Sarcoma[JOURNAL]
Sun
200 papers
Exploring Mentorship as a Novel Approach to Improving Quality of Life in Sarcoma Survivors: A Qualitative Pilot Study.
Våde L, Hompland I, Fauske L
Sarcoma · 2021 · PMID 34421328 · Full text
BACKGROUNDS: To investigate whether a formal mentoring program involving mentors from the business community could improve the quality of life (QoL) of sarcoma survivors struggling with the late effects of treatment. MET...
BACKGROUNDS: To investigate whether a formal mentoring program involving mentors from the business community could improve the quality of life (QoL) of sarcoma survivors struggling with the late effects of treatment. METHODS: Seven former sarcoma patients participated in an eight-month formal mentoring program. The program was assessed through a qualitative study involving a phenomenological approach that utilized a hermeneutical design. In-depth, semistructured interviews were conducted with the mentees after the intervention and six months later. The mentors were interviewed after the program was over. The gathered data were interpreted using a thematic analysis. RESULTS: The program facilitated dialogue between the mentors and mentees as well as between the mentees. Afterwards, the mentees were more willing to accept the challenges they faced following cancer treatment. During the program, the mentees were pushed out of their comfort zone, which led to mastery and personal growth in them all. However, the program also revealed some additional challenges, including unfulfilled expectations in two mentor-mentee relationships. CONCLUSIONS: The mentoring program facilitated the mentees' reorientation and enhanced their QoL. Its eight-month duration appeared important in terms of allowing the mentees to go through a long-lasting process with continued support. The program could serve as the basis for larger studies involving other cancer survivors.
Establishment of an Academic Tissue Microarray Platform as a Tool for Soft Tissue Sarcoma Research.
Lee CJ, Wozniak A, Van Cann T … +9 more , Timmermans I, Wellens J, Vanleeuw U, Briaire-de Bruijn IH, Britschgi C, Bovée JVMG, Zlobec I, Sciot R, Schöffski P
Sarcoma · 2021 · PMID 34413700 · Full text
Soft tissue sarcoma (STS) is a heterogeneous family of rare mesenchymal tumors, characterized by histopathological and molecular diversity. Tissue microarray (TMA) is a tool that allows performing research in orphan dise...
Soft tissue sarcoma (STS) is a heterogeneous family of rare mesenchymal tumors, characterized by histopathological and molecular diversity. Tissue microarray (TMA) is a tool that allows performing research in orphan diseases in a more efficient and cost-effective way. TMAs are paraffin blocks consisting of multiple small representative tissue cores from biological samples, for example, from multiple donors, diverse sites of disease, or multiple different diseases. In 2015, we began constructing TMAs using archival tumor material from STS patients. Specimens were well annotated in terms of histopathological diagnosis, treatment, and clinical follow-up of the tissue donors. Each TMA block contains duplicate or triplicate 1.0-1.5 mm tissue cores from representative tumor areas selected by sarcoma pathologists. The construction of TMAs was performed with TMA Grand Master (3DHistech). So far, we have established disease-specific TMAs from 7 STS subtypes: gastrointestinal stromal tumor (72 cases included in the array), alveolar soft part sarcoma ( = 12 + 47), clear cell sarcoma ( = 22 + 32), leiomyosarcoma ( = 55), liposarcoma ( = 42), inflammatory myofibroblastic tumor ( = 12 + 21), and alveolar rhabdomyosarcoma ( = 24). We also constructed a multisarcoma TMA covering a representative number of important histopathological subtypes on arrays for screening purposes, namely, angiosarcoma, dedifferentiated liposarcoma, pleomorphic liposarcoma, and myxoid liposarcoma, leiomyosarcoma, malignant peripheral nerve sheath tumor, myxofibrosarcoma, rhabdomyosarcoma, synovial sarcoma, and undifferentiated pleomorphic sarcoma, with 7-11 individual cases per subtype. We are currently expanding the list of TMAs with additional sarcoma entities, considering the heterogeneity of this family of tumors. Our extensive STS TMA platform is suitable for rapid and cost-effective morphological, immunohistochemical, and molecular characterization of the tumor as well as for the identification of potential novel diagnostic markers and drug targets. It is readily available for collaborative projects with research partners.
Prospects for Epigenetic Targeted Therapies of Bone and Soft-Tissue Sarcomas.
Wang J, Elkrief A, Guo W … +3 more , Shukla N, Gounder M, Ladanyi M
Sarcoma · 2021 · PMID 34349608 · Full text
Targeted therapies have revolutionized cancer treatment. It is well established that alterations of chromatin configuration and modifications affect tumorigenesis of some, possibly most, bone and soft-tissue sarcomas. As...
Targeted therapies have revolutionized cancer treatment. It is well established that alterations of chromatin configuration and modifications affect tumorigenesis of some, possibly most, bone and soft-tissue sarcomas. As epigenetic regulators play a major role in the development of bone and soft-tissue sarcomas, epigenetic drugs provide a novel potential avenue for rational targeted therapies for these aggressive cancers. The present review summarizes the application of epigenetic drugs for clinical utilization in bone and soft-tissue sarcomas and provides an overview of clinical trials currently evaluating epigenetic therapies in this space.
