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https://www.readbyqxmd.com/read/29686563/pulmonary-lymphangioleiomyomatosis-associated-with-aggressive-renal-angiomyolipoma
#1
Allison Cooper, Laura Baugh, Shannon Kelley, Howard Huang, Joseph Guileyardo
Lymphangioleiomyomatosis (LAM) is a rare cystic pulmonary disease that may occur in association with mutations in the tuberous sclerosis genes or arise sporadically. The histologic hallmark of the disease is the "LAM" cell, a spindled to epithelioid smooth muscle-like cell that bears morphologic and immunohistochemical resemblance to the perivascular epithelioid cell tumors (PEComas). The origin of the "LAM" cell is unknown; emerging theories suggest that a member of the PEComa family, the renal angiomyolipoma, may be the primary source and that both LAM and angiomyolipomas are associated with the genetic syndrome tuberous sclerosis...
January 2018: Proceedings of the Baylor University Medical Center
https://www.readbyqxmd.com/read/29668485/prolonged-activity-and-toxicity-of-sirolimus-in-a-patient-with-metastatic-renal-perivascular-epithelioid-cell-tumor-a-case-report-and-literature-review
#2
Alessandra Raimondi, Francesca Colombo, Giulia Pintarelli, Carlo Morosi, Salvatore L Renne, Anna M Frezza, Maristella Saponara, Angelo P Dei Tos, Arabella Mazzocchi, Salvatore Provenzano, Paolo G Casali, Silvia Stacchiotti
Perivascular epithelioid cell tumor (PEComa) is a family of mesenchymal tumors. Conventional chemotherapy has little activity in this disease, but case reports are available on the activity of mammalian target of rapamycin inhibitors (e.g. sirolimus and temsirolimus). Pharmacokinetic assays of sirolimus are available as this drug has a precise therapeutic window and blood levels might be influenced by CYP3A4 polymorphisms and drug interactions. We report on a case of a patient with metastatic, progressive PEComa who started sirolimus at a dose of 5 mg/day with evidence of grade (G) 3 mucositis, G2 thrombocytopenia, and G1 leucopenia 10 days after the treatment started, in absence of concomitant medications or prohibited food assumption...
April 17, 2018: Anti-cancer Drugs
https://www.readbyqxmd.com/read/29626599/expanding-the-histomorphologic-spectrum-of-tfe3-rearranged-pecomas
#3
Nolan Maloney, Krinio Giannikou, Joel Lefferts, Julia A Bridge, Konstantinos Linos
Perivascular epithelioid tumors (PEComas) are a family of mesenchymal neoplasms that have smooth muscle and melanocytic differentiation. They can be sporadic or associated with Tuberous Sclerosis Complex and commonly present in the kidney as angiomyolipoma or in the lung as pulmonary clear cell sugar tumors or lymphangioleiomyomatosis. However, they can present at any visceral or soft tissue site. They usually have a benign clinical course, but rarely can behave in a malignant fashion. Most PEComas demonstrate abnormalities of TSC2, but a recently described subset harbor TFE3 rearrangements that appear to be mutually exclusive of TSC2 alterations...
April 4, 2018: Human Pathology
https://www.readbyqxmd.com/read/29531006/tfe3-expressing-epithelioid-rich-perivascular-epithelioid-cell-neoplasm-pecoma-of-the-bladder-with-unusual-benign-course
#4
Xiu Fen Chen, Joe Yeong, Kenneth Tou En Chang, Alvin Soon Tiong Lim, Chik Hong Kuick, Tse Hui Lim, Jain Sudhanshi, Sathiyamoorthy Selvarajan, Valerie Huei Li Gan, Li Yan Khor
Perivascular epithelioid cell tumor (PEComa) is an uncommon tumor which presents with epithelioid and spindled cell morphology and is immunoreactive for myogenic and melanocytic markers. Recently, a subset of PEComas has been reported to harbor TFE3 gene rearrangement.In this case report, we describe a TFE3 -expressing primary bladder PEComa in a 27-year-old male patient with acute myeloid leukaemia in remission. The tumor displayed epithelioid morphology with surrounding delicate blood vessels and was devoid of a prominent spindle cell component...
January 2018: Annals of Clinical and Laboratory Science
https://www.readbyqxmd.com/read/29516164/cathepsin-k-expression-in-clear-cell-sugar-tumor-pecoma-of-the-lung
#5
Anna Caliò, Maria Cecilia Mengoli, Alberto Cavazza, Giulio Rossi, Claudio Ghimenton, Matteo Brunelli, Maurizio Pea, Marco Chilosi, Lisa Marcolini, Guido Martignoni
Clear cell "sugar" tumor is a rare benign neoplasm arising in the lung, considered as a part of the PEComa family. As PEComas of other sites, this tumor expresses melanocytic markers such as HMB45 and Melan-A. Despite cathepsin K, MITF and CD68 staining are known to be positive in a large number of PEComas and TFE3 rearrangement has been reported in a subset of PEComas, no data is available regarding the expression of these markers and the occurrence of TFE3 and TFEB rearrangement in clear cell "sugar" tumor of the lung...
