Disease In Life Research Articles

The effect of long-standing lymphopenia after radiation therapy on survival in rectal cancer

BackgroundLymphopenia and high neutrophil-to-lymphocyte ratio are known negative prognostic factors in rectal cancer. Until recently, however, lymphopenia was regarded as a minor sequela following radiation therapy (RT). The immune system's influence on rectal cancer treatment outcomes led us to evaluate the impact of lymphopenia at various time points, before, during, and following radiotherapy. We hypothesized that chronic lymphopenia following radiotherapy might negatively influence the survival of patients, and pre-treatment lymphopenia may be predictive of poor outcomes. MethodsThis retrospective study involved 110 patients treated for rectal cancer between 2015 and 2019. The oncological outcomes are defined as alive without disease (AWOD), alive with disease (AWD), and death. These outcome probabilities tested against variables of lymphopenia before RT, during RT, and at several post-RT follow-up time points. ResultsAt the end of the study, 69 patients were AWOD (63 %), 13 were AWD (12 %) and 28 had died (25 %). Treatment results were assessed with according level of lymphocytes measured one year following RT: 35 out of 39 patients (89.7 %) with normal values were AWOD. In 65 patients with sustained lymphopenia, 52 % were AWOD, 18.5 % AWD and 29 % died. A similar difference was found at all time-points up to 2 years following RT (p < 0.004).The results of our study shows that pre-existing lymphopenia (prior to RT) is associated with a 3 times greater chance of death compared to patients with normal lymphocyte levels prior to RT. The PFS significantly affected by lymphopenia at all time-points after RT. An NLR of more than 4 was associated with a 3-time higher risk of recurrence than lower NLR scores (p = 0.0054). ConclusionOur results support the relevance of lymphopenia and NLR in the prognosis of rectal cancer. We believe this is the first study showing a negative correlation between sustained lymphopenia and OS following RT.

The Utility of Phosphohistone-H3 Immunohistochemistry (PHH3) in Pheochromocytomas

Abstract Introduction/Objective Most pheochromocytomas (PCC) are sporadic; ~40% present as part of familial syndromes and some cases are associated with germline mutations of succinate dehydrogenase (SDH) genes. Few cases are malignant. The Pheochromocytoma of the Adrenal Gland Scaled Score (PASS) is a scoring system to predict behavior that includes evaluation of mitotic count, among other parameters. PHH3 has been used to assess mitosis in breast and glial tumors. The aim of this study was to assess the potential utility of PHH3 in pheochromocytomas. Methods/Case Report 50 cases of PCC diagnosed between 2016 and 2020 at our institution were included in the study. Clinicopathologic data was recorded. One hematoxylin and eosin (H&amp;E) slide was selected per case and mitotic count was evaluated at 40x high power-fields (HPF). PHH3 was performed on the same block to assess mitoses. Cells with dark and coarsely clumped staining were considered positive for PHH3. PHH3-positive cells were counted also at 40x HPF. In one case with bilateral pheochromocytomas, the largest tumor was evaluated. Follow-up was available in 39 cases and recorded as no evidence of disease (NED), alive with disease (AWD), or dead of disease (DOD). Results (if a Case Study enter NA) 21 patients were female, 29 were males. Age ranged from 24 to 95 (mean: 56). Tumor size ranged from 1.1 to 16.0 cm (mean: 5.6 cm). Mean follow-up was 13.6 months (1-32 months range). Mitotic count ranged from 0 to 7 and 0 to 21 per 10 HPFs, on H&amp;E and PHH3, respectively. PHH3 showed higher mitotic count in 33 cases compared to H&amp;E. In 11 cases, mitotic count was equal on H&amp;E and PHH3, and in the remaining cases, mitotic count was higher on H&amp;E than PHH3. In cases with PASS score ≥ 4, 50% and 27% of cases had mitotic count &amp;gt;3/10HPF on PHH3 and H&amp;E, respectively. Follow-up showed two patients AWD and one DOD, all of whom had PASS score ≥ 4, with mitotic count on H&amp;E 7, 1 and 6 and mitotic count on PHH3 8, 4, and 21 respectively. Conclusion This is the first study evaluating the use of PHH3 in pheochromocytomas. Overall, mitotic count was higher on PHH3 than on H&amp;E. This indicates PHH3 is a helpful tool in counting mitosis. Since the PASS score is an imperfect system to predict outcome, additional studies are needed to evaluate the use of PHH3 in prognostic scoring systems for PCC.

