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Endometrial stromal sarcoma

Chaitra P Adiga, Manju Gyanchandani, Lakshmikantha N Goolahally, Rishikesh M Itagi, Kiran V Kalenahalli
Endometrial stromal sarcoma (ESS) is an aggressive uterine sarcoma. We report a case of a large endometrial stromal sarcoma in a 42 year nulliparous woman with chronic kidney disease presenting with acute urinary retention and irregular per vaginal bleeding. Ultrasound and Doppler imaging revealed a heterogeneous mass in the endometrial cavity with internal vascularity. Magnetic resonance imaging (MRI) revealed a large lobulated mass in the endometrial cavity extending into the vagina, causing local mass effect...
September 2016: Journal of Radiology Case Reports
Ming-Shyen Yen, Jen-Ruei Chen, Peng-Hui Wang, Kuo-Chang Wen, Yi-Jen Chen, Heung-Tat Ng
Uterine sarcoma is a very aggressive and highly lethal disease. Even after a comprehensive staging surgery or en block cytoreduction surgery followed by multimodality therapy (often chemotherapy and/or radiation therapy), many patients relapse or present with distant metastases, and finally die of diseases. The worst outcome of uterine sarcomas is partly because of their rarity, unknown etiology, and highly divergent genetic aberration. Uterine sarcomas are often classified into four distinct subtypes, including uterine leiomyosarcoma, low-grade uterine endometrial stromal sarcoma, high-grade uterine endometrial stromal sarcoma, and undifferentiated uterine sarcoma...
October 2016: Taiwanese Journal of Obstetrics & Gynecology
S F Lax
The 2014 World Health Organization (WHO) classification of uterine tumors revealed simplification of the classification by fusion of several entities and the introduction of novel entities. Among the multitude of alterations, the following are named: a simplified classification for precursor lesions of endometrial carcinoma now distinguishes between hyperplasia without atypia and atypical hyperplasia, the latter also known as endometrioid intraepithelial neoplasia (EIN). For endometrial carcinoma a differentiation is made between type 1 (endometrioid carcinoma with variants and mucinous carcinoma) and type 2 (serous and clear cell carcinoma)...
October 13, 2016: Der Pathologe
Weimin Xie, Dongyan Cao, Jiaxin Yang, Xuan Jiang, Keng Shen, Lingya Pan, Huifang Huang, Jinghe Lang, Yan You, Jie Chen
PURPOSE: To assess the clinical outcomes and fertility of young women with stage I low-grade endometrial stromal sarcoma (ESS) treated with fertility-sparing surgery. RESULTS: Seventeen patients with stage I low-grade ESS (stage IA, n = 6; stage IB, n = 11) were entered into this study. Adjuvant hormone therapy was administered to 15 (88.2%) patients. At a median follow-up of 39 months (range, 4-106 months), 10 (58.8%) patients developed recurrence. All 10 patients had stage IB disease; among them, the first recurrence limited to the uterus was observed in 6 patients...
October 6, 2016: Oncotarget
Manfei Si, Lin Jia, Kun Song, Qing Zhang, Beihua Kong
OBJECTIVE: Uterine sarcomas are rare, highly aggressive tumors with an unfavorable prognosis. The role of lymphadenectomy (LAD) remains controversial for this particular tumor type. To examine whether LAD can assist in prognosis or clinical benefits for uterine sarcoma patients, we performed a meta-analysis based on published studies. METHODS: We initially identified published studies by searching the PubMed database up to 30 November 2015. Study quality was evaluated systematically using the Newcastle-Ottawa Scale for assessing the quality of studies for inclusion in meta-analyses...
September 23, 2016: International Journal of Gynecological Cancer
Lien N Hoang, Amandeep Aneja, Niamh Conlon, Deborah F Delair, Sumit Middha, Ryma Benayed, Martee L Hensley, Kay J Park, Travis J Hollmann, Meera R Hameed, Cristina R Antonescu, Robert A Soslow, Sarah Chiang
Endometrial stromal sarcomas (ESS) are often underpinned by recurrent chromosomal translocations resulting in the fusion of genes involved in epigenetic regulation. To date, only YWHAE-NUTM2 rearrangements are associated with distinctive high-grade morphology and aggressive clinical behavior. We identified 3 ESS morphologically mimicking myxoid leiomyosarcoma of the uterus and sought to describe their unique histopathologic features and identify genetic alterations using next-generation sequencing. All cases displayed predominantly spindled cells associated with abundant myxoid stroma and brisk mitotic activity...
September 14, 2016: American Journal of Surgical Pathology
Krystyna Serkies, Ewa Pawłowska, Jacek Jassem
Uterine endometrial stromal sarcomas including true low-grade endometrial stromal sarcoma (LG-ESS) and high-grade (HG-ESS) or undifferentiated endometrial sarcoma (UES) constitute a group of rare, aggressive malignancies. Most LG-ESSs express steroid receptors. Surgery is the principal primary therapy for endometrial stromal sarcomas and should be considered in all cases. These malignancies are relatively radio- and chemoresistant. Chemotherapy is used in recurrent and advanced HG-ESS and UES. Currently, the combination of gemcitabine and docetaxel is considered the most effective regimen, but at the expense of substantial toxicity...
