Management Of Sarcoma Research Articles

Surgical management of primary bone sarcomas

This article aims to address the principles of surgical treatment of primary bone sarcomas including chondrosarcoma, osteosarcoma and Ewing's sarcoma of bone. This piece is aimed at serving as a guide to experienced orthopaedic surgeons who have limited knowledge of dealing with musculoskeletal tumours especially primary bone sarcomas. Important principles surrounding surgery involving bone sarcomas, principles of biopsy of such lesions and reconstruction techniques have been discussed. Limb salvage is the way forward in today's era and though endoprosthesis replacement is an important tool in a surgeon's armoury, biological methods of reconstruction have also shown to be effective in many settings. Chemotherapy and radiotherapy are vital adjuvant therapies associated with these sarcomas, and they have been discussed in this article.

Next-generation sequencing for the management of sarcomas with no known driver mutations.

Next-generation sequencing (NGS) has enabled fast, high-throughput nucleotide sequencing and has begun to be implemented into clinical practice for genomic-guided precision medicine in various cancer types. This review will discuss recent evidence that highlights opportunities for NGS to improve outcomes in sarcomas that have complex genomic profiles with no known driver mutations. Global genomic signatures detectable by NGS including tumour mutational burden and microsatellite instability have potential as biomarkers for response to immunotherapy in certain sarcoma subtypes including angiosarcomas. Identification of hallmarks associated with 'BRCAness' and homologous recombination repair defects in leiomyosarcomas and osteosarcomas may predict sensitivity to poly(adenosine diphosphate-ribose) polymerase (PARP) inhibitors. Lastly, the use of NGS for evaluating cancer predisposition in sarcomas may be useful for early detection, screening and surveillance. Currently, the implementation of NGS for every sarcoma patient is not practical or useful. However, adopting NGS as a complementary approach in sarcomas with complex genomics and those with limited treatment options has the potential to deliver precision medicine to a subgroup of patients, with novel therapies such as immune checkpoint and PARP inhibitors. Moving forward, molecular tumour boards incorporating multidisciplinary teams of pathologists, oncologists and genomic specialists to interpret NGS data will complement existing tools in diagnosis and treatment decision making in sarcoma patients.

Management of Soft Tissue Sarcomas in Extremities: Variation in Treatment Recommendations and Surveillance According to Specialty and Continent

BackgroundThis study aimed to provide an insight into clinical decision-making and surveillance strategy of sarcoma specialists for patients with primary soft tissue sarcoma of the extremities (eSTS). The secondary aim was to quantify the role of patient- and tumor-specific factors in the perioperative management.MethodsMembers of sarcoma societies were sent a Web-based 21-item survey about eSTS management. The survey concerned only primary resectable high-grade eSTS in adults.ResultsThe study enrolled 396 respondents. The majority of the surgical specialists thought the evidence for perioperative chemotherapy (CTX) for high-grade eSTS was insufficient. Radiotherapy (RTX) was less frequently offered in Asia than in North America and Europe. The specialties and continents also differed regarding the importance of patient and tumor characteristics influencing RTX and CTX recommendation. For surveillance after initial treatment outpatient visits, chest computed tomography (CT) scans, and magnetic resonance images of the extremity were the methods primarily used. The specialists in North America preferred chest CT scan over chest x-ray, whereas those in Asia and Europe had no clear preference.DiscussionSpecialty and continent are important factors contributing to the variation in clinical practice, treatment recommendations, and surveillance of patients with primary resectable high-grade eSTS.

Systematic review of charged-particle therapy for chordomas and sarcomas of the mobile spine and sacrum.