In stage IV pulmonary adenocarcinoma patients, treatment with Traditional Chinese Medicine alone gives prognostically superior results to treatment with Platinum-Based Chemotherapy alone

BackgroundAbout 30% of pulmonary stage IV adenocarcinomas die within 3 months of diagnosis. Western medical treatments with Platinum-Based Chemotherapy=PBC and tyrosine-kinase inhibitors Targeted Therapy=TT can improve prognosis. In China, Traditional Chinese Medicine herbal treatments (TCM) are often used in addition to PBC and TT. A considerable number of patients refuse Western medical treatments and use TCM alone. However, the survival impact of the latter is unknown. Hypotheses testedTreatment with TCM alone is prognostically superior to PBC alone. Addition of PBC or TT or both TT to TCM improves survival. MethodsIn this prospective observational, non-interventional study of 1017 consecutive first-onset stage IV NSCLC patients with up to 10 years follow-up, 261 who Died of Disease (DOD) within 3 months were omitted, as they never got the optimal Western medical therapies. All 218 non-adenocarcinomas were also omitted, leaving 538 stage IV adenocarcinomas treated by TCM alone (n = 29), PBC alone (N = 19) and TCM and other Western medical combinations (299 TCM and PBC, 50 TCM and TT, 141 TCM and PBC and TT) with 3 – 120 months follow-up. Survivals were compared using Alive with Disease (AWD) and DOD as endpoints. ResultsThe patients treated only with TCM had 7 months better median survival than those that received PBC alone (17 and 10 months). The patients that received TCM and PBC had a better median survival (24 months) than TCM alone and much better than PBC alone. None of the patients that received TCM alone survived > 54 months, whereas 18% of TCM and PBC patients survived much longer. Over the observation period of 3 – 120 months, survivals of TCM and TT, TCM and PBC and TT, and TCM and PBC were not different and therefore grouped as TCM and Western medicines. Median survival times of PBC alone and TCM alone were lower than that of TCM and Western medical treatments (p < 0.0001, 10, 17 and 27 months). ConclusionsPulmonary stage IV adenocarcinoma patients with at least 3 months survival, treated with TCM alone have a significantly better survival than those treated with PBC alone. Adding Western PBC, TT or both to TCM further improves prognosis.

C-Reactive Protein Pretreatment-Level Evaluation for Ewing's Sarcoma Prognosis Assessment-A 15-Year Retrospective Single-Centre Study.

A pathological/inflamed cellular microenvironment state is an additional risk factor for any cancer type. The importance of a chronic inflammation state in most diffuse types of tumour has already been analysed, except for in Ewing's sarcoma. It is a highly malignant blue round cell tumour, with 90% of cases occurring in patients aged between 5 and 25 years. Worldwide, 2.9 out of 1,000,000 children per year are affected by this malignancy. The aim of this retrospective study was to analyse the role of C-reactive protein (CRP) as a prognostic factor for Ewing's sarcomas. This retrospective study at Klinikum rechts der Isar included 82 patients with a confirmed Ewing's sarcoma diagnosis treated between 2004 and 2019. Preoperative CRP determination was assessed in mg/dL with a normal value established as below 0.5 mg/dL. Disease-free survival time was calculated as the time between the initial diagnosis and an event such as local recurrence or metastasis. Follow-up status was described as death of disease (DOD), no evidence of disease (NED) or alive with disease (AWD). The exclusion criteria of this study included insufficient laboratory values and a lack of information regarding the follow-up status or non-oncological resection. Serum CRP levels were significantly different in patients with a poorer prognosis (DOD) and in patients who presented distant metastasis (p = 0.0016 and p = 0.009, respectively), whereas CRP levels were not significantly different in patients with local recurrence (p = 0.02). The optimal breakpoint that predicted prognosis was 0.5 mg/dL, with a sensitivity of 0.76 and a specificity of 0.74 (AUC 0.81). Univariate CRP analysis level &gt;0.5 mg/dL revealed a hazard ratio of 9.5 (95% CI 3.5-25.5). In Ewing's sarcoma cases, we consider a CRP pretreatment value &gt;0.5 mg/dL as a sensitive prognostic risk factor indication for distant metastasis and poor prognosis. Further research with more data is required to determine more sensitive cutoff levels.

