenous stroma, nests of primitive oval or stellate cells in basophilic mucoid stroma, and mature adipose tissue. We hypothesized that FHI, because of histologic overlap with mesenchymal overgrowth tumors seen in CLOVES (Congenital Lipomatous Overgrowth with Vascular, Epidermal, Skeletal anomalies) and Proteus syndromes, may harbor mutations in signaling pathways associated with cellular proliferation...
September 14, 2016: American Journal of Surgical Pathology
https://www.readbyqxmd.com/read/27610342/history-of-the-infantile-hepatic-hemangioma-from-imaging-to-generating-a-differential-diagnosis
#16
REVIEW
Maria Gnarra, Gerald Behr, Alison Kitajewski, June K Wu, Sudha A Anupindi, Carrie J Shawber, Nick Zavras, Dimitrios Schizas, Chris Salakos, Konstantinos P Economopoulos
We aim to provide an up-to-date summary of infantile hepatic hemangioma (IHH) and its misnomers and to dialectically present the differential diagnosis of these rare entities of the liver. Eligible peer-reviewed articles on hepatic infantile hemangiomas, published between 2000 and 2015, were reviewed for this study. IHH is the most common hepatic vascular tumor in children. Once a liver mass is identified in an infant, the differential diagnosis ranges from vascular malformations to benign and malignant tumors including mesenchymal hamartoma, hepatoblastoma, metastatic neuroblastoma, so careful physical examination, imaging studies, and, if indicated, tumor markers and biopsy, are of pivotal importance to ascertain the correct diagnosis...
August 8, 2016: World Journal of Clinical Pediatrics
https://www.readbyqxmd.com/read/27526937/mri-of-paediatric-liver-tumours-how-we-review-and-report
#17
REVIEW
Susan C Shelmerdine, Derek J Roebuck, Alexander J Towbin, Kieran McHugh
Liver tumours are fortunately rare in children. Benign tumours such as haemangiomas and cystic mesenchymal hamartomas are typically seen in infancy, often before 6 months of age. After that age, malignant hepatic tumours increase in frequency. The differentiation of a malignant from benign lesion on imaging can often negate the need for biopsy. Ultrasound is currently the main screening tool for suspected liver pathology, and is ideally suited for evaluation of hepatic lesions in children due to their generally small size...
August 15, 2016: Cancer Imaging: the Official Publication of the International Cancer Imaging Society
https://www.readbyqxmd.com/read/27525242/prolonged-air-leakage-caused-by-mesenchymal-cystic-hamartoma-of-the-lung
#18
Young Uk Lee, Jang Hoon Lee, Jong Hyun Baek
A 16-year-old girl was transferred to the department of thoracic and cardiovascular surgery because of a spontaneous pneumothorax with prolonged air leakage. Chest computed tomography demonstrated a cystic lesion measuring 2×3 cm and involving the left upper lobe. Left upper lobectomy was performed via video-assisted thoracoscopic surgery. A pathologic examination of the specimen revealed a mesenchymal cystic hamartoma. Despite the rarity of pulmonary mesenchymal cystic hamartoma, it should be considered a potential cause of pneumothorax for patients with a large pulmonary cyst...
August 2016: Korean Journal of Thoracic and Cardiovascular Surgery
https://www.readbyqxmd.com/read/27516388/catenin-delta-1-ctnnd1-phosphorylation-controls-the-mesenchymal-to-epithelial-transition-in-astrocytic-tumors
#19
Jin Yang, Alexander G Bassuk, Juliane Merl-Pham, Chun-Wei Hsu, Diana F Colgan, Xiaorong Li, Kit Sing Au, Lijuan Zhang, Scott Smemo, Sally Justus, Yasunori Nagahama, Andrew J Grossbach, Matthew A Howard, Hiroto Kawasaki, Neil A Feldstein, William B Dobyns, Hope Northrup, Stefanie M Hauck, Marius Ueffing, Vinit B Mahajan, Stephen H Tsang
Inactivating mutations of the TSC1/TSC2 complex (TSC1/2) cause tuberous sclerosis (TSC), a hereditary syndrome with neurological symptoms and benign hamartoma tumours in the brain. Since TSC effectors are largely unknown in the human brain, TSC patient cortical tubers were used to uncover hyperphosphorylation unique to TSC primary astrocytes, the cell type affected in the brain. We found abnormal hyperphosphorylation of catenin delta-1 S268, which was reversible by mTOR-specific inhibitors. In contrast, in three metastatic astrocytoma cell lines, S268 was under phosphorylated, suggesting S268 phosphorylation controls metastasis...
October 1, 2016: Human Molecular Genetics
https://www.readbyqxmd.com/read/27468623/-liver-transplantation-for-benign-liver-tumor-six-cases-report-and-literature-review
#20
L J Wang, Y M Zhang, Y L Deng, H Zheng, C Pan, Z Y Shen
OBJECTIVE: To analyse and explore the indications and efficacy of liver transplantation for benign liver tumor. METHODS: From Jan.2001 to Dec.2014, 6 patients, incluing 3 males and 3 females, with benign liver tumor underwent liver transplantation in our department.There were 2 cases of hepatic epithelioid hemangioendothelioma, 1 case of hepatic cavernous hemangioma, 1 case of liver mesenchymal hamartoma, 1 case of hilar bile duct mucinous cystadenoma, and 1 case of hepatic adenoma...
July 12, 2016: Zhonghua Yi Xue za Zhi [Chinese medical journal]
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