Rare Tumors最新文献

{"title":"Quality of life of patients with soft tissue sarcoma treated with doxorubicin in the ANNOUNCE phase III clinical trial","authors":"B. V. Van Tine, A. Krarup-Hansen, L. Hess, A. A. Abdul Razak, V. Soldatenkova, Jennifer Wright, S. Park","doi":"10.1177/20363613221100033","DOIUrl":"https://doi.org/10.1177/20363613221100033","url":null,"abstract":"Background: Patient-reported outcomes (PROs), including health-related quality of life, are recommended to be routinely collected in clinical trials, but data are limited from trials of sarcoma patients. In this analysis, pooled PRO data are reported from patients with advanced or metastatic soft tissue sarcoma (STS) enrolled to the ANNOUNCE phase III trial of doxorubicin-based therapy. Methods: ANNOUNCE was a phase III trial that randomized 509 patients with STS to receive up to eight cycles of doxorubicin with olaratumab or placebo, followed by single-agent olaratumab or placebo. Dexrazoxane was allowed at any cycle of treatment. Participants completed the European Organization for Research and Treatment of Cancer Quality of Life Questionnaire-Core 30 (EORTC QLQ-C30, which is scored 0–100), and Brief Pain Inventory Short Form Modified (mBPI-sf, scored from 0–10) at each treatment cycle. A descriptive analysis of the longitudinal data was conducted overall and by cumulative dose of doxorubicin received to inform the clinical care of patients with STS. Worsening on the QLQ-C30 was defined as a change of 10 points or more at any post-baseline assessment. Worsening on the mPBI-sf was defined as an increase of ≥2 points from baseline. Results: The majority of participants completed the baseline and at least one subsequent PRO assessment within the trial (n = 460, 90.4% EORTC QLQ-C30; n = 454, 89.2%, mBPI-sf). Patients with STS enrolled to the ANNOUNCE trial had clinically meaningful problems with physical function and pain before initiating doxorubicin. Overall, those with fewer symptoms or better function at baseline received higher cumulative doxorubicin dose throughout the study. At baseline, mean QLQ-C30 fatigue was 29.9 with a median time to first worsening of 0.9 months, and mean nausea/vomiting was 6.5 with 1.4 months until worsening; mean physical function was 78.3 with median time to worsening of 2.1 months and mean health status was 66.8 with median time to first worsening of 1.6 months. Median time to worsening of pain was 7.9 months. Conclusion: Patients with advanced or metastatic sarcoma reported a relatively rapid decline in PROs during doxorubicin-based treatment, with patients with poorer symptoms at baseline (specifically fatigue), subsequently receiving less doxorubicin therapy. The availability of detailed summary data from the patient perspective during doxorubicin-based treatment may inform future care of these patients and can provide a resource for the development of PRO endpoints in future trials.","PeriodicalId":46078,"journal":{"name":"Rare Tumors","volume":" ","pages":""},"PeriodicalIF":0.9,"publicationDate":"2022-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"47755898","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Polymorphous low-grade neuroepithelial tumor of the young: Rare tumor and review of the literature","authors":"Ali H. Palejwala, Christen M. O’Neal, Michael R Quinton, J. Battiste, J. Peterson, I. Dunn","doi":"10.1177/20363613221083360","DOIUrl":"https://doi.org/10.1177/20363613221083360","url":null,"abstract":"Polymorphous low-grade neuroepithelial tumor of the young (PLNTY) is a recently described low-grade neuroepithelial tumor with an infiltrative growth pattern and oligodendrocyte-like cells that are CD34 immunopositive. Correlating histology and results from molecular testing is critical to correctly diagnosing PLNTY, as its histologic appearance is similar to oligodendrogliomas and shares genetic abnormalities common to other low-grade epilepsy associated tumors (LEATs). In this case report, we describe a 31-year-old female with intractable epilepsy found to have a temporal mass and diagnosed with