Patterns of Perioperative Treatment and Survival of Localized, Resected, Intermediate- or High-Grade Soft Tissue Sarcoma: A 2000-2017 Netherlands Cancer Registry Database Analysis.
Van Meekeren M, Fiocco M, Ho VKY … +3 more , Bovée JVMG, Gelderblom H, Haas RL
Sarcoma · 2021 · PMID 34335076 · Full text
BACKGROUND: Standard therapy for localized soft tissue sarcoma (STS) is wide, limb-sparing resection. For intermediate- or high-grade tumors, (neo)adjuvant therapies are frequently added to the treatment plan. In this st...
BACKGROUND: Standard therapy for localized soft tissue sarcoma (STS) is wide, limb-sparing resection. For intermediate- or high-grade tumors, (neo)adjuvant therapies are frequently added to the treatment plan. In this study, data from a Dutch nationwide database are used to (1) assess whether perioperative management of STS follows ESMO guidelines, (2) characterize prognostic factors for overall survival (OS), and (3) assess the association between perioperative treatment and survival. METHODS: All intermediate- or high-grade, localized STS cases, who have undergone surgery and diagnosed between 2000 and 2017, were identified in the Netherlands Cancer Registry (NCR) database. Variables with demographic, treatment, and survival data were obtained. Survival curves were estimated by Kaplan-Meier's method, and the effect of prognostic factors on OS was assessed in a multivariable Cox regression analysis. RESULTS: A total of 4957 patients were identified. There were slightly more males (54.7%). Median age at diagnosis was 64 years, and 53.6% of the tumors were located in the extremities. Radiotherapy (RT) was administered to 2481 (50.1%) patients, and 252 (5.1%) patients were treated with perioperative systemic chemotherapy. The total use of perioperative RT did not significantly change in the last 20 years, but the timing followed clinical guidelines: preoperative RT increased significantly (2000-2008: 3.7%, 2009-2017: 22.3%; < 0.001), whereas the use of postoperative RT diminished (2000-2008: 45.9%, 2009-2017: 26.1%; < 0.001). The use of perioperative chemotherapy slightly decreased (2000-2008: 5.9%, 2009-2017: 4.4%; = 0.015). 5-year OS was 59.6% (95% CI: 58.2-61.0). Sex, age, year of diagnosis, tumor location, tumor size, histological grade, depth, histological subtype, surgical margins, and the use of perioperative RT were identified as independent predictors for OS. CONCLUSION: Preoperative RT is gradually replacing postoperative RT for localized STS in the Netherlands. The use of perioperative chemotherapy is rare and has slightly decreased in recent years. Identified baseline characteristics and treatment factors predicting OS may aid in future treatment decisions.
Uterine Sarcomas: Are There MRI Signs Predictive of Histopathological Diagnosis? A 50-Patient Case Series with Pathological Correlation.
Hélage S, Vandeventer S, Buy JN … +6 more , Bordonné C, Just PA, Jacob D, Ghossain M, Rousset P, Dion É
Sarcoma · 2021 · PMID 34305438 · Full text
PURPOSE: To make clear distinction between two radiological types of uterine sarcomas. METHODS: 50 preoperative MRI were analyzed retrospectively, blinded to histopathology: 11 endometrial stromal sarcomas (ESS), 19 leio...
PURPOSE: To make clear distinction between two radiological types of uterine sarcomas. METHODS: 50 preoperative MRI were analyzed retrospectively, blinded to histopathology: 11 endometrial stromal sarcomas (ESS), 19 leiomyosarcomas (LMS), 18 carcinosarcomas/malignant mixed Mullerian tumors (MMMT), and 2 smooth muscle tumors of uncertain malignant potential (STUMP). RESULTS: According to their locations, two radiological types of sarcomas were identified: type 1: intracavitary (ESS, MMMT) and type 2: intramyometrial (LMS, STUMP). In both types, all tumors displayed intermediate T2-weighted signal ( < 0.001) and high diffusion-weighted imaging (DWI) b1000 signal ( < 0.001). Dynamic contrast-enhanced (DCE) MRI showed intratumoral pathologic vessels (98%) and heterogeneity at venous phase ( < 0.001). In the type 1 subgroup, all tumors displayed local spread: invasion of junctional zone on T2-weighted imaging (T2WI), irregular margins on DWI, and disruption of arcuate arteries subendometrial ring on DCE-MRI. In the type 2 subgroup, all tumors displayed irregular margins on T2WI, DWI, and DCE-MRI. Tumor heterogeneity was due to necrosis ( < 0.001). Most commonly the tumor was single (61%). In both types, apparent diffusion coefficient (ADC) lesser than or equal to 0.86 × 10 mm/s (sensitivity = 73%, specificity = 92%) was suggestive of malignancy. CONCLUSION: It may be feasible to get close to histological type of a uterine sarcoma based on our topographic classification into two radiological subgroups, corresponding to two kinds of diagnostic difficulties. . MRI signs suggestive of histopathological malignancy are identifiable, considering the triad T2WI/DWI/DCE-MRI, easily for type 1 but less easily for type 2; the threshold value for ADC is 0.86 × 10 mm/s.
Predictors of Recurrence and Patterns of Initial Failure in Localized Ewing Sarcoma: A Contemporary 20-Year Experience.