Long-term local control in patients with primary chordoma and sarcoma of the spine and sacrum is increasingly reliant upon en bloc resection with negative margins. At many institutions, adjuvant radiation is recommended; definitive radiation is also recommended for the treatment of unresectable tumors. Because of the high off-target radiation toxicities associated with conventional radiotherapy, there has been growing interest in the use of proton and heavy-ion therapies. The aim of this study was to systematically review the literature regarding these therapies. The PubMed, OVID, Embase, and Web of Science databases were queried for articles describing the use of proton, combined proton/photon, or heavy-ion therapies for adjuvant or definitive radiotherapy in patients with primary sarcoma or chordoma of the mobile spine and sacrum. A qualitative synthesis of the results was performed, focusing on overall survival (OS), progression-free survival (PFS), disease-free survival (DFS), and disease-specific survival (DSS); local control; and postradiation toxicities. Of 595 unique articles, 64 underwent full-text screening and 38 were included in the final synthesis. All studies were level III or IV evidence with a high risk of bias; there was also significant overlap in the reported populations, with six centers accounting for roughly three-fourths of all reports. Five-year therapy outcomes were as follows: proton-only therapies, OS 67%-82%, PFS 31%-57%, and DFS 52%-62%; metastases occurred in 17%-18% and acute toxicities in 3%-100% of cases; combined proton/photon therapy, local control 62%-85%, OS 78%-87%, PFS 90%, and DFS 61%-72%; metastases occurred in 12%-14% and acute toxicities in 84%-100% of cases; and carbon ion therapy, local control 53%-100%, OS 52%-86%, PFS (only reported for 3 years) 48%-76%, and DFS 50%-53%; metastases occurred in 2%-39% and acute toxicities in 26%-48%. There were no studies directly comparing outcomes between photon and charged-particle therapies or comparing outcomes between radiation and surgical groups. The current evidence for charged-particle therapies in the management of sarcomas of the spine and sacrum is limited. Preliminary evidence suggests that with these therapies local control and OS at 5 years are comparable among various charged-particle options and may be similar between those treated with definitive charged-particle therapy and historical surgical cohorts. Further research directly comparing charged-particle and photon-based therapies is necessary.

Venous Tumor Thrombus in Primary Bone Sarcomas in the Pelvis: A Clinical and Radiographic Study of 451 Cases.

Few investigations of venous tumor thrombus (VTT) in primary pelvic bone sarcomas are available. We aimed to identify the prevalence, associated factors, and prognosis of VTT across different types of pelvic sarcomas and to propose an algorithm for management. We included 451 consecutive cases of primary, bone-derived, treatment-naive, pelvic sarcomas in this study. Demographic data and the results of initial laboratory tests, imaging examinations, and oncological evaluations were extracted and analyzed. Forty-four cases of VTT were diagnosed with radiographic examinations, and 18 of them were verified histologically. The cohort consisted of chondrosarcomas (41.2%), osteosarcomas (30.4%), Ewing sarcomas (15.5%), bone-derived undifferentiated pleomorphic sarcomas (5.8%), and other bone sarcomas (7.1%). The prevalence of VTT was 9.8% in the whole group, and associated factors included a lactate dehydrogenase (LDH) level of ≥230.5 U/L and invasion of the L5-S1 intervertebral foramen. Patients with pelvic osteosarcoma had a high prevalence of VTT (22.6%), and the associated factors in this group included a chondroblastic subtype, an LDH level of ≥187 U/L, and invasion of the obturator foramen and the L5-S1 intervertebral foramen. Patients with VTT had a poor prognosis with a median overall survival time of 14 months. Subgroup analyses of localized pelvic osteosarcoma indicated that the presence of VTT decreased the median overall survival time (21.5 versus 54.0 months for those without VTT, p = 0.003), median recurrence-free survival time (18.6 versus 32.4 months, p = 0.020), and median metastasis-free survival time (11.2 versus 41.0 months, p < 0.001). VTT is most common in patients with pelvic osteosarcoma as compared with patients with other primary bone sarcomas, and it is associated with several factors. It is a negative prognostic factor. An algorithm for management of pelvic sarcomas with VTT stratified by the classification of the VTT might be beneficial, but further validation is necessary. Prognostic Level IV. See Instructions for Authors for a complete description of levels of evidence.

Management of Primary Retroperitoneal Sarcoma (RPS) in the Adult: An Updated Consensus Approach from the Transatlantic Australasian RPS Working Group