March 7, 2018: Virchows Archiv: An International Journal of Pathology
https://www.readbyqxmd.com/read/29484084/radiological-findings-of-two-neoplasms-with-perivascular-epithelioid-cell-differentiation
#6
Timothy Diestelkamp, Zachary Mikes, Robin Wilson-Smith, Pauline Germaine
Perivascular epithelioid cell tumors (PEComas) constitute a rare subset of mesenchymal neoplasms classified by the World Health Organization in 2002. We present two cases of PEComas; the first is a cervical PEComa in a 35-year-old woman with no known past medical history who presented with a palpable pelvic mass; the second is an adnexal PEComa in a 39-year-old woman with a history of colitis who presented with abdominal pain and diarrhea. The rarity of these tumors has led to little information about imaging characteristics which we hope these two cases will help expand...
December 2017: Radiology Case Reports
https://www.readbyqxmd.com/read/29478828/primary-pancreatic-perivascular-epithelioid-cell-tumor-pecoma-a-surgical-enigma-a-systematic-review-of-the-literature
#7
REVIEW
Maurizio Zizzo, Lara Ugoletti, David Tumiati, Carolina Castro Ruiz, Stefano Bonacini, Michele Panebianco, Giuliana Sereni, Antonio Manenti, Filippo Lococo, Gabriele Carlinfante, Claudio Pedrazzoli
BACKGROUND: Perivascular epithelioid cell tumor (PEComa) is a rare mesenchymal tumor, with distinctive morphology and expression of myo-melanocytic markers. Current scientific literature reported just 24 cases of pancreatic PEComas. With our systematic review, we aimed at improving our understanding of the disease, focusing on the knowledge gained on epidemiology, etiology, clinical presentation, diagnosis, treatment and prognosis. METHODS: Based on the PRISMA guidelines, a systematic research was carried out on PubMed/MEDLINE, Web of Science, Scopus, EMBASE, and EBSCO using the search terms: ("perivascular epithelioid cell tumor" OR ″PEComa") and ("pancreas "OR″ pancreatic")...
April 2018: Pancreatology: Official Journal of the International Association of Pancreatology (IAP) ... [et Al.]
https://www.readbyqxmd.com/read/29463266/unresectable-hepatic-pecoma-a-rare-malignancy-treated-with-stereotactic-body-radiation-therapy-sbrt-followed-by-complete-resection
#8
Simon Kirste, Gian Kayser, Anne Zipfel, Anca-Ligia Grosu, Thomas Brunner
BACKGROUND: Perivascular epithelioid cell tumors (PEComas) are rare mesenchymal tumors occurring in various anatomic regions. Although diagnostic criteria and treatment management are not established, current treatment options consist of surgery and chemotherapy including mTOR inhibitors. Stereotactic body radiation therapy (SBRT) is a non-invasive ablative treatment which has shown excellent control rates for more common types of unresectable liver tumors and metastases. In this report we present a rare case of PEComa of the liver that was treated by stereotactic radiotherapy followed by resection...
February 20, 2018: Radiation Oncology
https://www.readbyqxmd.com/read/29435023/primary-malignant-perivascular-epithelioid-cell-neoplasm-pecoma-of-the-bone-mimicking-granular-cell-tumor-in-core-biopsy-a-case-report-and-literature-review
#9
Sam Sadigh, Preya Shah, Kristy Weber, Ronnie Sebro, Paul J Zhang
The present study investigated the case of a 46-year-old female with primary malignant perivascular epithelioid cell neoplasm (PEComa) of the femur. The patient presented with a 5-month history of right distal thigh pain following trauma. Radiographs of the right distal femur revealed a mixed lytic and sclerotic lesion with subtle areas of cortical destruction and soft tissue extension, consistent with an aggressive tumor. A core biopsy revealed an epithelioid tumor with granular cell features, but a definitive diagnosis could not be made...
