Rare Tumors最新文献

{"title":"Primary lung low-grade fibromyxoid sarcoma: A rare case with A diagnostic dilemma.","authors":"Aida Ayadi, Yoldez Houcine, Chirine Moussa, Houda Rouis, Tarek Abid, Begueret Hugues, François Le Loarer","doi":"10.1177/20363613241234201","DOIUrl":"10.1177/20363613241234201","url":null,"abstract":"<p><p>Low-grade fibromyxoid sarcoma (LGFMS) is a rare, low-grade malignant soft tissue tumor that is often mistaken for benign or more rarely malignant tumor types. Commonly, this tumor affects young adults and typically arises in the deep proximal extremities or trunk with frequent recurrences and can metastasize to the lungs many years late. Visceral LGFMS is extremely rare. Only a few cases of primary LGFMS of the lung have been reported. Here, we present the clinical, gross, microscopic, and immunohistochemical characteristics of Evans tumor occurring in the lung with a review of the literature and discuss the differential diagnosis in this exceptional localization.</p>","PeriodicalId":46078,"journal":{"name":"Rare Tumors","volume":"16 ","pages":"20363613241234201"},"PeriodicalIF":0.9,"publicationDate":"2024-02-29","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10906048/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"140023952","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":"Real-world evidence for ultra rare cancers.","authors":"Leonidas Mavroeidis, Andrea Napolitano, Paul Huang, Robin L Jones","doi":"10.1177/20363613241234207","DOIUrl":"10.1177/20363613241234207","url":null,"abstract":"","PeriodicalId":46078,"journal":{"name":"Rare Tumors","volume":"16 ","pages":"20363613241234207"},"PeriodicalIF":0.9,"publicationDate":"2024-02-15","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10874139/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"139900684","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":"Dermatofibrosarcoma protuberans: Case series in a tropical setting and review of literature.","authors":"John Adi Ashindoitiang, Victor Ikechukwu Canice Nwagbara, Ugbem Theophilus Ipeh, George Peter Owusu, Maurice Efana Asuquo","doi":"10.1177/20363613241234243","DOIUrl":"10.1177/20363613241234243","url":null,"abstract":"<p><p>Dermatofibrosarcoma protuberans (DFSP) is an infrequent cutaneous tumour that may involve subcutaneous fat and in some cases fascia, muscles and bone. The infrequent occurrence lessens its clinical awareness in addition to its clinical semblance to many common cutaneous lesions. It is characterized by proclivity for local recurrence. We evaluated the clinical presentation and treatment outcomes of six consecutive cases of DFSP with histologic diagnosis in the University of Calabar Teaching Hospital, Calabar seen between January 2013 and December 2022. This was compared with total cutaneous malignances in the same period. The six consecutive patients comprised of two males and four females (M = F: 1:2) whose ages ranged from 21 to 57 years (mean of 36.5 years) and accounted for 7% of cutaneous malignancies. The site distribution was trunk (back) involved in 3 (50%) of the patients, limbs 3 (50%); upper 1 (17%) and lower limb 2 (33%). Clinical presentation was in the form of firm cutaneous mass with some ulcerated lesions that bled and some fungated. 50 percent of the patients presented with recurrent lesions and in all there was no regional lymphadenopathy or evidence of metastasis. There was a patient with Neurofibromatosis- 1 who had a huge fungated limb lesion offered amputation, four had wide local excision and one incision biopsy. Follow up was poor and the period ranged from 2 to 14 months (mean 7 months). Dermatofibrosarcoma protuberans is an uncommon tumour with clinical semblance to other cutaneous lesions. Early presentation, preoperative histologic diagnosis will enhance the goal of ensuring adequate excision. Adjuvant therapy with Imatinib with or without adjuvant radiotherapy are recommended in the treatment plan in view of the frequency of late presentation with advanced recurrent lesions and poor follow up.</p>","PeriodicalId":46078,"journal":{"name":"Rare Tumors","volume":"16 ","pages":"20363613241234243"},"PeriodicalIF":0.9,"publicationDate":"2024-02-15","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10874159/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"139900683","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":"An extremely rare case of recurrent pleomorphic myxoidliposarcoma with response to eribulin chemotherapy - A case report.","authors":"Raghavendra Rao, Sameer Rastogi, Divya Kashyap, Shamim A Shamim, Adarsh Barwad","doi":"10.1177/20363613231212380","DOIUrl":"https://doi.org/10.1177/20363613231212380","url":null,"abstract":"<p><p>Pleomorphic myxoid liposarcoma (PML) is a newly recognized entity with aggressive clinical behavior and a tendency to recur. It has histological features of both myxoid and pleomorphic liposarcoma and lacks the molecular and structural chromosomal abnormalities associated with myxoid and pleomorphic liposarcoma. The data about their response to chemotherapy is quite sparse. We report a case of