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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
#1
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
#2
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
#3
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
#4
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
#5
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
#6
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
#7
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...
November 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
#8
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
#9
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
#10
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
#11
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
#12
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
#13
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
#14
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...
September 15, 2016: Diagnostic Cytopathology
https://www.readbyqxmd.com/read/27542598/intrahepatic-peribiliary-perivascular-epithelioid-cell-tumor-pecoma-associated-with-heterotopic-pancreas-a-case-report
#15
Yuka Kiriyama, Tetsuya Tsukamoto, Yoshikazu Mizoguchi, Shin Ishihara, Akihiko Horiguchi, Takamasa Tokoro, Yutaro Kato, Atsushi Sugioka, Makoto Kuroda
BACKGROUND: Perivascular epithelioid-cell tumor (PEComa) is a group of rare mesenchymal neoplasms that express myomelanocytic-cell markers and exhibit a wide variety of histopathological features. Although heterotopic pancreas has been reported to occur in the gastrointestinal tract, intrahepatic heterotopic pancreas has been reported only rarely. CASE PRESENTATION: We present a case of intrahepatic PEComa that showed a strong regional correlation with the presence of heterotopic pancreas...
2016: Diagnostic Pathology
https://www.readbyqxmd.com/read/27494029/whole-exome-sequencing-identifies-tsc1-tsc2-biallelic-loss-as-the-primary-and-sufficient-driver-event-for-renal-angiomyolipoma-development
#16
Krinio Giannikou, Izabela A Malinowska, Trevor J Pugh, Rachel Yan, Yuen-Yi Tseng, Coyin Oh, Jaegil Kim, Magdalena E Tyburczy, Yvonne Chekaluk, Yang Liu, Nicola Alesi, Geraldine A Finlay, Chin-Lee Wu, Sabina Signoretti, Matthew Meyerson, Gad Getz, Jesse S Boehm, Elizabeth P Henske, David J Kwiatkowski
Renal angiomyolipoma is a kidney tumor in the perivascular epithelioid (PEComa) family that is common in patients with Tuberous Sclerosis Complex (TSC) and Lymphangioleiomyomatosis (LAM) but occurs rarely sporadically. Though histologically benign, renal angiomyolipoma can cause life-threatening hemorrhage and kidney failure. Both angiomyolipoma and LAM have mutations in TSC2 or TSC1. However, the frequency and contribution of other somatic events in tumor development is unknown. We performed whole exome sequencing in 32 resected tumor samples (n = 30 angiomyolipoma, n = 2 LAM) from 15 subjects, including three with TSC...
August 2016: PLoS Genetics
https://www.readbyqxmd.com/read/27491291/primary-pure-spindle-cell-carcinoma-sarcomatoid-carcinoma-of-the-ovary-a-case-report-with-immunohistochemical-study
#17
Giovanna Giordano, Roberto Berretta, Enrico Silini
BACKGROUND: In the ovary, sarcomatoid carcinoma has been reported only as mural nodules in epithelial malignant or borderline serous or mucinous cystic neoplasms, and in teratomas. In this paper we report a rare case of a solid sarcomatoid carcinoma of the ovary, without accompanying component of giant cells, pleomorphic cells, or glandular and other epithelial structures. CASE PRESENTATION: This case report refers to a sarcomatoid carcinoma of the ovary in in a 57 year-old woman with abdominal pain...
August 5, 2016: Diagnostic Pathology
https://www.readbyqxmd.com/read/27452081/a-case-of-malignant-pecoma-of-the-uterus-associated-with-intramural-leiomyoma-and-endometrial-carcinoma
#18
Yoo Jin Choi, Jin Hwa Hong, Aeree Kim, Hankyeom Kim, Hyeyoon Chang
Perivascular epithelioid cell tumors (PEComas) refers to a family of mesenchymal neoplasms composed of angiomyolipomas, clear cell "sugar" tumors of the lung, and lymphangioleiomyomatoses. These tumors have a distinctive and common component of perivascular epithelioid cells that show an association with blood vessel walls and immunohistochemically display myomelanocytic differentiation. The unique neoplasms have been shown to have an expanded range through a variety of case reports, including visceral, intra-abdominal, soft tissue, and bone tumors...
November 2016: Journal of Pathology and Translational Medicine
https://www.readbyqxmd.com/read/27428182/a-systematic-review-perivascular-epithelioid-cell-tumor-of-gastrointestinal-tract
#19
Zehong Chen, Siqi Han, Jialin Wu, Minmin Xiong, Yanqiao Huang, Jianhui Chen, Yujie Yuan, Jianjun Peng, Wu Song
Perivascular epithelioid cell tumor (PEComa) is a rare entity with distinctive morphology and of expressing myomelanocytic markers. Gastrointestinal tract (GI) is one of the most common anatomic sites of origin and counts for 20% to 25% of all reported cases of perivascular epithelioid cell tumors not otherwise specified (PEComas-NOS). However, the biologic behavior of perivascular epithelioid cell tumors of gastrointestinal tract (GI PEComas-NOS) is still unclear. The aim of conducting this systematic review is to sum up what is known so far of the epidemiology, natural history, management and prognosis of GI PEComas-NOS...
July 2016: Medicine (Baltimore)
https://www.readbyqxmd.com/read/27404774/incidence-of-mature-adipocytic-component-within-cutaneous-smooth-muscle-neoplasms
#20
Chelsea Jones, Sara C Shalin, Jerad M Gardner
Angioleiomyoma is a benign smooth muscle tumor of the subcutis. The presence of mature adipocytes has been described in this tumor under the rubric of 'angiolipoleiomyoma' or, erroneously, 'angiomyolipoma' (these are not PEComas). Previous studies have found adipocytes in only 2-3% of angioleiomyoma. Anecdotally, the incidence appeared to be greater than this in our practice. Moreover, the presence of adipocytes has not been evaluated in pilar leiomyoma or cutaneous leiomyosarcoma. We searched the pathology archives from 2007 to 2014 for all cutaneous and subcutaneous leiomyoma and leiomyosarcoma; cases were reviewed to confirm the diagnosis and evaluate for mature adipocytes...
October 2016: Journal of Cutaneous Pathology
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