Aggressive Multimodality Management Research Articles

An urgent invasion: lower mandible Ewing’s sarcoma and unleashed massive abscess – a case report study

Introduction and importance: Ewing’s sarcoma (ES) is a rare and aggressive bone tumor that can affect any bone but is particularly rare when it involves the mandible. Mandible ES often presents with nonspecific symptoms and requires a multimodal approach to treatment. Case presentation: This case involves a 19-year-old woman who had ES of the mandible. At the initial stage, she stated that she was bothered by a small round mass that caused her discomfort during chewing. The first examination by a specialist required a more detailed examination by biopsy, which the immunohistochemistry results confirmed the diagnosis of ES based on this detailed information. Clinical discussion: Mandible ES is a rare and aggressive bone tumor that requires early diagnosis and prompt multimodal management to achieve good outcomes. Despite being a challenging diagnosis, histologic evaluation and molecular testing have revolutionized the diagnosis and management of these tumors in recent years. Conclusion: By the way of conclusion, recognizing the importance of early diagnosis and aggressive multimodal management, clinicians and researchers should continue to work collaboratively to improve the management of mandible ES and enhance outcomes for affected patients.

Feasibility, Safety, and Efficacy of Aggressive Multimodal Management of Elderly Patients with Pancreatic Ductal Adenocarcinoma.

We aimed to evaluate the safety and efficacy of NAT followed by surgical resection in patients with PDAC aged ≥75 years. Whether administration of neoadjuvant therapy (NAT) followed by surgical resection in elderly patients with pancreatic ductal adenocarcinoma (PDAC) is safe and effective is unknown. The present study is a three-part comparison of older (≥ 75 years) versus younger (< 75 years) patients in different settings throughout the continuum of PDAC care. The first analysis was a comparison of older versus younger consecutive patients with non-metastatic PDAC who were initiated on FOLFIRINOX. The second was a comparison of older vs. younger patients who underwent NAT followed by surgical resection, and the third and final analysis was a comparison of older patients who underwent either NAT followed by surgical resection vs. upfront surgical resection. Postoperative complications, overall survival (OS), and time to recurrence (TTR), were compared. Propensity-score matching (PSM) analysis was performed to adjust for potential confounders. In the first analysis, a lower proportion of older patients (n=40) were able to complete the intended neoadjuvant FOLFIRINOX (8) cycles compared to younger patients (n=214) (65.0% vs. 81.4%, P=0.021). However, older patients were just as likely to undergo surgical exploration as younger patients (77.5% vs 78.5%, P=0.89) as well as surgical resection (57.5% vs 55.6%, P=0.70). In the second analysis, PSM was conducted to compare older (n=54) vs. younger patients (n=54) who underwent NAT followed by surgical resection. There were no significant differences in postoperative complications between the matched groups. While there was a significant difference in overall survival (OS) between older and younger patients (median OS: 16.43 months vs. 30.83 months, P=0.002), importantly, there was no significant difference in time to recurrence (TTR, median: 7.65 months vs. 11.83 months, P=0.215). In the third analysis, older patients who underwent NAT followed by surgical resection (n=48) were compared with similar older patients who underwent upfront surgical resection (n=48). After PSM, there was a significant difference in OS (median OS: 15.78 months vs. 11.51 months, P=0.037) as well as TTR (median TTR: 8.81 months vs. 7.10 months, P=0.046) representing an association with improved outcomes that favored the neoadjuvant approach among older patients alone. This comprehensive three-part study showed that administration of NAT followed by surgical resection appears to be safe and effective among patients ≥ 75 years of age. An aggressive approach should be offered to older adults undergoing multimodal treatment of PDAC.

