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https://www.readbyqxmd.com/read/28063419/perivascular-epithelioid-cell-tumors-pecomas-of-the-orbit
#1
Panagiotis Paliogiannis, Giuseppe Palmieri, Francesco Tanda, Antonio Cossu
No abstract text is available yet for this article.
January 5, 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
#2
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
#3
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...
December 22, 2016: 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
#4
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...
December 22, 2016: American Journal of Surgical Pathology
https://www.readbyqxmd.com/read/28002331/hepatic-perivascular-epithelioid-cell-tumor-case-report-and-brief-literature-review
#5
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
#6
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
#7
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
#8
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
https://www.readbyqxmd.com/read/27865795/hepatic-angiomyolipomas-may-overexpress-tfe3-but-have-no-relevant-genetic-alterations
#9
Naoe Jimbo, Takashi Nishigami, Masayuki Noguchi, Hiroko Iijima, Seiichi Hirota, Takuma Tajiri, Takeshi Inoue, Takanori Hirose, Tomoo Itoh, Yoh Zen
The fusion or amplification of TFE3 has been identified as one of the molecular events underlying tumorigenesis in perivascular epithelioid cell tumors (PEComas). TFE3 rearrangements in PEComas are related to the morphological features of the epithelioid appearance and weaker expression of immunohistochemical muscular markers. This study aimed to clarify whether these genetic alterations are involved in hepatic angiomyolipomas (AMLs), which are a member of the PEComa tumor family. We examined 28 liver specimens (15 biopsies and 13 surgical specimens) of hepatic AMLs obtained from 26 patients...
November 16, 2016: Human Pathology
https://www.readbyqxmd.com/read/27858882/pigmented-perivascular-epithelioid-cell-tumor-pecoma-arising-from-kidney-a-case-report
#10
Hexi Du, Jun Zhou, Lingfan Xu, Cheng Yang, Li Zhang, Chaozhao Liang
INTRODUCTION: Perivascular epithelioid cell tumor (PEComa) is a mesenchymal neoplasm composed of perivascular epithelioid cells with clear to eosinophilic cytoplasm. Pigmented PEComa arising from kidney is extraordinarily rare and sometimes can exhibit aggressive biological behavior. CASE REPORT: We present here a rare case of pigmented renal PEComa in a 46-year-old female. The patient had complained of lumbago complicated with nausea and vomiting for 2 weeks and therefore was referred to our department...
November 2016: Medicine (Baltimore)
https://www.readbyqxmd.com/read/27842558/perivascular-epithelioid-cell-tumor-of-the-descending-colon-mimicking-a-gastrointestinal-stromal-tumor-a-case-report
#11
Ryuta Iwamoto, Tatsuki R Kataoka, Ayako Furuhata, Kazuo Ono, Seiichi Hirota, Kenji Kawada, Yoshiharu Sakai, Hironori Haga
BACKGROUND: We present a case of perivascular epithelioid cell tumor (PEComa), which clinically and histologically mimics a gastrointestinal stromal tumor (GIST). CASE PRESENTATION: A 42-year-old woman was found to have a mass in the left flank during her annual medical checkup. Computed tomography examination revealed a submucosal tumor of the descending colon. Surgeons and radiologists suspected that the lesion was a GIST, and left hemicolectomy was performed without biopsy...
November 14, 2016: World Journal of Surgical Oncology
https://www.readbyqxmd.com/read/27828802/agminated-clear-cell-tumor-an-impostor-of-pecoma-and-distinctive-dermal-clear-cell-mesenchymal-neoplasm
#12
Ana Isabel Teixeira, Luís Soares-Almeida, Heinz Kutzner
Cutaneous clear cell tumors are a heterogeneous group of cutaneous neoplasms, which may show a wide range of histogenesis. We report the clinicopathological features of an agminated clear cell tumor, arising in a 67-year-old man, otherwise asymptomatic, with distinct histopathological and immunohistochemical features, which did not fit into any existing diagnostic categories. The patient presented with several skin-colored papules at the lateral and posterior aspects of the neck, which on histopathological examination showed circumscribed lobular aggregates of clear cells within the dermis...
October 28, 2016: American Journal of Dermatopathology
https://www.readbyqxmd.com/read/27793946/treatment-of-advanced-malignant-uterine-perivascular-epithelioid-cell-tumor-with-mtor-inhibitors-single-institution-experience-and-review-of-the-literature
#13
Kristen D Starbuck, Richard D Drake, G Thomas Budd, Peter G Rose
: Uterine perivascular epithelioid cell tumors (PEComas) are rare mesenchymal tumors. Many have malignant behavior, and no successful treatment strategy has been established. Identification of mutations in the tuberous sclerosis 1 (TSC1) and TSC2 genes producing constitutive activation of the mammalian target of rapamycin (mTOR) pathway presents an opportunity for targeted therapy. Patients with advanced malignant uterine PEComa treated with mTOR inhibitors were identified and records were retrospectively reviewed for treatment response based on radiographic assessment...