incidentally detected pleomorphic myxoid liposarcoma of the mediastinum in a 32-year-old gentleman. After resection and adjuvant chemotherapy with doxorubicin and ifosfamide, there was no evidence of residual disease at the end of treatment. During a routine follow-up 5 months later, he was found to have a recurrence of the disease with histological confirmation. He received a trabectedin given its activity in myxoid liposarcoma. However, he had toxicities and progression leading to its discontinuation. Subsequently, eribulin was started as the next line of therapy. After 4 cycles of chemotherapy, response assessment was suggestive of partial response, which is still maintained after 7 cycles of eribulin. This is the first report of this entity responding to a newer chemotherapy regimen.</p>","PeriodicalId":46078,"journal":{"name":"Rare Tumors","volume":"15 ","pages":"20363613231212380"},"PeriodicalIF":0.9,"publicationDate":"2023-12-08","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10710107/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"138811991","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":"Letter re: Uveal melanoma metastasis to the liver: Unveiling effective strategies with HEPZATO KIT.","authors":"Ushna Zameer, Wajiha Shaikh, Anood Choudhary","doi":"10.1177/20363613231218172","DOIUrl":"https://doi.org/10.1177/20363613231218172","url":null,"abstract":"","PeriodicalId":46078,"journal":{"name":"Rare Tumors","volume":"15 ","pages":"20363613231218172"},"PeriodicalIF":0.9,"publicationDate":"2023-11-20","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10664426/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"138465539","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":"Talquetamab: A promising immunotherapy for multiple myeloma.","authors":"Amna Tariq, Abdul Wahid, Fatima Asif, Mahnoor Khan","doi":"10.1177/20363613231211051","DOIUrl":"https://doi.org/10.1177/20363613231211051","url":null,"abstract":"","PeriodicalId":46078,"journal":{"name":"Rare Tumors","volume":"15 ","pages":"20363613231211051"},"PeriodicalIF":0.9,"publicationDate":"2023-11-16","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10655669/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"138465540","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":"Follicular adenoma with bizarre nuclei and wild-type P53 expression: A case report and literature review.","authors":"Daniel Nguyen,&nbsp;Nyein Nyein Htun,&nbsp;Beverly Wang","doi":"10.1177/20363613231212383","DOIUrl":"https://doi.org/10.1177/20363613231212383","url":null,"abstract":"<p><strong>Introduction: </strong>Thyroid cancer is the most common endocrine tumor in humans. Follicular adenoma/carcinoma is the second most common subtype. Multiple histological patterns have been identified. Follicular adenoma with bizarre nuclei is one of the patterns associated with p53 mutation and has an unclear clinical prognosis.</p><p><strong>Case report: </strong>A 74-year-old female presented with incidental findings of elevated TSH levels and normal thyroid markers. Ultrasound was performed and revealed multiple bilateral thyroid nodules measuring up to 1.9 cm. Fine needle aspiration was performed, and cytology showed one Bethesda category 5 nodule. Total thyroidectomy with neck dissection was performed, and the pathology showed follicular adenoma with bizarre nuclei. Based on the results of immunohistochemistry, the neoplastic cells exhibited staining for wild-type p53 and low levels of the proliferation index Ki-67.</p><p><strong>Conclusions: </strong>We report a rare case of thyroid follicular adenoma with bizarre nuclei. In contrast to previous reports of this tumor, our patient showed a p53 wild-type pattern using immunohistochemistry. More studies are needed to better understand the etiology and clinical prognosis of this tumor.</p>","PeriodicalId":46078,"journal":{"name":"Rare Tumors","volume":"15 ","pages":"20363613231212383"},"PeriodicalIF":0.9,"publicationDate":"2023-10-28","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10614501/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"71427772","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":"Elrexfio™ (elranatamab-bcmm): The game-changer in treatment of multiple myeloma.","authors":"Taruba Rais,&nbsp;Afsheen Khan,&nbsp;Rumaisa Riaz","doi":"10.1177/20363613231207483","DOIUrl":"10.1177/20363613231207483","url":null,"abstract":"<p><p>Multiple myeloma is the second most common plasma cell malignancy, characterized by uncontrolled proliferation of plasma cells within the bone marrow. ELREXFIO™ (elranatamab-bcmm) is a recently FDA-approved drug for relapsed and refractory multiple myeloma. The progression of multiple myeloma involves interactions with various bone marrow cell types, and targeting this microenvironment has shown promising results in inhibiting its growth and osteolysis. ELREXFIO, a bispecific antibody targeting CD3 and BCMA, activates cytotoxic T-lymphocyte responses against BCMA-expressing myeloma cells. Clinical trials, such as MagnetisMM-3, demonstrated significant response rates and long-term tolerability. Its approval offers hope to multiple myeloma patients, especially those with relapsed or refractory cases, as innovative therapies like ELREXFIO continue to improve outcomes in this challenging malignancy.