scholarly journals High-grade Endometrial Stromal Sarcoma With BCOR Gene Alterations is Recommended as an Independent Subtype of Endometrial Carcinoma: A Case Report

2020 ◽  
Author(s):  
Feng LING ◽  
Sibei Ruan ◽  
Na Li ◽  
XiaoMing Xiong ◽  
DongMei Zhao ◽  
...  
Keyword(s):  
Endometrial Stromal Sarcoma ◽  
World Health ◽  
Uterine Leiomyosarcoma ◽  
Tumour Mass ◽  
High Grade ◽  
Case Presentation ◽  
Stromal Sarcoma ◽  
Health Organization ◽  
Pathological Report ◽  
Gene Alterations

Abstract Background:The uterine leiomyosarcoma combined high-grade endometrial stromal sarcoma (HGESS) with BCOR gene alterations is exceedingly rare. The subtype of YWHAE-FAM22 HGESS is shown in the current World Health Organization classification, but not HGESS with BCOR alterations. Case presentation: We reported such a case in which HGESS with BCOR gene alterations and leiomyosarcoma coexist in a patient. And, most impressively, HGESS with BCOR gene alterations caused ovarian and pelvic metastases, although its volume was less than 1% of leiomyosarcoma.Conclusions: Given the more aggressive of HGESS with BCOR gene alterations, we suggest that it is classified as an independent subtype of HGESS, and when it coexists with other types of tumors, the HGESS with BCOR gene alterations needs to be noted in the pathological report even if it accounts for less than 1% of the tumour mass.

Is high-grade endometrial stromal sarcoma the key to disease prognosis in cases of coexisting leiomyosarcoma? A case report

2020 ◽  
Author(s):  
Feng Ling ◽  
Sibei Ruan ◽  
XiaoMing Xiong ◽  
Na Li ◽  
DongMei Zhao ◽  
...  
Keyword(s):  
Case Report ◽  
Endometrial Stromal Sarcoma ◽  
Uterine Leiomyosarcoma ◽  
Tumour Mass ◽  
High Grade ◽  
Case Presentation ◽  
Stromal Sarcoma ◽  
Disease Prognosis ◽  
Pathological Report ◽  
Gene Alterations

Abstract Background:The uterine leiomyosarcoma combined high-grade endometrial stromal sarcoma (ESS) is quite rare.Case presentation: We reported such a case in which high-grade ESS with BCOR gene alterations and leiomyosarcoma coexist in a patient. And, most impressively, high-grade ESS with BCOR gene alterations caused ovarian and pelvic metastases, although its volume was less than 1% of leiomyosarcoma.Conclusions: We suggested that when both high-grade ESS BCOR gene alterations and leiomyosarcoma are present in a patient, the high-grade ESS needs to be noted in the pathological report even if it accounts for less than 1% of the tumour mass.

scholarly journals Can Proliferation Biomarkers Reliably Predict Recurrence in World Health Organization 2003 Defined Endometrial Stromal Sarcoma, Low Grade?

PLoS ONE ◽  
2013 ◽  
Vol 8 (10) ◽  
pp. e75899 ◽  
Author(s):  
Weiwei Feng ◽  
Anais Malpica ◽  
Ivar Skaland ◽  
Einar Gudlaugsson ◽  
Stanley J. Robboy ◽  
...  
Keyword(s):  
World Health Organization ◽  
Endometrial Stromal Sarcoma ◽  
World Health ◽  
Low Grade ◽  
Stromal Sarcoma ◽  
Health Organization

scholarly journals Primary ovarian high-grade endometrial stromal sarcoma: a case report

2021 ◽  
Vol 15 (1) ◽  
Author(s):  
Ji Sun Lee ◽  
Dayong Lee ◽  
Jisun Lee ◽  
Man-Hoon Han ◽  
Dae Gy Hong ◽  
...  
Keyword(s):  
Intraperitoneal Chemotherapy ◽  
Hyperthermic Intraperitoneal Chemotherapy ◽  
Standard Treatment ◽  
Therapeutic Option ◽  
Endometrial Stromal Sarcoma ◽  
Asian Woman ◽  
High Grade ◽  
Case Presentation ◽  
Stromal Sarcoma ◽  
Initial Surgery

