Rare Tumors最新文献

{"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":null,"pages":null},"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":null,"pages":null},"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":null,"pages":null},"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}

{"title":"The enigmatic ear: Unveiling a rare case of a primary cutaneous CD8+ acral T-cell lymphoproliferative disorder with a literature review.","authors":"Ghada Sahraoui,&nbsp;Farah Sassi,&nbsp;Lamia Charfi,&nbsp;Raoudha Doghri,&nbsp;Karima Mrad","doi":"10.1177/20363613231204046","DOIUrl":"10.1177/20363613231204046","url":null,"abstract":"<p><p><b>Introduction:</b> Primary cutaneous CD8+ acral T-cell lymphoproliferative disorder (CD8+ ATCLPD) is a rare form of cutaneous T-cell lymphoma that commonly presents on the acral regions of the body. We report a case of a 61-year-old man diagnosed with primary cutaneous CD8+ ATCLPD of the ear. <b>Case presentation:</b> A 61-year-old man presented with a non-healing, erythematous painful macule on the ear that had been evolving for the past 3 months. The lesion was resected, and histopathological examination revealed a primary cutaneous CD8+ ATCLPD with acral localization. Further investigations including CT scan of the thorax, abdomen and pelvis were done to stage the disease. The results showed no extracutaneous involvement. <b>Conclusion:</b> Accurate identification of primary cutaneous CD8+ ATCLPD is crucial due to its distinct prognostic and therapeutic implications compared to other CD8+ cytotoxic lymphoid proliferations. Primary cutaneous CD8+ ATCLPD can be treated conservatively and typically follows a slow clinical course, regardless of the treatment method. Understanding the clinical context, as well as the morphological and immunophenotypic characteristics, can assist in making a precise diagnosis.</p>","PeriodicalId":46078,"journal":{"name":"Rare Tumors","volume":null,"pages":null},"PeriodicalIF":0.9,"publicationDate":"2023-09-22","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://ftp.ncbi.nlm.nih.gov/pub/pmc/oa_pdf/b3/87/10.1177_20363613231204046.PMC10517602.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"41153089","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":"Teratocarcinosarcoma of the head and neck: Clinicopathologic review of a rare entity.","authors":"Rana S Al-Zaidi","doi":"10.1177/20363613231204047","DOIUrl":"https://doi.org/10.1177/20363613231204047","url":null,"abstract":"<p><p>Teratocarcinosarcoma is a rare, highly aggressive malignancy of the head and neck, characterized by multiphenotypic and triphasic growth of epithelial, mesenchymal, and primitive neuroepithelial elements. Owing to its rarity and morphological heterogeneity, as well as the lack of experience with this neoplasm, teratocarcinosarcoma is often misdiagnosed, particularly in small biopsy samples when only some of the elements are identified, thus leading to delayed management. Aggressive clinical behavior and poor survival outcomes, necessitate an accurate diagnosis and appropriate treatment. This review describes the main demographic and clinicopathological features of teratocarcinosarcoma, with an emphasis on the recent advances that have attempted to identify the molecular signature of this neoplasm.</p>","PeriodicalId":46078,"journal":{"name":"Rare Tumors","volume":null,"pages":null},"PeriodicalIF":0.9,"publicationDate":"2023-09-21","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://ftp.ncbi.nlm.nih.gov/pub/pmc/oa_pdf/76/9a/10.1177_20363613231204047.PMC10515544.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"41170265","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":"Recurrent pineal parenchymal tumor of intermediate differentiation with intratumoral hemorrhage: A case report and review of the literature.","authors":"Yu-Li Chen,&nbsp;Li-Hsin Tai,&nbsp;Ann-Shung Lieu","doi":"10.1177/20363613231177537","DOIUrl":"https://doi.org/10.1177/20363613231177537","url":null,"abstract":"<p><p>Pineal apoplexy is a rare clinical condition. Its common symptoms include headaches, nausea, vomiting, ataxia, and gaze paralysis. These symptoms are mainly caused by obstructive hydrocephalus or direct compression of the cerebellum or midbrain. There have been no previous reports on the development of a recurrent pineal parenchymal tumor of intermediate differentiation (PPTID) with intratumoral hemorrhage. We report a case of PPTID with intratumoral hemorrhage. A 44-year-old woman developed recurrent PPTID following tumor removal and ventriculoperitoneal shunting in 2010. She visited the emergency department in April 2021 for sudden-onset dizziness and generalized weakness. Blurring of vision occurred and progressed over the previous month. Neurological examination revealed upward conjugate gaze paralysis. Brain computed tomography revealed a hyperdense lesion in the pineal region, and a recurrent tumor with hemorrhage was suspected. Magnetic resonance imaging of the brain confirmed a pineal tumor with intratumoral hemorrhage. The pineal tumor and hematoma were surgically removed via the suboccipital transtentorial approach. The patient was discharged from the hospital 2 weeks after the surgery. The pathological findings were consistent with the diagnosis of recurrent PPTID. PPTID is a rare tumor, accounting for less than 0.1% of primary central nervous system tumors. Pineal apoplexy is rare, and its incidence and clinical significance remain unclear. There have only been nine reported cases of pineal apoplexy, associated with pineal parenchymal tumors. The recurrence of PPTID with apoplectic hemorrhage after 10 years has not been reported. Despite its rarity, PPTID with apoplexy should be considered in patients with PPTID who develop sudden-onset neurological symptoms.</p>","PeriodicalId":46078,"journal":{"name":"Rare Tumors","volume":null,"pages":null},"PeriodicalIF":0.9,"publicationDate":"2023-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://ftp.ncbi.nlm.nih.gov/pub/pmc/oa_pdf/b1/76/10.1177_20363613231177537.PMC10201390.