Chemotherapeutic drugs for soft tissue sarcomas: a review

Frontiers in Pharmacology, Journal Year: 2023, Volume and Issue: 14

Published: Aug. 11, 2023

Despite the low incidence of soft tissue sarcomas (STSs), hundreds thousands new STS cases are diagnosed annually worldwide, and approximately half them eventually progress to advanced stages. Currently, chemotherapy is first-line treatment for STSs. There difficulties in selecting appropriate drugs multiline chemotherapy, or combination different histological subtypes. In this study, we first comprehensively reviewed efficacy various chemotherapeutic STSs, then described current status sensitive anthracyclines most important systemic Ifosfamide, trabectedin, gemcitabine, taxanes, dacarbazine, eribulin exhibit certain activities Vinca alkaloid agents (vindesine, vinblastine, vinorelbine, vincristine) have therapeutic effects specific subtypes, such as rhabdomyosarcoma Ewing sarcoma family tumors, whereas their activity other subtypes weak. Other (methotrexate, cisplatin, etoposide, pemetrexed) weak STSs rarely used. It necessary select second- above-line depending on subtype. This review aims provide a reference selection multi-line therapy patients with who an increasingly long survival.

Language: Английский

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Case Report and review of the literature: esophageal pleomorphic rhabdomyosarcoma

Frontiers in Oncology, Journal Year: 2025, Volume and Issue: 15

Published: Feb. 28, 2025

Background Esophageal rhabdomyosarcoma is an exceedingly rare malignant tumor, with only three cases comprehensively documented in the literature since 1995. Due to its rarity, there limited information on epidemiology and diagnosis of this disease, no standardized treatment protocols have been established. As a result, both recognition management esophageal pose significant challenges. The present case report provides valuable insight into clinical approach highlighting need for further research investigation develop more effective diagnostic tools therapeutic strategies. Case Presentation We described 77-year-old male who presented dyspepsia anemia, leading discovery lesion. At index endoscopy, histological findings were consistent adenocarcinoma. Consequently, patient was treated neoadjuvant chemotherapy Ivor Lewis esophagectomy. Interestingly, pathological examination lesion identified as pleomorphic esophagus. Postoperatively, received adjuvant chemotherapy. Subsequently, subcutaneous metastatic his right shoulder combination radiotherapy surgical excision. patients died twenty-six months after initial diagnosis. Conclusions Our represents one few reported instances rhabdomyosarcoma, highly aggressive malignancy, insights challenges diagnosing managing disease. Moreover, first followed up than 24 months. However, given paucity data remains unmet comprehensive studies establish protocols.

Language: Английский

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Pediatric Rhabdomyosarcomas of the Genitourinary Tract

Cancers, Journal Year: 2023, Volume and Issue: 15(10), P. 2864 - 2864

Published: May 22, 2023

Rhabdomyosarcoma (RMS) is the most common soft tissue sarcoma in pediatric and adolescent population, with 350 new cases diagnosed each year. While they can develop anywhere body, genitourinary tract second primary location for an RMS to develop. Overall survival has improved through increased use of protocols multidisciplinary approaches. However, guidelines management continue change as systemic radiation therapeutics advance. Given relative rarity this disease compared other non-solid childhood malignancies, healthcare providers not directly managing may be familiar their presentation updated management. This review aims provide foundational knowledge RMSs emphasis on specific paradigms those arising from tract. The but varies greatly symptomology depending organ origin. As clinical understanding these tumors advances, treatment have evolved. Herein, we describe breadth presentations diagnostic considerations, incorporating recently available societal consensus recommendations.