2016: Ginekologia Polska
Mauro Cozzolino, Dimitrios Nasioudis, Giovanni Sisti, Maria Elisabetta Coccia
BACKGROUND: The study aimed to examine all available evidence regarding primary vaginal cancer arising from endometriosis from an evidence-based perspective. METHODS: A literature search in Medline and EMBASE databases was conducted. The following key words were employed: 'vaginal cancer' AND 'endometriosis malignant transformation' OR 'endometriosis'. RESULTS: A total of 23 eligible studies were identified and included in the present review providing information for 37 patients...
September 13, 2016: Gynecologic and Obstetric Investigation
Birgit Rommel, Carsten Holzmann, Jörn Bullerdiek
Sarcomas are rare uterine tumors with leiomyosarcomas and endometrial stromal sarcomas constituting the predominant entities often making their first appearance in young and middle-aged women. By histology combined with immunostaining alone some of these tumors can offer diagnostic challenges e.g. for the differential diagnosis between leiomyosarcomas and smooth muscle tumors of uncertain malignant potential (STUMP). Areas covered: Recent advances in the genetic classification and subclassification, respectively, have shown that genetic markers can offer a valuable adjunct to conventional diagnostic tools...
November 2016: Expert Review of Anticancer Therapy
Huann-Cheng Horng, Kuo-Chang Wen, Peng-Hui Wang, Yi-Jen Chen, Ming-Shyen Yen, Heung-Tat Ng
Endometrial stromal tumors are rare uterine tumors (<1%). Four main categories include endometrial stromal nodule, low-grade endometrial stromal sarcoma (LG-ESS), high-grade endometrial stromal sarcoma (HG-ESS), and uterine undifferentiated sarcoma (UUS). This review is a series of articles discussing the uterine sarcomas. LG-ESS, a hormone-dependent tumor harboring chromosomal rearrangement, is an indolent tumor with a favorable prognosis, but characterized by late recurrences even in patients with Stage I disease, suggesting the requirement of a long-term follow-up...
August 2016: Taiwanese Journal of Obstetrics & Gynecology
Ju A Back, Myeong Gyune Choi, U Chul Ju, Woo Dae Kang, Seok Mo Kim
Endometrial stromal sarcoma (ESS) is a rare malignancy. Development of extrauterine ESS form endometriosis is particularly rare. The majority of extrauterine ESS occurs in areas with preexisting endometriosis. The most common site is the ovary. We experienced a case of ESS of the ovary that arose from endometriosis with multiple disseminated lesions. This disease was managed by total abdominal hysterectomy, bilateral salpingo-oophorectomy, both pelvic lymph nodes dissection, omentectomy, and appendectomy followed by postoperative high-dose progesterone therapy...
July 2016: Obstetrics & Gynecology Science
Supawon Srettabunjong, Tuenjai Chuangsuwanich
Pulmonary tumor thromboembolus (PTTE) secondary to uterine cancer is a rare cause of sudden unexpected deaths in the elderly population. Additionally, tumor extension to the inferior vena cava (IVC) makes it rarer. No such cases have been previously reported in the forensic literature. We report this phenomenon in a previously healthy 70-year-old woman with no other known risk factors for the development of deep vein thrombosis. The deceased was found dead while watching television with her relatives. Autopsy examination revealed that her death was attributed to a saddle embolus lodged in the main pulmonary arteries...
March 2016: Journal of Forensic Sciences
L-C Horn, D Mayr, C E Brambs, J Einenkel, I Sändig, K Schierle
Histopathological assessment of the tumor grade and cell type is central to the management and prognosis of various gynecological malignancies. Conventional grading systems for squamous carcinomas and adenocarcinomas of the vulva, vagina and cervix are poorly defined. For endometrioid tumors of the female genital tract as well as for mucinous endometrial, ovarian and seromucinous ovarian carcinomas, the 3‑tiered FIGO grading system is recommended. For uterine neuroendocrine tumors the grading system of the gastrointestinal counterparts has been adopted...
July 2016: Der Pathologe
Colin J R Stewart, Maxine Crook, Adeline Tan
The aim of this study was to perform an immunohistochemical comparison of uterine tumour resembling ovarian sex cord-stromal tumour (UTROSCT) and other uterine lesions with sex cord-like (SCL) differentiation. Six UTROSCTs and 10 potential histological mimics with focal SCL elements were examined, the latter comprising three endometrial stromal nodules, three low-grade endometrial stromal sarcomas, three Müllerian adenosarcomas, and one case of adenomyosis. All cases were stained immunohistochemically for SF1, FOXL2, calretinin and inhibin, and for the less specific markers smooth muscle actin, desmin, CD10, CD56, CD99, cytokeratin, oestrogen receptor and progesterone receptor...