Stachelek GC, Ligon JA, Vogel J … +8 more , Levin AS, Llosa NJ, Ladle BH, Meyer CF, Terezakis SA, Morris CD, Ladra MM, Pratilas CA
Sarcoma · 2021 · PMID 33953640 · Full text
BACKGROUND: The majority of patients with localized Ewing sarcoma will remain disease-free long term, but for those who suffer recurrence, successful treatment remains a challenge. Identification of clinicopathologic fac...
BACKGROUND: The majority of patients with localized Ewing sarcoma will remain disease-free long term, but for those who suffer recurrence, successful treatment remains a challenge. Identification of clinicopathologic factors predictive of recurrence could suggest areas for treatment optimization. We sought to describe our experience regarding predictors of recurrence and patterns of first failure in patients receiving modern systemic therapy for nonmetastatic Ewing sarcoma. METHODS: The medical records of pediatric and adult patients treated for localized Ewing sarcoma between 1999 and 2019 at Johns Hopkins Hospital were retrospectively analyzed. Local control was surgery, radiotherapy, or both. Recurrence-free survival (RFS) was calculated using the Kaplan-Meier method. Univariable and multivariable Cox proportional-hazards modeling was performed to obtain hazard ratios (HR) for recurrence. RESULTS: In 94 patients with initially localized disease, there were 21 recurrences: 4 local, 14 distant, and 3 combined. 5-year and 10-year RFS were 75.6% and 70.5%, respectively. On multivariable analysis including age at diagnosis and tumor size, <95% tumor necrosis following neoadjuvant chemotherapy (NAC; HR 14.3, = 0.028) and radiological tumor size change during NAC (HR 1.04 per 1% decrease in size change, = 0.032) were independent predictors of recurrence. Among patients experiencing distant recurrence, pulmonary metastases were present in 82% and were the only identifiable site of disease in 53%. CONCLUSIONS: Poor pathologic or radiologic response to NAC is predictive of recurrence in patients with localized Ewing sarcoma. Suboptimal tumor size reduction following chemotherapy provides a means to risk-stratify patients who do not undergo definitive resection. Isolated pulmonary recurrence was a common event.
Adult Pleomorphic Rhabdomyosarcomas: Assessing Outcomes Associated with Radiotherapy and Chemotherapy Use in the National Cancer Database.
Reddy VK, Jain V, Wilson Ii RJ … +6 more , Hartner LP, Diamond M, Sebro RA, Weber KL, Maki RG, Shabason JE
Sarcoma · 2021 · PMID 33814964 · Full text
PURPOSE: Practice patterns for treatment of localized adult pleomorphic rhabdomyosarcoma (PRMS) remain quite variable given its rarity. Current national guidelines recommend management similar to that of other high-grade...
PURPOSE: Practice patterns for treatment of localized adult pleomorphic rhabdomyosarcoma (PRMS) remain quite variable given its rarity. Current national guidelines recommend management similar to that of other high-grade soft tissue sarcomas (STS), which include surgery with perioperative radiation (RT) with or without chemotherapy. Using the National Cancer Database (NCDB), we assessed practice patterns and overall outcomes of patients with localized PRMS. . Patients with stage II/III PRMS treated with surgical resection from 2004 to 2015 were identified from the NCDB. Predictors of RT and chemotherapy use were assessed using multivariable logistic regression analysis. The association of radiation and chemotherapy status on overall survival was assessed using Kaplan-Meier and Cox proportional hazards analyses. RESULTS: Of 243 total patients, RT and chemotherapy were not uniformly utilized, with 44% receiving chemotherapy and in those who did not undergo amputation 62% receiving RT. In those who did not undergo amputation, RT was associated with improved survival on both univariate (HR: 0.49, 95% CI 0.32-0.73, < 0.001) and multivariate analysis (HR: 0.40, 95% CI 0.26-0.62, < 0.001), corresponding to greater 5-year overall survival (59% vs. 38%, < 0.001). Chemotherapy was associated with a higher rate of 5-year overall survival (63% vs. 39%, < 0.001). However, the survival benefit of chemotherapy did not reach statistical significance on multivariate analysis (HR: 0.65, 95% CI 0.41-1.03, =0.064). Notable predictors of omission of RT included female gender (OR: 0.40, 95% CI 0.22-0.74, < 0.01) and age ≥ 70 (OR: 0.55, 95% CI 0.30-1.00, =0.05). Correspondingly, factors associated with omission of chemotherapy included age ≥70 (OR: 0.17, 95% CI 0.08-0.39, < 0.001). CONCLUSIONS: A significant proportion of patients with localized adult PRMS are not receiving RT. Likewise, use of chemotherapy was heterogeneous. Our findings note potential benefits and underutilization of RT, for which further investigation is warranted.
Reconstructive Challenges of Distal Tibia Bone Tumors: Extracorporeally Irradiated Autograft Combined with a Nonvascularized Autograft Fibula for Superior Reconstruction and Functional Outcomes When Compared to Ipsilateral Pedicled Fibula Transfer Alone.
Gundavda MK, Agarwal MG, Reddy R … +1 more , Bary A
Sarcoma · 2021 · PMID 33814963 · Full text
INTRODUCTION: Traditionally, centralization of the fibula with fusion across the tibiotalar joint has been used to reconstruct distal tibial defects. Although effective, it requires long periods of protected weight-beari...