Real-Time Online Adaptive Magnetic Resonance Image Guided Stereotactic Body Radiation Therapy with or without Elective Nodal Irradiation for Inoperable Pancreas Cancer Patients

<h3>Purpose/Objective(s)</h3> Stereotactic body radiation therapy (SBRT) to patients with inoperable pancreas cancer has shown promising outcomes. Dose escalated SBRT is usually challenging given the associated risks to nearby organs. This study aims to report treatment outcomes and adverse events of Real-time Online Adaptive Magnetic Resonance-Guided SBRT (MRgSBRT). <h3>Materials/Methods</h3> This IRB approved retrospective study included inoperable pancreas cancer patients who were treated consecutively with MRgSBRT from 2020 to 2021. Most patients (89%) received 50Gy prescribed to gross tumor volume (GTV), two patients (11%) received 40 Gy to GTV. Ten patients (56%) received elective nodal irradiation (ENI) to celiac and super mesenteric lymph nodes, & 8 patients (44%) did not receive ENI. Planning Target Volume (PTV) received a median dose of 35 Gy (range 25-50 Gy) and 40 Gy (range 40-50 Gy), in patients with, and without ENI respectively. PTV was defined as 3 mm expansion of either GTV, or a clinical target volume encompassing GTV and ENI areas when treated. Real-time Online Adaptive MRgSBRT was utilized in 59% of the treated fractions (53 of 90 fractions), while gated MRgSBRT was utilized in all fractions (100%). Real-time Online Adaptive MRgSBRT was utilized whenever the predicted radiation plan did not meet the organs at risk (OARs) constraints based on daily anatomic variations. Reoptimized plans were generated after re-contouring OARs within a 3 cm ring, & verifying GTV & PTV, & always met OARs constraints. All patients were treated twice weekly. <h3>Results</h3> The study included 18 eligible patients with median age of 72.5 years (range 56-85). Patients had local-regional advanced (44%), borderline (33%) & metastatic (22%) diseases. At a median follow up of 10 months from MRgSBRT (range 2-17 months), in patients with & without ENI local-regional progression was 20% versus 67% (p 0.12 Fisher's Exact Test), distant progression was 30% versus 67% (p 0.3), & overall survival was 80% and 37.5% (p 0.14) respectively. Of those who developed disease progression, first site of progression was distant metastases (75%), versus regional nodal progression (75%) in patients with & without ENI respectively. In the non-ENI group, 50%, 12.5%, 12.5%, & 25% were dead of disease (DOD), dead without disease progression (DWODP), alive with disease (AWD), & alive without disease progression (AWODP) respectively. In the ENI group, 10%, 10%, 60%, & 20% were DOD, DWODP, AWD, & AWODP respectively. Median follow up from diagnosis was 19 months (range 6-103 months). Acute & chronic toxicities were uncommon, & included grade 1-2 nausea (22%), fatigue (17%), & abdominal discomfort (11%). No grades 3-5 toxicities were reported. <h3>Conclusion</h3> Real-time Online Adaptive MRgSBRT is a feasible, & safe approach with minimal treatment related toxicities & promising local-regional control in a cohort of advanced pancreas cancer patients. ENI is safe & shows a trend for improved local-regional control. Larger controlled prospective trials are recommended.

Clinicopathological assessment of cancer/testis antigens NY‑ESO‑1 and MAGE‑A4 in osteosarcoma.

The cancer/testis antigens (CTAs), New York esophageal squamous cell carcinoma-1 (NY-ESO-1) and melanoma antigen gene (MAGE)-A4 are normally restricted to male germ cells but are aberrantly expressed in several cancers. Considering the limited information regarding their significance in osteosarcoma (OS), the purpose of this study was to determine the clinical significance of NY-ESO-1 and MAGE-A4 expression in OS. Nine patients with OS treated at Kindai University Hospital were included in the study. The median age was 27 years, and median follow-up period was 40 months. The specimens obtained at the time of biopsy were used to perform immunostaining for NY-ESO, MAGE-A4, p53, and Ki-67. The positive cell rates and positive case rates of NY-ESO, MAGE-A4, p53, and Ki-67 were calculated. The correlation between the positive cell rate of immunohistochemical markers was also calculated. The correlation between the positive cell rate of NY-ESO-1 or MAGE-A4 and tumor size or maximum standardized uptake (SUV-max) was also determined. The positive cell rates of NY-ESO-1 or MAGE-A4 in continuous disease-free (CDF) cases were also compared with those in alive with disease (AWD) or dead of disease (DOD) cases. The average positive cell rates of NY-ESO, MAGEA4, p53, and Ki-67 were 71.7%, 85.1%, 16.2%, and 14.7%, and their positive case rates were 33.3%, 100%, 44.4%, and 100%, respectively. The positivity rates of NY-ESO-1 and p53 were strongly correlated, whereas those of NY-ESO-1 and Ki-67 were moderately correlated. The MAGE-A4 and p53 positivity rates and the MAGE-A4 and Ki-67 positive cell rates were both strongly correlated. The NY-ESO-1 and MAGE-A4 positivity rates were moderately correlated. The positive correlation between the NY-ESO-1 positive cell rate and tumor size was medium, and that between the MAGE-A4 positivity rate and SUV-max was very strong. There was no significant difference in the positive cell rates of NY-ESO-1 or MAGE-A4 between CDF cases and AWD or DOD cases. Overall, our results suggest that NY-ESO-1 and MAGE-A4 may be involved in the aggressiveness of OS.