Retroperitoneal soft tissue sarcomas comprise a heterogeneous group of rare tumors of mesenchymal origin that include several well-defined histologic subtypes. In 2015, the Transatlantic Australasian RPS Working Group (TARPSWG) published consensus recommendations for the best management of primary retroperitoneal sarcoma (RPS). Since then, through international collaboration, new evidence and knowledge have been generated, creating the need for an updated consensus document. The primary aim of this study was to critically evaluate the current evidence and develop an up-to-date consensus document on the approach to these difficult tumors. The resulting document applies to primary RPS that is non-visceral in origin, with exclusion criteria as previously described. The relevant literature was evaluated and an international group of experts consulted to formulate consensus statements regarding the best management of primary RPS. A level of evidence and grade of recommendation were attributed to each new/updated recommendation. Management of primary RPS was considered from diagnosis to follow-up. This rare and complex malignancy is best managed by an experienced multidisciplinary team in a specialized referral center. The best chance of cure is at the time of primary presentation, and an individualized management plan should be made based on the 29 consensus statements included in this article, which were agreed upon by all of the authors. Whenever possible, patients should be enrolled in prospective trials and studies. Ongoing international collaboration is critical to expand upon current knowledge and further improve outcomes of patients with RPS. In addition, prospective data collection and participation in multi-institution trials arestrongly encouraged.

P135 Undifferentiated round cell and spindle cell sarcomas: the current paradigm

IntroductionThe classification of soft tissue and bone neoplasms is experiencing a transformation as methods to detect gene fusions expand. Next-generation sequencing panels, anchored multiplex polymerase chain reaction systems and comprehensive RNA sequencing have all contributed to new histological categorisation. The EWSR1-NFATC2 is one such novel translocation found in a round cell sarcomas. These sarcoma sit within the WHO group of undifferentiated round and spindle cell sarcomas. They presents as either a primary bone or soft tissue tumour and exhibits distinctive histopathologic features.MethodsThis is a case study of a soft tissue mesenchymal tumour with the recently described gene fusion EWSR1-NFATC2. The case is described, with 3-month outcomes and considered in the most recent literature on this sarcoma subset (26 cases).ResultsThis case study prompted a look at the current NHS paradigm for these emerging histopathologically distinctive sarcoma subgroups. At present the expertise with the sarcoma MDT, along with second opinion from other national MDTs is the format for subtype specific sarcoma management, informed by recent literature.ConclusionThe EWSR1-NFATC2 fusion round cell sarcoma tumours are an example of a very recently emerged sarcoma subset which display oncological variance to the wider Ewing’s family. The novel literature guided the Southmead and Royal Marsden MDT opinions to proceed straight to surgical excision.The Authors recommend a national prospective database for rare sarcoma subsets, particularly those that appear to exhibit distinctive clinicopathology characteristics. The format could be through the RSTN collaborative, secure anonymised data collection can be collected through REDCap.

Management of Synovial Sarcoma in a Tertiary Referral Center: A Retrospective Analysis of 134 Patients

Background: Synovial sarcomas (SS) are malignant mesenchymal neoplasms that account for about 10% of all sarcomas. Complete surgical excision is the mainstay of primary treatment for localized disease, but SS have a high tendency for local relapse and metastases. Metastatic disease is commonly treated with systemic chemotherapy. Methods: We designed a retrospective analysis to describe the clinical presentation, course of treatment, outcome, and prognosis of patients with SS. Univariate and multivariate analyses were performed for potential prognostic factors. Results: We identified 134 patients treated between 1987 and 2018, with a cutoff date of December 2018. Demographics, disease characteristics, treatment, and survival rates were collected and analyzed. The median overall survival (mOS) from the date of diagnosis was 96.7 months. The median progression-free survival was 6.37 months. Disease-free survival was 26 months. Age over 65 years was found to be a prognostic factor with statistically significant value in the univariate analysis regarding mOS (p = 0.015) and mOS after local relapse (p = 0.0228). Conclusions: Even though our study is limited by the retrospective nature of the analysis, it adds an important amount of clinical data regarding the treatment and outcome of SS.

Testicular, Spermatic Cord, and Scrotal Soft Tissue Sarcomas: Treatment Outcomes and Patterns of Failure.

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 (p=0.15), the use of chemotherapy, (p=0.36), or final margin status (p=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.

