Rose, Peter S.

Background:Myoepithelioma of the soft tissues is a rare entity and little is known about how best to manage locally recurrent and high-grade disease. Here, we retrospectively examined outcomes of surgery, chemotherapy, and radiation therapy (RT) for treatment of low-grade and high-grade myoepithelioma of soft tissues.Methods:We reviewed 20 cases of myoepithelioma of soft tissues seen at Mayo Clinic between 1994 and 2014. The effect of histologic grade and therapies received on relapse and survival were assessed.Results:We identified 13 patients with low-grade disease and 7 patients with high-grade disease. We found that low-grade disease was frequently effectively managed with surgical resection alone, whereas high-grade disease frequently metastasized and was often fatal. The 5-year event-free survival was 88% (confidence interval, 46%-98%) for low-grade disease versus 36% (confidence interval, 7%-75%;P=0.04) for high-grade disease. The relapse rate in low-grade disease was 29% at 5 years versus 64% (P=0.04) in high-grade disease. No significant responses to chemotherapy were noted, however, excellent responses to perioperative RT were seen.Conclusions:Surgery continues as the primary modality of treatment for myoepithelioma of soft tissues. Our study did not show a clear benefit of chemotherapy in the metastatic disease setting, but supports the use of perioperative RT in the management of high-grade disease; further investigation is warranted.

Introduction Symptomatic Metastatic Epidural Spinal Cord Compression (MESCC) afflicts up to 10% of all cancer patients and is associated with shortened survival and worsened quality of life. This study aims to identify the key survival prognostic factors in MESCC patients who were surgically treated for a single symptomatic lesion. Material and Methods 142 MESCC patients were enrolled in a prospective North American multi-center study and followed postoperatively for 12 months. Using univariate analyses, Kaplan-Meier methods, and log-rank tests the prognostic value of various clinical predictors were assessed. Non-collinear predictors with p < 0.05 in univariate analyses were included in the final Cox proportional hazards model. Results The overall median survival was 7.7 months (range: 3 days – 35.6 months); breast cancer had the longest median survival (12.1 months). Ten patients (7%), whose primary cancer were lung (3), kidney (3), sarcoma (2), prostate (1), and breast (1), died within 30-days postoperatively and 88 had died at 12 months (62%). Univariate analyses yielded eight significant predictors for survival: the growth of primary tumor (Tomita Grade 1 vs Grade 2 and 3), BMI, gender, preoperative SF-36 physical component, EQ-5D, and ODI scores as well as the presence of either visceral or extraspinal bony metastasis. The multiple regression analysis revealed that the Tomita grade (Grade 1 vs Grade 2 and 3; HR: 2.81, p = 0.007), the absence of visceral metastasis (HR: 2.01; p = 0.0044), and higher score on SF-36 physical component (HR: 0.95, p < 0.0001) were independent predictors for longer survival regardless of the selection method used (backward, forward, or stepwise). Conclusion Slow growing tumor (Tomita Grade 1), absence of visceral metastasis, and lower degree of preoperative physical disability, as reflected by a higher score on the SF-36 physical component questionnaire, are good prognostic factors for survival in selected patients who underwent surgical treatment for a focal symptomatic MESCC lesion.

Introduction Although surgery is being increasingly used in patients with Metastatic Epidural Spinal Cord Compression (MESCC) as a complementary strategy to radiation and chemotherapy, the impact of surgery on quality of life (QoL) is not well established. This study aimed to prospectively evaluate survival, neurological, functional, and QoL outcomes in MESCC patients undergoing operative management. Material and Methods A total of 142 surgically treated patients with a single symptomatic MESCC lesion enrolled in a prospective North American multi-center study were followed for 12 months. Clinical data, such as Brief Pain Inventory (BPI), ASIA, SF-36, Oswestry Disability Index (ODI), and EQ-5D scores, were obtained both pre- and post-operatively. Results The median survival was 7.7 months. The 30-day and 12-month mortality rates were 9% and 62%, respectively. Six weeks post-operatively, ambulatory status ( p = 0.02) and bladder control ( p = 0.03) were significantly improved. Overall, 67.5% of ASIA B, C, or D patients gained at least 1 grade after surgery, 25% remained stable, and 7.5% deteriorated. ODI, EQ-5D, BPI scores were significantly improved at each follow-up (p ≤ 0.01). SF-36 scores were generally higher after surgery for mental and physical components, and for all domains except energy/fatigue. The incidence of wound complications was 10% and 2 patients required a second surgery (screw malposition and epidural hematoma). Conclusion Surgical intervention, as a complementary adjunct to radiation and chemotherapy, provides immediate and sustained improvement in pain, neurological, functional, and QoL outcomes with acceptable risks in patients with a focal symptomatic MESCC lesion.