Real-world experience of tyrosine kinase inhibitors in patients (pt) with recurrent bone tumours (BT): A CanSaRCC study.

11530 Background: Survival after relapse in osteosarcoma (OST), Ewing Sarcoma (ES) and chondrosarcoma (CS) remains dismal. Recent reports suggest a role of tyrosine kinase inhibitors (TKI) including regorafenib (R) and cabozantinib (C). We conducted a retrospective multi-centre pan-Canadian study to assess real-word outcomes with these novel treatments in recurrent BT. Methods: After ethics approval, data from pts treated in 7 different institutions was extracted from the CanSaRCC (Canadian Sarcoma Research and Clinical Collaboration) Database. Pt characteristics, treatment and outcomes were analyzed. Response was assessed per RECIST 1.1. PFS, OS were estimated using Kaplan-Meier. TTP was defined as time from TKI start to progression. Results: From June 2018-Dec 2021, 44 pts received R or C and best response by histology are listed in Table, with an overall clinical benefit rate of 63.6%. Median time to best response was 2.3 mo (range 1 – 17). 15 pts (34.1%) required dose reduction; most common reasons were hand-foot syndrome (13.6%), mucositis (9.1%) and hypertension (9.1%). At median FU of 6.4 mo (range 1.6 – 29), 25 pts (56.8%) died, 19 (43.2%) were alive with disease (AWD). Median PFS was 4.1 mo (95%CI 2.9 – 5.7), for OST was 5.0 (N = 25, 95%CI 2.6 – 10.6), for ES was 4.1 (N = 10, 95%CI 2-5.9), and for CS 4.0 (N = 9, 1 Progressed). Median OS was 10.5 mo (95%CI 7 – 14). By univariate analysis, age, line of therapy, gender, location of primary, or R vs. C did not correlate with PFS. Conclusions: Consistent with previous published studies, our pan-country real-world analysis shows that TKI have meaningful activity in the setting of recurrent BT with acceptable toxicities. Inclusion in earlier lines of treatment and/or maintenance therapy could be questions for future research. [Table: see text]

"How Long Have I Got?" in Stage IV NSCLC Patients With at Least 3Months Up to 10Years Survival, Accuracy of Long-, Intermediate-, and Short-Term Survival Prediction Is Not Good Enough to Answer This Question.

BackgroundMost lung cancer patients worldwide [stage IV nonsmall cell lung cancer (NSCLC)] have a poor survival: 25%–30% die <3 months. Yet, of those surviving >3 months, 10%–15% (70,000–105,000 new patients worldwide per year) survive (very) long. Surprisingly, little scientific attention has been paid to the question, which factors cause the good prognosis in these NSCLC stage IV long survivors. Therefore, “How long do I still have?” currently cannot be accurately answered. We evaluated in a large group of 737 stage IV NSCLC patients surviving 3.2–120.0 months, the accuracies of short- and long-term survival predictive values of baseline factors, radiotherapy (RT), platinum-based chemotherapy (PBT), and tyrosine kinase inhibitor targeted therapy (TKI-TT).MethodsThis is a noninterventional study of 998 consecutive first-onset stage IV NSCLC patients. A total of 737 (74%) survived 3.2–120.0 months, 47 refused RT, PBT, and TKI-TT. Single and multivariate survival analysis and receiver operating curve (ROC) analysis were used with dead of disease (DOD) or alive with disease (AWD) as endpoints.ResultsThe median survival (16.1 months) of 47 patients who refused PBT, RT, and TKI-TT was significantly worse than those with RT, PBT, and/or TKI-TT (23.3 months, HR = 1.60, 95% CI = 1.06–2.42, p = 0.04). Of these latter 690 patients, 42% were females, 58% males, median age 63 years (range 27–85), 1-, 2-, 5-, and 10-year survival rates were 74%, 49%, 16%, and 5%. In total, 16% were alive with disease (AWD) at the last follow-up. Pathology subtype (adenocarcinoma vs. all others), performance score, TNM substage, the number of PBT cycles and TKI-TT had independent predictive value. However, with the multivariate combination of these features, identification results of short-term nonsurvivors and long-term survivors were poor.ConclusionsIn stage IV NSCLC patients with >3 months survival, baseline features, and systemic therapeutic modalities have strong survival predictive value but do not accurately identify short- and long-term survivors. The predictive value of other features and interventions discussed should be investigated in the worldwide very large group of stage IV NSCLC patients with >3 months survival.