Abstract Background Primary ovarian high-grade endometrial stromal sarcoma is a very rare disease. Even though it has poor prognosis, the gold standard treatment has not been established owing to its rarity. This report aimed to present therapeutic options for primary ovarian high-grade endometrial stromal sarcoma. Case presentation A 49-year-old Asian woman presented with disseminated intravascular coagulation due to ruptured primary high-grade ovarian endometrial stromal sarcoma with multiple intraperitoneal metastases. After the initial surgery, the patient underwent adjuvant chemotherapy with three courses of Adriamycin (75 mg/m2). We performed the secondary debulking operation including total hysterectomy, metastasectomy, omentectomy, peritonectomy, appendectomy, and hyperthermic intraperitoneal chemotherapy (paclitaxel 175 mg/m2). Currently she has been alive for 28 months under a new chemotherapy regimen. Conclusion We suggest cytoreductive surgery with hyperthermic intraperitoneal chemotherapy could be a therapeutic option for primary high-grade ovarian endometrial stromal sarcoma with peritoneal dissemination.

scholarly journals Case Report: Unusual High-Grade Diffuse Leptomeningeal Glioneuronal Tumor Mimicking Tuberculous Meningitis in a Child From an Endemic Region

2021 ◽  
Vol 9 ◽  
Author(s):  
Yong Guang Teh ◽  
Nornazirah Azizan ◽  
Nur Atifah Mohd Naim ◽  
Chiak Yot Ng ◽  
Ke Juin Wong ◽  
...  
Keyword(s):  
Mr Spectroscopy ◽  
Who Classification ◽  
World Health ◽  
Glioneuronal Tumor ◽  
High Grade ◽  
Endemic Region ◽  
Case Presentation ◽  
Diffuse Leptomeningeal Glioneuronal Tumor ◽  
Health Organization

Background: Diffuse leptomeningeal glioneuronal tumor (DL-GNT) is a new entity described in the 2016 World Health Organization (WHO) classification of brain tumors. While DL-GNT is predominantly an indolent tumor that affects young boys, high-grade DL-GNT is unusual and seldom reported in children.Case Presentation: In this report, we describe the challenges and pitfalls associated with diagnosing this high-grade variant in a tuberculosis-endemic region. We highlight the importance of identifying non-typical imaging findings, i.e., non-enhancing cystic lesions with high T2 signal along the leptomeningeal surface, that may expedite the diagnosis of this condition. Histopathologic correlations with MR spectroscopy findings are also discussed.Conclusion: We provide the first clinical imaging report of utilizing MR spectroscopy to distinguish DL-GNT from tuberculosis with histopathologic correlation.

Metody cytogenetyki molekularnej w różnicowaniu agresywnych B-NHL

2019 ◽  
Vol 50 (3) ◽  
pp. 109-115
Author(s):  
Beata Grygalewicz
Keyword(s):  
B Cell ◽  
Cell Lymphoma ◽  
B Cell Lymphoma ◽  
World Health ◽  
High Grade ◽  
Non Hodgkin Lymphoma ◽  
Large B Cell Lymphoma ◽  
Health Organization ◽  
Large B Cell

StreszczenieB-komórkowe agresywne chłoniaki nieziarnicze (B-cell non-Hodgkin lymphoma – B-NHL) to heterogenna grupa nowotworów układu chłonnego, wywodząca się z obwodowych limfocytów B. Aberracje cytogenetyczne towarzyszące B-NHL to najczęściej translokacje onkogenów takich jak MYC, BCL2, BCL6 w okolice genowych loci dla łańcuchów ciężkich lub lekkich immunoglobulin. W niektórych przypadkach dochodzi do wystąpienia kilku wymienionych aberracji jednocześnie, tak jak w przypadkach przebiegających z równoczesną translokacją genów MYC i BCL2 (double hit), niekiedy także z obecnością rearanżacji BCL6 (triple hit). Takie chłoniaki cechuje szczególnie agresywny przebieg kliniczny. Obecnie molekularna diagnostyka cytogenetyczna przy użyciu techniki fluorescencyjnej hybrydyzacji in situ (FISH) oraz, w niektórych przypadkach, aCGH jest niezbędnym narzędziem rozpoznawania, klasyfikowania i oceny stopnia zaawansowania agresywnych, nieziarniczych chłoniaków B-komórkowych. Technika mikromacierzy CGH (aCGH) była kluczowym elementem wyróżnienia prowizorycznej grupy chłoniaków Burkitt-like z aberracją chromosomu 11q (Burkitt-like lymphoma with 11q aberration – BLL, 11q) w najnowszej klasyfikacji nowotworów układu chłonnego Światowej Organizacji Zdrowia (World Health Organization – WHO) z 2016 r. Omówione zostaną sposoby różnicowania na poziomie cytogenetycznym takich chłoniaków jak: chłoniak Burkitta (Burkitt lymphoma – BL), chłoniak rozlany z dużych komórek B (diffuse large B-cell lymphoma – DLBCL) oraz 2 nowych jednostek klasyfikacji WHO 2016, czyli chłoniaka z komórek B wysokiego stopnia złośliwości z obecnością translokacji MYC i BCL2 i/lub BCL6 (high-grade B-cell lymphoma HGBL, with MYC and BCL2 and/or BCL6 translocations) oraz chłoniaka BLL, 11q.