Complete pathological response to neoadjuvant treatment is associated with better survival outcomes in patients with soft tissue sarcoma: Results of a retrospective multicenter study

BackgroundLocally advanced soft tissue sarcoma (STS) management may include neoadjuvant or adjuvant treatment by radiotherapy (RT), chemotherapy (CT) or chemoradiotherapy (CRT) followed by wide surgical excision. While pathological complete response (pCR) to preoperative treatment is prognostic for survival in osteosarcomas, its significance for STS is unclear. We aimed to evaluate the prognostic significance of pCR to pre-operative treatment on 3-year disease-free survival (3y-DFS) in STS patients. MethodsThis is an observational, retrospective, international, study of adult patients with primary non-metastatic STS of the extremities and trunk wall, any grade, diagnosed between 2008 and 2012, treated with at least neoadjuvant treatment and surgical resection and observed for a minimum of 3 years after diagnosis. The primary objective was to evaluate the effect of pCR. (≤5% viable tumor cells or ≥95% necrosis/fibrosis) on 3y-DFS. Effect on local recurrence-free survival (LRFS), distant recurrence-free survival (MFS) overall survival (OS) at 3 years was also analyzed. Statistical univariate analysis utilized chi-square independence test and odds ratio confidence interval (CI) estimate, multivariate analysis was performed using LASSO. ResultsA total of 330 patients (median age 56 years old, range:19–95) treated by preoperative RT (67%), CT (15%) or CRT (18%) followed by surgery were included. pCR was achieved in 74/330 (22%) of patients, of which 56/74 (76%) had received RT. 3-yr DFS was observed in 76% of patients with pCR vs 61% without pCR (p < 0.001). Multivariate analysis showed that pCR is statistically associated with better MFS (95% CI, 1.054–3.417; p = 0.033), LRFS (95% CI, 1.226–5.916; p = 0.014), DFS (95% CI, 1.165–4.040; p = 0.015) and OS at 3 years (95% CI, 1.072–5.210; p = 0.033). ConclusionsIn a wide, heterogeneous STS population we showed that pCR to preoperative treatment is prognostic for survival.

Soft-tissue reconstruction after soft-tissue sarcoma resection: the clinical outcomes of 24 patients.

Pedicle or free-flap reconstruction is important in surgical sarcoma management. Free flaps are indicated only when pedicle flaps are considered inadequate; however, they are associated with a higher risk of flap failure, longer surgical times, and technical difficulty. To determine the skin defect size that can be covered by a pedicle flap, we investigated the clinical outcomes and complications of reconstruction using pedicle flaps vs. free flaps after sarcoma resection. We retrospectively studied the medical records of 24 patients with soft-tissue sarcomas who underwent reconstruction using a pedicle (n = 20) or free flap (n = 4) following wide tumour resection. All skin defects of the knee, lower leg, and ankle were reconstructed using a pedicle flap. Skin defects of the knee, lower leg, and ankle were covered by up to 525cm2, 325cm2, and 234cm2, respectively. The amount of blood loss was significantly greater in the free-flap group than in the pedicle flap group (p = 0.011). Surgical time was significantly shorter in the pedicle flap group than in the free-flap group (p = 0.006). Total necrosis was observed in one (25%) patient in the free-flap group; no case of total necrosis was observed in the pedicle flap group. Less blood loss, shorter surgical time, and lower risk of total flap necrosis are notable advantages of pedicle flaps over free flaps. Most skin defects, even large ones, of the lower extremities following sarcoma resection can be covered using a single pedicle flap or multiple pedicle flaps.

The Management of Radiation-Induced Sarcomas: A Cohort Analysis from a Sarcoma Tertiary Center.

(1) Background: Radiation-induced sarcomas (RIS) are rare diseases with poor prognoses. The aim of the study was to analyze outcomes and identify factors affecting survival in a cohort of patients with RIS. (2) Methods: We included consecutive patients with RIS that we found in the available electronic medical records of a sarcoma tertiary center. We analyzed patients’ RIS characteristics, management of RIS, the occurrence of local recurrence and distant metastases, the date of disease progression, the date of death, and the date of the last follow-up. (3) Results: Fifty-eight patients met the inclusion criteria. The most frequent sites of RIS development were the thorax and pelvis. The majority of RIS were poorly differentiated, high-grade tumors. Forty patients underwent surgery or radiotherapy with curative intent. The others were referred to palliative chemotherapy. Median progression-free survival and overall survival were 15 and 21 months, respectively. Treatment with curative intent and tumor localization on breasts and upper extremities were associated with a lower risk of death in univariate analysis. (4) Conclusions: The study confirms the poor prognosis of RIS. Treatments with locally curative intent at the tumor site are of prognostic value. Secondary radiotherapy is rarely used in RIS.