Exploring the association with disease recurrence of miRNAs predictive of colorectal cancer.

Disease recurrence after surgery is a crucial predictor of poor prognosis in colorectal cancer, where disseminated disease at the time of intervention can also be observed in localized early-stage cases. We evaluated the ability to predict disease recurrence of miRNAs from two signatures that we have found linked to the presence of colorectal cancer (CL signature) or adenoma (HgA signature) in higher-risk subjects. miRNAs from the signatures were studied longitudinally by quantitative real-time polymerase chain reaction in plasma from 24 patients with resectable colorectal cancer collected at the time of surgery and during scheduled follow-up across 36 months. Patients either showed relapse within 36 months (alive with disease (AWD)), or remained disease-free (no evidence of disease (NED)) for the same period. Although the signatures did not predict recurrence, expression of the miRNAs from the CL signature decreased 1 year after surgery, and one miRNA of the signature, miR-378a-3p, almost reached significance in the NED subgroup (Wilcoxon signed-rank test: p-value = 0.078). Also, miR-335-5p from the HgA signature was higher in AWD patients before surgery (Kruskal-Wallis test: p-value = 0.019). These data, although from a small cohort of patients, support the possible use of miRNAs as non-invasive biomarkers in liquid biopsy-based tests to identify patients at risk of relapse and to monitor them during follow-up.

Outcomes of patients with mucoepidermoid carcinoma of minor salivary gland in palate undergoing radical resection followed by submental flap reconstruction

ObjectiveTo investigate the outcomes of patients with mucoepidermoid carcinoma of the palate undergoing pedicled facial-submental artery island flap (FSIF) reconstruction following resection. Patients and methods41 patients with early stage disease and 9 patients with advanced-stage disease underwent radical excision and neck dissection. 37 IIb, 4 class IIa and 9 IIIb maxillary defects were reconstructed with FSIF, folded FSIF or folded FSIF with titanium mesh respectively. The skin paddles were 3 × 8 to 5 × 15 cm and 3 × 8 to 5 × 14 cm, respectively. 5 patients with high grade disease were treated with cobalt 60 adjuvant radiotherapy after operation. ResultsOne flap failure occurred, yielding a success rate of 98.0% in the reconstruction of palate II and III defects with FSIF or titanium mesh. The patients were seen for follow-up for 16–60 months postoperative. 76.0% patients alive with no disease (AND); 14.0% patients alive with disease (AD) and 10.0% died of disease (DD). Rates of AND, AD and DD differed significantly according to histopathologic grade and TNM stage (P < 0.001); rates of AND, AD and DD differed obviously according to necrosis of the tumors lymph node metastasis, and tumour cell anaplasia and treatment (P < 0.05). ConclusionsRadical resection with wide safety margins of normal tissues including neck dissection is the mainstay of treatment modality. The patients with high grade disease should be treated with postoperative radiotherapy. The FSIF is a reliable and safe method for repairing Brown class II maxillary defects.

One-Stage Soft Tissue Reconstruction Following Sarcoma Excision: A Personalized Multidisciplinary Approach Called "Orthoplasty".

Background and Objectives. Wide surgical resection is a relevant factor for local control in sarcomas. Plastic surgery is mandatory in demanding reconstructions. We analyzed patients treated by a multidisciplinary team to evaluate indications and surgical approaches, complications and therapeutic/functional outcomes. Methods. We analyzed 161 patients (86 males (53%), mean age 56 years) from 2006 to 2017. Patients were treated for their primary tumor (120, 75.5%) or after unplanned excision/recurrence (41, 25.5%). Sites included lower limbs (36.6%), upper limbs (19.2%), head/neck (21.1%), trunk (14.9%) and pelvis (8.1%). Orthoplasty has been considered for flaps (54), skin grafts (42), wide excisions (40) and other procedures (25). Results. At a mean follow-up of 5.3 years (range 2–10.5), patients continuously showed no evidence of disease (NED) in 130 cases (80.7%), were alive with disease (AWD) in 10 cases (6.2%) and were dead with disease (DWD) in 21 cases (13.0%). Overall, 62 patients (38.5%) developed a complication (56 minor (90.3%) and 6 major (9.7%)). Flap loss occurred in 5/48 patients (10.4%). The mean Musculoskeletal Tumor Society (MSTS) and Toronto Extremity Salvage Score (TESS) was 74.8 ± 14 and 79.1 ± 13, respectively. Conclusions. Orthoplasty is a combined approach effective in management of sarcoma patients, maximizing adequate surgical resection, limb salvaging and functional recovery. One-stage reconstructions are technically feasible and are not associated with increased risk of complications.

