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Pecomas

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https://www.readbyqxmd.com/read/28584685/renal-epithelioid-angiomyolipoma-associated-with-pulmonary-lymphangioleiomyomatosis-imaging-findings
#1
Athina C Tsili, Alexandra Ntorkou, Maria I Argyropoulou
Renal angiomyolipomas (AMLs) and pulmonary lymphangioleiomyomatosis (LAM) are the most common tumors of the perivascular epithelioid cell (PEComa) family. Both may be associated with tuberous sclerosis (TS) complex. Epithelioid AML (EAML) is a rare variety of AMLs, with a potential aggressive behavior. There are few reports in the English literature addressing on the imaging findings of renal EAMLs, which are considered nonspecific. We present the sonographic, computed tomographic, and magnetic resonance imaging findings of a renal EAML in a pregnant woman with concomitant pulmonary lesions indicative of LAM, without stigmata of TS...
2017: Journal of Clinical Imaging Science
https://www.readbyqxmd.com/read/28562565/perivascular-epithelial-cell-tumor-pecoma-of-the-pancreas-a-case-report-and-review-of-literature
#2
Shuisheng Zhang, Fang Chen, Xiaozhun Huang, Qinglong Jiang, Yajie Zhao, Yingtai Chen, Jianwei Zhang, Jie Ma, Wei Yuan, Quan Xu, Jiuda Zhao, Chengfeng Wang
RATIONALE: Perivascular epithelial cell tumors (PEComas) of the pancreas are rare mesenchymal tumors and, to our knowledge, only 20 cases have been reported to date. PATIENT CONCERNS: We report a 43-year-old female who presented with upper abdominal pain for 1 year. She underwent an exploratory laparotomy at a local hospital, which failed to resect the tumor. Five months later, she came to the Chinese National Cancer Center for surgery. Preoperative imaging revealed an 11...
June 2017: Medicine (Baltimore)
https://www.readbyqxmd.com/read/28562547/hepatic-perivascular-epithelioid-cell-tumor-treated-by-transarterial-embolization-plus-radiofrequency-ablation-a-case-report-and-literature-review
#3
Haitao Guan, Yinghua Zou, Yongxing Lv, Chao Wang
BACKGROUND: Perivascular epithelioid cell tumors (PEComas) are extremely rare mesenchymal entities with potentially malignant properties; the liver cases are not encountered frequently. Owing to themalignant potential, these tumors are treated by surgical methods to ensure total resection. In the present report, a case of liver PEComa treated by embolization combined with radiofrequency ablation (RFA) has been described. CASE SUMMARY: A 40-year-old female was admitted for the detection of a liver mass during an annual physical examination...
June 2017: Medicine (Baltimore)
https://www.readbyqxmd.com/read/28556973/primary-cutaneous-perivascular-epithelioid-cell-tumor-pecoma-five-new-cases-and-review-of-the-literature
#4
Lauren N Stuart, Russell G Tipton, Michael DeWall, Douglas C Parker, Christina D Stelton, Annie O Morrison, Landon W Coleman, Scott W Fosko, Claudia I Vidal, M Yadira Hurley, Amy H Deeken, Jerad M Gardner
PEComas represent a family of uncommon mesenchymal tumors composed of "perivascular epithelioid cells" with a distinct immunophenotype that typically shows both myogenic and melanocytic differentiation. The PEComa family includes angiomyolipoma (AML), clear cell "sugar" tumor of the lung and extra pulmonary sites, lymphangioleiomyomatosis and clear cell myomelanocytic tumor of the falciform ligament/ligamentum teres. Very rarely, PEComas may arise in the skin. Primary cutaneous PEComas typically display a dermal proliferation of epithelioid cells with pale, clear, or granular pink cytoplasm arranged in nests and trabeculae with an intervening arborizing network of delicate capillaries...
May 30, 2017: Journal of Cutaneous Pathology
https://www.readbyqxmd.com/read/28547987/perirenal-perivascular-epithelioid-cell-tumor-pecoma-coexisting-with-other-malignancies-a-case-report
#5
Marian Danilewicz, Janusz M Strzelczyk, Małgorzata Wagrowska-Danilewicz
Perivascular epithelioid cell tumor (PEComa) is a very rare lesion and is described by the World Health Organization (WHO) as a mesenchymal tumor composed of histologically and immunohistochemically distinctive perivascular epithelioid cells. In this report we describe PEComa with perirenal manifestation, which is exceedingly rare and to our best knowledge up to now worldwide only three cases have been described. Despite the reports that most PEComas are benign, this tumor met criteria for malignancy and coexisted with mucinous gallbladder cancer and nonresectable pancreatic head tumor...
