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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.
October 18, 2016: Virchows Archiv: An International Journal of Pathology
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
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
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
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
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
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
Yoo Jin Choi, Jin Hwa Hong, Aeree Kim, Hankyeom Kim, Hyeyoon Chang
The concept of perivascular epithelioid cell tumors (PEComas) was first introduced. It 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...
July 25, 2016: Journal of Pathology and Translational Medicine
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)
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
Yusuke Mizuuchi, Kazuyoshi Nishihara, Akifumi Hayashi, Sadafumi Tamiya, Satoshi Toyoshima, Yoshinao Oda, Toru Nakano
Perivascular epithelial cell tumors (PEComas), firstly described by Bonetti in 1992, are a family of mesenchymal tumor composed of perivascular epithelioid cells having epithelioid or spindle morphology and exhibiting melanocytic and myogenic immunoreactivities. We herein described a 61-year-old woman who presented with epigastric pain. Preoperative imaging studies showed that 7-cm-sized mass was located in pancreatic head and body, and pancreaticoduodenectomy was performed. Histological findings showed that the tumor was composed of clear epithelioid cells with abundant glycogen granules, which grew in a nested and alveolar pattern around blood vessels...
December 2016: Surgical Case Reports
Martin D Hyrcza, Daniel A Winer, Mary Shago, Karolyn Au, Gelareh Zadeh, Sylvia L Asa, Ozgur Mete
We report a primary central nervous system (CNS) perivascular epithelioid cell tumor (PEComa) in a middle-aged female patient. The tumor occurred in suprasellar location with secondary extension into the sella turcica. The patient presented with intracranial hemorrhage and an altered level of consciousness. The tumor had morphologic features matching those of other previously described TFE3-translocated PEComas, including epithelioid morphology, diffuse and strong nuclear immunoreactivity for TFE3, and minimal staining with myoid markers...
May 17, 2016: Endocrine Pathology
David D'Andrea, Esther Hanspeter, Carolina D'Elia, Thomas Martini, Armin Pycha
Perivascular epithelioid cell neoplasms (PEComa) are rare mesenchymal tumors that can occur in any part of the body and have unpredictable pathological behavior. They are usually benign, but may be malignant. We present a case of malignant PEComa of the pelvic retroperitoneum treated with radical surgery.
May 2016: Urology Case Reports
Bedoor Al Omran, Naseem Ansari
Angiomyolipomas (AMLs) are the most common mesenchymal renal neoplasms and are classified as neoplasms of perivascular epithelioid cells (PEComa). AML is usually a benign neoplasm arising most often in the kidney although it has been described in a wide variety of sites. Most patients are adults, and one-third suffer from tuberous sclerosis. We describe a case of renal AML in a 54-year-old Bahraini woman who presented to the Bahrain Defence Force Hospital with right flank pain and hematuria, and who was known to have rheumatoid arthritis but had no cutaneous or other stigmata of tuberous sclerosis...
May 2016: Oman Medical Journal
Megan Fitzpatrick, Tanya Pulver, Molly Klein, Paari Murugan, Mahmoud Khalifa, Khalid Amin
BACKGROUND: Perivascular epithelioid cell tumors (PEComas) are a rare group of neoplasms composed of epithelioid cells that express both melanocytic and myoid markers. When considering PEComas of the female genital tract, the uterus is the most common location. Involvement of the ovary in the context of a primary uterine PEComa, in the absence of systemic disease associated with tuberous sclerosis, however, has only been reported in 1 previous case. CASE REPORT: We report a case of a PEComa of the uterus with metastasis to the left ovary in a 61-year-old Caucasian woman...
2016: American Journal of Case Reports
Atsuhiko Handa, Kazutoshi Fujita, Tatsuo Kono, Koji Komori, Seiichi Hirobe, Ryuji Fukuzawa
Perivascular epithelioid cell tumour (PEComa) encompasses a group of mesenchymal tumours composed of histologically and immunohistochemically distinctive perivascular epithelioid cells. A subset of PEComa that typically arises from the falciform ligament and/or ligamentum teres is termed clear cell myomelanocytic tumour of the falciform ligament/ligamentum teres. To date, its imaging findings have not been described. Here, we report the first radiological description of a pathologically confirmed tumour. The patient was a 5-year-old girl with a palpable abdominal mass...
April 20, 2016: Journal of Medical Imaging and Radiation Oncology
E Kudela, K Biringer, P Kasajova, M Nachajova, M Adamkov
The World Health Organization (WHO) defines PEComas as mesenchymal tumors composed of histologically and immunohistochemically distinctive perivascular cells. Uterus is the most common site of a subgroup of PEComas not otherwise specified(NOS). PEComas of the uterine cervix are extremely rare, and only thirteen cases have been described in the English literature to date. In this review, we summarize the available data concerning diagnostics, immunohistochemical analysis, genetics and treatment of cervical PEComas...
August 2016: Pathology, Research and Practice
Yin Zhi Lan, Xiao En Hua
Perivascular epithelioid cell neoplasm (PEComa) is a rare tumor type and primary hepatic multiple perivascular is rarer still. The present case report investigated the case of a 40-year-old woman who was admitted to The Second Xiangya Hospital for hepatic multiple tumor by physical examination without any history of abdominal pain or body weight loss. Abdominal plain computed tomography revealed three lesions in the liver. All lesions exhibited a well-defined boarder and only one mass contained fatty tissue in S6 of the liver...
April 2016: Molecular and Clinical Oncology
Ha Yeon Kim, Jin Hyuk Choi, Hye Seung Lee, Yoo Jin Choi, Aeree Kim, Han Kyeom Kim
Benign perivascular epithelioid cell tumor (PEComa) of the lung is a rare benign neoplasm, a sclerosing variant of which is even rarer. We present a case of 51-year-old man who was diagnosed with benign sclerosing PEComa by percutaneous fine needle aspiration cytology and biopsy. The aspirate revealed a few cell clusters composed of bland-looking polygonal or spindle cells with fine granular or clear cytoplasm. Occasional fine vessel-like structures with surrounding hyalinized materials were seen. The patient later underwent wedge resection of the lung...
May 2016: Journal of Pathology and Translational Medicine
Hui Jiang, Na Ta, Xiao-Yi Huang, Ming-Hua Zhang, Jing-Jing Xu, Kai-Lian Zheng, Gang Jin, Jian-Ming Zheng
Perivascular epithelioid cell tumor (PEComa) of the pancreas is an unusual tumor deriving from mesenchyma. This paper described a case of pancreatic PEComa, which was initially suspected as neuroendocrine carcinoma by biopsy, and therefore surgical treatment was recommended due to undetermined diagnosis. Examination of the surgical specimen under a microscope showed that the tumor cell's morphology was epithelioid or spindle-shaped, and ranged in a nested pattern. Additionally, these cells had a large extent of acidophilic cytoplasm, no mitotic figures, and expressed HMB-45, melan-p, and smooth muscle actin immunohistochemically...
April 7, 2016: World Journal of Gastroenterology: WJG
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