Discontinuous Involvement of Spermatic Cord Soft Tissue in Testicular Germ Cell Tumors: A Multi-Institution Experience

Abstract Introduction/Objective In the 8th Edition AJCC Cancer Staging Manual, discontinuous involvement of spermatic cord soft tissue (DISC) by testicular germ cell tumors (GCT) is regarded as metastatic deposit (pM1), placing the patient in clinical prognostic stage group (CPSG) III. We conducted a multi-institution study to corroborate or refute the current recommendations. Methods: Thirty-eight cases of GCT with spermatic cord involvement were collected from 13 institutions in Europe, Phillipines and America. Clinical and pathologic data was obtained. Results Tumors included 28 (73%) non-seminomatous and 10 (26%) seminomatous GCTs. Mean testicular tumor size was 6.6 cm (range 1.3-18). After review by an uropathologist, cases were classified as cord LVI [T2] (n=3), continuous cord involvement (CCI) [T3] (n=13), and DISC (n=22). Mean cord tumor size for DISC was 0.9 cm (range 0.1-4.5). CPSG was available for 33 and follow-up (FU) for22 patients with a mean length of FU of 38 months (range 2-144). Seven (39%) DISC patients were CPSG II (regional LN metastases), and 11 (61%) CPSG III (distant metastases). On FU, 5 (45%) DISC patients had no evidence of disease (NED); 6 (55%) were alive with disease (AWD). Three (25%) CCI patients were CPSG I (local disease), 6 (50%) CPSG II, and 3 (25%) CPSG III. On FU, 6 (60%) CCI patients were NED, 4 (40%) AWD. Cord LVI patients were one in each CPSG. One cord LVI patient was NED, the others were lost at FU. All DISC (100%) patients with available CPSG had advanced disease (CPSG II or III), compared to 75% of CCI, and 67% of cord LVI patients. Conclusion Although it did not reach statistical significance (p=0.054; Fisher’s exact test), DISC patients were more likely to have CPSG II and III compared to CCI patients. Our findings suggest a worse behavior in patients with DISC, supporting a higher pathologic stage than CCI.

Paraganglioma of the spine: A twenty-years clinical experience of a high volume tumor center

Paragangliomas (PGs) are rare tumours with a reported estimated annual incidence of up to 3 per million. Spinal involvement may possible with spinal metastasis and primary extradural localizations.The aim of this paper is to evaluate clinical outcomes of surgical treatment of a rare disease that can involve the spine and that should be considered in the differential diagnosis of spinal injuries.This is a retrospective observational study of the spinal paragangliomas treated at our institute. Five patients have been enrolled: three with metastatic PG and two with extradural PG. Metastatic PGs were treated with intralesional excision plus adjuvant therapies instead, extradural PGs with intralesional excision without adjuvant therapies.Among patients affected by metastastic paraganglioma two patients were Alive with disease (AWD) at the latest follow and one patient died for the spreading of disease at 240 months after surgery. Two patients with extradural paraganglioma of thoracic spine were AWD at the latest follow-up without pain and neurological deficits.Surgical management of spinal localizations can represent a challenge. Surgery has a main role in both diseases where intralesional excision plus adjuvant therapies seems to be able to achieve the local control and a satisfying prognosis in case of undisseminated tumour.

Feasibility of uterine preservation in the management of early-stage uterine adenosarcomas: a single institute experience

BackgroundWe aimed to evaluate the efficacy and the safety of uterine preservation in patients with early-stage uterine adenosarcoma who want to preserve future fertility.MethodsWe performed a retrospective review of patients with stage I uterine adenosarcoma diagnosed and treated at a single institute from 1998 through 2014.ResultsAmong the total of 31 patients, uterine preservation surgery was performed in 7 of the nulliparas. Of the 7 patients receiving uterine preservation surgery, 3 showed no evidence of disease (NED), 2 had persistent disease confined to the uterus, and 2 were alive with disease (AWD) after recurrence. One patient with an NED status had a vaginal delivery at term. In the uterine preservation group, 1 patient had sarcomatous overgrowth at the time of diagnosis and experienced disease recurrence. In the hysterectomy group, 3 of 24 patients had tumor recurrence. Of the five patients with tumor recurrence, four (80%) had sarcomatous overgrowth at diagnosis and it was significantly associated with recurrence by univariate analysis (OR 13.3, p = 0.027).ConclusionsUterine preservation represents a possible treatment option for young female patients who want to maintain fertility. However, a detailed explanation of the risk of recurrence is necessary, especially in patients with sarcomatous overgrowth, which seems to be associated with a higher risk of recurrence.Trial registrationRetrospectively registered.