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"10350373","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":"Axillary chest wall solid-papillary carcinoma: A case report on presentation and management.","authors":"Harper E Niver,&nbsp;Priscilla Strom","doi":"10.1177/20363613231155957","DOIUrl":"https://doi.org/10.1177/20363613231155957","url":null,"abstract":"<p><p>Papillary breast carcinomas comprise <1% of all breast cancers. They are notorious among surgical pathologists for posing diagnostic difficulty, especially with small sample sizes, such as a core-needle biopsy and carry potential for overtreatment. Solid-papillary carcinoma is a subtype of papillary breast carcinomas that affects elderly females and generally has a favorable diagnosis in its in-situ form. This report focuses on the unique and clinically aggressive presentation and treatment of invasive solid-papillary carcinoma that was discovered along the axillary chest wall after an ipsilateral mastectomy for multifocal ductal carcinoma in situ.</p>","PeriodicalId":46078,"journal":{"name":"Rare Tumors","volume":null,"pages":null},"PeriodicalIF":0.9,"publicationDate":"2023-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://ftp.ncbi.nlm.nih.gov/pub/pmc/oa_pdf/07/70/10.1177_20363613231155957.PMC9905025.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"10693807","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":"Epithelioid hemangioendothelioma of the distal lower extremity and the role of radiotherapy: A report of two cases.","authors":"Robin Go,&nbsp;Linus Lee,&nbsp;Gayathri Vijayakumar,&nbsp;Sarah Tepper,&nbsp;Steven Gitelis,&nbsp;Alan Blank","doi":"10.1177/20363613231172611","DOIUrl":"https://doi.org/10.1177/20363613231172611","url":null,"abstract":"<p><p>We report two cases of epithelioid hemangioendothelioma (EHE) in the distal lower extremity. Our first patient had unicentric EHE of the left os calcis initially treated with an intralesional procedure; however, later developed two recurrences which were managed with radiation therapy. Our second patient had multicentric EHE of the distal tibia and fibula managed with primary radiation therapy. Although EHE is typically treated with wide resection or an intralesional procedure, we present two cases of EHE in the distal lower extremity to discuss the therapeutic role of radiation therapy in the management of distal EHE.</p>","PeriodicalId":46078,"journal":{"name":"Rare Tumors","volume":null,"pages":null},"PeriodicalIF":0.9,"publicationDate":"2023-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://ftp.ncbi.nlm.nih.gov/pub/pmc/oa_pdf/73/40/10.1177_20363613231172611.PMC10134116.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"9450499","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":"Unusual cause of mediastinal tumor: A case of calcified pericardial cyst.","authors":"S M Tajdit Rahman,&nbsp;Mofizur Rhaman Mia,&nbsp;Mohammad Anamul Hoque,&nbsp;Sanghita Banik Proma","doi":"10.1177/20363613231177539","DOIUrl":"https://doi.org/10.1177/20363613231177539","url":null,"abstract":"<p><p>Pericardial cysts are rare benign intrathoracic lesions, and calcified pericardial cysts are even more uncommon. Most pericardial cysts are asymptomatic, but patients may present with chest pain, dyspnea and any complications of pericardial effusion. We present a case of a left-sided calcified pericardial cyst, highlighting its rarity and symptoms in relation to its location.</p>","PeriodicalId":46078,"journal":{"name":"Rare Tumors","volume":null,"pages":null},"PeriodicalIF":0.9,"publicationDate":"2023-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://ftp.ncbi.nlm.nih.gov/pub/pmc/oa_pdf/ad/d3/10.1177_20363613231177539.PMC10192659.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"10663137","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 qualitative study to assess the psychological experiences and coping strategies of families affected with Li-Fraumeni syndrome in the Indian population.","authors":"Poonam Joshi,&nbsp;Sunidhi Bhandari,&nbsp;Ajesh Tk,&nbsp;Simran Kaur,&nbsp;Rachna Bhargava,&nbsp;Ghazal Tansir,&nbsp;Sameer Rastogi","doi":"10.1177/20363613231186300","DOIUrl":"https://doi.org/10.1177/20363613231186300","url":null,"abstract":"<p><p><b>Background:</b> Li-Fraumeni syndrome (LFS) is a rare autosomal dominant hereditary cancer syndrome. Due to the high risk of occurrence of multiple cancers, families with LFS may have an overwhelming psychosocial burden. <b>Methods:</b> This cross-sectional study was conducted at a tertiary care center using face-to-face interviews through a grounded theory approach. Statistical analysis was done using Smith's Interpretative Phenomenological Approach. Themes and sub-themes were extracted, and a thematic schema was developed. <b>Results:</b> A total of five themes were identified. The extracted themes were psychological experiences, behavioural responses, stressors, coping strategies and perceived needs. The interlay of the themes deepened the impact of LFS on the affected ones and brought into light the turmoil of emotions and difficulties that these individuals were going through in the face of the disease. <b>Conclusions:</b> LFS-affected individuals had a range of experiences with this rare and little-known disease. The lack of information seems to be a precursor to the denial of diagnosis. Their experience with the illness sheds light on the grey areas like guilt and helplessness that demand immediate attention. Future policies need to be developed in accordance with the identified perceived needs to potentially guide the treatment and rising needs of LFS-affected individuals.</p>","PeriodicalId":46078,"journal":{"name":"Rare Tumors","volume":null,"pages":null},"PeriodicalIF":0.9,"publicationDate":"2023-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://ftp.ncbi.nlm.nih.gov/pub/pmc/oa_pdf/0f/ed/10.1177_20363613231186300.PMC10327410.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"10292272","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}