1,721,181 research outputs found
Perivascular epithelioid cell tumors (PEComa) of the female genital tract: A challenging question for gynaecologic oncologist and pathologist
Perivascular epithelioid cell tumors (PEComa)s are mesenchymal neoplasms composed of perivascular epithelioid cells, which express both melanocytic and myogenic markers. These neoplastic cells are thought to arise from undifferentiated cells of the neural crest, or from myoblastic cells harbouring a molecular alteration leading to expression of melanocytic markers, or from pericytic cells. The majority of cases are benign, but a small subset behaves in a malignant fashion. Unfortunately, given their rarity, reliable criteria for predicting malignancy have yet to be established. PEComas of the female genital tract account for nearly 25% of PEComas of all body sites, and the most common site of occurrence is the uterine corpus; less common sites include the cervix, adnexa, vagina/vulva or broad or round ligament exceptionally. The present review aims to elucidate the clinical, pathological and molecular features of gynecological PEComas. Moreover, prognostic and therapeutic implications are also discussed
Endometriosis-associated extraovarian malignancies: A challenging question for the clinician and the pathologist
Endometriosis is an estrogen-dependent disease, which affects 10% of women in the reproductive age. Malignant transformation is an uncommon event, which affects approximately 0.7-2.5% of women, and, when it occurs, it involves ovarian and extraovarian sites in 75% and 25% of the cases, respectively. Endometriosis correlates with presentation of clear cell and endometrioid carcinoma of the ovary. Activation of phosphatidylinositol 3-kinase (PIK3) - protein kinase B (AKT) - mammalian target of rapamycin (mTOR) pathway, aberrant chromatin remodeling due to AT-rich interactive domain-containing protein 1A (ARID1A) mutation and inactivation of estrogen receptor-α signaling seem to play a major role in the carcinogenesis. To date, little data are available regarding endometriosisassociated extraovarian malignancies. The aim of the present study was to review the clinical, pathological and prognostic features of endometriosis-related neoplasms arising from extraovarian sites, with particular focus on intestinal malignancies, urinary tract malignancies and tumors arising from surgical scars
Perivascular epithelioid cell tumors (PEComa) of the female genital tract: A challenging question for gynaecologic oncologist and pathologist
Perivascular epithelioid cell tumors (PEComa)s are mesenchymal neoplasms composed of perivascular epithelioid cells, which express both melanocytic and myogenic markers. These neoplastic cells are thought to arise from undifferentiated cells of the neural crest, or from myoblastic cells harbouring a molecular alteration leading to expression of melanocytic markers, or from pericytic cells. The majority of cases are benign, but a small subset behaves in a malignant fashion. Unfortunately, given their rarity, reliable criteria for predicting malignancy have yet to be established. PEComas of the female genital tract account for nearly 25% of PEComas of all body sites, and the most common site of occurrence is the uterine corpus; less common sites include the cervix, adnexa, vagina/vulva or broad or round ligament exceptionally. The present review aims to elucidate the clinical, pathological and molecular features of gynecological PEComas. Moreover, prognostic and therapeutic implications are also discussed
Uterine smooth muscle tumors of unknown malignant potential: A challenging question
Uterine smooth muscle tumors of unknown malignant potential [STUMP]s are neoplasms with pathological features that preclude an equivocal diagnosis of leiomyosarcoma, but that do not fulfill the criteria for leiomyoma or its variants, and raise concerns that the tumors may behave in a malign fashion. Total hysterectomy with or without bilateral salpingo-oophorectomy is the standard treatment if fertility is completed, whereas myomectomy alone can be taken into consideration in young patients who desire to preserve childbearing potential. A careful surveillance every 6 months for 5 years and then yearly is strongly warranted. Patients with STUMP can relapse as either STUMP or leiomyosarcoma in approximately 11–13% of the cases, and their 5-year overall survival ranges from 92 to 100%. The present paper reviews the clinicopathological features of uterine STUMPs with a particular focus on most commonly accepted histopathological criteria for the diagnosis and on biological behaviour of these controversial neoplasms
Granular Cell Tumor of the tongue (Abrikossoff’ s tumor). A case report and review of the literature
Presumed early ovarian cancer with isolated tumor cells in para-aortic sentinel nodes