Craniofacial resection and reconstruction in patients with recurrent cancer involving the craniomaxillofacial region

Purpose Head and neck tumors that involve the craniomaxillofacial region are classified as stage IVb disease and are clinically challenging. In this study, the outcomes of craniofacial resection and craniofacial reconstruction in patients with recurrent malignant tumors involving the craniomaxillofacial region were evaluated. Patients and Methods This retrospective observational study was conducted from January 2008 to August 2015. Data collected for each patient included age, gender, tumor site, initial treatment, craniofacial resection, reconstruction flaps and complications after craniofacial resection, adjuvant treatment, and reported outcomes of craniofacial resection and craniofacial reconstruction. The χ2 test in SPSS was used to analyze the data. Results Twenty-four patients with recurrent malignant tumors involving the craniomaxillofacial region were identified who had undergone craniofacial resection at the Center of Craniomaxillofacial Surgery of Sun Yat-sen University (Guangzhou, Guangdong, China). The study population was comprised of 24 patients (15 men and 9 women; age range, 21 to 73 yr) with recurrent tumors (58.3% with squamous cell carcinoma [SCC], 41.7% with sarcoma [SA]) involving the craniomaxillofacial region who underwent craniofacial resection. Craniofacial resection consisted of orbital exenteration and maxillotomy; anterior skull base surgery, facial resection, and mandibulotomy; or ipsilateral radical neck dissection. The resultant craniomaxillofacial defects were reconstructed using extended vertical lower trapezius island myocutaneous flaps (TIMFs), temporalis myofascial flaps, or submental flaps. All patients with recurrent malignant tumor involving the craniomaxillofacial region underwent gross total resection of the tumor; 22 patients underwent craniofacial reconstruction. There were no major surgical complications. Minor flap failure and wound dehiscence in the donor site occurred in 4 patients. The follow-up period ranged from 8 to 36 months. Seven patients in the SCC group and 7 in the SA group were alive with no evidence of disease (AND), 3 in the SCC group and 2 in the SA group were alive with disease (AWD), and 4 in the SCC and 1 in the SA group died of the disease (DOD) after local recurrence or distant metastases at 8 to 18 months. There were no statistical differences among the AND, AWD, and DOD groups. Conclusions Craniofacial resection remains an effective salvage treatment for patients with recurrent SCC and SA involving the craniomaxillofacial region. The extended vertical lower TIMF is a large, simple, and reliable flap for reconstructing major defects after a craniofacial resection.

Craniofacial Resection and Reconstruction in Patients With Recurrent Cancer Involving the Craniomaxillofacial Region

Head and neck tumors that involve the craniomaxillofacial region are classified as stage IVb disease and are clinically challenging. In this study, the outcomes of craniofacial resection and craniofacial reconstruction in patients with recurrent malignant tumors involving the craniomaxillofacial region were evaluated. This retrospective observational study was conducted from January 2008 to August 2015. Data collected for each patient included age, gender, tumor site, initial treatment, craniofacial resection, reconstruction flaps and complications after craniofacial resection, adjuvant treatment, and reported outcomes of craniofacial resection and craniofacial reconstruction. The χ2 test in SPSS was used to analyze the data. Twenty-four patients with recurrent malignant tumors involving the craniomaxillofacial region were identified who had undergone craniofacial resection at the Center of Craniomaxillofacial Surgery of Sun Yat-sen University (Guangzhou, Guangdong, China). The study population was comprised of 24 patients (15 men and 9 women; age range, 21 to 73yr) with recurrent tumors (58.3% with squamous cell carcinoma [SCC], 41.7% with sarcoma [SA]) involving the craniomaxillofacial region who underwent craniofacial resection. Craniofacial resection consisted of orbital exenteration and maxillotomy; anterior skull base surgery, facial resection, and mandibulotomy; or ipsilateral radical neck dissection. The resultant craniomaxillofacial defects were reconstructed using extended vertical lower trapezius island myocutaneous flaps (TIMFs), temporalis myofascial flaps, or submental flaps. All patients with recurrent malignant tumor involving the craniomaxillofacial region underwent gross total resection of the tumor; 22 patients underwent craniofacial reconstruction. There were no major surgical complications. Minor flap failure and wound dehiscence in the donor site occurred in 4 patients. The follow-up period ranged from 8 to 36months. Seven patients in the SCC group and 7 in the SA group were alive with no evidence of disease (AND), 3 in the SCC group and 2 in the SA group were alive with disease (AWD), and 4 in the SCC and 1 in the SA group died of the disease (DOD) after local recurrence or distant metastases at 8 to 18months. There were no statistical differences among the AND, AWD, and DOD groups. Craniofacial resection remains an effective salvage treatment for patients with recurrent SCC and SA involving the craniomaxillofacial region. The extended vertical lower TIMF is a large, simple, and reliable flap for reconstructing major defects after a craniofacial resection.