INTRODUCTION: Traditionally, centralization of the fibula with fusion across the tibiotalar joint has been used to reconstruct distal tibial defects. Although effective, it requires long periods of protected weight-bearing. The fibula or the fixation often fails before fibular hypertrophy necessitating multiple additional surgeries. A method of using ECRT with the available ipsilateral fibula (nonvascularized) to reconstruct the distal tibia defect with the aim of early return to weight-bearing was evolved. This paper documents our early experience. . Four patients; with the diagnosis of osteosarcoma in 3 patients and recurrent giant cell tumor of the bone in 1 patient, underwent resection of the distal tibia for tumors between 2017 and 2019. Extracorporeally irradiated (50 Gy) distal tibia along with ipsilateral nonvascularized fibula was used to bridge the defect and fuse the tibiotalar joint. A plate was used to rigidly hold the construct. The final outcome was compared to the historical control group that underwent only pedicled ipsilateral fibula transposition and ankle arthrodesis without recycled autograft or allograft between 2009 and 2017. Oncological reconstruction and functional outcomes were compared for each group. Patient reported outcomes on the acceptability of ankle fusion; cosmesis and function were analyzed and compared between the two groups. RESULTS: The mean resection length in the study group (4 patients) was 7.75 cm (7 to 8.5 cm). As compared to the historical cohort of 7 patients, the study population showed statistically superior results in all reconstruction, functional, and patient-reported outcomes except time to proximal junction union (=0.068). There were no reconstruction failures, infection, or nonunions in the study group, whereas the control comparative group had 2 proximal junction nonunions and a mean time to fibular hypertrophy of 143 weeks (82 to 430 weeks) with fibula centralization. Earlier weight-bearing was allowed (mean 26.75 weeks; median 27 weeks) compared to (mean 80.75 weeks; median 80 weeks) in the control group. CONCLUSION: We think that ECRT with ipsilateral vascularized fibula is a promising method of reconstructing the distal tibia. The recycled autograft tibia added strength to the distal tibia construct in our study and aided the anatomical reconstruction of the distal tibia. The patient-reported outcomes for cosmesis and acceptability add to the benefits of performing this procedure. Consistent early union across the proximal junction and earlier weight-bearing were clear advantages of this method.
Postradiation Fractures after Combined Modality Treatment in Extremity Soft Tissue Sarcomas.
Bartelstein MK, Yerramilli D, Christ AB … +5 more , Kenan S, Ogura K, Fujiwara T, Fabbri N, Healey JH
Sarcoma · 2021 · PMID 33790687 · Full text
Soft tissue sarcoma (STS) of the extremities is typically treated with limb-sparing surgery and radiation therapy; with this treatment approach, high local control rates can be achieved. However, postradiation bone fract...
Soft tissue sarcoma (STS) of the extremities is typically treated with limb-sparing surgery and radiation therapy; with this treatment approach, high local control rates can be achieved. However, postradiation bone fractures, fractures occurring in the prior radiation field with minimal or no trauma, are a serious late complication that occurs in 2-22% of patients who receive surgery and radiation for STS. Multiple risk factors for sustaining a postradiation fracture exist, including high radiation dose, female sex, periosteal stripping, older age, femur location, and chemotherapy administration. The treatment of these pathological fractures can be difficult, with complications including delayed union, nonunion, and infection posing particular challenges. Here, we review the mechanisms, risk factors, and treatment challenges associated with postradiation fractures in STS patients.
Testicular, Spermatic Cord, and Scrotal Soft Tissue Sarcomas: Treatment Outcomes and Patterns of Failure.
Chowdhry VK, Kane JM, Wang K … +3 more , Joyce D, Grand'Maison A, Mann GN
Sarcoma · 2021 · PMID 33746565 · Full text
INTRODUCTION: Paratesticular sarcomas are defined as tumors that arise within the scrotum and include the subsites of epididymis, spermatic cord, and tunica vaginalis and represent the most common type of GU sarcoma. The...
INTRODUCTION: Paratesticular sarcomas are defined as tumors that arise within the scrotum and include the subsites of epididymis, spermatic cord, and tunica vaginalis and represent the most common type of GU sarcoma. The mainstay of treatment is often surgical resection, combined with histology specific chemotherapy and radiotherapy. Due to the rare nature of the disease, there are limited data to guide management. We present our single-institution retrospective experience regarding the management and treatment of paratesticular sarcomas. MATERIALS AND METHODS: We queried our oncology registry database for patients treated for testicular, spermatic cord, and scrotal soft tissue sarcomas between 1971 and 2017. Patients in this series had pathological confirmation of a sarcoma diagnosis by a sarcoma-specialized pathologist. Only patients with localized disease were included in this analysis with the exception of patients with a diagnosis of rhabdomyosarcoma where patients with both localized and metastatic disease were included on this study. RESULTS: A total of 34 patients were included in this retrospective analysis. The median was 24 (range, 5-78), and the median tumor size was 6.25 cm. Twenty-six patients had localized disease (76.6%) at the time of diagnosis. A predominance of patients had tumors involving the spermatic cord (45.5%), and the most common histology was rhabdomyosarcoma (35.3%), leiomyosarcoma (26.5%), and well-differentiated liposarcoma (23.5%). The median follow-up was 71.0 months (range, 2.5-534.4 months). A total of 7 patients experienced an isolated local failure (20.6%), four patients developed distant metastatic disease (11.8%), and one patient (2.9%) with synovial sarcoma of the spermatic cord experienced a regional recurrence. The median progression-free survival (PFS) was 99.6 months, 95% CI (45.8-534.3 months), with a three-year PFS rate of 71%, 95% CI (53%-83%), and a 5-year PFS rate of 64% (range, 46%-78%). We did not find any statistically significant associations based on surgery type (=0.15), the use of chemotherapy, (=0.36), or final margin status (=0.21). Two patients who were treated with preoperative radiotherapy had significant wound healing complication with chronic sinus tracts, though these patients did not experience a local recurrence. CONCLUSIONS: We provide a characterization of the natural history and treatment patterns of paratesticular sarcomas. While effective at reducing a local recurrence, preoperative radiotherapy was associated with significant toxicity. As a result, we prefer the use of postoperative radiotherapy in patients as clinically indicated. We did not find any specific treatment patterns associated with an improvement in clinical outcomes.