O143 Management strategies and outcomes for small bowel neuroendocrine tumours with involvement of the superior mesenteric vascular root: a systematic review

Abstract Introduction Small intestine neuroendocrine tumours (SI-NETs) are the most common small bowel tumours. A particularly challenging subset of these tumours are those that involve the superior mesenteric artery (SMA) or vein (SMV) for which the role and feasibility of surgery is often questioned. This review aimed to identify and evaluate the management strategies used for these complex SI-NETs. Methods Primary studies concerning SI-NETs with SMA and/or SMV involvement were identified from PubMed and Embase review and data abstraction was performed. Results Initial literature search identified 155 potentially eligible studies, from which nine non-randomised primary retrospective studies met inclusion criteria. Treatments identified include stenting the SMV, debulking and resection, auto-transplantation, and endovascular occlusion and tumour excision (EVOTE) procedures. The level of involvement of the SMA and SMV was associated with resectability such that more proximal involvement yielded a lower likelihood of successful resection. Auto-transplantation strategies may show promise in more proximal mesenteric root involvement. To date, one study reported a case of a patient with SMV encasement who was successfully treated with auto-transplantation of ileocolic and ileojejunal segments after resection of the affected structures. Conclusion The present study highlights a role for aggressive surgical and multimodal management in cases of SI-NETs with vascular involvement of the mesenteric root. The use of classification systems to stratify mesenteric masses by level of vascular involvement can help identify patients who could benefit from resection. Large scale prospective studies are needed to better elucidate the effectiveness of surgical management strategies in these challenging SI-NETs.

Metastatic NUT Carcinoma in a Child.

Nuclear protein of the testis (NUT) carcinoma is a rare and aggressive malignancy associated with rearrangements of the NUT gene on chromosome 15q14 .This entity is often under diagnosed and there is limited literature regarding its biology and optimal management. We report a case of pediatric NUT carcinoma presenting as a mass involving the nasal septum with associated bilateral cervical lymphadenopathy. CT and MRI of PNS revealed soft tissue lesion involving cartilaginous and bony septum and tip of nose .Histopathology of cervical lymph node and IHC with NUT confirmed the diagnosis of metastatic NUT carcinoma. NUT carcinoma has a dismal prognosis despite aggressive multimodality management. As new NUT targeting drugs using BET inhibitors are emerging, the correct and prompt recognition of NC is the key to improve patient outcome.

Primary spinal glioblastoma multiforme. Single center experience and literature review

ObjectivesSpinal glioblastomas represent a rare entity accounting for ca 1–3% of all intramedullary tumors; data about survival, prognostic factors and therapeutic protocols are quite poor. Even with an aggressive multimodal management the spinal glioblastoma patients’ survival remains poor, with rapid progression of the disease. This study reports our experience with the management of the primary intramedullary glioblastomas, also in regard to the current literature data. Patients and MethodsWe retrospectively analyzed the medical records of 5 patients treated at the Department for Neuro-oncology and Spine Surgery of the Clinical Center of Belgrade, Serbia, between January 2007 and December 2016 for a primary intramedullary glioblastoma. Demographic characteristics, pre-operative data and post-operative results were then compared with previous literature regarding spinal GBMs and attempt to identify potential prognostic factors. ResultsGross total resection was achieved in two patients, while a subtotal resection was performed in the latter 3 cases; as per protocol, all patients underwent to surgery, followed by radio and chemotherapy. There were no intraoperative complications and no patients developed a new postoperative neurological defect; the median overall survival was 6 months. Progression or recurrence of disease was noted in all patients at the 3-months follow-up, despite the adjuvant treatments. ConclusionsTo the date, there is a lack of consensus on specific management of spinal glioblastomas: the extent of resection can play an important role, but it appears to be not preeminent. A shorter interval between symptoms onset and treatment and a smaller extension of the tumor seem to be correlated with better outcomes and a longer overall survival. However, there is not an adjunctive viable standardized postoperative therapy yet, which results in concrete and persistent improvement of overall survival and progression free survival.

Pediatric Dystonic Storm: A Hospital-Based Study.