2017: Polish Journal of Pathology: Official Journal of the Polish Society of Pathologists
https://www.readbyqxmd.com/read/28459168/malignant-pecoma-with-metastatic-disease-at-diagnosis-and-resistance-to-several-chemotherapy-regimens-and-targeted-therapy-m-tor-inhibitor
#6
Isidro Machado, Julia Cruz, Javier Lavernia, José M Rayon, Andrés Poveda, Antonio Llombart-Bosch
Perivascular epithelioid cell tumors (PEComas) are infrequent neoplasms with peculiar myomelanocytic differentiation. The aggressive abdominopelvic variant is rare, with only a small number of published cases. We present an additional case of this unusual variant, which showed an aggressive histologic and clinical behavior with multiple liver metastases and resistance to several therapies. We also discuss the histological and immunohistochemical profiles as well as the differential diagnosis.
April 1, 2017: International Journal of Surgical Pathology
https://www.readbyqxmd.com/read/28415905/cardiac-perivascular-epithelioid-cell-tumor
#7
Sanjay Rao, P Pavithra, Sunil Bhat, Colin John, Ashley J D'Cruz
Cardiac tumors presenting as mediastinal masses in childhood are rare. This report describes the case of a 6-year-old girl who presented to us after a failed attempt at resection of a mass arising from the left atrial appendage. Computed tomography-guided biopsy suggested a perivascular epithelioid cell tumor (PEComa). Sirolimus was started in order to reduce the mass size. She subsequently underwent resection of the mass by midline sternotomy approach and cardiopulmonary bypass. Recovery was uneventful and the child is doing well at two-year follow-up...
January 1, 2017: World Journal for Pediatric & Congenital Heart Surgery
https://www.readbyqxmd.com/read/28413654/hepatic-perivascular-epithelioid-cell-neoplasm-a-clinical-and-pathological-experience-in-diagnosis-and-treatment
#8
Wenying Chen, Yeqing Liu, Yanyan Zhuang, Juanfei Peng, Fengting Huang, Shineng Zhang
Hepatic perivascular epithelioid cell neoplasm (PEComa) is a rare type of neoplasm derived from mesenchymal tumors that is often misdiagnosed as hepatocellular carcinoma (HCC), hepatic hemangioma or other liver malignancies. The clinical and histological characteristics of PEComa have yet to be fully documented. To optimize the diagnosis and treatment of the disease, a retrospective analysis was performed to investigate the clinicopathological characteristics of 7 patients diagnosed with hepatic PEComa in the Sun Yat-Sen Memorial Hospital between January 2004 and December 2015...
April 2017: Molecular and Clinical Oncology
https://www.readbyqxmd.com/read/28412212/mucoepidermoid-carcinoma-with-extensive-spindled-morphology-and-melanocytic-marker-expression
#9
Takashi Oide, Kenzo Hiroshima, Yoko Takahashi, Kazunori Fugo, Masanobu Yamatoji, Atsushi Kasamatsu, Yosuke Endo-Sakamoto, Masashi Shiiba, Katsuhiro Uzawa, Hideki Tanzawa, Toshitaka Nagao, Yukio Nakatani
Mucoepidermoid carcinoma (MEC) is the most common malignant neoplasm of the salivary gland. Albeit common, histological variants have rarely been noted in MEC. Here, we report a 49-year-old man with a sublingual gland tumor. Histologically, the tumor was composed of spindle cells arranged in interlacing fascicules or globular nests. A few bland small glands containing mucous cells were also scattered. The spindle tumor cells completely lacked immunoreactivity for cytokeratin, and exhibited immunoreactivity for vimentin, S-100, HMB-45, Melan A, and SOX10...
April 12, 2017: Human Pathology
https://www.readbyqxmd.com/read/28372349/perivascular-epithelioid-cell-tumor-in-the-stomach
#10
Sun Ah Shin, Jiwoon Choi, Kyung Chul Moon, Woo Ho Kim
Perivascular epithelioid cell tumors or PEComas can arise in any location in the body. However, a limited number of cases of gastric PEComa have been reported. We present two cases of gastric PEComas. The first case involved a 62-year-old woman who presented with a 4.2 cm gastric subepithelial mass in the prepyloric antrum, and the second case involved a 67-year-old man with a 5.0 cm mass slightly below the gastroesophageal junction. Microscopic examination revealed that both tumors were composed of perivascular epithelioid cells that were immunoreactive for melanocytic and smooth muscle markers...