OBJECTIVE Primary spinal osteosarcomas are rare and aggressive neoplasms. Poor outcomes can occur, as obtaining marginal margins is technically demanding; further Enneking-appropriate en bloc resection can have significant morbidity. The goal of this study is to identify prognostic variables for local recurrence and mortality in surgically treated patients diagnosed with a primary osteosarcoma of the spine. METHODS A multicenter ambispective database of surgically treated patients with primary spine osteosarcomas was developed by AOSpine Knowledge Forum Tumor. Patient demographic, diagnosis, treatment, perioperative morbidity, local recurrence, and cross-sectional survival data were collected. Tumors were classified in 2 cohorts: Enneking appropriate (EA) and Enneking inappropriate (EI), as defined by pathology margin matching Enneking-recommended surgical margins. Prognostic variables were analyzed in reference to local recurrence and survival. RESULTS Between 1987 and 2012, 58 patients (32 female patients) underwent surgical treatment for primary spinal osteosarcoma. Patients were followed for a mean period of 3.5 ± 3.5 years (range 0.5 days to 14.3 years). The median survival for the entire cohort was 6.7 years postoperative. Twenty-four (41%) patients died, and 17 (30%) patients suffered a local recurrence, 10 (59%) of whom died. Twenty-nine (53%) patients underwent EA resection while 26 (47%) patients underwent EI resection with a postoperative median survival of 6.8 and 3.7 years, respectively (p = 0.048). EI patients had a higher rate of local recurrence than EA patients (p = 0.001). Patient age, previous surgery, biopsy type, tumor size, spine level, and chemotherapy timing did not significantly influence recurrence and survival. CONCLUSIONS Osteosarcoma of the spine presents a significant challenge, and most patients die in spite of aggressive surgery. There is a significant decrease in recurrence and an increase in survival with en bloc resection (EA) when compared with intralesional resection (EI). The effect of adjuvant and neoadjuvant chemotherapeutics, as well as method of biopsy, requires further exploration.

Background. Reconstruction of intrapelvic defects can be a challenging problem in patients with limited regional muscle flap options and previously resected omentum. In such situations, alternative methods of mobilizing vascularized tissue may be required.Methods. A case of a patient that underwent pelvic extirpation for recurrent rectal cancer who had limited donor sites for flap reconstruction is presented. The mucosa was removed from a blind loop of colon, and a pedicled seromuscular flap based on the colonic mesentery was placed into the pelvis for vascularized soft-tissue coverage and elimination of dead space.Results. The postoperative course was only complicated by a small subcutaneous fluid collection beneath the sacrectomy skin incision, which was drained with radiological assistance. The patient recovered without any major postoperative complications.Conclusion. Seromuscular colonic flap is a useful option for soft-tissue coverage after pelvic extirpation and should be considered by plastic surgeons when other reconstruction options are not available.