Pediatric dystonic storm is an underrecognized entity. We aimed to evaluate the profiles of children presenting with dystonic storm in a referral hospital. Management schema and treatment responsiveness of this uncommonly reported entity were analyzed. Retrospective review of all children (up to 18 years) hospitalized with dystonic storm over 39 months in the aforementioned facility. Twenty-three children whose ages ranged from 2 years 2 months to 14 years 4 months years (median: 6 years 11 months) (males: 13, females: 11) presented with dystonic storm. The annual incidence was 0.4 per 1,000 fresh admissions with an event rate of 0.9 per 1,000 for all admissions. All had Dystonia Severity Action Plan grades 4/5 with identifiable trigger in 13 (50%). Underlying dystonic disorder preexisted in 10 (43.4%); 8 of these had cerebral palsy. Polypharmacotherapy with >4 drugs out of trihexyphenidyl, tetrabenazine, clonazepam, gabapentin, levodopa-carbidopa, trichlorophos, and melatonin was needed. Supportive care and adequate sedation helped in symptom control. All children were managed with midazolam infusion over 2-10 days (median: 5 days). Mechanical ventilation was resorted to in 6 children (3-22 days). Vecuronium and propofol were used in 3/23 (13%) and 4/23 (17%) children, respectively. Deep brain stimulation was curative in 1 child. Hospitalization ranged from 5 to 31 (median: 11) days. Although there were no deaths, rhabdomyolysis was noted in 1 child. Postdischarge, 6 (26%) children relapsed. Dystonic storm is a medical emergency mandating aggressive multimodal management. Supportive care, antidystonic drugs, and early elective ventilation alongside adequate sedation with benzodiazepines ameliorate complications. Relapses of dystonic storm are not uncommon.

Evaluating Surgical Resection Extent and Adjuvant Therapy in the Management of Gliosarcoma.

Introduction: Gliosarcomas are clinically aggressive tumors, histologically distinct from glioblastoma. Data regarding the impact of extent of resection and post-operative adjuvant therapy on gliosarcoma outcomes are limited.Methods: Patients with histologically confirmed gliosarcoma diagnosed between 1999 and 2019 were identified. Clinical, molecular, and radiographic data were assembled based on historical records. Comparisons of categorical variables used Pearson's Chi-square and Fisher's exact test while continuous values were compared using the Wilcoxon signed-rank test. Survival comparisons were assessed using Kaplan-Meier statistics and Cox regressions.Results: Seventy-one gliosarcoma patients were identified. Secondary gliosarcoma was not associated with worse survival when compared to recurrent primary gliosarcoma (median survival 9.8 [3.8 to 21.0] months vs. 7.6 [1.0 to 35.7], p = 0.7493). On multivariable analysis, receipt of temozolomide (HR = 0.02, 95% CI 0.001–0.21) and achievement of gross total resection (GTR; HR = 0.13, 95% CI 0.02–0.77) were independently prognostic for improved progression-free survival (PFS) while only receipt of temozolomide was independently associated with extended overall survival (OS) (HR = 0.03, 95% CI 0.001–0.89). In patients receiving surgical resection followed by radiotherapy and concomitant temozolomide, achievement of GTR was significantly associated with improved PFS (median 32.97 [7.1–79.6] months vs. 5.45 [1.8–26.3], p = 0.0092) and OS (median 56.73 months [7.8–104.5] vs. 14.83 [3.8 to 29.1], p = 0.0252).Conclusion: Multimodal therapy is associated with improved survival in gliosarcoma. Even in patients receiving aggressive post-operative multimodal management, total surgical removal of macroscopic disease remains important for optimal outcomes.

An Analysis of Primary Site Radiation, Surgery, and Survival in Metastatic Rectal Cancer: A National Cancer Database Population Study

