IU Indianapolis ScholarWorks :: Browsing by Subject "Sarcoma"

Browsing by Subject "Sarcoma"

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Alternative lengthening of telomeres (ALT) influences survival in soft tissue sarcomas: a systematic review with meta-analysis
(BMC, 2019-03-14) Lawlor, Rita T.; Veronese, Nicola; Pea, Antonio; Nottegar, Alessia; Smith, Lee; Pilati, Camilla; Demurtas, Jacopo; Fassan, Matteo; Cheng, Liang; Luchini, Claudio; Pathology and Laboratory Medicine, School of Medicine
Alternative lengthening of telomeres (ALT) is a telomerase-independent mechanism used by a broad range of neoplasms to maintain telomere length, permitting uncontrolled replication during their progression. ALT has been described in different types of sarcoma, but a comprehensive analysis of its clinical significance is still lacking. Therefore, we provide here the first meta-analysis on this topic. METHODS: We searched SCOPUS and PubMed through July 2018 to identify all studies that investigated the prognostic role of ALT in sarcomas. We considered the risk of death (risk ratio, RR) calculated as the number of death vs. total participants during follow-up in ALT+ versus ALT- patients as the primary outcome. The secondary outcome was the hazard ratio (HR), adjusted for the maximum number of covariates available, using ALT- patients as reference. RESULTS: Eight articles comprising a total of 551 patients with sarcomas (226 ALT+ and 325 ALT-) were selected. The ALT+ group showed a higher mitotic count and a higher tumor grade compared with the ALT- group (p < 0.01). Furthermore, we demonstrate a strong impact of ALT on survival. In fact, ALT+ patients showed a statistically significant higher risk of death than ALT- patients, when also considering data from multivariate analyses (RR = 1.50; 95% CI: 1.15-1.96; p = 0.003; HR = 2.02; 95% CI: 1.22-3.38; p = 0.007). CONCLUSIONS: Our results indicate that ALT is associated with an increased risk of death in patients with sarcoma. In these neoplasms, ALT should be taken into account for a precise prognostic stratification and design of potential therapeutic strategies.
Approach to wild-type gastrointestinal stromal tumors
(AME Publishing Company, 2018-11-15) Kays, Joshua K.; Sohn, Jeffrey D.; Kim, Bradford J.; Goze, Katherine; Koniaris, Leonidas G.; Surgery, School of Medicine
Gastrointestinal stromal tumors (GISTs) arise from the intestinal pacemaker cells of Cajal. Wild-type gastrointestinal stromal tumors (WT-GIST) are a unique and uncommon subtype of GISTs that lack activating mutations in the tyrosine kinase c-KIT or platelet derived growth factor receptor alpha (PDGFRA) receptors. The lack of these growth-stimulating mutations renders tyrosine kinase receptor inhibitors, such as imatinib mesylate, relatively ineffective against these tumors. WT-GIST arises most commonly due to underlying alternate proliferative signals associated with germ-line, genetic mutations. WT-GIST frequently arises in patients with BRAF mutations, Carney’s Triad or neurofibromatosis type-1 (NF-1). All patients with WT-GIST require a careful examination for germ-line mutations and very close observation for recurrent tumors. Surgery remains a mainstay therapy for these patients. This review aims to discuss the most recent data available on the diagnosis and treatment of WT-GIST.
Bilateral Renal Auto-Transplantation for Retroperitoneal Sarcomas: Is It Underutilized?
(MDPI, 2023-08-14) Robinson, Tyler P.; Milgrom, Daniel P.; Nagaraju, Santosh; Goggins, William C.; Samy, Kannan P.; Koniaris, Leonidas G.; Surgery, School of Medicine
Sarcomas are a rare tumor of mesenchymal origin. The liposarcoma is the most common sarcoma of the retroperitoneum. Liposarcomas are typically low grade, and present at an advanced stage and a large size. We report a case of a large retroperitoneal liposarcoma, approximately 50 kg, encasing both kidneys, which was managed via a two-stage resection and staged renal auto-transplantation into the intra-peritoneal pelvis. The patient maintained normal renal function throughout, and remains disease free two years post-resection. Renal auto-transplantation with pelvic placement may facilitate improved margin-free resection. Renal relocation may allow the use of curative-intent ablative therapies such as radiofrequency ablation and radiation in cases of retroperitoneal recurrence.