Language: Английский

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Rhabdomyosarcoma: Updates on classification and the necessity of molecular testing beyond immunohistochemistry

Human Pathology, Journal Year: 2023, Volume and Issue: 147, P. 72 - 81

Published: Dec. 21, 2023

Language: Английский

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Gender Differences in Soft Tissue and Bone Sarcoma: A Narrative Review

Cancers, Journal Year: 2023, Volume and Issue: 16(1), P. 201 - 201

Published: Dec. 31, 2023

Sarcomas, uncommon malignancies, stem from mesenchymal tissues, distinct epithelial originating in the embryonic mesodermal layer. These sarcomas have been categorized as either bone or soft tissue sarcomas, depending on their tissue. The majority of occur sporadically with etiology being unknown, but there are several, well-established genetic predisposition syndromes and some environmental exposures associated specific sarcomas. Recently, many studies shown that analogy colorectal, skin, head neck, esophageal, lung, liver carcinomas, also a male sex predilection. Significant gender differences already observed childhood Among tumors strongly sex, identified particularly sensitive to biological between sexes, special regard As mechanisms underlying incidence remain largely unexplored, this review aims highlight factors these inform prevention treatment.

Language: Английский

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The Rat Sarcoma Virus (RAS) Family of Proteins in Sarcomas

Cureus, Journal Year: 2024, Volume and Issue: unknown

Published: March 27, 2024

The rat sarcoma virus (RAS) protein family plays a crucial role in facilitating communication both within and between cells, thereby governing fundamental cellular processes such as growth, survival, differentiation. RAS comprises four members of small GTPases, namely Harvey (H-RAS), Kirsten (K-RAS, two splice variants, 4A 4B), Neuroblastoma (N-RAS), these are encoded by three genes. Mutations genes play significant cancer development progression. Accordingly, here we review discuss currently available literature about the fate function proteins sarcomas.

Language: Английский

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Emerging role and function of Hippo-YAP/TAZ signaling pathway in musculoskeletal disorders

Stem Cell Research & Therapy, Journal Year: 2024, Volume and Issue: 15(1)

Published: Oct. 29, 2024

Hippo pathway is an evolutionarily conservative key that regulates organ size and tissue regeneration by regulating cell proliferation, differentiation apoptosis. Yes-associated protein 1 (YAP)/ WW domain-containing transcription regulator (TAZ) serves as a pivotal factor within the signaling pathway, which undergoes negative regulation pathway. The expression of YAP/TAZ affects various biological processes, including osteoblasts (OB) osteoclasts (OC), cartilage homeostasis, skeletal muscle development, quality maintenance. At same time, dysregulation can concurrently contribute to development musculoskeletal disorders, bone tumors, osteoporosis (OP), osteoarthritis (OA), intervertebral disc degeneration (IDD), muscular dystrophy, rhabdomyosarcoma (RMS). Therefore, targeting has emerged promising therapeutic strategy for treatment disorders. focus this review elucidate mechanisms maintains homeostasis in bone, cartilage, muscle, while also providing comprehensive summary role played core components diseases. efficacy feasibility pathway-related drugs targeted therapy diseases are discussed our study. These endeavors offer novel insights into application effective targets potential drug candidates treating such conditions.

Language: Английский

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Primary Intracranial Sarcoma, DICER1-Mutant, With Prominent Chondroid Differentiation: Case Report and Summary of Reported Patients in Literature

International Journal of Surgical Pathology, Journal Year: 2025, Volume and Issue: unknown

Published: Jan. 7, 2025

Primary intracranial sarcoma, DICER1-mutant, included as a new diagnostic entity in the 2021 WHO Classification of Central Nervous System Tumors, is rare, but aggressive neoplasm generally identified supratentorial forebrain. The prognostic implications these uncommon tumors and optimal treatment strategy remain unclear. A 19-year-old woman was found unresponsive after reporting severe headache. CT demonstrated an intra-axial, mass-like hemorrhage left temporal lobe which subsequently resected. review electronic medical record, histologic characterization by hematoxylin eosin-stained sections targeted panel immunohistochemical stains, molecular pursued. tumor pleomorphic varying cellularity. cells had hyperchromatic nuclei with spindled to round appearance. Numerous mitoses, interspersed islands mature hyaline cartilage, scattered eosinophilic globules associated marked nuclear atypia were noted. positive for desmin, myogenin, SMSA (focal) negative other lineage markers, suggestive mesenchymal myogenic differentiation. Next-generation sequencing revealed DICER1 (E1705K P1805fs) KRAS (Q61H) variants; composite methylation profile prompted final diagnosis primary DICER1-mutant. This lesion underscores immunophenotypic variability rare tumors. Notable features include prominent cartilaginous islands, retained H3K27me3 expression, expression presence unique P1805fs variant, has not previously been reported this type. We compile findings all sarcomas, DICER1-mutant date.