Optimal management of the primary site in metastatic rectal cancer remains undefined. This analysis evaluates patterns of care, primary site treatment, outcomes, and factors associated with survival in metastatic rectal cancer. A National Cancer Database (NCDB) inquiry was performed to identify patients diagnosed with metastatic rectal cancer between 2004 and 2013 who received treatment coded as non-palliative to the primary cancer site. This included either radiation therapy (RT) alone or a combination of RT with primary site surgery. Patients with squamous histology, unknown or palliative surgery, unknown RT dose, or no pelvic RT were excluded. Kaplan Meier method was used to estimate overall survival (OS). Univariate and multivariate analyses (MVA) were conducted to identify associated risk factors. In total, 4,461 patients were analyzed. The majority of patients (79%) were under the age of 70 with a Charlson-Deyo score of 0 (84%). Clinical stage was T1-T2 (7%), T3 (50%), T4 (16%), Tx (27%), N0 (27%), N1 (37%), N2 (13%), Nx (23%). Grade was 1-2 (63%), 3-4 (16.3%), and unknown (20.5%). 93% received chemotherapy. A total of 2,163 of patients received non-palliative RT to the pelvis alone, and 2,298 patients received RT and surgery coded as non-palliative. Patients who had both RT and surgery had superior OS compared to patients who received RT alone (HR 2.4, p<0.001). For patients who received RT and surgery, 92% of patients received 45-60 Gy, 5% received >25 Gy but <45 Gy, and 3% received 25 Gy (3%). There was no difference in OS comparing doses of 25 Gy vs. ≥45 Gy, with five year OS of 42% and 34.2%, respectively (HR 1.1, p=0.65). Patients receiving doses between 25 and 45 Gy with surgery had worse OS (HR 1.9 <0.001). On MVA, the following additional variables were associated with worse OS: male sex (HR 1.2, p=0.005), N2 disease (HR 1.2, p=0.025), poorly differentiated histology (HR 1.7, p<0.001), and treatment at a non-academic center (1.3, p<0.001). Receipt of chemotherapy was associated with improved OS (HR 0.6, p=0.009). Patients with multi-organ metastases had worse OS relative to patients with single organ metastases, or other distributions such as multiple distant lymph node regions, or metastases to one distant organ and one distant lymph node region (HR 1.7, p=0.02). As of 2013, long course radiotherapy (≥45 Gy) is the most frequently delivered dose schedule. Survival amongst patients receiving long and short course (25 Gy) was not statistically different. Long-term survival is attainable in metastatic rectal cancer patients who undergo aggressive multimodality management of the primary site. Future prospective studies are warranted to evaluate the role of aggressive primary site management and optimal radiation doses in patients with metastatic rectal cancer.

Global Quantitative Proteomics reveal Deregulation of Cytoskeletal and Apoptotic Signalling Proteins in Oral Tongue Squamous Cell Carcinoma

Oral malignancies remain to have higher morbidity and mortality rates owing to the poor understanding of the carcinogenesis and the lack of early detection and diagnosis. The lack of established biomarkers for oral tongue squamous cell carcinoma (OTSCC) resulted in aggressive multi-modality management less effective. Here, we report for the first time that a panel of potential markers identified from tongue tumor samples using two-dimensional-differential-in-gel-electrophoresis (2D-DIGE). Our approach of combining 2D-DIGE with tandem mass spectrometry identified 24 candidate proteins including cofilins, myosin light chain family members, annexins, serpins, HSPs and tropomyosins, with significant differential expression in tongue carcinomas as compared with their matched adjacent normal tissues. The expression levels of the identified proteins were further validated in larger cohort of Indian samples using qPCR. Most of the differentially regulated proteins are involved in actin cytoskeletal dynamics, drug resistance, immune system, inflammation and apoptotic signalling pathways and are known to play critical role in oral tumorigenesis. Taken together, the results from present investigation provide a valuable base for understanding the development and progression of OTSCC. The validated panel of proteins may be used as potential biomarkers for early detection as well as in predicting therapeutic outcome of OTSCC.

Gene Delivery in Neuro-Oncology.

Glioblastoma multiforme (GBM) is the most common primary malignant brain tumor in adults with a dismal prognosis despite aggressive multimodal management thus novel treatments are urgently needed. Gene therapy is a versatile treatment strategy being investigated in multiple cancers including GBM. In gene therapy, a variety of vectors or "carriers" are used to deliver genes designed for different anti-tumoral effects. Gene delivery vehicles and approaches to treatment will be addressed in this review. The most commonly studied vectors are viral based, however, driven by advances in biomedical engineering, mesenchymal and neural stem cells, as well as multiple different types of nanoparticles have been developed to improve tumor tropism and also increase gene transfer into tumor cells. Different genes have been studied including suicide genes, which convert non-toxic prodrug into cytotoxic drug; immunomodulatory genes, which stimulate the immune system; and tumor suppressor genes which repair the defect that allow cells to divide unchecked. Gene therapy may be a promising treatment strategy in neuro-oncology as it is versatile and flexible due to the ability to tailor vectors and genes for specific therapeutic activity. Pre-clinical studies and clinical trials have demonstrated feasibility and safety of gene therapy; however, further studies are required to determine efficacy.