Introduction Primary spinal osteosarcomas are rare and aggressive neoplasms with poor outcomes. Enneking appropriate, en bloc resection is recommended for appendicular osteosarcomas. Obtaining even marginal margins is technically demanding in the spine, resulting in significant morbidity and possible functional sacrifice. The aim of this study was to identify prognostic variables on local recurrence and mortality, in surgically treated patients diagnosed with a primary osteosarcoma of the spine. Methods A multicenter ambispective database of surgically treated patients with primary spine osteosarcomas was developed by the AOSpine knowledge forum tumor. Patient demographic, diagnosis, treatment, perioperative morbidity, local recurrence, and cross-sectional survival data were collected. Tumors were classified according to Enneking principles and analyzed in the following two cohorts: Enneking appropriate (EA) and Enneking inappropriate (EI). EA was defined by the final pathology margin matching the Enneking recommended surgical margin and EI by not matching. Prognostic variables including age, previous spine tumor operation, biopsy type, spine level, tumor size, and chemotherapy timing were analyzed in reference to local recurrence and survival. Results Between 1987 and 2012, 57 patients (31 females and 26 males) underwent surgical treatment for a primary spinal osteosarcoma at a mean age of 36 ± 16 years. Patients were followed for a mean period of 3.4 ± 3.5 years (range, 0.5 days–14.3 years). Median survival for the entire cohort was 6.7 years postoperative. Overall, 24 (42%) patients died and 17 (30%) patients suffered a local recurrence, 10 (59%) of which died. Overall, 28 (52%) patients underwent EA resection while 26 (48%) patients were treated by EI resection. Median survival for patients with EA was 6.8 years postoperative, whereas median survival for EI patients was 3.7 years postoperatively ( p = 0.062). EI patients were at a higher risk of local recurrence than EA patients ( p = 0.002). Patient age, previous spine tumor operation, tumor size, spine level, and chemotherapy timing did not significantly influence recurrence and survival. Conclusion Osteosarcoma of the spine presents a significant challenge and most patients die from their disease in spite of aggressive surgery. There is a significant decrease in recurrence with en bloc resection when compared with intralesional resection.

Abstract Metastatic epidural spinal cord compression (MESCC) is an oncologic emergency and if left untreated, permanent paralysis will ensue. The treatment of MESCC is governed by disease, patient, and treatment factors. Patient's preferences and goals of care are to be weighed into the treatment plan. Ideally, a patient with MESCC is evaluated by an interdisciplinary team promptly to determine the urgency of the clinical scenario. Treatment recommendations must take into consideration the risk-benefit profiles of surgical intervention and radiotherapy for the particular individual's circumstance, including neurologic status, performance status, extent of epidural disease, stability of the spine, extra-spinal disease status, and life expectancy. In patients with high spinal instability neoplastic score (SINS) or retropulsion of bone fragments in the spinal canal, surgical intervention should be strongly considered. The rate of development of motor deficits from spinal cord compression may be a prognostic factor for ultimate functional outcome, and should be taken into account when a treatment recommendation is made. The American College of Radiology Appropriateness Criteria are evidence-based guidelines for specific clinical conditions that are reviewed every three years by a multidisciplinary expert panel. The guideline development and review include an extensive analysis of current medical literature from peer-reviewed journals and the application of a well-established consensus methodology (modified Delphi) to rate the appropriateness of imaging and treatment procedures by the panel. In those instances where evidence is lacking or not definitive, expert opinion may be used to recommend imaging or treatment.

Background. Radiotherapy has been utilized for metastatic and recurrent osteosarcoma and Ewing sarcoma (ES), in order to provide palliation and possibly prolong overall or progression-free survival. Stereotactic body radiotherapy (SBRT) is convenient for patients and offers the possibility of increased efficacy. We report our early institutional experience using SBRT for recurrent and metastatic osteosarcoma and Ewing sarcoma.Methods. We reviewed all cases of osteosarcoma or ES treated with SBRT between 2008 and 2012.Results. We identified 14 patients with a total of 27 lesions from osteosarcoma(n=19)or ES(n=8). The median total curative/definitive SBRT dose delivered was 40 Gy in 5 fractions (range, 30–60 Gy in 3–10 fractions). The median total palliative SBRT dose delivered was 40 Gy in 5 fractions (range, 16–50 Gy in 1–10 fractions). Two grade 2 and 1 grade 3 late toxicities occurred, consisting of myonecrosis, avascular necrosis with pathologic fracture, and sacral plexopathy. Toxicity was seen in the settings of concurrent chemotherapy and reirradiation.Conclusions. This descriptive report suggests that SBRT may be a feasible local treatment option for patients with osteosarcoma and ES. However, significant toxicity can result, and thus systematic study is warranted to clarify efficacy and characterize long-term toxicity.