The role of radiation therapy in the management of primary retroperitoneal sarcoma: A systematic review and clinical practice guidelines from the Australia and New Zealand Sarcoma Association.

While surgery is the mainstay of treatment for localised retroperitoneal sarcoma, the use of radiotherapy (RT) remains controversial. This systematic review aimed to evaluate the role of RT for retroperitoneal sarcoma. A systematic review using the population, intervention, comparison, and outcome model from 1990 to 2022 identified 66 studies (a mixture of preoperative and postoperative RT); one randomised controlled trial (RCT) with two publications, 18 registry studies, and 46 retrospective studies. In the RCT of preoperative RT, there was no difference in local/abdominal recurrence. The pooled analysis of this RCT and a retrospective study showed a significant abdominal recurrence free survival benefit with preoperative RT in low grade liposarcoma. The RCT and the majority of retrospective series found RT did not improve recurrence free survival (11 of 16 no difference in combined local and distant RFS, 11 of 13 no difference in distant metastasis free survival), disease specific survival (9 of 12 studies) or overall survival (33 of 49 studies). The majority of studies found no association between RT and perioperative morbidity. In summary, preoperative RT may improve local control for low grade (well-differentiated or grades 1-2 dedifferentiated) liposarcoma, but not other histological subtypes. There is no strong evidence that perioperative RT provides an overall survival benefit. Patients with low grade retroperitoneal liposarcoma can be considered for preoperative RT to improve abdominal recurrence free survival. The rationale and level of evidence in this scenario should be carefully discussed by the multidisciplinary team with patients. RT should not be routinely recommended for other histological subtypes.

Comparison of three analgesic regimens in women undergoing cervical brachytherapy.

PURPOSE: Effective periprocedural analgesia is an important aspect of cervical brachytherapy delivery, with implications for patient comfort and attendance for subsequent fractions. We compared the efficacy and safety of three analgesic modalities: intravenous patient-controlled analgesia (IV-PCA), continuous epidural infusion (CEI) and programmed-intermittent epidural bolus with patient-controlled epidural analgesia (PIEB-PCEA). METHODS AND MATERIALS: Ninety-seven brachytherapy episodes involving 36 patients between July 2016 and June 2019 in a single tertiary center were retrospectively reviewed. Episodes were divided into two key phases: Phase 1 (while applicator remained in situ) and Phase 2 (following applicator removal until discharge or 4 h). For the primary endpoint, pain scores were retrieved and analyzed by analgesic modality with respect to median score and an internally defined "unacceptable" pain experience (>20% of scores being ≥4/10; i.e., moderate or greater). Total nonepidural oral morphine equivalent dose (OMED) and toxicity/complication events were reported as secondary endpoints. RESULTS: In Phase 1, there was a significantly higher median pain score (p < 0.001) and more episodes with unacceptable pain scores (46%) in the IV-PCA group compared with either epidural modality (6-14%; p < 0.001). In Phase 2, we observed a greater median pain score (p = 0.007) and higher proportion of patient episodes with unacceptable pain scores (38%) in the CEI group compared with both the IV-PCA (13%) and PIEB-PCEA (14%) groups (p = 0.001). There was a significant difference in median OMED used throughout all phases across the PIEB-PCEA (0 mg), IV-PCA (70 mg), and CEI (15 mg) groups (p < 0.001). CONCLUSIONS: PIEB-PCEA is safe and offers superior analgesia compared to IV-PCA or CEI for pain control after applicator placement in cervical brachytherapy.

Surgery at specialised sarcoma centres improves patient outcomes - A systematic review by the Australia and New Zealand sarcoma association clinical practice guidelines working party.