Giant adenoid basal cell carcinoma of the scalp with intracranial involvement—successful management strategies for a rare entity

Adenoid basal cell carcinoma (BCC) is a rare but aggressive histologic subtype of BCC. The aim of this report is to present our multidisciplinary management strategy and a literature review on this seldom reported entity. A 72-year-old female with fungating giant BCC of the scalp, ear, and bone underwent preoperative biopsy revealing adenoid histology with infiltrating and nodular components. The patient underwent radical resection of the biparietal and occipital scalp, auriculectomy and craniectomy with dural resection. Immediate reconstruction of the intracranial and bony defect was performed with dural substitute and a titanium cranioplasty. The soft tissue defect was reconstructed with a latissimus dorsi free flap and split thickness skin graft using the right superficial temporal artery and vein as recipient vessels. Adjuvant external beam radiation was initiated postoperatively. Nearly 2 years postoperatively, she presented mesh exposure necessitating secondary reconstruction with debridement, hardware removal, and an anterolateral thigh (ALT) free flap, followed by flap debulking. Four years following initial presentation, she remains disease-free. To our knowledge, this is the only reported case of a giant, locally invasive scalp BCC with the adenoid subtype treated with composite resection and single stage reconstruction. Using an aggressive multimodal management paradigm, treatment and surveillance of the rarely reported, giant, locally invasive scalp BCC with adenoid subtype can result in a durable disease-free response and a functional, esthetic reconstruction. Evidence-based medicine level.

The Management Challenges in an Unusual Case of Primary Osteosarcoma of the Rib in an Adult Patient.

Primary osteosarcomas are derived from primitive bone-forming mesenchymal stem cells and most often originate in the metaphysis of the long bones of children and adolescents, but it occurs very rare in adults. Approximately 10% of osteosarcomas are located in the flat bones, pelvis being the main site. Involvement of flat bones like rib may be seen as a metastatic process or secondary to chemotherapy, but a primary osteosarcoma rib is extremely rare. Osteosarcoma originating from such rare sites poses numerous diagnostic and therapeutic challenges, and we report one such case in a 24-year-old male patient. Our case highlights the fact that osteosarcoma of the rib, although rare, should be considered in the differential diagnosis of primary malignant neoplasms of the rib even in the adult population. Further, early prompt diagnosis and aggressive multimodality management are cornerstones of management and will help improve the survival outcomes.

Infected hardware after surgical stabilization of rib fractures: Outcomes and management experience.

Surgical stabilization of rib fracture (SSRF) is increasingly used for treatment of rib fractures. There are few data on the incidence, risk factors, outcomes, and optimal management strategy for hardware infection in these patients. We aimed to develop and propose a management algorithm to help others treat this potentially morbid complication. We retrospectively searched a prospectively collected rib fracture database for the records of all patients who underwent SSRF from August 2009 through March 2014 at our institution. We then analyzed for the subsequent development of hardware infection among these patients. Standard descriptive analyses were performed. Among 122 patients who underwent SSRF, most (73%) were men; the mean (SD) age was 59.5 (16.4) years, and median (interquartile range [IQR]) Injury Severity Score was 17 (13-22). The median number of rib fractures was 7 (5-9) and 48% of the patients had flail chest. Mortality at 30 days was 0.8%. Five patients (4.1%) had a hardware infection on mean (SD) postoperative day 12.0 (6.6). Median Injury Severity Score (17 [range, 13-42]) and hospital length of stay (9 days [6-37 days]) in these patients were similar to the values for those without infection (17 days [range, 13-22 days] and 9 days [6-12 days], respectively). Patients with infection underwent a median (IQR) of 2 (range, 2-3) additional operations, which included wound debridement (n = 5), negative-pressure wound therapy (n = 3), and antibiotic beads (n = 4). Hardware was removed in 3 patients at 140, 190, and 192 days after index operation. Cultures grew only gram-positive organisms. No patients required reintervention after hardware removal, and all achieved bony union and were taking no narcotics or antibiotics at the latest follow-up. Although uncommon, hardware infection after SSRF carries considerable morbidity. With the use of an aggressive multimodal management strategy, however, bony union and favorable long-term outcomes can be achieved. Therapeutic study, level V.