</p>","PeriodicalId":46078,"journal":{"name":"Rare Tumors","volume":"15 ","pages":"20363613231207483"},"PeriodicalIF":0.9,"publicationDate":"2023-10-11","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://ftp.ncbi.nlm.nih.gov/pub/pmc/oa_pdf/6b/fc/10.1177_20363613231207483.PMC10571667.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"41239758","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":"\"A beacon of hope for relapsed multiple myeloma patients: TALVEY™\".","authors":"Nawal Khaliq,&nbsp;Rumaisa Riaz,&nbsp;Aleeza Hasan,&nbsp;Sara Alauddin","doi":"10.1177/20363613231205749","DOIUrl":"https://doi.org/10.1177/20363613231205749","url":null,"abstract":"Dear Editor, Multiple myeloma, a significant hematological malignancy affecting about 10% of such cases globally, presents a substantial health challenge. Despite medical advancements, most patients eventually face relapse and resistance to treatment. Particularly, patients with triple-class exposed relapsed/refractory multiple myeloma (RRMM), pretreated with immunomodulatory drugs, proteasome inhibitors, and anti-CD38 monoclonal antibodies, have poor overall survival rates. Recent breakthroughs in immunotherapy, including CAR T-cell therapy and bispecific antibodies (BispAbs), offer promising options for RRMM management. CAR T-cell therapy is effective but has a timeconsuming manufacturing process. On the other hand, BispAbs are readily available and show remarkable efficacy in RRMM treatment. Recognizing this, the Food and Drug Administration (FDA) has recently given accelerated approval to Talquetamab, a pioneering second bispecific antibody that targets GPRC5D and CD3 receptors. This offthe-shelf therapy shows remarkable therapeutic achievement for heavily pretreated RRMM patients. Multiple myeloma (MM), the second most prevalent hematologic cancer after non-Hodgkin lymphoma, predominantly affects high-income countries. It is defined by the infiltration of bone marrow with monoclonal plasma cells, producing monoclonal immunoglobulins detectable in the blood or urine. The buildup of these immunoglobulins can result in organ dysfunction, commonly referred to as CRAB symptoms (hypercalcemia, kidney problems, anemia, and bone abnormalities), which signify the onset of symptomatic disease. TALVEYTM (talquetamab-tgvs), a GPRC5D-targeted therapeutic bispecific antibody, effectively engages T-cells and demonstrates the capacity to attach to both the CD3 receptor situated on T-cells and the G protein-coupled receptor class C group 5 member D (GPRC5D), which is present on the surfaces of both multiple myeloma cells and non-cancerous plasma cells. Furthermore, it also interacts with normal tissues such as epithelial cells in keratinized regions of the skin and tongue. In experimental settings, talquetamab-tgvs prompted the activation of T-cells, resulting in the emission of inflammatory signaling molecules and leading to the elimination of multiple myeloma cells. The effectiveness of TALVEY as a standalone treatment was assessed in patients with relapsed or refractory multiple myeloma. This evaluation took place in a study called MMY1001 (MonumenTAL-1) (NCT03399799, NCT4634552), involving 187 patients who had received at least four previous systemic treatments. Patients were given either talquetamab-tgvs 0.4 mg/kg subcutaneously weekly after initial step-up doses, or talquetamab-tgvs 0.8 mg/kg subcutaneously every 2 weeks following initial step-up doses. Treatment will be administered until either the disease advances or intolerable side effects occur. The main group under analysis consisted of patients who had undergone at least four prior therapies, i","PeriodicalId":46078,"journal":{"name":"Rare Tumors","volume":"15 ","pages":"20363613231205749"},"PeriodicalIF":0.9,"publicationDate":"2023-09-27","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://ftp.ncbi.nlm.nih.gov/pub/pmc/oa_pdf/44/05/10.1177_20363613231205749.PMC10536835.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"41169242","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":"Advancing precision oncology in metastatic colorectal cancer: The food and drug administration approval of foundation one liquid CDx as a companion diagnostic a correspondence.","authors":"Syeda Shahnoor,&nbsp;Manahil Mansha,&nbsp;Solay Farhat,&nbsp;Adeena Maryyum,&nbsp;Adil Naseer Khan,&nbsp;Abdul Moiz Khan","doi":"10.1177/20363613231204798","DOIUrl":"https://doi.org/10.1177/20363613231204798","url":null,"abstract":"malignant","PeriodicalId":46078,"journal":{"name":"Rare Tumors","volume":"15 ","pages":"20363613231204798"},"PeriodicalIF":0.9,"publicationDate":"2023-09-25","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://ftp.ncbi.nlm.nih.gov/pub/pmc/oa_pdf/a4/b9/10.1177_20363613231204798.PMC10521303.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"41172464","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}