PLNTY after histopathologic and molecular testing. We describe the radiographic, histologic, and genetic features in relation to the epileptic and oncologic outcomes for this patient. Then, we compare these features and outcomes to other cases of PLNTY described in the literature.","PeriodicalId":46078,"journal":{"name":"Rare Tumors","volume":" ","pages":""},"PeriodicalIF":0.9,"publicationDate":"2022-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"43570520","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Gastric adenocarcinoma of the fundic gland: A review of clinicopathological characteristics, treatment and prognosis.","authors":"Xiang-Yu Meng,&nbsp;Guang Yang,&nbsp;Cheng-Ji Dong,&nbsp;Ru-Yi Zheng","doi":"10.1177/20363613211060171","DOIUrl":"https://doi.org/10.1177/20363613211060171","url":null,"abstract":"<p><p>Gastric adenocarcinoma of the fundic gland is a rare, well-differentiated gastric cancer entity, and very few patients transition to poorly differentiated tubular adenocarcinoma during progression. Gastric adenocarcinoma of the fundic gland originates from the mucosa of the gastric fundic gland, usually without chronic gastritis or intestinal metaplasia. Histologically, the tumor cells are closely arranged to form anastomosing tubular glands, and more than 95% of tumor cells differentiate towards chief cells. Most gastric adenocarcinoma of the fundic gland cases are characterized by submucosal involvement, but the tumor volume is usually small, with lymphatic and vascular invasion rarely observed. Therefore, endoscopic submucosal dissection can be an ideal treatment, leading to a favorable prognosis, and recurrence and metastasis of the disease are uncommon.</p>","PeriodicalId":46078,"journal":{"name":"Rare Tumors","volume":" ","pages":"20363613211060171"},"PeriodicalIF":0.9,"publicationDate":"2021-12-14","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://ftp.ncbi.nlm.nih.gov/pub/pmc/oa_pdf/1e/33/10.1177_20363613211060171.PMC8679019.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"39739295","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"The challenge to differentiate between sarcoma or adrenal carcinoma-an observational study.","authors":"Eva M Dobrindt,&nbsp;Wolfgang Saeger,&nbsp;Hendrik Bläker,&nbsp;Martina T Mogl,&nbsp;Marcus Bahra,&nbsp;Johann Pratschke,&nbsp;Nada Rayes","doi":"10.1177/20363613211057746","DOIUrl":"https://doi.org/10.1177/20363613211057746","url":null,"abstract":"<p><strong>Background: </strong>Adrenal sarcomas are rare malignant tumors with structural and clinical similarities to sarcomatoid adrenocortical carcinoma. Preoperative diagnosis of tumors of the adrenal gland can be challenging and often misleading thus detaining patients from appropriate oncological strategies.</p><p><strong>Objective: </strong>This analysis of a case series evaluated the predictive capability of the primary clinical diagnosis in case of malignancies of the adrenal gland.</p><p><strong>Methods: </strong>Thirty two patients were treated from 2009 to 2015 at our clinic and analyzed retrospectively. All patients had computed tomography and/or magnet resonance imaging and a primary histopathological examination at our institution after surgery. Ten questionable cases were surveyed by a reference pathologist.</p><p><strong>Results: </strong>Twelve out of 32 diagnoses had to be revised (37.5%). Only 15 out of 24 tumors primarily classified as adrenocortical carcinoma were finally described as primary adrenal cancer. We found two leiomyosarcomas, one liposarcoma, one sarcomatoid adrenocortical carcinoma, and one epitheloid angiosarcoma among 12 misleading diagnoses. Other tumors turned out to be metastases of lung, hepatocellular, and neuroendocrine tumors. Larger tumors were significantly more often correctly diagnosed compared to smaller tumors. Four patients of the group of revised diagnoses died whereas all patients with confirmed diagnoses survived during the follow-up.