BACKGROUND: Optimal management of sarcoma requires multidisciplinary team input throughout the process of diagnosis, treatment and follow up. This systematic review aimed to evaluate the impact of surgery performed at specialised sarcoma centres on outcomes. METHODS: A systematic review was conducted using the population, intervention, comparison and outcome (PICO) model. Medline, Embase, Cochrane Central databases were queried for publications that evaluated the local control, limb salvage rate, 30-day and 90-day surgical mortality, and overall survival in patients undergoing surgery in a specialist sarcoma centre compared with non-specialist centre. Each study was screened by two independent reviewers for suitability. A qualitative synthesis of the results was performed. RESULTS: Sixty-six studies were identified. The majority of studies were Level III-3 as assessed by the NHMRC Evidence Hierarchy, whilst just over half of the studies were of good quality. Definitive surgery performed at specialised sarcoma centres was associated with improved local control as defined by lower rate of local relapse, higher rate of negative surgical margins, improved local recurrence free survival and higher limb conservation rate. Available evidences show a favourable pattern of lower 30-day and 90-day mortality rates, and greater overall survival when surgery was performed in specialist sarcoma centres compared with non-specialised centres. CONCLUSIONS: Evidences support better oncological outcomes when surgery is performed at specialised sarcoma centre. Patients with suspected sarcoma should be referred early to a specialised sarcoma centre for multidisciplinary management, which includes planned biopsy and definitive surgery.

Long term outcomes of gastric mucosa-associated lymphoid tissue lymphoma treated with radiotherapy: A multi-center retrospective cohort study.

Gastric mucosa-associated lymphoid tissue (MALT) lymphoma is a rare disease. Radiotherapy remains an important definitive modality. We assessed the long term outcomes of patients with early stage gastric MALT marginal zone lymphoma (MZL) treated with definitive radiotherapy at three institutions in New South Wales, Australia. A retrospective, multi-center study of patients with gastric MALT MZ lymphoma treated with radiotherapy between 1st of March 1999 and 31st of May 2020 was conducted. Eligible patients were: age ≥18 years, treated with curative-intent radiotherapy, pathological diagnosis of MALT MZ lymphoma. There were 33 eligible patients. Complete response (CR) was reported in 30/31 (96.7%) of endoscopically assessed cases. During median follow up of 66.2 months (IQR 22-119 months), estimated 5 and 10 years local relapse free survival were 92.6% (95% CI: 83-100) and 92.6% (95% CI: 83-100); distant relapse free survival 95.8% (95% CI 88.2-100) and 64.7% (95% CI 43.4-96.4); freedom from treatment failure 92.6% (95% CI; 83.1-100) and 62.5% (95% CI; 41.7-93.7), respectively. There were six documented recurrences; one local, four distant, and both in one patient; two cases were high grade recurrences. 5 and 10 years OS were 92.4% and 73.5% respectively. There were no grade 3-5 late toxicities or treatment related deaths. Patients with gastric MALT MZL treated with definitive radiotherapy have excellent outcomes. In long term follow up a significant proportion developed distant low grade disease. Extended follow up should be considered in these patients. Treatment is well tolerated with minimal toxicity. Radiotherapy remains an important modality in the treatment of gastric MALT MZ lymphoma.

Chemoradiotherapy with concurrent durvalumab for the palliative treatment of oligometastatic oesophageal and gastrooesophageal carcinoma with dysphagia: a single arm phase II clinical trial (PALEO, sponsored by the Australasian Gastro-Intestinal Trials Group).