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Prognostic significance of the estrogen receptor beta (ERβ) isoforms ERβ1, ERβ2, and ERβ5 in advanced serous ovarian cancer
Objective In the present study we have examined the pattern of expression of the full length estrogen receptor β (ERβ1) and two ERβ splice variant isoforms (ERβ2, ERβ5) in well-characterized advanced serous ovarian cancers. Methods Immunohistochemistry was performed with ERβ1, ERβ2, and ERβ5 antibodies and results were correlated with pathological and clinical follow-up data. Expression of ERβ isoforms in a panel of ovarian cancer cell lines and human tumor xenografts was also assessed. Results Immunohistochemical staining revealed cellular compartment-specific distribution for each isoform in malignant ovarian tissues exhibiting both nuclear staining and cytoplasmic staining. Patients with cytoplasmic ERβ2 expression had significantly worse outcome (p = 0.006 at the multivariate analysis), the 5-year survival rate being nearly 28% for patients who did express cytoplasmic ERβ2, and 60% in negative patients. Cytoplasmic ERβ2 expression was also found to be significantly associated with chemoresistance. In concordance with clinical results both nuclear and cytoplasmic expressions were observed for the three isoforms in the cancer cell lines and human tumor xenografts tested. Conclusions This is the first study to uncover an unfavorable prognostic role of ERβ2 in advanced serous ovarian cancer. If anomalies of ERβ2 cytoplasmic expression could be demonstrated to represent an independent unfavorable prognostic marker and/or a marker predicting chemoresistance in advanced serous ovarian cancer, its immunohistochemical assessment at the time of surgery, could help to recognize candidates for clinical trials of new interventions. © 2013 Elsevier Inc. All rights reserved
TCGA Molecular Subgroups in Endometrial Undifferentiated/Dedifferentiated Carcinoma
We aimed to classify undifferentiated/dedifferentiated carcinoma (UDC/DDC) according to the four TCGA molecular subgroups of endometrial cancer: microsatellite-instable/hypermutated (MSI), POLE-mutant/ultramutated (POLE), copy-number-low/p53-wild-type (p53wt), and copy-number-high/p53-abnormal (p53abn), through a systematic review and meta-analysis. Electronic databases were searched from January 2013 to July 2019 for studies assessing the TCGA classification in endometrial UDC/DDC series. Pooled prevalence of each TCGA subgroup on the total UDC/DDCs was calculated. Three studies with 73 patients were included. Pooled prevalence of the TCGA subgroups were: 12.4% for the POLE subgroup, 44% for the MSI subgroup, 18.6% for the p53abn subgroup, 25% for the p53wt group. All TCGA groups are represented in UDC/DDC, with a predominance of the MSI group, indicating a biological heterogeneity. Hypermutated/ultramutated cancers constitute the majority of UDC/DDC, suggesting a crucial difference with other high-risk histologies of endometrial carcinoma
Pathologic response to neoadjuvant chemotherapy in advanced ovarian cancer: Utility of a scoring system to predict outcomes
Background Growing evidence supports the role of neoadjuvant chemotherapy in patients with advanced epithelial ovarian cancer. Currently, there is no shared histopathologic scoring system to assess pathologic response in the specimens obtained at interval surgery after neoadjuvant chemotherapy This review aims to summarize the literature on pathologic response, focusing on proposed scoring systems. Methods The systematic review was conducted according to the preferred reporting items for systematic reviews and meta-analyses (PRISMA) guidelines, focusing on the definition of pathologic response, its prognostic value, possible predictors, and future implications. Eighteen manuscripts focusing on pathologic response in epithelial ovarian cancer were selected for analysis. Results Overall, eight histopathologic scoring systems to evaluate pathologic response have been proposed. There are currently no available markers (serum, radiological, genomic) to select which patients could achieve the highest benefit from neoadjuvant chemotherapy experiencing a complete pathologic response. A three-tier scoring system (CRS) based on omental assessment and which classifies the response to neoadjuvant chemotherapy has been validated in external cohorts of epithelial ovarian cancer. This scoring system demonstrated adequate interobserver reproducibility. Data is limited on the pathologic complete response rate changes according to chemotherapy regimen. Conclusions A histopathologic scoring system endowed with prognostic value could be helpful in personalizing the treatment decision in patients with epithelial ovarian cancer
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