April 4, 2017: Journal of Pathology and Translational Medicine
https://www.readbyqxmd.com/read/28359636/hepatic-perivascular-epithelioid-tumor-pecoma-a-case-report
#11
Hélder Cardoso, Marco Silva, Filipe Vilas-Boas, Rui Cunha, Joanne Lopes, José Costa Maia, Guilherme Macedo
A 37-year-old female had liver nodules found in an abdominal ultrasound scan. The radiological features were suggestive of hepatocellular adenomas, although there were some atypical findings. Two years later, one of the nodules showed dimensional progression and intralesional hemorrhage. The patient underwent a left hepatectomy and the postoperative course was uneventful. The histological exam and the immunohistochemistry were consistent with the diagnosis of PEComa. The PEComa is a mesenchymal tumor rarely described in the liver...
March 27, 2017: Clinics and Research in Hepatology and Gastroenterology
https://www.readbyqxmd.com/read/28318774/-epithelioid-angiomyolipoma-of-the-kidney-about-one-case-and-malignant-features-evaluation
#12
Pierre-Marie Lavrut, Philippe Paparel, Myriam Decaussin-Petrucci
Renal epithelioid angiomyolipoma (E-AML) is a rare mesenchymal tumor of the kidney included in the family of tumor with perivascular epithelioid cell differentiation (PEComas) and is frequently associated with tuberous sclerosis complex. Since its clinical and radiological features are not specific, the diagnosis remained mostly pathological. Microscopically, E-AML demonstrate proliferation of more than 80% of epithelioid cells with atypia, often associated with necrosis, hemorrhage, mitotic activity and vascular invasion...
April 2017: Annales de Pathologie
https://www.readbyqxmd.com/read/28315422/sfpq-psf-tfe3-renal-cell-carcinoma-rcc-a-clinicopathologic-study-emphasizing-extended-morphology-and-reviewing-the-differences-between-sfpq-tfe3-rcc-and-the-corresponding-mesenchymal-neoplasm-despite-an-identical-gene-fusion
#13
Xiao-Tong Wang, Qiu-Yuan Xia, Hao Ni, Sheng-Bing Ye, Rui Li, Xuan Wang, Shan-Shan Shi, Xiao-Jun Zhou, Qiu Rao
Xp11 translocation renal cell carcinoma (RCC) with SFPQ/PSF-TFE3 gene fusion is a rare epithelial tumor. Of note, the appearance of the gene fusion does not necessarily mean that it is renal cell carcinoma. The corresponding mesenchymal neoplasms, including Xp11 neoplasm with melanocytic differentiation, TFE3 rearrangement-associated perivascular epithelioid cell tumor (PEComa) and melanotic Xp11 translocation renal cancer, can also harbor the identical gene fusion. However, the differences between Xp11 translocation RCC and the corresponding mesenchymal neoplasm have only recently been described...
March 14, 2017: Human Pathology
https://www.readbyqxmd.com/read/28288506/hepatic-perivascular-epithelioid-cell-tumor-pecoma-a-case-report-with-a-review-of-literatures
#14
REVIEW
Hyun-Jin Son, Dong Wook Kang, Joo Heon Kim, Hyun Young Han, Min Koo Lee
Hepatic perivascular epithelioid cell tumors (PEComas) are very rare. We report a primary hepatic PEComa with a review of the literature. A 56-year-old women presented with a nodular mass detected during the management of chronic renal failure and chronic hepatitis C. Diagnostic imaging studies suggested a nodular hepatocellular carcinoma in segment 5 of the liver. The patient underwent partial hepatectomy. A brown-colored expansile mass measuring 3.2×3.0 cm was relatively demarcated from the surrounding liver parenchyma...
March 2017: Clinical and Molecular Hepatology
https://www.readbyqxmd.com/read/28285963/clear-cell-colorectal-carcinoma-time-to-clarify-diagnosis
#15
Andrea Remo, Federica Grillo, Luca Mastracci, Matteo Fassan, Sokol Sina, Caterina Zanella, Pietro Parcesepe, Emanuele Damiano Urso, Massimo Pancione, Germana Bortuzzo, Aldo Scarpa, Erminia Manfrin
Primary clear cell colorectal carcinoma (CCC) is a very rare entity accounting for only 35 cases reported in the Literature. CCC is neither classified as a distinct entity nor is it defined as a CRC variant because its ontogeny remains unclear. Most of the reported CCC were found in the distal colon in patients with a mean age of 56 years. Histologically, clear cell change is the main morphologic feature and may present in a "pure" form, composed exclusively of clear cells, or in a "composite" form, admixed with other morphologically different components...