Language: Английский

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Head and Neck Rhabdomyosarcoma in Pediatric Patients: An International Collaborative Study

Journal of Oral Pathology and Medicine, Journal Year: 2025, Volume and Issue: 54(2), P. 81 - 90

Published: Jan. 6, 2025

ABSTRACT Background Rhabdomyosarcoma (RMS), a rare malignant tumor, frequently affects pediatric patients, with 35%–40% occurring in the head and neck. This study analyzes clinicopathologic profile of neck rhabdomyosarcomas from Brazil, Guatemala, Mexico, South Africa. Methods We reviewed 44 cases 10 Oral Maxillofacial Pathology services, conducting immunohistochemical analyses desmin, myogenin, Myo‐D1, Ki67, quantification via QuPath software. Cases ≥ 50% myogenin expression were tested for fusion status using AP2β, NOS‐1, HMGA2. Statistical included Kruskal–Wallis test age marker comparisons, Fisher's exact categorical variables, Spearman's rank correlation relationships, multinomial logistic regression to assess likelihood. Results predominantly Brazil (40.9%), followed by Africa (27.3%), Guatemala (22.7%), Mexico (9.1%). Two‐thirds patients diagnosed their first decade no gender predilection. Nonparameningeal sites (45.5%) more affected than parameningeal (40.9%) orbital sites. Microscopically, embryonal RMS (77.3%) was most common, alveolar (18.2%) spindle cell (2.3%) tumors. Immunohistochemistry revealed positivity myogenic markers, significant differences between variants ( p < 0.05). Fusion prediction identified two potential fusion‐positive cases, while all one case appeared fusion‐negative. Significant positive found only AP2β NOS1 Conclusion Although there are slight clinical‐demographic variations among these regions, identifying through immunohistochemistry remains diagnostic challenge.

Language: Английский

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Ocular Hypertropia Mimicking Inferior Rectus Palsy as an Atypical Presentation of Parameningeal Rhabdomyosarcoma in a Child

International Medical Case Reports Journal, Journal Year: 2025, Volume and Issue: Volume 18, P. 99 - 104

Published: Jan. 1, 2025

Introduction: Rhabdomyosarcoma (RMS) originates from undifferentiated mesenchymal cells that give rise to striated muscles.The symptoms of para-meningeal RMS often resemble those allergic rhinosinusitis, including nasal congestion, mucus discharge, headache, and occasional nosebleeds.We report a child with atypical clinical presentation ocular hypertropia secondary RMS.Case Presentation: A presented an upward deviation the left eye.He had history blunt trauma face before 5 days.Computed tomography (CT) head revealed soft tissue density involving maxillary sinus.The magnetic resonance imaging (MRI) showed 37.6 mm x 38.4 lesion within sinus extending orbit, cavity, premaxillary retro areas heterogeneous signal mild enhancements.A biopsy histopathology confirmed alveolar RMS.The was treated chemotherapy radiotherapy.Conclusion: Pediatric orbital extension mimicking benign conditions is challenging diagnose manage.Hypertropia following eye can obscure severe underlying conditions, such as inferior rectus palsy stresses thorough evaluation, biopsy.Early accurate diagnosis crucial for effective management children aggressive malignancy.

Language: Английский

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