Complex Regional Pain Syndrome: Diagnostic and Treatment Conundrum

A 54-year-old man presented with a 12-week history of right lower extremity radicular pain with symptoms and signs of complex regional pain syndrome (CRPS). He reported a recent medical history of transient ischemic attack followed by cerebrovascular accident for which he underwent carotid endarterectomy. Following carotid endarterectomy, the patient was left with minimal residual speech and memory deficits, but concomitant presentation of right lower extremity radicular pain in the postoperative period was the reason for seeking pain relief. Right lumbar radiculopathy was suspected at the time of presentation to the pain clinic, and magnetic resonance imaging revealed bilateral moderate to severe lumbar foraminal stenosis, worse on the right side. Parasagittal lumbar epidural steroid injection, and aggressive multimodal pain management strategy was started resulting in significant improvement in pain scores, functionality and stress levels, albeit for a short period. Patient continued to obtain incomplete resolution of symptoms with conventional treatment. He underwent spinal cord stimulation at an early stage but only derived moderate benefit. The presented case is unusual because of overlapping etiologies that influenced our treatment plans. In this article, we will discuss the evidence for each of the questions raised by practitioners while treating aforementioned patient with CRPS. J Med Cases. 2016;7(1):33-42 doi: http://dx.doi.org/10.14740/jmc2378w

Nonmelanoma cutaneous cancers involving the skull base: outcomes of aggressive multimodal management.

A relative paucity of information exists regarding outcomes from craniofacial resection for advanced nonmelanoma skin cancers involving the skull base. In light of advances in surgical technique and adjuvant therapy protocols, the authors reviewed their surgical experience to determine disease control rates, overall survival (OS), morbidity, and mortality. A retrospective review of 24 patients with nonmelanoma cutaneous cancers with skull base involvement treated with craniofacial resection at The University of Texas MD Anderson Cancer Center from 1994 to 2012 was performed. Of these patients, 19 (79%) had squamous cell carcinoma (SCC), 4 (17%) had basosquamous carcinoma (BSCC), and 1 patient (4%) had adenocarcinoma. Factors as assessed were prior treatment, TNM staging, tumor involvement, extent of intracranial extension, margin status, postoperative complications, recurrence, disease status at last follow-up, and long-term survival. The majority of tumors were T4 (67%) according to the TNM classification; perineural extension was noted in 58%, cavernous sinus involvement in 25%, and dural involvement in 29%. Postoperative complications occurred in 4 patients (17%) including 1 death. Kaplan-Meier estimates were calculated for OS and progression-free survival (PFS). Median OS was 43.2 months with an 82% 1-year OS and 37% 5-year OS; the median PFS was 91.2 months. Margin status was positively associated with median OS in SCC (91 months [for negative margins] vs 57 months, p = 0.8) and in BSCC (23.7 vs 3.2 months, p < 0.05). Postoperative radiotherapy was associated with improved median OS (43.2 vs 22 months, p = 0.6). Brain involvement was uniformly fatal after 1 year, while cavernous sinus involvement (31 vs 43 months, p = 0.82), perineural disease (31 vs 54 months, p = 0.30), and T4 stage (22 vs 91.2 months, p = 0.09) were associated with worsened OS. Similar associations were found with median PFS. Aggressive multimodality management with surgery and postoperative radiotherapy can positively impact locoregional control and OS. With improvements in technique and adjuvant therapy protocols, treatment can still be considered in situations of perineural disease and cavernous sinus involvement and as a salvage option for patients in whom prior treatment has failed. As patients with advanced NMSCs often have few options, craniofacial resection, as part of a coordinated multimodal management plan, is justified if it can be performed safely.

Management of patients with recurrent glioblastoma using hypofractionated stereotactic radiotherapy.

