Rare TumorsPub Date : 2021-10-08eCollection Date: 2021-01-01DOI: 10.1177/20363613211052506
Xiujie Yu, Qiuyue Du, Xiaojing Zhang, Yixin Liu, Yan Shen
{"title":"Pure primary non-gestational choriocarcinoma originating in the ovary: A case report and literature review.","authors":"Xiujie Yu, Qiuyue Du, Xiaojing Zhang, Yixin Liu, 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}
Rare TumorsPub Date : 2021-10-08eCollection Date: 2021-01-01DOI: 10.1177/20363613211052498
Brian A Van Tine, Mia C Weiss, Angela C Hirbe, Peter J Oppelt, Sarah Abaricia, Kathryn Trinkaus, Jingqin Luo, Shellie Berry, Tyler Ruff, Cheryl Callahan, Jacqui Toensikoetter, Jessica Ley, Marilyn J Siegel, Farrokh Dehdashti, Barry A Siegel, Douglas R Adkins
{"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, Mia C Weiss, Angela C Hirbe, Peter J Oppelt, Sarah Abaricia, Kathryn Trinkaus, Jingqin Luo, Shellie Berry, Tyler Ruff, Cheryl Callahan, Jacqui Toensikoetter, Jessica Ley, Marilyn J Siegel, Farrokh Dehdashti, Barry A Siegel, 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/m<sup>2</sup> 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}
Rare TumorsPub Date : 2021-10-07eCollection Date: 2021-01-01DOI: 10.1177/20363613211050355
Martin Arinzechukwu Nzegwu, Onyekachi Nwokoro, Christian Nnamani, Vincent C Enemuo, Victor Ifeanyichukwu Nzegwu, Ogochukwu Nwoye, Anthony Edeh, Kenneth Nwankwo
{"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, Onyekachi Nwokoro, Christian Nnamani, Vincent C Enemuo, Victor Ifeanyichukwu Nzegwu, Ogochukwu Nwoye, Anthony Edeh, 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}
Rare TumorsPub Date : 2021-09-28eCollection Date: 2021-01-01DOI: 10.1177/20363613211045252
Ken Tatebe, Claudia Perez, Lydia Usha, Ritu Ghai, Dian Wang, Parul Barry
{"title":"Hypofractionated radiation in secretory breast cancer: A case report.","authors":"Ken Tatebe, Claudia Perez, Lydia Usha, Ritu Ghai, Dian Wang, 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}
Rare TumorsPub Date : 2021-09-23eCollection Date: 2021-01-01DOI: 10.1177/20363613211044566
Sobiya Ansari, Yixiang Liao, Summer Dewdney, Dian Wang, Parul Barry
{"title":"Vaginal oligometastatic disease of colorectal primary: Report of a novel therapeutic approach.","authors":"Sobiya Ansari, Yixiang Liao, Summer Dewdney, Dian Wang, Parul Barry","doi":"10.1177/20363613211044566","DOIUrl":"https://doi.org/10.1177/20363613211044566","url":null,"abstract":"<p><p>Vaginal oligometastatic disease of colorectal primary is a rare malignancy with few reported cases in the literature and no standardized treatment paradigm. We report on the definitive management of an unusual case of an elderly woman with the aforementioned disease. A 78-year-old African-American woman presented with vaginal spotting and was found to have a vaginal lesion. Final pathology was consistent with moderately differentiated adenocarcinoma of colorectal primary. Extensive work up, which included endoscopies, pathologic analyzes, and imaging workup, did not reveal a primary gastrointestinal malignancy. The patient underwent partial vaginectomy and final pathology once again confirmed moderately differentiated adenocarcinoma of colorectal primary (CDX 2 and CEA positive, ER/PR, and CK 7 negative) with negative margins. She went on to receive adjuvant concurrent chemoradiation with 5-FU based chemotherapy. She received 45 Gy in 25 fractions to the whole pelvis followed by an HDR brachytherapy boost to 12 Gy in two fractions. Unfortunately, 10 months after completing radiation, she was found to have adenocarcinoma arising from a hepatic flexure colon polyp on colonoscopy. She required definitive surgical resection and was staged as mpT3N0M1. She received 12 cycles of 5-FU and at 2-year follow-up was found to be disease free with no evidence of locoregional recurrence or distant metastatic disease. Continued long-term follow up is warranted.</p>","PeriodicalId":46078,"journal":{"name":"Rare Tumors","volume":" ","pages":"20363613211044566"},"PeriodicalIF":0.9,"publicationDate":"2021-09-23","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://ftp.ncbi.nlm.nih.gov/pub/pmc/oa_pdf/cd/6f/10.1177_20363613211044566.PMC8474297.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"39494352","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}
Rare TumorsPub Date : 2021-08-29eCollection Date: 2021-01-01DOI: 10.1177/20363613211043662
Akwasi Ofori Abayie, Kofi Mensah Nyarko, Markus Bährle, Alfred Brütting