Comparison of Publication Rates for Musculoskeletal Oncology Abstracts Presented at National Meetings.
Collier CD, Quereshy HA, Getty PJ
Sarcoma · 2021 · PMID 33746564 · Full text
BACKGROUND: Scientific meetings provide a forum to disseminate new research and advance patient care. The American Academy of Orthopaedic Surgeons (AAOS), Connective Tissue Oncology Society (CTOS), and Musculoskeletal Tu...
BACKGROUND: Scientific meetings provide a forum to disseminate new research and advance patient care. The American Academy of Orthopaedic Surgeons (AAOS), Connective Tissue Oncology Society (CTOS), and Musculoskeletal Tumor Society (MSTS) annual meetings are examples of such gatherings in the field of musculoskeletal oncology. After a review of select MSTS abstracts from 1991 to 1999 revealed a 41% publication rate in scientific journals, previous authors cautioned meeting attendees that the majority of abstracts may not survive rigorous peer review and may not be scientifically valid. Since two decades have passed, this study reexamined publication rates and characteristics in a contemporary and expanded cohort of oncology abstracts presented at the AAOS, CTOS, and MSTS annual meetings. METHODS: 1408 podium and poster abstracts from the AAOS (oncology-focused from 2013 to 2015), CTOS (2012 to 2014), and MSTS (2012 to 2014) annual meetings were reviewed to allow for a four-year publication window. Searches were performed with PubMed and Google Scholar databases to identify full-text publications using abstract keywords. Characteristics of each abstract and resulting publication were collected. Statistical analysis was performed using the chi-square and Kruskal-Wallis tests for time-independent comparisons, and the log-rank test after reverse Kaplan-Meier analysis for time-dependent comparisons. RESULTS: Abstract publication rates overall were higher for podium presentations (67%, 280 of 415) compared to poster presentations (53%, 530 of 993; < 0.001). When both abstract types were combined, differences between meetings did not meet statistical significance (AAOS: 65%, 106 of 162; CTOS: 57%, 521 of 909; MSTS: 54%, 183 of 337, =0.06). Abstracts from AAOS meetings were more often published prior to the first day of the meeting (AAOS: 24%, 25 of 106; CTOS: 10%, 52 of 521; MSTS: 14%, 25 of 183; < 0.01). After excluding previously published abstracts, AAOS abstracts had the shortest time to publication (median: 10.8 months, interquartile range (IQR): 4.4 to 18.8 months), compared to those from CTOS (16.0 months, 8.4 to 25.9 months, < 0.01) and MSTS (15 months, 7.9 to 25.0 months, < 0.01) meetings. CTOS abstracts were published in higher impact journals (median: 3.7, IQR: 2.9 to 5.9), compared to those from AAOS (2.9, 1.9 to 3.2, < 0.01) and MSTS (3.1, 2.3 to 3.1, < 0.01) meetings. Finally, 7.7% (62 of 810) of published abstracts were presented at more than one meeting. CONCLUSIONS: Publication rates in this study were higher than previous reports in musculoskeletal oncology and comparable or better than recent reports for other orthopedic meetings. Comparisons across the AAOS, CTOS, and MSTS annual meetings highlight notable differences but suggest similarity overall in the quality of evidence presented with little overlap between meetings. Taken together, this study points to progress in the review processes used by the program committees, reaffirms the importance of critical appraisal when considering abstract findings, and supports the continued organization of multiple scientific meetings in musculoskeletal oncology.
Corrigendum to "Clinical Presentation, Natural History, and Therapeutic Approach in Patients with Solitary Fibrous Tumor: A Retrospective Analysis".
Schöffski P, Timmermans I, Hompes D … +15 more , Stas M, Boecxstaens V, Sinnaeve F, De Leyn P, Coosemans W, Van Raemdonck D, Hauben E, Sciot R, Clement P, Bechter O, Beuselinck B, Woei-A-Jin FJSH, Dumez H, Nafteux P, Wessels T
Sarcoma · 2021 · PMID 33628084 · Full text
[This corrects the article DOI: 10.1155/2020/1385978.].
[This corrects the article DOI: 10.1155/2020/1385978.].
Prognostic and Therapeutic Utility of Variably Expressed Cell Surface Receptors in Osteosarcoma.