Glioblastoma (GBM) is the most common primary malignant brain tumor in adults. The chance of cure is very limited due to treatment-refractory disease course with frequent recurrences despite aggressive multimodality management. In this retrospective study, we evaluated treatment outcomes of hypofractionated stereotactic radiotherapy (HFSRT) in the management of recurrent GBM and report our single-center experience. Twenty-eight patients receiving HFSRT for recurrent GBM between September 2008 and February 2014 were retrospectively assessed. Total radiotherapy dose was 25 Gy delivered in 5 fractions over 5 consecutive days for all patients. High-precision, image-guided volumetric modulated arc therapy was delivered with a linear accelerator using 6-MV photons using the frameless technique. Analyzed prognostic factors were age, gender, Karnofsky performance status (KPS), tumor location, planning target volume (PTV) size, overall survival (OS), progression-free survival (PFS), time interval between completion of treatment with Stupp protocol at primary diagnosis and recurrence. Median follow-up time was 42 months (range 2-68). Median time interval between primary chemoradiotherapy and HFSRT was 11.2 months (range 4-57.9). Median OS and PFS calculated from reirradiation was 10.3 months and 5.8 months, respectively. Longer interval between initial treatment and recurrence (p = 0.01), smaller PTV size (p = 0.001), KPS ≥70 (p = 0.005) and younger age (p = 0.004) were associated with longer OS on statistical analysis. HFSRT offers a feasible and effective salvage treatment option for recurrent GBM management. Prognostic factors associated with longer OS in our study were longer interval between initial treatment and recurrence, smaller PTV size, KPS ≥70 and younger age.

Multimodality Management of Metastatic Patients With Soft Tissue Sarcomas May Prolong Survival

Patients who develop metastatic disease from soft tissue sarcoma have a poor prognosis. The purpose of this study was to identify metastatic survival rates and identify prognostic variables that predict for these outcomes. Between 2000 and 2010, 182 patients with stage I to IV primary soft tissue sarcomas of the extremity and trunk were treated with multimodality treatment. Fifty-five patients developed or presented with metastasis. We retrospectively analyzed prognostic factors for metastatic survival. Metastatic survival between groups was compared with the log-rank test. Survival curves were estimated by Kaplan-Meier plots. Multivariate analysis was performed using the Cox proportional hazards model. Median follow-up was 3.1 years. Median metastatic survival was 24.2 months. Median metastatic survival in those undergoing multimodality therapies was 40 versus 22 months in those receiving single modality treatments. In single predictor Cox models, age, stage, number of lung metastases, location of metastases, and primary disease were significant for metastatic survival. On multivariate analysis, number of pulmonary metastases, histology, stage, and location of primary disease predicted for metastatic survival. Patients who had pulmonary-only disease had improved metastatic survival versus those that had extrapulmonary with or without pulmonary metastatic disease (38 vs. 15 mo). Patients who had ≤5 pulmonary metastasis had improved metastatic survival versus those that had >5 pulmonary lesions (55 vs. 22 mo). This analysis shows that >5 pulmonary metastasis, malignant fibrous histiocytoma histology, stage III disease, and proximal lower extremity sarcomas are associated with decreased metastatic survival. Moreover, aggressive multimodality management of metastatic disease may prolong metastatic survival.

Pericardial synovial sarcoma: challenges in diagnosis and management.