March 2018: Oncology Letters
https://www.readbyqxmd.com/read/29373116/uncommon-hereditary-gynaecological-tumour-syndromes-pathological-features-in-tumours-that-may-predict-risk-for-a-germline-mutation
#10
REVIEW
Karuna Garg, Anthony N Karnezis, Joseph T Rabban
The most common hereditary gynaecological tumour syndromes are hereditary breast and ovarian cancer syndrome and Lynch syndrome. However, pathologists also may encounter gynaecological tumours in women with rare hereditary syndromes. Many of these tumours exhibit distinctive gross and microscopic features that are associated with a risk for an inherited gene mutation. The sensitivity and specificity of these tumour pathology features for predicting an inherited mutation vary depending on the syndrome. By recognising these tumour features, pathologists may potentially contribute to the diagnosis of an unsuspected syndrome by recommending referral of the patient for formal risk assessment by genetic counselling...
February 2018: Pathology
https://www.readbyqxmd.com/read/29340997/immunohistochemical-biomarkers-of-mesenchymal-neoplasms-in-endocrine-organs-diagnostic-pitfalls-and-recent-discoveries
#11
Yin P Hung, Jason L Hornick
Mesenchymal neoplasms rarely present in or adjacent to endocrine organs. In this context, the recognition of these rare tumor types can be challenging, with significant potential for misdiagnosis as sarcomatoid carcinomas (i.e., anaplastic thyroid carcinoma and sarcomatoid adrenal cortical carcinoma) or neuroendocrine carcinomas, depending upon the dominant histologic patterns. In this review, we address potential pitfalls in diagnosing selected mesenchymal neoplasms arising within or near endocrine organs, including dedifferentiated liposarcoma, synovial sarcoma, angiosarcoma, PEComa, proximal-type epithelioid sarcoma, Ewing sarcoma, and neuroblastoma...
January 16, 2018: Endocrine Pathology
https://www.readbyqxmd.com/read/29340502/recurrence-of-retroperitoneal-localized-perivascular-epithelioid-cell-tumor-two-years-after-initial-diagnosis-case-report
#12
Yasemin Benderli Cihan, Engin Kut, Ali Koç
CONTEXT: Perivascular epithelioid cell tumors (PEComas) are rare mesenchymal tumors. Adjuvant radiotherapy and/or chemotherapy are administered according to the patient's clinical characteristics. CASE REPORT: A 42-year-old female patient was operated to treat a retroperitoneal mass. The diagnosis was established as PEComa with benign behavior. Two years after the diagnosis, chest and abdominal computed tomography scans showed intra-abdominal recurrence and lymphangioleiomyomatosis in the lung...
January 15, 2018: São Paulo Medical Journal, Revista Paulista de Medicina
https://www.readbyqxmd.com/read/29329131/alk-gene-fusions-in-epithelioid-fibrous-histiocytoma-a-study-of-14-cases-with-new-histopathological-findings
#13
Dmitry V Kazakov, Liubov Kyrpychova, Petr Martinek, Petr Grossmann, Petr Steiner, Tomas Vanecek, Michal Pavlovsky, Vladimir Bencik, Michael Michal, Michal Michal
Previous studies showed that ALK is often positive in epithelioid fibrous histiocytoma (EFH). Two cases of EFH with ALK gene fusions have been recorded. Our objective was to study a series of EFH to present histopathological variations of EFH, identify novel ALK gene fusions, and determine whether there is a correlation between histopathological features and particular gene. We investigated 14 cases of EFH, all ALK immunopositive. The cases were assessed histopathologically as well as for ALK and TFE-3 rearrangements using FISH and ALK gene fusions using next-generation sequencing...
January 11, 2018: American Journal of Dermatopathology
https://www.readbyqxmd.com/read/29324470/fibroma-like-pecoma-a-tuberous-sclerosis-complex-related-lesion
#14
Ana B Larque, Richard L Kradin, Ivan Chebib, G Petur Nielsen, Martin K Selig, Elizabeth A Thiele, Anat Stemmer-Rachamimov, Miriam A Bredella, Pawel Kurzawa, Vikram Deshpande
Perivascular epithelioid cell tumor (PEComa), mesenchymal tumors morphologically characterized by epithelioid cells, coexpress melanocytic and muscle markers. Herein, we describe a heretofore-undescribed tuberous sclerosis complex (TSC)-related neoplasm, morphologically resembling a soft tissue fibroma-like lesion, but showing an immunophenotype resembling PEComa. We identified 3 soft tissue fibroma-like lesions in individuals with TSC. We also evaluated 6 TSC-related periungual fibroma as well as a range of non-TSC fibroma-like lesions (n=19)...
April 2018: American Journal of Surgical Pathology
https://www.readbyqxmd.com/read/29318761/suitability-of-the-cellient-tm-cell-block-method-for-diagnosing-soft-tissue-and-bone-tumors
#15
W Song, B M van Hemel, A J H Suurmeijer
BACKGROUND: The diagnosis of tumors of soft tissue and bone (STB) heavily relies on histological biopsies, whereas cytology is not widely used. CellientTM cell blocks often contain small tissue fragments. In addition to Hematoxylin and Eosin (H&E) interpretation of histological features, immunohistochemistry (IHC) can be applied after optimization of protocols. The objective of this retrospective study was to see whether this cytological technique allowed us to make a precise diagnosis of STB tumors...