Correction: Exploring transcriptional regulators Ref-1 and STAT3 as therapeutic targets in malignant peripheral nerve sheath tumours
(Springer Nature, 2022) Gampala, Silpa; Shah, Fenil; Zhang, Chi; Rhodes, Steven D.; Babb, Olivia; Grimard, Michelle; Wireman, Randall S.; Rad, Ellie; Calver, Brian; Bai, Ren-Yuan; Staedtke, Verena; Hulsey, Emily L.; Saadatzadeh, M. Reza; Pollok, Karen E.; Tong, Yan; Smith, Abbi E.; Clapp, D. Wade; Tee, Andrew R.; Kelley, Mark R.; Fishel, Melissa L.; Pediatrics, School of Medicine
Correction to: British Journal of Cancer 10.1038/s41416-021-01270-8, published online 03 March 2021 The original version of this article unfortunately contained an error in Figure 4, specifically: Figure 4f: the middle cell image was originally a duplicate of the middle cell image from Fig. 4d; the correct image is now used. The corrected figure is displayed below. The correction does not have any effect on the final conclusions of the paper. The original article has been corrected.
Histiocytic sarcoma arising from a lymph node: a diagnostic conundrum
(Springer, 2021-05-21) Afzal, Anoshia; Asadbeigi, Sepideh N.; Farooque, Umar; Mather, Christy; Khan, Shahbaz; Pathology and Laboratory Medicine, School of Medicine
Histiocytic Sarcoma (HS) is extremely rare, with only a few hundred cases reported in the literature. The majority of patients present with symptoms due to unifocal or multifocal extra-nodal disease. Less than 20% of these cases show solitary involvement of a lymph node. We report a case of a solitary HS in a 53-year-old woman presenting with a 2.7-cm right groin mass arising from an inguinal lymph node. The initial cytologic examination of the tissue showed a high-grade spindle-shaped morphology with high-grade mitotic activity. A high-grade sarcoma was initially considered considering the absence of normal lymphoid aggregate and the presence of high-grade cytologic features in the cells. To evaluate the tumor in its entirety, the mass was surgically excised. A histological examination of the tumor showed focal rimming of the lymphoid tissue at the periphery and a centrally located stellate necrotic focus. The tumor cells had an epithelioid to spindle cell morphology along with large uniform nuclei and prominent nucleoli. A high mitotic index was present. Immunohistochemistry (IHC) stains showed strong positivity for CD68, CD163, and Vimentin, and were weakly positive for SMA and CD45. Based on the histologic and clinical examination, a diagnosis of HS was made. Multiple malignancies can mimic HS histopathology and the rarity of this tumor makes the diagnosis more challenging. No fine-needle aspiration (FNA) criteria for its diagnosis have been recognized. Herein, we report a rare case of an isolated HS involving a lymph node which resembled high-grade sarcoma on the FNA biopsy to raise awareness among our surgical pathologist colleagues.
Incisions and reconstruction approaches for large sarcomas
(AME Publishing Company, 2018-10-31) Spera, Leigh J.; Danforth, Rachel M.; Hadad, Ivan; Surgery, School of Medicine
Large intraabdominal, retroperitoneal, and abdominal wall sarcomas provide unique challenges in treatment due to their variable histology, potential considerable size at the time of diagnosis, and the ability to invade into critical structures. Historically, some of these tumors were considered inoperable if surgical access was limited or the consequential defect was unable to be closed primarily as reconstructive options were limited. Over time, there has been a greater understanding of the abdominal wall anatomy and mechanics, which has resulted in the development of new techniques to allow for sound oncologic resections and viable, durable options for abdominal wall reconstruction. Currently, intra-operative positioning and employment of a variety of abdominal and posterior trunk incisions have made more intraabdominal and retroperitoneal tumors accessible. Primary involvement or direct invasion of tumor into the abdominal wall is no longer prohibitive as utilization of advanced hernia repair techniques along with the application of vascularized tissue transfer have been shown to have the ability to repair large area defects involving multiple quadrants of the abdominal wall. Both local and distant free tissue transfer may be incorporated, depending on the size and location of the area needing reconstruction and what residual structures are remaining surrounding the resection bed. There is an emphasis on selecting the techniques that will be associated with the least amount of morbidity yet will restore and provide the appropriate structure and function necessary for the trunk. This review article summarizes both initial surgical incisional planning for the oncologic resection and a variety of repair options for the abdominal wall spanning the reconstructive ladder.