Introduction: Pericardial synovial sarcoma is an extremely rare tumor with poor prognosis. Timely diagnosis and aggressive multimodal management improves patient outcome. We present our experience of diagnosis and management of a young patient with monophasic synovial sarcoma arising from pericardium. Case: A 27-year-old man presented with dyspnea and cough of three weeks duration. Examination revealed sinus tachycardia, distant heart sounds and elevated jugular venous pressure. Chest X-ray showed widened mediastinum. Transthoracic echocardiogram (TTE) noted large pericardial effusion with tamponade physiology. Therapeutic pericardiocentesis yielded hemorrhagic fluid. Computed tomography (CT) of the chest showed persistent pericardial effusion and a left anterior mediastinal mass. Left anterior thoracotomy, pericardial window and left anterior mediastinotomy were done, revealing a well-encapsulated gelatinous tumor originating from the pericardium. Histology and immunohistochemical profile showed the tumor to be a monophasic synovial sarcoma. Fluorescent in-situ hybridization (FISH) was positive for SS18 (SYT) gene rearrangement on chromosome 18q11, substantiating the diagnosis. Work-up for metastases was negative. Neo-adjuvant chemotherapy with high dose ifosfamide led to substantial reduction in the size of the tumor. The patient underwent surgical resection and external beam radiation therapy (EBRT) post surgery. He had symptom-free survival for 8 months prior to local recurrence. This was managed with left lung upper lobectomy and follow-up chemotherapy with docetaxel. The patient is currently stable with an acceptable functional status. Conclusion: In patients with pericardial effusions of unknown etiology, multiple modalities of cardiac imaging must be employed if there is suspicion of a pericardial mass. CT and magnetic resonance imaging (MRI) are useful to evaluate for pericardial thickening or masses in addition to TTE. Treatment of synovial sarcoma is not well established. Surgery is the cornerstone of treatment. In non-resectable tumors, aggressive neo-adjuvant chemotherapy with ifosfamide followed by surgical resection and EBRT may lead to improved outcome.

Pericardial synovial sarcoma: challenges in diagnosis and management

Pericardial synovial sarcoma is an extremely rare tumor with poor prognosis. Timely diagnosis and aggressive multimodal management improves patient outcome. We present our experience of diagnosis and management of a young patient with monophasic synovial sarcoma arising from pericardium.A 27-year-old man presented with dyspnea and cough of three weeks duration. Examination revealed sinus tachycardia, distant heart sounds and elevated jugular venous pressure. Chest X-ray showed widened mediastinum. Transthoracic echocardiogram (TTE) noted large pericardial effusion with tamponade physiology. Therapeutic pericardiocentesis yielded hemorrhagic fluid. Computed tomography (CT) of the chest showed persistent pericardial effusion and a left anterior mediastinal mass. Left anterior thoracotomy, pericardial window and left anterior mediastinotomy were done, revealing a well-encapsulated gelatinous tumor originating from the pericardium. Histology and immunohistochemical profile showed the tumor to be a monophasic synovial sarcoma. Fluorescent in-situ hybridization (FISH) was positive for SS18 (SYT) gene rearrangement on chromosome 18q11, substantiating the diagnosis. Work-up for metastases was negative. Neo-adjuvant chemotherapy with high dose ifosfamide led to substantial reduction in the size of the tumor. The patient underwent surgical resection and external beam radiation therapy (EBRT) post surgery. He had symptom-free survival for 8 months prior to local recurrence. This was managed with left lung upper lobectomy and follow-up chemotherapy with docetaxel. The patient is currently stable with an acceptable functional status.In patients with pericardial effusions of unknown etiology, multiple modalities of cardiac imaging must be employed if there is suspicion of a pericardial mass. CT and magnetic resonance imaging (MRI) are useful to evaluate for pericardial thickening or masses in addition to TTE. Treatment of synovial sarcoma is not well established. Surgery is the cornerstone of treatment. In non-resectable tumors, aggressive neo-adjuvant chemotherapy with ifosfamide followed by surgical resection and EBRT may lead to improved outcome.

Aggressive multimodality management of locally advanced retromolar trigone tumors

Retromolar trigone tumors are rare and aggressive malignancies. There is lack of quality evidence pertaining to their management due to the heterogeneity in treatment policies adopted. We retrospectively reviewed the patients of locally advanced retromolar trigone tumors treated with a standard and uniform multimodality management. A retrospective review of patients with locally advanced retromolar trigone tumors was performed and an analysis of clinicopathologic profile, treatment details, and survival outcomes was carried out. Forty-two patients of locally advanced retromolar trigone tumors underwent the standard multimodality treatment. The majority of them presented with stage IVa disease. Margin negative resection could be achieved in 93% of patients. Histopathologically proven bone and node involvement was seen in 20 patients (47.6%) and 21 patients (50%), respectively. The 3-year disease-free and overall survival rates were 64% and 71%, respectively. Treatment of patients with locally advanced retromolar trigone tumors is challenging. However, good oncologic outcomes can be achieved by advocating an aggressive surgical approach with postoperative radiation therapy.