BACKGROUND: Oesophageal and gastrooesophageal junction (GOJ) carcinoma frequently present with dysphagia and de novo metastatic disease. There is scope to improve treatment paradigms to both address symptoms and improve survival. One method is integrating immune checkpoint inhibition with novel treatment combinations. METHODS: PALEO is a single arm, phase II clinical trial in patients with previously untreated, oligometastatic or locoregionally advanced oesophageal or GOJ carcinoma and dysphagia. PALEO is sponsored by the Australasian Gastro-Intestinal Trials Group (AGITG). Participants receive 2 weeks of therapy with concurrent hypofractionated radiotherapy of 30Gy in 10 fractions to the primary tumour, weekly carboplatin AUC2, weekly paclitaxel 50 mg/m2 and durvalumab 1500 mg q4 weekly, followed by durvalumab monotherapy continuing at 1500 mg q4weekly until disease progression, unacceptable toxicity or 24 months of therapy. A single metastasis is treated with stereotactic radiotherapy of 24Gy in 3 fractions in week 7. The trial primary endpoint is the progression free survival rate at 6 months. Secondary endpoints include duration of dysphagia relief, nutritional status change, quality of life, response rate, toxicity, progression free survival and overall survival. The tertiary endpoint is prediction of outcome based on biomarkers identified from patient serial blood samples collected pre- and post-radiotherapy. DISCUSSION: This unique investigator-initiated clinical trial is designed to simultaneously address the clinically relevant problems of dysphagia and distant disease control. The overarching aims are to improve patient nutrition, quality of life and survival with low toxicity therapy. AGITG PALEO is a multidisciplinary collaboration and will add to the understanding of the relationship between radiotherapy and the anti-tumour immune response. TRIAL REGISTRATION: Australian and New Zealand Clinical Trials Registry: ACTRN12619001371189 , registered 8 October 2019.

The benefit for radiotherapy at specialised sarcoma centres: A systematic review and clinical practice guidelines from the Australia and New Zealand Sarcoma Association.

Patients with sarcoma are best managed at specialised sarcoma centres as supported by published literature. Optimal management requires multidisciplinary team input to formulate the diagnosis and treatment sequencing taking into consideration multiple clinical and pathologic factors. This systematic review aimed to evaluate the impact on outcomes of radiotherapy at specialised sarcoma centres. A systematic review was conducted using the population, intervention, comparison and outcome model. A literature search was performed using Medline, Embase, Cochrane Central databases for publications from 1990 to February 2022 that evaluated the local control, survival and toxicity of radiotherapy at specialised sarcoma centres. A total of 21 studies were included (17 cancer registry studies, four retrospective comparative studies). Four studies reported the local recurrence endpoint when radiotherapy was part of limb conservation treatment and showed better conformity to clinical practice guidelines and an improved local recurrence free rate when radiotherapy treatment is supported through, but may not be necessarily delivered at a specialised sarcoma centres. Only one retrospective study analysed toxicity specifically and demonstrated that patients who received preoperative radiotherapy at community centres compared to radiotherapy at a specialised sarcoma centre were more likely to develop a major wound complication. Fourteen studies reported overall survival, and 12 of these showed significantly better 5-year overall survival for patients managed at specialised sarcoma centres, however the specific impact of radiotherapy delivered at sarcoma centres could not be determined. In conclusion, patients with sarcoma should be managed through specialised sarcoma centres for better oncological outcomes. Radiotherapy in specialised sarcoma centre is associated with a lower rate of wound complications and may contribute to improved oncological outcomes as part of the limb conservation treatment at a specialised sarcoma centre.

Population-level utilisation of neoadjuvant radiotherapy for the treatment of rectal cancer.

PURPOSE: International clinical guidelines recommend long- or short-course neoadjuvant radiotherapy for locally advanced rectal cancer. This study aims to examine variation in the use of neoadjuvant radiotherapy for rectal cancer and identify patient and hospital factors that underpin this variation. METHODS AND MATERIALS: We conducted a retrospective, consecutive cohort study using statewide hospitalisation and radiotherapy data from New South Wales, Australia, 2013-2018. Included participants had a primary rectal adenocarcinoma and underwent surgical resection. Factors associated with the use or not of any neoadjuvant radiotherapy, and short versus long-course were explored using multilevel logistic regression models. RESULTS: Of the 2912 people included in the study, 43% received neoadjuvant radiotherapy. There was significant variation in the use of neoadjuvant radiotherapy depending on geographic location. Abdominoperineal excision (odds ratio [OR] = 1.87, 95% confidence interval [CI] = 1.53-2.28) and having surgery in a public hospital (OR = 2.34, 95% CI = 1.92-2.87) were both predictors of use. Among those receiving neoadjuvant radiotherapy, 17% received short-course therapy, with short-course declining over the study period. CONCLUSIONS: The use of neoadjuvant radiotherapy for rectal cancer is highly variable, with differences only partially explained by assessable patient-or hospital-level factors. Understanding neoadjuvant radiotherapy utilisation patterns may assist in identifying barriers and opportunities to improve adherence to clinical guidelines.