</p><p><strong>Conclusion: </strong>Preoperative assessment of tumors of the adrenal gland is still challenging. In case of wrong primary diagnosis, the prognosis could be impaired due to inadequate surgical procedures or insufficient preoperative oncological treatment.</p>","PeriodicalId":46078,"journal":{"name":"Rare Tumors","volume":" ","pages":"20363613211057746"},"PeriodicalIF":0.9,"publicationDate":"2021-12-10","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://ftp.ncbi.nlm.nih.gov/pub/pmc/oa_pdf/89/7f/10.1177_20363613211057746.PMC8669116.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"39821737","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Understanding the role of indoleamine-2,3-dioxygenase and stromal differentiation in rare subtype endometrial cancer.","authors":"Dongling Wu,&nbsp;Sean Hacking,&nbsp;Jin Cao,&nbsp;Mansoor Nasim","doi":"10.1177/20363613211044690","DOIUrl":"https://doi.org/10.1177/20363613211044690","url":null,"abstract":"<p><p>Endometrial cancer (EC) is a disease with good and poor prognostic subtypes. Dedifferentiated endometrial carcinoma (DEC), undifferentiated endometrial carcinoma (UEC), and clear cell endometrial carcinoma (CEC) are rare high-grade tumors, associated with a poor prognosis and high pathologic stage. Many studies have been performed on the programmed death-ligand 1 (PD-L1) axis mainly focus on endometrioid adenocarcinomas and little research has been done on rare subtypes. The present body of work aims to evaluate the role of indoleamine-2,3-dioxygenase (IDO-1) and stromal differentiation (SD), their correlation with clinicopathologic features and overall survival. Here we found that positive IDO-1 expression in immune cells correlated with worse disease-free survival (<i>p</i> = 0.02), recurrence (<i>p</i> = 0.03), high pathologic tumor stage (<i>p</i> = 0.024), lymph node metastasis (<i>p</i> = 0.028), and myometrial invasion (<i>p</i> = 0.03). Our findings suggest IDO-1 to be relevant in both MMR intact and deficient tumors; however, >20% immune cell staining was restricted to MMR deficient cancers. For the stroma, immature, myxoid differentiation was found to correlate with worse disease-free survival (<i>p</i> = 0.04). We also found the correlation between IDO-1 expression and immature stroma. Looking forward, IDO-1 could be promising for immunotherapy and SD could be the answer to clinical heterogeneity.</p>","PeriodicalId":46078,"journal":{"name":"Rare Tumors","volume":" ","pages":"20363613211044690"},"PeriodicalIF":0.9,"publicationDate":"2021-12-07","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://ftp.ncbi.nlm.nih.gov/pub/pmc/oa_pdf/86/45/10.1177_20363613211044690.PMC8655461.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"39719293","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"National advisory panels for paediatric cancer in the UK as an example of rare cancer multidisciplinary team meetings.","authors":"S Brown,&nbsp;J Bate","doi":"10.1177/20363613211052503","DOIUrl":"https://doi.org/10.1177/20363613211052503","url":null,"abstract":"Creative Commons Non Commercial CC BY-NC: This article is distributed under the terms of the Creative Commons Attribution-NonCommercial 4.0 License (http://www.creativecommons.org/licenses/by-nc/4.0/) which permits non-commercial use, reproduction and distribution of the work without further permission provided the original work is attributed as specified on the SAGE and Open Access pages (https://us.sagepub.com/en-us/nam/open-access-at-sage). We recognise the proposed advantages of multidisciplinary team (MDT) working that Rosell et al.1 describe in their evaluation paper. In the UK, multidisciplinary team (MDT) working is mandated in the National Health Service (NHS) Cancer Plan. Benefits of this model include improved evidence-based treatment decisions, coordination of care and education for MDT members, along with improved patient outcomes and clinical trial recruitment.2–5 Childhood cancer is rare. There are approximately 1900 new cases per year in the UK, with childhood cancer cases comprising less than 1% of all new cancer cases.6 All children with suspected cancer in the UK are referred to one of 19 principle