May 2017: Pathology, Research and Practice
https://www.readbyqxmd.com/read/28270196/two-cases-of-perivascular-epithelioid-cell-tumor-of-the-uterus-clinical-radiological-and-pathological-diagnostic-challenge
#16
Byung Su Kwon, Dong Soo Suh, Nam Kyung Lee, Yong Jung Song, Kyung Un Choi, Ki Hyung Kim
BACKGROUND: Perivascular epithelioid cell tumor (PEComa) is a rare subtype of mesenchymal origin tumor composed of epithelioid cells which exhibits immunohistochemical co-expressions of melanocytic markers and smooth muscle markers. CASE PRESENTATION: In the first case, malignant uterine PEComa with vaginal and multiple lung metastasis was misdiagnosed preoperatively as uterine leiomyosarcoma despite a preoperative punch biopsy and immunohistochemical analysis of the metastatic vaginal mass...
March 7, 2017: European Journal of Medical Research
https://www.readbyqxmd.com/read/28246920/primary-perivascular-epithelioid-cell-tumors-of-the-liver-ct-mri-findings-and-clinical-outcomes
#17
Martin E O'Malley, Tanya P Chawla, Lisa P Lavelle, Sean Cleary, Sandra Fischer
OBJECTIVES: The purpose of our study was to describe the CT and MRI features of primary PEComas of the liver and to document the associated clinical outcomes. METHODS: Retrospective study included 20 patients with primary hepatic perivascular epithelioid cell tumors (PEComa) with pathology and clinical outcomes for correlation. RESULTS: Study group included 20 patients: 16 women, 4 men; mean age 53 (range 35-77) years. Initial pathology diagnoses were classic angiomyolipoma (AML) (n = 11), epithelioid AML (n = 7), and PEComa not otherwise specified (n = 2)...
February 28, 2017: Abdominal Radiology
https://www.readbyqxmd.com/read/28105182/long-lasting-stable-disease-with-mtor-inhibitor-treatment-in-a-patient-with-a-perivascular-epithelioid-cell-tumor-a-case-report-and-literature-review
#18
Ezequiel Flechter, Yaniv Zohar, Ludmila Guralnik, Maria Passhak, Gil Bar Sela
Perivascular epithelioid cell tumor (PEComa) of the small intestine is extremely rare, and there is no established treatment at the present time. In 10% of patients with PEComas, genetic alterations of tuberous sclerosis complex have been reported. These genetic alterations activate mechanistic target of rapamycin (mTOR) in AMP-activated protein kinase and Ras/mitogen-activated protein kinase pathways, resulting in high mTOR activity. Since 2007, several cases of treatment with mTOR inhibitors in advanced PEComa have been reported...
December 2016: Oncology Letters
https://www.readbyqxmd.com/read/28063419/perivascular-epithelioid-cell-tumors-pecomas-of-the-orbit
#19
Panagiotis Paliogiannis, Giuseppe Palmieri, Francesco Tanda, Antonio Cossu
No abstract text is available yet for this article.
January 2017: Journal of Pathology and Translational Medicine
https://www.readbyqxmd.com/read/28053374/ang-1-and-ang-2-expression-in-angiomyolipoma-and-pecoma-family-tumors
#20
Swati Shrestha, Carolyn Meyers, Jia Shen, Paulina Giacomelli, Michelle A Scott, Chia Soo, Sarah M Dry, Kang Ting, Aaron W James
OBJECTIVE: Perivascular epithelioid cell tumors (PEComa) are an uncommon family of soft tissue tumors. Previously, we described that the presence of pericyte antigens among PEComa family tumors differs extensively by histologic appearance. METHODS: Here, we extend our findings using the pericyte antigens Angiopoietin-1 (Ang-1) and Angiopoietin-2 (Ang-2), using immunohistochemical detection in human tumor samples. RESULTS: While Ang-1 showed no expression across any PEComa family tumor, Ang-2 showed expression that like other pericyte markers was largely determined by cytologic appearance...
March 2017: Journal of Orthopaedics
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