Systemic treatments and outcomes in CIC-rearranged Sarcoma: A national multi-centre clinicopathological series and literature review.

CIC-rearranged sarcoma is a recently established, ultra-rare, molecularly defined sarcoma subtype. We aimed to further characterise clinical features of CIC-rearranged sarcomas and explore clinical management including systemic treatments and outcomes. METHODS: A multi-centre retrospective cohort study of patients diagnosed between 2014-2019. RESULTS: Eighteen patients were identified. The median age was 27 years (range 13-56), 10 patients were male (56%), 11 patients (61%) had localised disease and 7 patients had advanced (metastatic or unresectable) disease at diagnosis. Of 11 patients with localised disease at diagnosis, median overall survival (OS) was 40.6 months and the 1-, 2- and 5-year OS estimates were 82%, 64% and 34% respectively. Nine patients (82%) underwent surgery (all had R0 resections), 8 (73%) patients received radiotherapy to the primary site (median dose 57Gy in 28 fractions), and 8 (73%) patients received chemotherapy (predominantly Ewing-based regimens). Metastases developed in 55% with a median time to recurrence of 10.5 months. In patients with advanced disease at diagnosis, median OS was 12.6 months (95% CI 5.1-20.1), 1-year OS was 57%. Median progression-free survival was 5.8 months (95% CI 4.5-7.2). Durable systemic therapy responses occurred infrequently with a median duration of systemic treatment response of 2.1 months. One durable complete response of metastatic disease to VDC/IE chemotherapy was seen. Responses to pazopanib (n = 1) and pembrolizumab (n = 1) were not seen. CONCLUSION: In this series, CIC-rearranged sarcomas affected young adults and had a high incidence of presenting with, or developing, metastatic disease. The prognosis overall was poor. In advanced disease, durable systemic therapy responses were infrequent.

Outcomes of patients with metastatic cutaneous squamous cell carcinoma to the axilla: a multicentre cohort study.

BACKGROUND: Metastatic cutaneous squamous cell carcinoma to the axilla is uncommon, with limited data to guide management. We sought to assess the outcomes of patients with this condition after surgery and radiotherapy. METHODS: A retrospective cohort study of patients treated at two Australian hospitals from 1994 through 2016 was performed. RESULTS: A total of 74 patients were identified, including 48 treated curatively with surgery-plus-radiotherapy and 15 with surgery alone. Compared with patients treated with surgery alone, a higher proportion of patients treated with surgery-plus-radiotherapy had lymph nodes larger than 6 cm (53% versus 8%, P = 0.012) and multiple adverse histopathological features (75% versus 47%, P = 0.04). The groups had similar 5-year disease-free survival (45% versus 46%) and overall survival (51% versus 48%). Presence of multiple positive lymph nodes was associated with reduced disease-free survival (hazard ratio 4.57, P = 0.01) and overall survival (hazard ratio 3.53, P = 0.02). Regional recurrence was higher in patients treated with surgery alone (38% versus 22%, P = 0.22) and patients with lymph nodes larger than 6 cm (34% versus 10%, P = 0.03). All recurrences occurred within 2 years following treatment. CONCLUSION: Combined-modality therapy for metastatic cutaneous squamous cell carcinoma to the axilla is recommended for high-risk patients, although outcomes remain modest. The key period for recurrence is within 2 years following treatment.