Integrating therapies for surgical adult soft tissue sarcoma patients
(AME Publishing Company, 2018-11-02) Milgrom, Daniel P.; Sehdev, Amikar; Kays, Joshua K.; Koniaris, Leonidas G.; Surgery, School of Medicine
Sarcomas are an uncommon group of over 50 different individual histological malignancies arising from mesenchymal (non-epithelial or connective) tissues. Overall, they constitute 1% of human malignancies with an annual incidence rate of fewer than 5 patients per million. Sarcoma may arise from any mesenchymal cell lineages including fat, muscle, or other connective tissues. Due to the rarity of these groups of malignancies, many subtypes were, and still today, are managed as a single entity. This review focused on soft tissue sarcomas with an emphasis on how to integrate therapies for patients with this rare disorder. The role for surgical resection in cure and palliation as well as the relative benefits of adjuvant therapies such as chemotherapy and radiation therapy are discussed.
Precision Medicine Highlights Dysregulation of the CDK4/6 Cell Cycle Regulatory Pathway in Pediatric, Adolescents and Young Adult Sarcomas
(MDPI, 2022-07-25) Barghi, Farinaz; Shannon, Harlan E.; Saadatzadeh, M. Reza; Bailey, Barbara J.; Riyahi, Niknam; Bijangi-Vishehsaraei, Khadijeh; Just, Marissa; Ferguson, Michael J.; Pandya, Pankita H.; Pollok, Karen E.; Medical and Molecular Genetics, School of Medicine
Despite improved therapeutic and clinical outcomes for patients with localized diseases, outcomes for pediatric and AYA sarcoma patients with high-grade or aggressive disease are still relatively poor. With advancements in next generation sequencing (NGS), precision medicine now provides a strategy to improve outcomes in patients with aggressive disease by identifying biomarkers of therapeutic sensitivity or resistance. The integration of NGS into clinical decision making not only increases the accuracy of diagnosis and prognosis, but also has the potential to identify effective and less toxic therapies for pediatric and AYA sarcomas. Genome and transcriptome profiling have detected dysregulation of the CDK4/6 cell cycle regulatory pathway in subpopulations of pediatric and AYA OS, RMS, and EWS. In these patients, the inhibition of CDK4/6 represents a promising precision medicine-guided therapy. There is a critical need, however, to identify novel and promising combination therapies to fight the development of resistance to CDK4/6 inhibition. In this review, we offer rationale and perspective on the promise and challenges of this therapeutic approach.
Solitary fibrous tumor
(AME Publishing Company, 2018-11-21) Davanzo, Brian; Emerson, Robert E.; Lisy, Megan; Koniaris, Leonidas G.; Kays, Joshua K.; Surgery, School of Medicine
Solitary fibrous tumor (SFT) is a rare tumor of mesenchymal origin that account for less than 2% of all soft tissue masses. Initially identified in the pleura, SFT has been identified in multiple anatomic locations and can arise anywhere in the body. The varying histologic features along with non-specific means of identification have led SFT to be associated with several different names. Over the last several decades, sustained advances through research and technology have led to more reliable methods for differentiating this distinct soft tissue tumor. Advances specifically in immunohistochemistry and molecular diagnostics have identified CD34 as the most consistent marker in SFT, however even this lacks specificity to conclusively narrow down the broad differential for exact identification. More recently the discovery of the NAB2-STAT6 fusion gene has led to more precise diagnosis of SFT. Like many other soft tissue tumors, surgical management is the mainstay of treatment for SFT with emphasis on obtaining tumor-negative margins. Radiation therapy and chemotherapy regimens have not demonstrated global effectiveness, and thus no standardized treatments have been identified. Given the rarity of SFT and current supportive evidence for therapies, management should be focused on tumor extirpation. Nonetheless, individualized therapy, determined within a multidisciplinary setting should be considered.

Browsing by Subject "Sarcoma"

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