Zvi Y, Ugur E, Batko B … +10 more , Gill J, Roth M, Gorlick R, Hall D, Tingling J, Barkauskas DA, Zhang J, Yang R, Hoang BH, Geller DS
Sarcoma · 2021 · PMID 33603563 · Full text
BACKGROUND: Six cell surface receptors, human epidermal growth factor receptor-2 (Her-2), platelet-derived growth factor receptor- (PDGFR-), insulin-like growth factor-1 receptor (IGF-1R), insulin receptor (IR), c-Met, a...
BACKGROUND: Six cell surface receptors, human epidermal growth factor receptor-2 (Her-2), platelet-derived growth factor receptor- (PDGFR-), insulin-like growth factor-1 receptor (IGF-1R), insulin receptor (IR), c-Met, and vascular endothelial growth factor receptor-3 (VEGFR-3), previously demonstrated variable expression across varying patient-derived and standard osteosarcoma (OS) cell lines. The current study sought to validate previous expression patterns and evaluate whether these receptors offer prognostic and/or therapeutic value. METHODS: Patient-derived OS cell lines ( = 52) were labeled with antibodies to Her-2, PDGFR-, IGF-1R, IR, c-Met, and VEGFR-3. Expression was characterized using flow cytometry. The difference in geometric mean fluorescent intensity (geoMFI = geoMFI - geoMFI) was calculated for each receptor across all cell lines. Receptor expression was categorized as low (Q1), intermediate (Q2, Q3), or high (Q4). The event-free survival (EFS) and overall survival for the six cell surface receptors were estimated by the Kaplan-Meier method. Differences in hazard for EFS event and overall survival event for patients in each of the three expression levels in each of the six cell surface receptors were assessed using the log-rank test. RESULTS: All 6 receptors were variably expressed in the majority of cell lines. IR and PDGFR- expressions were found to be significant predictors for EFS amongst patients with nonmetastatic disease (=0.02 and 0.01, respectively). The hazard ratio for EFS was significantly higher between high IR and intermediate IR expression (HR = 2.66, =0.02), as well as between high PDGFR- and intermediate PDGFR- expression (HR = 5.68, =0.002). Her-2, c-Met, IGF-1R, and VEGFR-3 were not found to be significant predictors for either EFS or overall survival. CONCLUSION: The six cell surface receptors demonstrated variable expression across the majority of patient-derived OS cell lines tested. Limited prognostic value was offered by IR and PDGFR- expression within nonmetastatic patients. The remaining receptors do not provide clear prognostic utility. Nevertheless, their consistent, albeit variable, surface expression across a large panel of patient-derived OS cell lines maintains their potential use as future therapeutic targets.
Sarcoma of the Breast: Clinical Characteristics and Outcomes of 991 Patients from the National Cancer Database.
Lee JS, Yoon K, Onyshchenko M
Sarcoma · 2021 · PMID 33542674 · Full text
BACKGROUND: Sarcoma of the breast is a rare malignancy with heterogeneous histology. Angiosarcoma, including secondary angiosarcoma from previous radiation, is the most common type of sarcoma of the breast. Other types o...
BACKGROUND: Sarcoma of the breast is a rare malignancy with heterogeneous histology. Angiosarcoma, including secondary angiosarcoma from previous radiation, is the most common type of sarcoma of the breast. Other types of sarcomas of the breast have limited clinical and survival information. METHODS: We obtained clinicopathological data and survival outcomes from the patients with sarcoma of the breast, excluding angiosarcoma, that were registered in the National Cancer Database (NCDB) from 2004 to 2016. The treatment patterns and prognostic factors were analyzed. RESULTS: A total of 991 patients had sarcoma of the breast other than angiosarcoma. The most common histology was spindle cell sarcoma (13.4%), followed by leiomyosarcoma (11.7%) and giant cell sarcoma (10.1%). Surgical resection was performed in 894 out of 991 patients (90.2%), including R0 resection achieved in 781 (87.4%). The patients who received surgery showed better survival than the patients without surgery regardless of radiation therapy. When radiation was added to the surgical management, the OS (overall survival) benefit was marginally significant (hazard ratio 1.30 (CI 1.01-1.67), =0.044). Adding chemotherapy did not improve OS. CONCLUSIONS: Surgical resection seems to be the most important treatment modality in sarcoma of the breast from the analysis of a large database. Radiation therapy added a minor survival benefit to the patients who received surgical resection. Systemic chemotherapy did not play a clear role in sarcoma of the breast.
Potential Association between Kaposi Sarcoma and Gout: An Exploratory Observational Study.
Moore A, Peretz I, Yosef L … +5 more , Goldstein DA, Goldvaser H, Horn S, Edel Y, Zer A
Sarcoma · 2020 · PMID 33519292 · Full text
BACKGROUND: Kaposi sarcoma is a rare vascular mesenchymal neoplasm, associated with Human Herpes Virus 8 (HHV8). Gout is a condition clinically characterized by recurrent flares of arthritis and hyperuricemia. Following...