18F-Fluorodeoxyglucose PET/CT-Guided Palliative Radiotherapy Provides Durable Responses to Over a Dozen Sites of Disease in Relapsed Myeloid Sarcoma.

Myeloid sarcoma (MS) is a rare entity, and FDG PET/CT is a useful tool for staging at diagnosis and response assessment. We present a case of a 72-year-old woman diagnosed with multifocal extramedullary MS, using FDG PET/CT to guide palliative radiotherapy to 13 sites of disease over 2 separate relapses with complete and durable local responses and minimal toxicity. This case represents the largest reported burden of disease in MS successfully treated with FDG PET/CT-guided radiotherapy.

Treatment Algorithm for Surgical Site Infections Following Extensor Mechanism Repair.

¼: Despite general agreement regarding techniques for extensor mechanism repair, there is very limited guidance in the literature for the management of surgical site infections (SSIs) that may occur after these procedures. ¼: Early or mild superficial SSIs, such as cellulitis, can be managed on an outpatient basis while monitoring for improvement, with escalated intervention if the symptoms do not resolve within 1 week. ¼: Deep SSIs should be managed more aggressively with surgical irrigation and debridement (I&D), including the knee joint, depending on the results of the aspiration, removal of all braided nonabsorbable suture (if necessary) with immediate or delayed exchange with monofilament suture, and the administration of parenteral antibiotics based on culture results and an infectious disease consult. ¼: Arthrocentesis should be performed early to monitor for the spread of infection to the joint space, and diagnosis of a septic knee joint should be immediately followed by arthroscopic or open I&D. ¼: For refractory cases (i.e., wound coverage issues or persistent infections despite multiple attempts at debridement), a consult with a plastic surgeon for consideration of a gastrocnemius flap is recommended, and surgeons should remain suspicious of the possibility of the contiguous spread of osteomyelitis.

Multimodality treatment of oligometastatic anal squamous cell carcinoma: A case series and literature review.

BACKGROUND AND OBJECTIVES: There is limited evidence to guide the management of patients with oligometastatic anal squamous cell carcinoma (SCC). We aimed to address this question by reporting the outcome of SCC patients who were treated with organ-directed therapies at two large cancer centers. METHODS: Patients with advanced anal SCC who were treated with surgery, stereotactic radiotherapy, or radiofrequency ablation (RFA) with a curative intent from 2008 to 2017 were retrospectively identified from the institutional electronic patient records. RESULTS: Eight patients with liver or lung metastases met the study inclusion criteria. Seven were treated with surgery while one received RFA and radiotherapy. Median progression-free survival was 5 months (range, 4-39). Three patients underwent repeat organ-directed treatment upon failure of the initial surgery with no evidence of further recurrent disease at the last follow-up. Median overall survival from the time of the first organ-directed therapy was 31 months (range, 11-96) with two out of eight patients being alive and disease-free at 5 years. CONCLUSIONS: Our study confirms that consideration should be given to the adoption of a multidisciplinary treatment approach in carefully selected, oligometastatic anal SCC patients as organ-directed therapies may offer the chance of achieving a relatively long disease control.

Using behaviour change and implementation science to address low referral rates in oncology.