treatment centres (PTC) which together comprise a network of tertiary specialist cancer services for diagnosis, treatment and coordination of care for each patient. It is standard of care for every child with a new diagnosis to be discussed at a PTC MDT meeting at which recommendations are formulated for management. In addition to PTC MDT meetings, there has been a recent rise in a number of virtual national advisory panels (NAP) for children’s cancer in the UK. These panels have developed alongside increasing centralisation of cancer services and enhanced complexities of patient management. Advice may be sought from these panels for individual patients with specific disease types including sarcoma (panel established in 2011), histiocytosis (2013), ependymoma (2015) leukaemia (2016), neuroblastoma (2017) and renal cancers (2017) As of January 2019, collectively there had been 920 referrals to the NAPs in the UK. Rosell et al. acknowledge rare cancers and complex cases may benefit from referrals to such forums to gather further clinical expertise, particularly when the evidence base is lacking or treatment pathway is not clearly defined. In contrast to the Swedish model described in Rosell’s evaluation, NAP referral is not mandated at either diagnosis or relapse. Furthermore, NAPs are distinct from PTC MDT meetings, involving national experts for particular cancer types offering an advisory role only ensuring overall responsibility for the patient remains that of the referring team. While the primary role of the NAPs is not to ensure equitable access to treatment, there is a degree of overlap with the UK Experimental Cancer Medicine Network regional meetings (ECMC). These meetings are designed to discuss cases at time of relapse to ensure equitable access to clinical trials, irrespective of geography. In contrast to the NAPs, it is an","PeriodicalId":46078,"journal":{"name":"Rare Tumors","volume":" ","pages":"20363613211052503"},"PeriodicalIF":0.9,"publicationDate":"2021-10-29","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://ftp.ncbi.nlm.nih.gov/pub/pmc/oa_pdf/0b/93/10.1177_20363613211052503.PMC8559199.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"39588927","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Pure primary non-gestational choriocarcinoma originating in the ovary: A case report and literature review.","authors":"Xiujie Yu,&nbsp;Qiuyue Du,&nbsp;Xiaojing Zhang,&nbsp;Yixin Liu,&nbsp;Yan Shen","doi":"10.1177/20363613211052506","DOIUrl":"https://doi.org/10.1177/20363613211052506","url":null,"abstract":"<p><p>Non-gestational choriocarcinoma (NGCO) of the ovary is rare, with a prevalence of less than 0.6% of all ovarian germ-cell tumors; and when found with other germ cell tumors, pure NGCO is exceedingly rare. We herein report the case of a 22-year-old woman who complained of menstrual disorders for over 2 months. MRI examination revealed an 11.4 cm right adnexal mass of the uterus, and the patients manifested an elevated serum level of β-hCG of 77,928 mIU/ml. Fertility-preserving surgery was performed, and the pathologic diagnosis was pure NGCO; immunohistochemical staining showed cancer cells that were positive for β-hCG, CK, hPL, SALL4, and Ki-67 (>80% of cells stained). We performed polymorphic DNA analysis and non-gestational origin was confirmed. The patient was then treated with six courses of chemotherapy with a BEP regimen, after which her serum β-hCG levels declined to normal levels, and she was free of disease at the 30-month follow-up.</p>","PeriodicalId":46078,"journal":{"name":"Rare Tumors","volume":" ","pages":"20363613211052506"},"PeriodicalIF":0.9,"publicationDate":"2021-10-08","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://ftp.ncbi.nlm.nih.gov/pub/pmc/oa_pdf/ba/f0/10.1177_20363613211052506.PMC8504641.