scholarly journals T2 mapping of the peritumoral infiltration zone of glioblastoma and anaplastic astrocytoma

2021 ◽  
pp. 197140092198932
Author(s):  
Timo Alexander Auer ◽  
Maike Kern ◽  
Uli Fehrenbach ◽  
Yasemin Tanyldizi ◽  
Martin Misch ◽  
...  
Keyword(s):  
Relaxation Time ◽  
Isocitrate Dehydrogenase ◽  
Anaplastic Astrocytoma ◽  
T2 Mapping ◽  
Relaxation Times ◽  
Region Of Interest ◽  
World Health ◽  
High Grade ◽  
High Grade Gliomas ◽  
Health Organization

Purpose To characterise peritumoral zones in glioblastoma and anaplastic astrocytoma evaluating T2 values using T2 mapping sequences. Materials and methods In this study, 41 patients with histopathologically confirmed World Health Organization high grade gliomas and preoperative magnetic resonance imaging examinations were retrospectively identified and enrolled. High grade gliomas were differentiated: (a) by grade, glioblastoma versus anaplastic astrocytoma; and (b) by isocitrate dehydrogenase mutational state, mutated versus wildtype. T2 map relaxation times were assessed from the tumour centre to peritumoral zones by means of a region of interest and calculated pixelwise by using a fit model. Results Significant differences between T2 values evaluated from the tumour centre to the peritumoral zone were found between glioblastoma and anaplastic astrocytoma, showing a higher decrease in signal intensity (T2 value) from tumour centre to periphery for glioblastoma ( P = 0.0049 – fit-model: glioblastoma –25.02± 19.89 (–54–10); anaplastic astrocytoma –5.57±22.94 (–51–47)). Similar results were found when the cohort was subdivided by their isocitrate dehydrogenase profile, showing an increased drawdown from tumour centre to periphery for wildtype in comparison to mutated isocitrate dehydrogenase ( P = 0.0430 – fit model: isocitrate dehydrogenase wildtype –10.35±16.20 (–51) – 0; isocitrate dehydrogenase mutated 12.14±21.24 (–15–47)). A strong statistical proof for both subgroup analyses ( P = 0.9987 – glioblastoma R2 0.93±0.08; anaplastic astrocytoma R2 0.94±0.15) was found. Conclusion Peritumoral T2 mapping relaxation time tissue behaviour of glioblastoma differs from anaplastic astrocytoma. Significant differences in T2 values, using T2 mapping relaxation time, were found between glioblastoma and anaplastic astrocytoma, capturing the tumour centre to the peritumoral zone. A similar curve progression from tumour centre to peritumoral zone was found for isocitrate dehydrogenase wildtype high grade gliomas in comparison to isocitrate dehydrogenase mutated high grade gliomas. This finding is in accordance with the biologically more aggressive behaviour of isocitrate dehydrogenase wildtype in comparison to isocitrate dehydrogenase mutated high grade gliomas. These results emphasize the potential of mapping techniques to reflect the tissue composition of high grade gliomas.

scholarly journals Oral Abstract

2016 ◽  
Author(s):  
Dharma Ram
Keyword(s):  
Survival Data ◽  
Eligible Patient ◽  
Stage Iv ◽  
Endometrial Stromal Sarcoma ◽  
Uterine Sarcoma ◽  
Low Grade ◽  
Uterine Leiomyosarcoma ◽  
High Grade ◽  
Lost To Follow Up