BACKGROUND: Kaposi sarcoma is a rare vascular mesenchymal neoplasm, associated with Human Herpes Virus 8 (HHV8). Gout is a condition clinically characterized by recurrent flares of arthritis and hyperuricemia. Following our clinical impression that patients with classical Kaposi sarcoma (CKS) have a high rate of gout, we explored this in a retrospective manner. METHODS: All consecutive patients diagnosed with sarcoma or carcinosarcoma within a single tertiary center between 1/2012-12/2017 were identified through the pathology department database. A cohort of CKS patients was compared with the non-Kaposi sarcoma and carcinosarcoma cohort. Data were extracted from patients' electronic medical records. Patients younger than 18 and patients without clinical data available were excluded. Association between diagnosis of gout and CKS was assessed and adjusted for risk factors. RESULTS: Three hundred and sixty-one patients were eligible for this analysis, 61 were diagnosed with CKS and 300 with other types of sarcoma. We found a higher incidence of gout in CKS patients, 11/61 (18%) patients, compared with 8/300 (2.6%) with other types of sarcoma, odds ratio (OR) 8.0 ( < 0.00001). This association persisted when adjusted for age >39 years (OR = 6.7, < 0.00001), age and male sex (OR = 4.97, < 0.0001), and when adjusting for multiple confounding factors and medical comorbidities. CONCLUSIONS: We have demonstrated a statistically significant association between gout and CKS. As risk factors for gout were accounted for, this association may be explained by HHV8 immune-related effects. This should be further explored in vitro and in population-based studies.
Assessment of Resection Margins in Bone Tumor Surgery.
Malherbe C, Crutzen B, Schrooyen J … +5 more , Caruso G, Lecouvet F, Detrembleur C, Schubert T, Docquier PL
Sarcoma · 2020 · PMID 33488268 · Full text
Limb salvage surgery is now the preferred procedure for bone tumor surgery. To decrease the risk of local recurrence, it is crucial to obtain adequate resection margins. The obtained margins must be evaluated postoperati...
Limb salvage surgery is now the preferred procedure for bone tumor surgery. To decrease the risk of local recurrence, it is crucial to obtain adequate resection margins. The obtained margins must be evaluated postoperatively because they influence what treatment is given subsequently when margins are not adequate (e.g., surgical revision and radiotherapy). The study aims to evaluate margin assessment of tumor specimen by MRI compared to conventional histology (to establish the viability of using MRI) and assess the accuracy of a patient-specific instrument when narrow margins were aimed. The resection margins in 12 consecutive patients that were operated on for bone tumor resection were prospectively analyzed using three methods: MRI of the resection specimen, macroscopic evaluation of specimen slices, and microscopic pathological evaluation. The assessments were qualitative (R0, R1, and R2) and quantitative (distance in mm). MRI, macroscopic, and microscopic margins generated similar results for both the qualitative (all resections were R0) and quantitative assessments. The median error in safe margins was 2 mm with a surgical guide (PSI) and 5 mm without a surgical guide. Local recurrences were not detected after a mean follow-up period of 3.7 years (range, 2.1-5 years); however, four patients died during the study. In conclusion, MRI is a valuable tool for assessing safe margins. When specimens are not available for pathological assessment (e.g., extracorporeally irradiated autograft or autoclaved autograft), MRI could be used to evaluate margins. In particular, when tumor volume is high, MRI could also help to focus the pathological examination on areas of concern.
Survey of Paediatric Oncologists and Pathologists regarding Their Views and Experiences with Variant Translocations in Ewing and Ewing-Like Sarcoma: A Report of the Children's Oncology Group.
Kinnaman MD, Zhu C, Weiser DA … +8 more , Mohiuddin S, Hingorani P, Roth M, Gill J, Janeway KA, Gorlick R, Lessnick SL, Grohar PJ
Sarcoma · 2020 · PMID 33488267 · Full text
Advances in molecular diagnostics have identified subsets of Ewing and Ewing-like sarcomas driven by variant translocations with unique biology. It is likely that patients with these tumours will have different clinical...
Advances in molecular diagnostics have identified subsets of Ewing and Ewing-like sarcomas driven by variant translocations with unique biology. It is likely that patients with these tumours will have different clinical features and therapeutic outcomes. Nevertheless, the management of these patients both locally and within cooperative group trials depends on the local pathological diagnosis. It is not known what molecular diagnostic approaches are employed by local pathologists or if the exact translocation is commonly determined. In addition, it is not known what therapeutic approaches are employed for these patients or what cooperative trials are deemed appropriate for these patients by expert consensus. To answer these questions, we performed an international survey of oncologists and pathologists to better understand the diagnostic approaches used to identify variant translocations and the influence the findings have on therapy and clinical trial eligibility. An online survey was distributed to oncologists and pathologists primarily in North America. A total of 141 surveys were completed, representing a 28% response rate. The majority of respondents considered EWSR1-ETS gene family translocations (range 61-96%) to be Ewing sarcoma and would include them on the primary arm of a Ewing sarcoma clinical trial. There was a lack of consensus on how to classify and stratify BCOR-CCNB3, CIC-DUX4, and EWSR1+ with non-ETS partner fusions. Most respondents were either unsure how their institution tested, or their institution did not perform the test. In cases with atypical Ewing morphology, most respondents favoured additional fusion transcript testing. There is a lack of consensus regarding the classification and stratification of rare molecular subtypes in Ewing sarcoma. It is not clear how these alternative translocations have impacted outcomes for past clinical studies. This suggests a need for molecular confirmation of diagnoses and centralized or minimum standardization of testing for future trial enrolment.