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"39518124","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Phase II study of dacarbazine given with modern prophylactic anti-emetics and growth factor support to patients with metastatic, resistant soft tissue, and bone sarcoma.","authors":"Brian A Van Tine,&nbsp;Mia C Weiss,&nbsp;Angela C Hirbe,&nbsp;Peter J Oppelt,&nbsp;Sarah Abaricia,&nbsp;Kathryn Trinkaus,&nbsp;Jingqin Luo,&nbsp;Shellie Berry,&nbsp;Tyler Ruff,&nbsp;Cheryl Callahan,&nbsp;Jacqui Toensikoetter,&nbsp;Jessica Ley,&nbsp;Marilyn J Siegel,&nbsp;Farrokh Dehdashti,&nbsp;Barry A Siegel,&nbsp;Douglas R Adkins","doi":"10.1177/20363613211052498","DOIUrl":"https://doi.org/10.1177/20363613211052498","url":null,"abstract":"<p><p>Historically, administration of dacarbazine to sarcoma patients was limited by frequent treat-ment-related nausea/vomiting and neutropenia. These toxicities are now largely preventable with contemporary antiemetics and growth factor support. In this single-arm, phase II study, dacarbazine 850 mg/m² was given on day 1 of each 3-week cycle until disease progression or intolerance with prophylactic serotonin-3 receptor, neurokinin-1 antagonists, corticosteroids, and pegfilgrastim. Coprimary endpoints included clinical benefit rate (CBR), and any grade of nausea/vomiting and/or grade 3-4 neutropenia. With a sample size of 80 patients, >24 patients with clinical benefit would indicate that the CBR exceeds the historical (<20%) [Power 0.80; alpha 0.05]. In addition, we hypothesized that the rates of nausea/vomiting would be 27% and grade 3-4 neutropenia would be 1% (historical: 90% and 36%, respectively) [power 0.95; alpha 0.05]. The CBR was 30% (24 patients: PR-2 and stable-22). The rate of nausea/vomiting was 37.5% (31 patients) and grades 3-4 neutropenia was 10% (8 patients). Median time-to-progression was 8.1 weeks (95% CI 8-9.7) and median overall survival was 35.8 weeks (95% CI 26.2-55.4). PET scans demonstrated no association with response. Modern prophylactic anti-emetics and pegfilgrastim given with dacarbazine reduced the rates of treatment related nausea/vomiting and serious neutropenia.</p>","PeriodicalId":46078,"journal":{"name":"Rare Tumors","volume":" ","pages":"20363613211052498"},"PeriodicalIF":0.9,"publicationDate":"2021-10-08","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://ftp.ncbi.nlm.nih.gov/pub/pmc/oa_pdf/ae/23/10.1177_20363613211052498.PMC8504645.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"39518123","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"TP 53 status and estrogen receptor-beta in triple negative breast cancer management in Africa: Time to rethink regime management of triple negative breast cancer and save more lives in Nigeria.","authors":"Martin Arinzechukwu Nzegwu,&nbsp;Onyekachi Nwokoro,&nbsp;Christian Nnamani,&nbsp;Vincent C Enemuo,&nbsp;Victor Ifeanyichukwu Nzegwu,&nbsp;Ogochukwu Nwoye,&nbsp;Anthony Edeh,&nbsp;Kenneth Nwankwo","doi":"10.1177/20363613211050355","DOIUrl":"https://doi.org/10.1177/20363613211050355","url":null,"abstract":"Creative Commons Non Commercial CC BY-NC: This article is distributed under the terms of the Creative Commons Attribution-NonCommercial 4.0 License (https://www.creativecommons.org/licenses/by-nc/4.0/) which permits non-commercial use, reproduction and distribution of the work without further permission provided the original work is attributed as specified on the SAGE and Open Access pages (https://us.sagepub.com/en-us/nam/open-access-at-sage). Although Estrogen receptor alpha (ESR1) is now routinely used in typing breast cancers in most of Eastern Nigeria, where it is used as a major prognostic and predictive factor in treatment outcome.1,2 ESR1 negative breast cancer remains a significant subtype contributing to (38.4%) and usually the predominant triple negative breast cancers.1,2 For these patients no further treatment is given after surgery and neoadjuvant chemotherapy and radiotherapy. A comparative study done by Wright et al.3 shows that comparatively by 50 weeks after diagnosis and management survival probability of triple negative breast cancers in Nigeria; fall from 1 to 0.3, while in UK survival probability only falls from 1.0 to 0.6 (twice as good). By 100 weeks it has flattened to 0.1 in Nigeria and in UK 0.353. Although the differences can be explained