BACKGROUND: Patients undergoing surgery for bowel cancer now have a routine screening test to assess their genetic predisposition to this and other cancers (Lynch syndrome). A result indicating a high risk should trigger referral to a genetic clinic for diagnostic testing, information, and management. Appropriate management of Lynch syndrome lowers morbidity and mortality from cancer for patients and their family, but referral rates are low. The aim of this project was to increase referral rates for patients at high risk of Lynch syndrome at two Australian hospitals, using the Theoretical Domains Framework (TDF) Implementation approach. METHODS: Multidisciplinary teams at each hospital mapped the referral process and discussed barriers to referral. A 12-month retrospective audit measured baseline referral rates. The validated Influences on Patient Safety Behaviours Questionnaire was administered to evaluate barriers using the TDF. Results were discussed in focus groups and interviews, and interventions co-designed, guided by theory. Continuous monitoring audits assessed change in referral rates. RESULTS: Teams (n = 8, 11) at each hospital mapped referral processes. Baseline referral rates were 80% (4/5) from 71 screened patients and 8% (1/14) from 113 patients respectively. The questionnaire response rate was 51% (36/71). Most significant barrier domains were: 'environmental context;' 'memory and decision making;' 'skills;' and 'beliefs about capabilities.' Focus groups and interviews with 19 healthcare professionals confirmed these domains as significant. Fifteen interventions were proposed considering both emerging and theory-based results. Interventions included: clarification of pathology reports, education, introduction of e-referrals, and inclusion of genetic status in documentation. Audits continued to December 2016 showing a change in pathology processes which increased the accuracy of screening. The referral rate remained low: 46% at Hospital A and 9% Hospital B. Results suggest patients who have their referral deferred for some reason are not referred later. CONCLUSION: Lynch syndrome is typical of low incidence problems likely to overwhelm the system as genomic testing becomes mainstream. It is crucial for health researchers to test methods and define generalizable solutions to address this problem. Whilst our approach did not improve referrals, we have deepened our understanding of barriers to referral and approaches to low frequency conditions.

Radiotherapy for anal squamous cell carcinoma: must the upper pelvic nodes and the inguinal nodes be treated?

BACKGROUND: Loco-regional failure is the predominant cause of death in anal squamous cell carcinoma. We assessed patterns of loco-regional recurrence to determine the impact of radiotherapy (RT) volumes on patient outcome. METHODS: Retrospective clinical study, including patients treated curatively with RT or chemo-radiotherapy between 1994 and 2007. RT fields/volumes were reviewed and compared with patterns of failure. Patients were classified as having whole pelvic radiotherapy (WPRT) if RT extended to L5/S1 or lower pelvic radiotherapy (LPRT) if it extended to the lower sacroiliac joints or below. Patients with negative inguinal nodes either underwent prophylactic inguinal radiotherapy (PIRT) or had inguinal observation (IO). Patterns of failure were compared. RESULTS: Twenty-seven patients (53%) had WPRT and 24 (47%) had LPRT. Forty-two patients had negative inguinal nodes: 29 (69%) had PIRT and 13 (31%) had IO. Median follow-up was 5.8 years. Twelve regional failures occurred in eight patients: three pelvic, one inguinal and four pelvic and inguinal. All patients with regional failure died of disease. Pelvic nodal failure was 7.7% in N0 and 33% in N1-3 patients (P = 0.012). There was no difference in pelvic regional failure between WPRT and LPRT (11% versus 16%, P = 0.64). There was only one possible regional failure above LPRT in this group (4%). Inguinal failure was 0% in the PIRT group compared with 23% in IO group (P = 0.009). CONCLUSION: There was no difference in pelvic regional failure between WPRT and LPRT. LPRT is likely to be safe in N0 patients. Inguinal nodes should be treated in all patients.

Impact of salvage treatment modalities in patients with positive FDG-PET/CT after R-CHOP chemotherapy for aggressive B-cell non-Hodgkin lymphoma.