Clinical Characteristics, Management, and Outcomes of 19 Nonpediatric Patients with Desmoplastic Small Round Cell Tumor: A Cohort of Brazilian Patients.
Campos F, Coutinho DL, Silva MLG … +7 more , Lopes A, Nascimento A, Aguiar Júnior S, Nicolau UR, Formiga MN, D'Almeida Costa F, Mello C
Sarcoma · 2020 · PMID 33192158 · Full text
Desmoplastic small round cell tumor (DSRCT) is a rare and aggressive mesenchymal malignancy, usually affecting young males. There is no consensus on the best therapeutic approach. We seek to characterize a cohort of nonp...
Desmoplastic small round cell tumor (DSRCT) is a rare and aggressive mesenchymal malignancy, usually affecting young males. There is no consensus on the best therapeutic approach. We seek to characterize a cohort of nonpediatric patients with DSRCT treated at a large Brazilian cancer center. We performed a retrospective analysis of patients with histologically confirmed DSRCT referred to our institution (2007-2020). Clinical and imaging data were extracted and summarized with descriptive statistics. Survival analyses were conducted by the Kaplan-Meier method and compared with the log-rank test. We included 19 patients with DSRCT, the median age at diagnosis was 26 years (range: 15-41 years), and 68% were male. Ninety percent presented with abdominopelvic masses, and 32% had extra-abdominal metastasis at diagnosis. Eleven patients (58%) underwent surgery, four patients (21%) received whole abdominal adjuvant radiotherapy, and five patients (26%) had hyperthermic intraperitoneal chemotherapy. Median OS was 27 months (interquartile range: 18-51 m). The five-year OS rate was 12%. Our data confirm the aggressiveness of DSRCT despite intense multimodality treatment. Outcomes of patients treated in a reference cancer center in a developing country are similar to cancer centers in developed nations. Multicenter cooperation is urgent to the development of clinical trials and to improve diagnosis and treatment efficacy.
Striving towards Normality in Daily Life: A Qualitative Study of Patients Living with Metastatic Gastrointestinal Stromal Tumour in Long-Term Clinical Remission.
Fauske L, Hompland I, Lorem G … +2 more , Hall KS, Bondevik H
Sarcoma · 2020 · PMID 33082706 · Full text
BACKGROUND: This study explored how patients with metastatic gastrointestinal stromal tumour (GIST) experience the psychosocial challenges associated with their disease and its treatment, as well as how that experience i...
BACKGROUND: This study explored how patients with metastatic gastrointestinal stromal tumour (GIST) experience the psychosocial challenges associated with their disease and its treatment, as well as how that experience influenced their practical, relational, vocational, and existential life. METHODS: This qualitative study has an explorative design and applied a phenomenological and hermeneutical approach. We conducted in-depth, semistructured interviews with 20 patients with metastatic GIST in long-term clinical remission. The gathered data were interpreted using a thematic analysis. RESULTS: Living with metastatic GIST, as well as the side effects of the required medication, led to changes that limited the participants' daily life. They expressed how tiredness, impaired memory, and physical challenges were among the detrimental impacts of the disease on their family life, vocational life, social life, and leisure time. Adjustments were necessary to ensure they had sufficient energy to cope with the practical and relational aspects of everyday life. Feelings of uncertainty stemming from drug resistance, disease progression, and the possibility of early death were also experienced as challenging. Half the participants stated that it was difficult to keep negative mental health issues at bay, and all of them considered the time spent waiting for their scheduled follow-up scan to be burdensome. CONCLUSIONS: It is important to focus increased attention on how the daily practical and psychosocial life of patients with chronic cancer, including metastatic GIST, is affected by their disease. Doing so might provide health-care workers with clues regarding how best to guide and support such patients throughout their emotional journey and, therefore, to improve their quality of life. As new medical treatments can also prolong survival and induce long-term clinical remission in relation to several other forms of metastatic cancer, the findings concerning GIST reported in this study might have widespread implications.
Diagnostic Differences in Expert Second-Opinion Consultation Cases at a Tertiary Sarcoma Center.
Rupani A, Hallin M, Jones RL … +2 more , Fisher C, Thway K
Sarcoma · 2020 · PMID 33061792 · Full text
Soft tissue tumors are diagnostically challenging, and it is recommended that these are reported or reviewed by specialist soft tissue pathologists. We present our experience with second-opinion (consultation) cases in a...
Soft tissue tumors are diagnostically challenging, and it is recommended that these are reported or reviewed by specialist soft tissue pathologists. We present our experience with second-opinion (consultation) cases in a specialist tertiary sarcoma center. The aim of this study was to determine areas of diagnostic difficulty in soft tissue pathology. We assessed 581 second-opinion cases which were reviewed by two experienced pathologists in a period of one year. There was 62% concordance between the original and the second-opinion diagnosis, with diagnostic discrepancy in 38%. The largest group of soft tissue neoplasms received for second opinion was fibroblastic/myofibroblastic tumors, and most major diagnostic problems were encountered in adipocytic and so-called "fibrohistiocytic" tumors. Major diagnostic errors impacting management were found in 148 cases (25%). Morphologic assessment of tumors, judicious use of molecular techniques, newer immunostains and their interpretation, along with importance of knowledge of rarer entities were found to be most useful in avoiding errors.
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