Introduction: Uterine sarcoma accounts for nearly 3% of all uterine malignancies. They have 4 major pathology includes endometrial stromal sarcoma high grade, ESS low grade, uterine leiomyosarcoma (uLMS) and undifferentiated uterine sarcoma (UUS). Recent WHO classification 2014, recognizes low grade ESS and high grade ESS as distinct entity. They differ from endometrial carcinoma in their aggressive nature and poor prognosis. We review our database and found total 44 eligible patient treated at our institute. Materials and Methods: Its retrospective analysis of computer based database of our institute from January 2009 to December 2015. We analyzed demographic, pathological, treatment and survival data. Results: Total 44 patient treated for uterine sarcoma at our institute. Among these 16 were operated at our institute during study period. Here we reporting results of operated patients at our institute. The histological diagnosis LMS (5/16), ESS-L (4/16), MMMT (3/16), UUS (3/16) and ESS-H (1/16). Stage distribution was stage I, (6/16) stage II, (5/16) stage III, (3/16) stage IV, (0/16) and unknown stage (2/16). Two patients underwent completion surgery for outside myomectomy. The adjuvant treatment was CT in 3/16, CT with RT in 7/16, HT in 4/16 and one lost to follow up with one was put on observation. Median follow up is 30 month with 14 patients alive and one lost to follow up. At last follow up 4 patients alive with metastatic disease and 10 patients alive with no evidence of disease. Conclusion: Uterine sarcoma are uncommon disease with

scholarly journals Nasopharyngeal non-intestinal-type adenocarcinoma: a case report and updated review of the literature

2017 ◽  
Vol 24 (1) ◽  
pp. 55 ◽  
Author(s):  
C. Jain ◽  
L. Caulley ◽  
K.I. Macdonald ◽  
B. Purgina ◽  
C.K. Lai ◽  
...  
Keyword(s):  
Radiation Treatment ◽  
Rare Condition ◽  
Intestinal Type ◽  
World Health ◽  
Primary Radiation ◽  
High Grade ◽  
Rare Tumours ◽  
Intestinal Type Adenocarcinoma ◽  
Health Organization ◽  
Nasopharyngeal Masses

Background Non-intestinal-type adenocarcinoma is a malignancy traditionally found in the sinonasal cavity. To our knowledge, this case is the first reported of this rare condition originating in the nasopharynx.Case Presentation A 67-year-old woman with nasopharyngeal non-intestinal-type adenocarcinoma, with an accompanying parapharyngeal mass received primary radiation treatment for both lesions. Her tumour subsequently persisted, with a concomitant conversion in pathology from a low- to a high-grade malignancy.Results Non-intestinal-type and intestinal-type adenocarcinomas of the nasopharynx are extremely rare tumours and do not appear in the World Health Organization classification system. We review the pathophysiologic features of these malignancies and propose modifications to the current classification system.Conclusions Non-intestinal-type adenocarcinoma should be included in the differential diagnosis of nasopharyngeal masses. In our experience, this tumour in this location showed a partial response to primary radiation but later converted from a low- to a high-grade adenocarcinoma.

scholarly journals Genetic Abnormalities, Clonal Evolution, and Cancer Stem Cells of Brain Tumors

2018 ◽  
Vol 6 (4) ◽  
pp. 85 ◽  
Author(s):  
Ugo Testa ◽  
Germana Castelli ◽  
Elvira Pelosi
Keyword(s):  
Brain Tumors ◽  
Clonal Evolution ◽  
Pediatric Brain Tumors ◽  
Genetic Alterations ◽  
World Health ◽  
Driver Mutations ◽  
Low Grade ◽  
High Grade ◽  
Genetic Profiling ◽  
Health Organization

Brain tumors are highly heterogeneous and have been classified by the World Health Organization in various histological and molecular subtypes. Gliomas have been classified as ranging from low-grade astrocytomas and oligodendrogliomas to high-grade astrocytomas or glioblastomas. These tumors are characterized by a peculiar pattern of genetic alterations. Pediatric high-grade gliomas are histologically indistinguishable from adult glioblastomas, but they are considered distinct from adult glioblastomas because they possess a different spectrum of driver mutations (genes encoding histones H3.3 and H3.1). Medulloblastomas, the most frequent pediatric brain tumors, are considered to be of embryonic derivation and are currently subdivided into distinct subgroups depending on histological features and genetic profiling. There is emerging evidence that brain tumors are maintained by a special neural or glial stem cell-like population that self-renews and gives rise to differentiated progeny. In many instances, the prognosis of the majority of brain tumors remains negative and there is hope that the new acquisition of information on the molecular and cellular bases of these tumors will be translated in the development of new, more active treatments.

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