INTRODUCTION: To compare outcomes of different salvage treatment modalities in patients with aggressive B-cell non-Hodgkin lymphoma (NHL) who remain FDG-PET positive after R-CHOP chemotherapy. Existing data on these patients with FDG-PET primary refractory disease are limited. METHODS: Patients with diffuse large B-cell lymphoma or grade 3 follicular lymphoma were retrospectively reviewed from the Prince of Wales Hospital databases. Eligibility criteria were: age≥18 years, treated with R-CHOP, with positive post-chemotherapy FDG-PET. Salvage treatment modalities were: radical radiotherapy (RT, dose≥30 Gy), high dose chemotherapy and autologous stem cell transplant (ASCT), or non-radical management. Survival was calculated from date of post-chemotherapy FDG-PET to last follow-up. RESULTS: Twenty-six patients from 2003-2015 met the inclusion criteria. Median age was 60 (range 19-84). Most had adverse baseline features: 21 (81%) stage III-IV, 24 (92%) bulky disease and nine (35%) skeletal involvement. Characteristics of PET-positivity post-chemotherapy were single site in 16 (62%), sites of prior bulk in 24 of 24, skeletal sites in five of nine, and able to be encompassed by RT in 21 (81%). Salvage treatment was: radical RT in 17 (65%), ASCT in four (15%) and non-radical in five (20%). Median follow-up of surviving patients was 31 months. Kaplan-Meier estimates of 3-year PFS and OS were 41% and 52%, respectively. By salvage modality, 3-year PFS was 51% for RT, 25% for ASCT and 20% for non-radical treatment, (P = 0.453); 3-year OS was respectively 65%, 25% and 40% (P = 0.173). CONCLUSION: Patients with FDG-PET positive disease after R-CHOP for aggressive B-cell NHL are salvageable with radiotherapy.

Patterns of care of superficial soft tissue sarcomas: it is not always just a lump.

AIM: Superficial soft tissue sarcomas (S-STS) are generally considered low-risk tumors and have an excellent prognosis when treated with appropriate surgery and adjuvant therapy. However, they are often misdiagnosed then mistreated, leading to significant morbidity. This study aims to examine the patterns of care and outcomes of patients with S-STS, comparing those initially managed through sarcoma units versus elsewhere. METHODS: Patients with S-STS from Prince of Wales Hospital in NSW (1995-2013) and Peter MacCallum Cancer Centre in Victoria (2009-2013) were identified from a national sarcoma database. Baseline variables, treatment and disease outcomes were recorded. Statistical tests performed included univariate and multivariate analyses, chi-square tests, as well as the Kaplan-Meier method for 5-year local recurrence and survival rates. RESULTS: Eighty-nine patients were identified, with 35% initially managed at a sarcoma unit and 65% elsewhere. Patients initially managed at sarcoma units had larger tumors (>5 cm 39% vs 17%; P  =  0.036) with a trend to higher grade (61% vs 48%; P = 0.39). Patients that were initially managed outside a sarcoma unit more often underwent open surgical biopsies (P < 0.0005), had multiple operations (P < 0.0005) and had higher rates of local recurrences (24% vs 6.5%, P  =  0.038). They also had lower 5-year local recurrence-free survival rates (P = 0.022), but had higher metastasis-free survival (P = 0.014). On multivariate analysis, only larger STS size and male gender predicted for poorer metastasis-free survival (P = 0.042 and 0.018, respectively). CONCLUSION: Patients with S-STS initially managed outside specialized sarcoma units undergo more operations, with risk of greater morbidity, and have greater risk of local recurrence.

Treatment of breast cancer 2 (BRCA2)-mutant follicular dendritic cell sarcoma with a poly ADP-ribose polymerase (PARP) inhibitor: a case report.

BACKGROUND: Follicular dendritic cell sarcoma is a rare tumour with clinical behaviour covering a spectrum from indolent to aggressive disease. Treatment recommendations are currently based on case reports and small series describing combinations of surgery, chemotherapy and radiotherapy providing the best patient outcomes. Recent knowledge on molecular aberrations in this disease have not yet impacted on therapeutic decisions. CASE PRESENTATION: We describe a case of progressive follicular dendritic cell sarcoma of the lung and pleura, treated based on knowledge of the tumour's molecular aberrations. The patient was initially treated with surgery, chemotherapy and radiotherapy and developed disease progression. Mutation testing by Caris molecular intelligence demonstrated a breast cancer 2 gene mutation and further treatment with carboplatin and veliparib achieved disease stabilisation. CONCLUSION: Understanding of the molecular profile of rare tumours is key to improve therapeutic decision making and patient outcomes.