in part by our late presentations, poorer health care systems and lack of good health insurance. We note that Adding Transcription factor 53 status as well as the estrogen receptor beta status evaluation only for triple negative breast cancers will make a significant difference in survival. Estrogen receptor beta (ESB2) shares structural homology at DNA and ligand binding domains (98% and 56%, respectively) with (ESR1) the major type of estrogen receptor in breast cancer.4,5 ESR2 functions and expression patterns are different from ESR1 and is widely expressed in both basal and luminal epithelial cells.6–8 The precise role of ESR2 in breast cancer is unclear, with both antiproliferative and proliferative roles described.9,10 The mechanisms for these opposing actions of ESR2 in breast tumorigenesis have not been fully elucidated.11 Mukhopadhyay et al.12 provides an explanation for the dual nature of ESR2 function in triple-negative breast cancer (TNBC) related to its interactions with TP53 status (wildtype or mutant). In wild-type TP53-expressing cells, silencing of ESR2 augmented apoptosis, whereas its over expression resulted in increased proliferation. Opposite effects were observed following silencing or overexpression of ESR2 in mutant TP53 cells, suggesting the important role of TP53 status in determining ESR2’s function. Mechanistically, ESR2-mutant TP53 interaction mediates sequestration of mutant TP53, leading to the TP73 activation and antiproliferative effects. Treatment with tamoxifen (4-hydroxy tamoxifen) also increases ESR2 expression and reactivates TP73 in mutant TP53 cells, providing an TP 53 status and estrogen receptorbeta in triple negative breast cancer management ","PeriodicalId":46078,"journal":{"name":"Rare Tumors","volume":" ","pages":"20363613211050355"},"PeriodicalIF":0.9,"publicationDate":"2021-10-07","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://ftp.ncbi.nlm.nih.gov/pub/pmc/oa_pdf/54/94/10.1177_20363613211050355.PMC8511903.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"39528716","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Hypofractionated radiation in secretory breast cancer: A case report.","authors":"Ken Tatebe,&nbsp;Claudia Perez,&nbsp;Lydia Usha,&nbsp;Ritu Ghai,&nbsp;Dian Wang,&nbsp;Parul Barry","doi":"10.1177/20363613211045252","DOIUrl":"https://doi.org/10.1177/20363613211045252","url":null,"abstract":"<p><p>Secretory carcinoma is a rare and indolent breast cancer with a lack of established treatment paradigms. We describe a case of a woman who underwent breast conservative therapy in the modern era. A 48 year old woman with a screen-detected left breast cancer was found to have early-stage secretory carcinoma after definitive breast conservation surgery. Further management with adjuvant radiation was recommended. After definitive breast conservative surgery, final pathology was notable for secretory breast carcinoma due to the immunohistologic characteristics of the tumor, ETV6-NTRK3 gene fusion, and histologic findings. After multi-disciplinary discussion, it was recommended that the patient proceed with adjuvant radiation. She was treated using a modestly hypofractionated regimen of 4256 cGy in 16 fractions. She tolerated the treatment well, developing only grade 1 radiation dermatitis. At 1 year follow-up she was clinically and radiographically free of disease. With a shift in management toward breast conservative therapy, defining the role of adjuvant radiation for secretory carcinomas in the modern era is of increasing importance. Modestly hypofractionated radiation is well-tolerated. Oncologic outcomes will be assessed with continued long-term follow-up.</p>","PeriodicalId":46078,"journal":{"name":"Rare Tumors","volume":" ","pages":"20363613211045252"},"PeriodicalIF":0.9,"publicationDate":"2021-09-28","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://ftp.ncbi.nlm.nih.gov/pub/pmc/oa_pdf/05/37/10.1177_20363613211045252.PMC8481704.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"39481597","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}