April 2018: Diagnostic Cytopathology
https://www.readbyqxmd.com/read/29245304/computed-tomography-imaging-features-of-hepatic-perivascular-epithelioid-cell-tumor-a-case-report-and-literature-review
#16
REVIEW
Xu Han, Mei-Yu Sun, Jing-Hong Liu, Xiao-Yan Zhang, Meng-Yao Wang, Rui Fan, Sahrish Qamar
RATIONALE: Perivascular epithelioid cell tumor (PEComa) is a rare tumor which is most frequently found in uterus. The tumor arising from liver is extremely uncommon. PATIENT CONCERNS: A 36-year-old female with abdominal distention, cramps, and low-grade fever for over 15 days. The patient had a history of gastric adenocarcinoma with ovarian, celiac lymph nodes, and retroperitoneal lymph nodes metastases. DIAGNOSES: Computed tomography (CT) imaging demonstrated an ill-defined heterogeneous hypo-dense mass in segment 8 (S8) of the liver...
December 2017: Medicine (Baltimore)
https://www.readbyqxmd.com/read/29208563/malignant-perivascular-epithelioid-cell-tumor-of-the-oropharynx-with-strong-tfe3-expression-mimicking-alveolar-soft-part-sarcoma-a-case-report-and-review-of-the-literature
#17
Karan Saluja, Jaiyeola Thomas, Songlin Zhang, Erich M Sturgis, Kunal S Jain, Victor G Prieto, Adel K El-Naggar, Diana Bell
Perivascular epithelioid cell tumors (PEComas) in the head and neck region are rare with 26 cases described in literature. These distinct mesenchymal tumors normally express both myoid and melanocytic markers. We here report an interesting and challenging case of malignant PEComa that showed transcription factor E3 (TFE3) protein expression and rearrangement, paucity of muscle and melanocytic marker expression, and morphologically mimicked as alveolar soft part sarcoma. Awareness of this morphologic pitfall and recognition TFE3 gene rearranged PEComa, as a distinct subtype of PEComa is essential to avoid misdiagnosis...
December 2, 2017: Human Pathology
https://www.readbyqxmd.com/read/29127695/management-of-the-other-retroperitoneal-sarcomas
#18
REVIEW
Piotr L Rutkowski, John T Mullen
The focus of this review is on the management of the less common sarcomas occurring in the retroperitoneal space, including solitary fibrous tumor (SFT), malignant peripheral nerve sheath tumor (MPNST), perivascular epithelioid cell tumor (PEComa), and undifferentiated pleomorphic sarcoma (UPS) of the psoas muscle. As for other retroperitoneal sarcomas, surgical resection is the mainstay of curative therapy, and multidisciplinary preoperative assessment, including percutaneous needle biopsy for histologic confirmation, is the basis for personalized management, as the surgical management, and the integration of systemic therapy and radiation therapy is unique to each histologic subtype...
January 2018: Journal of Surgical Oncology
https://www.readbyqxmd.com/read/29052596/t-6-11-renal-cell-carcinoma-a-study-of-seven-cases-including-two-with-aggressive-behavior-and-utility-of-cd68-pg-m1-in-the-differential-diagnosis-with-pure-epithelioid-pecoma-epithelioid-angiomyolipoma
#19
Anna Caliò, Matteo Brunelli, Diego Segala, Serena Pedron, Regina Tardanico, Andrea Remo, Stefano Gobbo, Emanuela Meneghelli, Claudio Doglioni, Ondrej Hes, Claudia Zampini, Pedram Argani, Guido Martignoni
Renal cell carcinomas with t(6;11) chromosome translocation involving the TFEB gene are indolent neoplasms which often occur in young patients. In this study, we report seven cases of renal cell carcinoma with TFEB rearrangement, two of whom had histologically proven metastasis. Patients (4F, 3M) ranged in age from 19 to 55 years (mean 37). One patient developed paratracheal and pleural metastases 24 months after surgery and died of disease after 46 months; another one recurred with neoplastic nodules in the perinephric fat and pelvic soft tissue...
March 2018: Modern Pathology: An Official Journal of the United States and Canadian Academy of Pathology, Inc
https://www.readbyqxmd.com/read/29044701/malignant-pecoma
#20
Daniela Haiges, Philipp Kurz, Helmut Laaff, Frank Meiss, Heinz Kutzner, Kristin Technau-Hafsi
No abstract text is available yet for this article.
January 2018: Journal of Cutaneous Pathology
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