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https://www.readbyqxmd.com/read/28415905/cardiac-perivascular-epithelioid-cell-tumor
#1
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
#2
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
#3
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
#4
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
#5
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
#6
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
#7
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
#8
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
#9
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...
February 24, 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
#10
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
#11
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
#12
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
#13
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
#14
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
https://www.readbyqxmd.com/read/28009610/alveolar-soft-part-sarcoma-of-the-female-genital-tract-a-morphologic-immunohistochemical-and-molecular-cytogenetic-study-of-10-cases-with-emphasis-on-its-distinction-from-morphologic-mimics
#15
MULTICENTER STUDY
J Kenneth Schoolmeester, Joseph Carlson, Gary L Keeney, Karen J Fritchie, Esther Oliva, Robert H Young, Marisa R Nucci
Alveolar soft part sarcoma (ASPS) is a morphologically distinctive neoplasm of unknown differentiation that bears a characteristic gene fusion involving ASPSCR1 and TFE3. ASPS can occur in the female genital tract, but is rare. Eleven cases with an initial diagnosis of ASPS at female genital tract sites were evaluated for their morphologic features and immunoprofile using a panel of antibodies (TFE3, HMB45, melan-A, smooth muscle actin, desmin, and h-Caldesmon). In addition, the presence of TFE3 rearrangement and subsequent ASPSCR1-TFE3 fusion were determined by fluorescence in situ hybridization...
May 2017: American Journal of Surgical Pathology
https://www.readbyqxmd.com/read/28009605/melanotic-pecoma-of-the-sinonasal-mucosa-with-nono-tfe3-fusion-an-elusive-mimic-of-sinonasal-melanoma
#16
Stephanie M McGregor, Mir B Alikhan, Rahel A John, Howard Kotler, Julia A Bridge, Ibro Mujacic, Sabah Kadri, Jeremy Segal, Thomas Krausz
Perivascular epithelioid cell neoplasms (PEComas) are a family of mesenchymal tumors with features of both smooth muscle and melanocytic differentiation, with or without true melanin pigment. The highly variable morphology of PEComas results in a broad differential diagnosis that is also dependent on anatomic site. A subset demonstrates rearrangements involving the TFE3 (Xp11) locus, which can be used in diagnostically difficult cases. Here we describe a case of a melanotic PEComa with NONO-TFE3 fusion occurring in the sinonasal mucosa, as demonstrated by both next-generation sequencing and molecular cytogenetic studies...
May 2017: American Journal of Surgical Pathology
https://www.readbyqxmd.com/read/28002331/hepatic-perivascular-epithelioid-cell-tumor-case-report-and-brief-literature-review
#17
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/27919857/hepatic-perivascular-epithelioid-cell-tumor-in-three-patients
#18
REVIEW
Bao-Bin Hao, Jian-Hua Rao, Ye Fan, Chuang-Yong Zhang, Xin-Zheng Dai, Xiao Li, Yan Leng, Feng Zhang
Perivascular epithelioid cell tumor (PEComa) is a rare, soft tissue tumor that can occur in various locations. The present report included three patients (one male and two females; age range, 25-51 years) with hepatic PEComas. The collected data included the clinical manifestations, diagnosis, management, treatment, and prognosis. Since it is difficult to diagnose hepatic PEComas by imaging, the patients were diagnosed by tumor tissue examination such as immunohistochemistry, which was positive for HMB-45, Melan-A, and SMA on all slides...
December 2016: Hepatobiliary & Pancreatic Diseases International: HBPD INT
https://www.readbyqxmd.com/read/27895874/computerized-tomography-and-magnetic-resonance-imaging-findings-in-malignant-perivascular-epithelioid-cell-tumors-of-the-ovaries-with-pulmonary-metastasis
#19
Han Yoo-Bee, Shin Yu Ri, Kim Ki Jun, Kim Jiyoung
Perivascular epithelioid cell tumor (PEComa) is a rare mesenchymal tumor composed of immunohistochemically and histologically distinctive perivascular epithelioid cells. Here, we report on the computed tomography (CT) and magnetic resonance imaging (MRI) findings of ovarian PEComa with pulmonary metastasis. The tumor was visible as a multilocular hemorrhagic mass that encased the ovarian vessels. These findings were different to those of other common ovarian tumors.
October 2016: Iranian Journal of Radiology: a Quarterly Journal Published By the Iranian Radiological Society
https://www.readbyqxmd.com/read/27871249/benign-clear-cell-sugar-tumor-of-the-lung-in-a-patient-with-birt-hogg-dub%C3%A3-syndrome-a-case-report
#20
Yoko Gunji-Niitsu, Toshio Kumasaka, Shigehiro Kitamura, Yoshito Hoshika, Takuo Hayashi, Hitoshi Tokuda, Riichiro Morita, Etsuko Kobayashi, Keiko Mitani, Mika Kikkawa, Kazuhisa Takahashi, Kuniaki Seyama
BACKGROUND: Birt-Hogg-Dubé (BHD) syndrome is a rare inherited autosomal genodermatosis and caused by germline mutation of the folliculin (FLCN) gene, a tumor suppressor gene of which protein product is involved in mechanistic target of rapamycin (mTOR) signaling pathway regulating cell growth and metabolism. Clinical manifestations in BHD syndrome is characterized by fibrofolliculomas of the skin, pulmonary cysts with or without spontaneous pneumothorax, and renal neoplasms. There has been no pulmonary neoplasm reported in BHD syndrome, although the condition is due to deleterious sequence variants in a tumor suppressor gene...
November 21, 2016: BMC Medical Genetics
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