A Prolonged Response and Characteristics of Trabectedin Treatment of Metastatic Soft Tissue Sarcoma

Unique features and treatment effects of trabectedin are presented in consideration of soft tissue sarcoma management. A prolonged time on trabectedin through 59 cycles is shown. This is one of the longer reported uses of trabectedin successfully to control disease. Adjunctive cytoreduction options with surgery, radiation or ablation are presented. Future studies would be helpful to investigate treatment holidays, the impact of multi-modality care and assessment of genetics of clonal metastases. This may assist in guiding and selecting patients for priority treatment with trabectedin. J Med Cases. 2021;12(4):160-163 doi: https://doi.org/10.14740/jmc3655

Computer Navigation and 3D Printing in the Surgical Management of Bone Sarcoma.

The long-term outcomes of osteosarcoma have improved; however, patients with metastases, recurrence or axial disease continue to have a poor prognosis. Computer navigation in surgery is becoming ever more commonplace, and the proposed advantages, including precision during surgery, is particularly applicable to the field of orthopaedic oncology and challenging areas such as the axial skeleton. Within this article, we provide an overview of the field of computer navigation and computer-assisted tumour surgery (CATS), in particular its relevance to the surgical management of osteosarcoma.

SEOM Clinical Guideline of management of soft-tissue sarcoma (2020)

Soft-tissue sarcomas constitute an uncommon and heterogeneous group of tumors of mesenchymal origin. Diagnosis, treatment, and management should be performed by an expert multidisciplinary team. MRI/CT of the primary tumor and biopsy is mandatory before any treatment. Wide surgical resection with tumor-free tissue margin is the mainstay for localized disease. Radiotherapy is indicated in large, deep, high-grade tumors, or after marginal resection not suitable for re-excision. Perioperative chemotherapy should be discussed for high-risk sarcomas of the extremities and trunk-wall. In the case of oligometastatic disease, patients should be considered for local therapies. First-line treatment with anthracyclines (or in combination with ifosfamide) is the treatment of choice. Other drugs have shown activity in second-line therapy and in specific histological subtypes but options are limited and thus, a clinical trial should always be discussed.

Management of bulky high-grade pleomorphic sarcoma using grid therapy technique

Management of bulky high-grade pleomorphic sarcoma using grid therapy technique

Evaluating the Soft Tissue Sarcoma Paradigm for the Local Management of Extraskeletal Ewing Sarcoma.

We reviewed our experience treating patients with localized extraskeletal Ewing sarcoma (EES) to determine optimal local management strategies for this rare disease. Sixty patients with localized EES treated at our institution between 1994 and 2018 were reviewed. The Kaplan-Meier method was used to estimates disease outcomes. The median follow-up time was 74 months (interquartile range [IQR], 17-121). Half the patients (n = 30) received combined-modality local therapy (CMT) with both surgery and radiation therapy (RT), whereas the other half received single-modality local therapy (SMT) with either surgery or RT. All patients received chemotherapy. The 5-year overall survival was 76%. Twenty-two patients (37%) developed recurrence at a median time of 15 months (IQR, 5-56 months) resulting in 3-year progression-free survival (PFS) of 65%. On univariate analysis, the use of both neoadjuvant and adjuvant chemotherapy was associated with improved 5-year PFS (71% vs. 50%, p = .04) compared with those who received one or the other. Furthermore, 11 patients (18%) developed local recurrences at a median time of 14 months (IQR, 2-19 months), resulting in a 5-year local control (LC) rate of 77%. Use of CMT was not associated with improved LC (83% vs. 72% SMT, p = .41). Also, use of CMT was the only factor associated with poorer disease-specific survival (vs. SMT; hazard ratio, 3.4; p = .047; 95% confidence interval, 1.01-11.4). For patients with EES, CMT was not associated with a decreased rate of local relapse. These data suggest that SMT alone may be sufficient for LC in select patients. A multi-institutional collaborative effort should be considered to validate these findings. Extraskeletal Ewing sarcoma is a rare chemosensitive sarcoma whose clinical course more closely follows Ewing sarcoma of bone rather than that of other soft tissue sarcomas. Based on this study, combined-modality local therapy did not confer a local control advantage compared with single-modality local therapy. Therefore, single-modality local therapy is likely adequate in select patients with favorable disease features, which has the advantage of ensuring prompt administration of systemic therapy. A multi-institutional collaborative effort is warranted to determine which patients may benefit from de-escalated local therapy.