2016: Anticancer Research
https://www.readbyqxmd.com/read/27793610/primary-perivascular-epithelioid-cell-tumor-pecoma-of-the-bladder-a-case-report-with-2-years-of-follow-up-and-review-of-current-literature
#14
Stefano Creti, Daniele Romagnoli, Enrico Severini, Cristina Baldoni, Arrigo Bondi, Alfonso Di Campli, Mauro Dicuio, Giorgio Gentile, Rosario Dipietro, Carlo Saltutti, Riccardo Schiavina, Eugenio Brunocilla, Pierfrancesco Buli
No abstract text is available yet for this article.
August 13, 2016: Clinical Genitourinary Cancer
https://www.readbyqxmd.com/read/27784493/-rapidly-progressive-pulmonary-malignant-perivascular-epithelioid-cell-tumor-a-case-report-and-literature-review
#15
X Y Shi, F Long, B Liang, L L Su, H C Li, S J Jiang
Objective: To analyze the pathogenesis, clinical features, diagnosis and differential diagnosis of primary perivascular epithelioid cell tumor(PEComa). Methods: The clinical features, auxiliary examinations and diagnosis of a case with rapidly progressive pulmonary malignant PEComa were reported and the related literatures were reviewed.The literature review was carried out respectively in Wanfang Data, CNKI and PubMed from Jan. 1975 to Jul. 2015 with "pulmonary malignant perivascular epithelioid cell tumor" and "PEComa" being the search terms...
October 12, 2016: Chinese Journal of Tuberculosis and Respiratory Diseases
https://www.readbyqxmd.com/read/27773162/a-case-of-perivascular-epithelioid-cell-tumour-pecoma-nos
#16
Sagarika Tripathy, Anthony Kelmann, Yuen Chan
No abstract text is available yet for this article.
February 2016: Pathology
https://www.readbyqxmd.com/read/27757534/pancreatic-pecoma-is-a-novel-member-of-the-family-of-tuberous-sclerosis-complex-associated-tumors-case-report-and-review-of-the-literature
#17
Christopher P Hartley, David J Kwiatkowski, Lana Hamieh, Joel A Lefferts, Kerrington D Smith, Mikhail Lisovsky
No abstract text is available yet for this article.
December 2016: Virchows Archiv: An International Journal of Pathology
https://www.readbyqxmd.com/read/27689526/perivascular-epithelioid-cell-tumor-of-the-uterus-report-of-two-cases-and-mini-review-of-the-literature
#18
Charalampos Theofanakis, Nikolaos Thomakos, Maria Sotiropoulou, Alexandros Rodolakis
INTRODUCTION: Perivascular Epithelioid Cell tumor (PEComa) is a rare neoplasm of mesenchymal origin, with the uterus being the most common site of appearance, regarding the female genital tract. CASE REPORT: We present two cases of PEComas of the uterus in patients aged 57 and 42-years-old, presented to our department with palpable abdominal masses and abnormal vaginal bleeding. During follow up period, both patients are free of recurrent disease one and two years after surgery, respectively, without receiving any adjuvant treatment...
September 24, 2016: International Journal of Surgery Case Reports
https://www.readbyqxmd.com/read/27684885/primary-ovarian-malignant-pecoma-a-case-report
#19
Joseph D Westaby, Nesreen Magdy, Cyril Fisher, Mona El-Bahrawy
Perivascular epithelioid cell tumor (PEComa) is a rare mesenchymal neoplasm characterized by expression of both melanocytic and smooth muscle markers. PEComas are rarely encountered in the female genital tract. We here report a case of malignant primary PEComa of the ovary, and discuss the differential diagnosis. This represents the first case of primary typical malignant PEComa of the ovary.
September 28, 2016: International Journal of Gynecological Pathology
https://www.readbyqxmd.com/read/27628738/perivascular-epithelioid-cell-tumor-pecoma-of-pancreas-diagnosed-preoperatively-by-endoscopic-ultrasound-guided-fine-needle-aspiration-a-case-report-and-review-of-literature
#20
Katrina Collins, Tinera Buckley, Kevin Anderson, Michael Karasik, Saverio Ligato
Perivascular epithelioid cell tumors (PEComas) of the pancreas are extremely rare mesenchymal tumors and to our knowledge, only 17 cases have been reported in the English literature to date. We report our experience with a new case of primary pancreatic PEComa diagnosed preoperatively by endoscopic ultrasound-guided fine-needle aspiration (EUS-FNA) complemented by tissue cell block and immunohistochemistry. The patient was a 54-year-old female whose chief complaint was intermittent severe right upper quadrant abdominal pain...
January 2017: Diagnostic Cytopathology
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