{"title":"The first case report of primary thyroid teratocarcinosarcoma: An analog to sinonasal teratocarcinosarcoma.","authors":"Akwasi Ofori Abayie, Kofi Mensah Nyarko, Markus Bährle, Alfred Brütting","doi":"10.1177/20363613211043662","DOIUrl":"https://doi.org/10.1177/20363613211043662","url":null,"abstract":"<p><p>Teratocarcinosarcoma is a rare and aggressive tumor usually affecting the sinonasal tract. It arises primarily from the nasal cavity, paranasal sinuses with some reported cases arising from the nasopharynx and oral cavity and commonly referred to as Sinonasal Teratocarcinosarcoma (SNTC). We present the first case of teratocarcinosarcoma as a primary thyroid cancer in a 17-year-old male patient who presented with a rapidly growing anterior neck mass with no symptoms. Physical examination revealed circa 4 cm × 5 cm slightly right sided, non-tender, firm anterior neck swelling. A thyroid ultrasound revealed an enlarged thyroid gland with multiple thyroid nodes. Magnetic Resonance Imaging (MRI) of the head and neck showed no sinonasal tract tumor. Thyroidectomy and surgical resection of the tumor was performed. Histological examination revealed teratocarcinosarcoma of the thyroid gland, an analog to SNTC with no primary sinonasal tissue involvement. This implies that, teratocarcinosarcoma can occur in primary tissues other than sinonasal origin contrary to conventional knowledge.</p>","PeriodicalId":46078,"journal":{"name":"Rare Tumors","volume":" ","pages":"20363613211043662"},"PeriodicalIF":0.9,"publicationDate":"2021-08-29","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://ftp.ncbi.nlm.nih.gov/pub/pmc/oa_pdf/ea/1b/10.1177_20363613211043662.PMC8408892.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"39386457","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}
Rare TumorsPub Date : 2021-08-29eCollection Date: 2021-01-01DOI: 10.1177/20363613211034710
Karlton Wong, Jomjit Chantharasamee, Scott Nelson, Mark A Eckardt, Kambiz Motamedi, Francis J Hornicek, Arun S Singh
{"title":"Aggressive osteoblastoma with a secondary aneurysmal bone cyst treated with denosumab.","authors":"Karlton Wong, Jomjit Chantharasamee, Scott Nelson, Mark A Eckardt, Kambiz Motamedi, Francis J Hornicek, Arun S Singh","doi":"10.1177/20363613211034710","DOIUrl":"https://doi.org/10.1177/20363613211034710","url":null,"abstract":"<p><p>Osteoblastomas and aneurysmal bone cysts (ABC) are rare benign bone tumors that make up about 1%-2% of primary bone malignancies, typically occurring in young patients with a median age of 20 years, most commonly effecting the axial skeleton. ABCs may develop independently as primary lesions, or secondary to other bony lesions including osteoblastomas, chondroblastomas, and giant cell tumors. Treatment of unresectable or extensive osteoblastomas can be challenging. In 2013, the Food and Drug Administration (FDA) approved denosumab for the treatment of giant cell tumors of the bone due to its efficacy in these morbid bony lesions. Various case reports have shown that osteoblastomas can respond to denosumab. Furthermore, numerous ABC case reports have described the efficacy of denosumab in these situations. We herein describe a unique case of a young patient with an aggressive osteoblastoma and secondary ABCs who was successfully treated with denosumab.</p>","PeriodicalId":46078,"journal":{"name":"Rare Tumors","volume":" ","pages":"20363613211034710"},"PeriodicalIF":0.9,"publicationDate":"2021-08-29","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://ftp.ncbi.nlm.nih.gov/pub/pmc/oa_pdf/2c/5f/10.1177_20363613211034710.PMC8408893.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"39388559","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}
Rare TumorsPub Date : 2021-08-20eCollection Date: 2021-01-01DOI: 10.1177/20363613211039724
Mousa ElKhaldi, Rakan Radi, Maysa Al-Hussaini
{"title":"Choriocarcinoma metastatic to the skin: A rare occurrence associated with dismal outcome.","authors":"Mousa ElKhaldi, Rakan Radi, Maysa Al-Hussaini","doi":"10.1177/20363613211039724","DOIUrl":"https://doi.org/10.1177/20363613211039724","url":null,"abstract":"<p><p>Germ cell tumors (GCTs) are a histologically heterogeneous group of tumors that arise from the primitive germ cell of the embryonic gonad. Choriocarcinoma is a variant of GCTs that is prone to hematogenous metastasis to the liver, lung, and brain. Cutaneous metastasis in choriocarcinoma is rarely encountered with only a few cases reported in literature. We report the case of a 28-year-old male presenting with lower back pain that, upon further work-up, was diagnosed with pure choriocarcinoma of the testes. Around 9 months after his initial presentation, he developed a cutaneous back lesion. Microscopic examination confirmed the presence of choriocarcinoma composed of mononuclear cytotrophoblasts which interweave with multinucleated syncytiotrophoblasts. The patient passed away 3 weeks after the onset of cutaneous metastasis.</p>","PeriodicalId":46078,"journal":{"name":"Rare Tumors","volume":" ","pages":"20363613211039724"},"PeriodicalIF":0.9,"publicationDate":"2021-08-20","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://ftp.ncbi.nlm.nih.gov/pub/pmc/oa_pdf/91/25/10.1177_20363613211039724.PMC8381407.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"39344182","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}