Anti-Angiogenic Agents in Management of Sarcoma Patients: Overview of Published Trials.

We reviewed all fully published clinical trials assessing anti-angiogenic agents in sarcoma patients (last issue, January 13, 2020). Anti-angiogenic macromolecules (e.g., bevacizumab or ombrabulin) provide disappointing results. Many multikinase inhibitors have been assessed with non-randomized phase II trials with limited samples and without stratification according to histological subtypes, therefore interpretation of such trials is very challenging. On the contrary, pazopanib, regorafenib, and sorafenib have been assessed using double-blind placebo-controlled randomized phase II or phase III trials. Compared to placebo, sorafenib demonstrates activity in desmoid-type fibromatosis patients. Based on results of phase 3 trial, pazopanib had obtained approval for treatment of pretreated non-adipocytic soft tissue sarcoma. Regorafenib is currently assessed in several clinical settings and provides significant improvement of progression-free survival in pre-treated non-adipocytic soft tissue sarcoma and in advanced pretreated osteosarcoma. Multikinase inhibitors are a breakthrough in sarcoma management. Many trials are ongoing. Nevertheless, predictive factors are still missing.

The importance of the margin of resection and radiotherapy in retroperitoneal liposarcoma

BackgroundPrior studies evaluating the impact of adjuvant or neoadjuvant radiotherapy on clinical outcomes in retroperitoneal liposarcoma have been underpowered. MethodsWe queried the National Cancer Database for patients undergoing resection of retroperitoneal liposarcoma from 2004 to 2016. Cox proportional hazards modeling stratified by tumor size was used to identify factors associated with overall survival. Results4018 patients met inclusion criteria. 251 had small (<5 cm), 574 intermediate (5–10 cm), and 3193 large (>10 cm) tumors. Positive surgical margins were correlated with risk of death across all tumor size categories (<5 cm HR 2.33, CI [1.20, 4.55]; 5–10 cm HR 1.49, CI [1.03, 2.14]; >10 cm HR 1.30, CI [1.12, 1.51]). Adjuvant radiotherapy was associated with improved survival for patients with large tumors only (HR 0.75, CI [0.64, 0.89]). ConclusionsIn retroperitoneal liposarcoma, adjuvant radiation is associated with improved survival only for patients with tumors larger than 10 cm. Radiation should be used sparingly in patients with smaller tumors. SummaryThe use of radiotherapy in the management of retroperitoneal sarcoma remains controversial. We isolated retroperitoneal liposarcomas only and identified a survival benefit from radiotherapy treatment only in tumors larger than 10 cm and only in the adjuvant setting.

DICER1-associated embryonal rhabdomyosarcoma and adenosarcoma of the gynecologic tract: Pathology, molecular genetics, and indications for molecular testing.

Gynecologic sarcomas are uncommon neoplasms, the majority occurring in the uterus. Due to the diverse nature of these, the description of "new" morphological types and the rarity of some of them, pathological diagnosis and treatment is often challenging. Finding genetic alterations specific to, and frequently occurring, in a certain type can aid in the diagnosis. DICER1 is a highly conserved ribonuclease crucial in the biogenesis of microRNAs and mutations in DICER1 (either somatic or germline) have been detected in a wide range of sarcomas including genitourinary embryonal rhabdomyosarcomas (ERMS) and adenosarcomas. Importantly, DICER1-associated sarcomas share morphological features irrespective of the site of origin such that the pathologist can strongly suspect a DICER1 association. A review of the literature shows that almost all gynecologic ERMS reported (outside of the vagina) harbor DICER1 alterations, while approximately 20% of adenosarcomas also do so. These two tumor types exhibit significant morphological overlap and DICER1 tumor testing may be helpful in distinguishing between them, because a negative result makes ERMS unlikely. Given that germline pathogenic DICER1 variants are frequent in uterine (corpus and cervix) ERMS and pathogenic germline variants in this gene cause a hereditary cancer predisposition syndrome (DICER1 syndrome), patients diagnosed with these neoplasms should be referred to medical genetic services. Cooperation between pathologists and geneticists is crucial and will help in improving the diagnosis and management of these uncommon sarcomas.