August 2016: Pathology
Wataru Kudaka, Hitoshi Inafuku, Yuko Iraha, Tomoko Nakamoto, Yusuke Taira, Rie Taira, Hisashi Kamiya, Maho Tsubakimoto, Yuichi Totsuka, Yukio Kuniyoshi, Tomoko Tamaki, Hajime Aoyama, Masanao Saio, Naoki Yoshimi, Yoichi Aoki
Background. A rare case of low-grade endometrial stromal sarcoma (LG-ESS) extending to inferior vena cava (IVC) and cardiac chambers. Case Report. A 40-year-old woman had IVC tumor, which was incidentally detected by abdominal ultrasonography during a routine medical checkup. CT scan revealed a tumor in IVC, right iliac and ovarian veins, which was derived from the uterus and extended into the right atrium and ventricle. The operation was performed, the heart and IVC were exposed, and cardiopulmonary bypass was initiated...
2016: Case Reports in Obstetrics and Gynecology
Francesca Micci, Ludmila Gorunova, Antonio Agostini, Lene E Johannessen, Marta Brunetti, Ben Davidson, Sverre Heim, Ioannis Panagopoulos
Recent cytogenetic and molecular investigations have improved our understanding of endometrial stromal tumors, including sarcomas (ESS), and helped redefine their classification into more pathogenetically meaningful categories. Because much more can be gained through such studies, we add information on another 22 ESS examined by karyotyping, PCR analysis, expression array analysis, and transcriptome sequencing. In spite of the known preference for certain pathogenetic pathways, we found considerable genetic heterogeneity in high-grade (HG) as well as in low-grade (LG) ESS...
November 2016: Genes, Chromosomes & Cancer
S Sivakumari, R Rajaraman, S Subbiah
Uterine sarcomas are rare, highly malignant tumours comprising < 1 % of all gynaecologic malignancies. To evaluate clinical presentation, histolopathologic pattern and outcome of uterine sarcomas presenting to a tertiary referral centre over an 8 year period (2004-2012). All histologically proven uterine sarcomas were retrospectively analysed. Clinical presentation, histology, treatment and outcome were analysed. Mean age was 42 years. Predominant histopathology was endometrial stromal sarcoma (n = 13); 9 were low grade, carcinosarcoma (n = 8) and leiomyosarcoma (n = 2)...
September 2015: Indian Journal of Surgical Oncology
Ayoma D Attygalle, Katherine Vroobel, Dorte Wren, Desmond P J Barton, Stephen J Hazell, Paola Dal Cin, Konrad Koelble, W Glenn McCluggage
Endometrial stromal sarcoma (ESS) characterized by YWHAE-NUTM2A/B genetic fusion is a recently recognized entity that is classified as a high-grade (HG) ESS in the 2014 World Health Organization Classification. These are myoinvasive neoplasms and typically contain a monomorphous HG round-cell cyclinD1-positive component with or without an accompanying low-grade (LG) component that is only focally positive/negative for cyclinD1. We report a case of YWHAE-NUTM2A/B ESS in a 46-yr-old woman that showed a number of unusual histologic features, including being entirely confined to the endometrium with no myoinvasion or lymphovascular space invasion...
May 11, 2016: International Journal of Gynecological Pathology
Josefa E O Vella, Raji Ganesan, Lynn Hirschowitz
Review of pulmonary biopsies received by Birmingham Women's Hospital to identify which gynecologic tumors most commonly metastasize to lung or pleura, and which may first present with pulmonary metastases. We reviewed all pulmonary biopsies over a 14-yr period. There were 25 lung and 9 pleural biopsies, from 33 patients. Twenty-one patients had known gynecologic tumors (1 vulval, 1 cervical, 9 endometrial, 4 uterine mesenchymal, and 6 ovarian). Eighteen of the 21 biopsies had been referred from other hospitals; in 4 cases review lead to an altered diagnosis...
May 10, 2016: International Journal of Gynecological Pathology
Jennelle C Hodge, Patrick P Bedroske, Kathryn E Pearce, William R Sukov
Diagnosis of endometrial stromal tumors (ESTs) can be challenging, particularly endometrial stromal sarcomas (ESSs) because of variable histologic appearance, long latency to recurrence, frequent metastases with unknown primary, and overlap with endometrial stromal nodules and undifferentiated uterine sarcomas. To enhance EST diagnosis, a break-apart strategy fluorescence in situ hybridization panel to detect JAZF1, PHF1, and YWHAE rearrangements was applied to a cohort of primary or metastatic endometrial stromal nodules, ESSs, or undifferentiated uterine sarcomas (36 cases for JAZF1, 24 of which were also assessed for PHF1 and YWHAE), 24 myometrium/endometrium controls, and 37 non-ESTs in the differential diagnosis...
July 2016: Journal of Molecular Diagnostics: JMD
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