Rare TumorsPub Date : 2021-07-18eCollection Date: 2021-01-01DOI: 10.1177/20363613211034115
Maroun Bou Zerdan, Aram H Bidikian, Ibrahim Alameh, Clara El Nakib, Hazem I Assi
{"title":"Olaratumab's failure in soft tissue sarcoma.","authors":"Maroun Bou Zerdan, Aram H Bidikian, Ibrahim Alameh, Clara El Nakib, Hazem I Assi","doi":"10.1177/20363613211034115","DOIUrl":"https://doi.org/10.1177/20363613211034115","url":null,"abstract":"<p><p>Soft tissue sarcomas remain one of the rarest malignancies with numerous subtypes that go undiagnosed. The PDGFRα antagonist Olaratumab (Lartruvo) was withdrawn from the market due to disappointing findings in the phase III studies. We share our experience with this medication in a tertiary care center in the Middle East and North Africa region. Monitor the effect of Olaratumab on sarcomas when it was used prior to its withdrawal, and compare our findings with the literature. We performed a retrospective analysis of adult patients with advanced-/metastatic soft tissue sarcomas treated with at least two cycles of Olaratumab at a tertiary care center in Lebanon during the period from January 1, 2017 to December 31, 2018. Fifteen patients were included in the study. The mean age was 49 with a range of 26-75 years. The median duration of the use of Olaratumab was 21.3 months with a range of 7.3-37 months. The average number of number of cycles received per patient was four. Five patients were deceased. Median PFS was 7.87 months (95% CI 5.28-10.45), and mean OS was 12.26 months (95% CI 8.47-16.05) Median OS was 9.8 months (95% CI 6.07-13.53). Olaratumab has been withdrawn from the market, and it is currently being investigated as part of the phase II ANNOUNCE 2 trial. Our experience from a tertiary care center shows results similar to those reported in the literature. The immunogenicity and heterogeneity of soft tissue sarcomas pose a challenge to the treatment of soft tissue sarcomas, but they also allow a wide array of possible management solutions.</p>","PeriodicalId":46078,"journal":{"name":"Rare Tumors","volume":" ","pages":"20363613211034115"},"PeriodicalIF":0.9,"publicationDate":"2021-07-18","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://sci-hub-pdf.com/10.1177/20363613211034115","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"39276934","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}
Rare TumorsPub Date : 2021-07-02eCollection Date: 2021-01-01DOI: 10.1177/20363613211029493
Philip T Sobash, Krishna Vedala, Daniel Alfano, Heather Pinckard-Dover, Jason L Muesse, Raman Desikan
{"title":"A rare case of chordoma presenting as a Pancoast tumor.","authors":"Philip T Sobash, Krishna Vedala, Daniel Alfano, Heather Pinckard-Dover, Jason L Muesse, Raman Desikan","doi":"10.1177/20363613211029493","DOIUrl":"https://doi.org/10.1177/20363613211029493","url":null,"abstract":"<p><p>The notochord is the defining structure of all chordate embryos. It is a midline structure ventral to the ectoderm, neural plates, and neural arch. Remnants of the notochord ultimately give rise to the nucleus pulposus. The function of the notochord is to organize the surrounding structures. Chordoma is a rare malignant bone tumor arising from remnants of the notochord. These tumors are indolent and can present as incidental or locally advanced involving adjacent structures. These tumors typically present at the skull base and sacral spine but more rarely can be seen on the cervical and thoracic spine. Rare cases of chordoma invading the brachial plexus have been recorded. Surgical resection is the mainstay of treatment for chordomas. We would like to discuss a novel presentation of a chordoma as a Pancoast tumor, and aim to highlight the clinical importance of accurate diagnosis and planning therapy along with poor prognosis of incomplete surgical resection.</p>","PeriodicalId":46078,"journal":{"name":"Rare Tumors","volume":" ","pages":"20363613211029493"},"PeriodicalIF":0.9,"publicationDate":"2021-07-02","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://sci-hub-pdf.com/10.1177/20363613211029493","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"39199384","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}