Epithelioid Sarcoma of the Upper Extremity

Objective: Epithelioid sarcoma (ES) is a relatively rare soft tissue tumor that occurs in the extremities. Slow-growing, the seemingly benign appearance often results in misdiagnosis. Inadequate surgeries, as well as mistaken treatment as chronic ulcer or wound infection may lead to delayed recognition. ES is usually resistant to chemotherapy; thus, once metastasized, few options remain for its treatment. We reviewed upper limb ES cases that underwent treatment at our institution, all of which had experienced misdiagnosis. Patients: Ten patients (7 male, 3 female) were treated for ES in the upper limb between 1992 and 2015. The age at presentation was 6 to 88 years (mean, 36.7 ± 2.4 years). The initial tumor site was the digits in 4, palm in 2, forearm in 3, and upper arm in 1 case. All 10 had been treated previously at other institutions before diagnosis as a malignancy and subsequent referral to the orthopedic oncology division at our hospital. Results: The time between onset of symptoms and histological diagnosis was 3 months to 14 years (mean, 66.7 ± 6.2 months). The initial symptoms were a nodule in 5 cases (3 asymptomatic), persistent swelling after trauma in 2 cases, and a chronic ulcer in 1. Two cases were presented as neuropathies; a tumor at the wrist was diagnosed as Guyon canal syndrome and another in the forearm had caused anterior interosseous nerve palsy. Before recognition as a malignancy, 4 had been operated on as a benign tumor, and the others had been treated surgically as osteomyelitis, hematoma, wound infection, neuropathy, or chronic ulcer. None had evidence of metastasis on positron emission tomography (PET) scans at the time of referral to our services. Once correctly diagnosed, all cases received additional surgery. As adjuvant therapy, 3 received chemotherapy. Three were given radiotherapy; one at the primary site and 2 at sites of metastasis. At last follow-up, 5 cases were continuous disease free (CDF) and 2 were alive with disease (AWD); their follow-up was 19 months to 13 years and 9 months (mean, 95.9 ± 7.6 months). Five had experienced metastasis. Of the metastasis cases, 3 had died of disease. All 3 had been given radiotherapy and 2 were given chemotherapy. The duration of disease before death in these cases was 185, 197, and 30 months. There seemed to be no apparent correlation between the duration of symptoms and disease progression. A case treated 6 months after onset experienced multiple metastases and died 22 months after diagnosis, while cases of more than 10 years of duration are currently CDF or AWD. As surgery still seems to be the only curative option, early diagnosis and immediate treatment before metastasis are advisable. Conclusions: ES is not only rare, but does not always present as a typical sarcoma. Misdiagnosis is common, and in some instances, the mistake may be fatal. This tumor should be kept in mind when observing and treating a seemingly benign skin lesion that is taking time to heal.

Prothèses totales de coude dans les tumeurs primitives et secondaires

BackgroundProstheses can be used in elbow reconstruction in both primary and metastatic lesions. Several authors have reported their experience with different types of implant, but not with modular prostheses. HypothesisLimb salvage using an elbow prosthesis is effective in obtaining good functional results and reliable local tumor control. Material and methodsForty-seven patients treated at the Rizzoli Institute for elbow neoplasm from 1990 to 2012 were evaluated. There were 30 primary tumors (64%), 24 bone tumors and 6 soft tissue sarcomas, and 17 bone metastases. Elbow reconstruction used a modular prosthesis in 25 patients and a standard prosthesis in 22. Reconstruction was primary in 30 patients and secondary in 17. ResultsAt last control, 15 (32%) were dead of disease (DOD) at a mean follow-up of 35months, 12 (25%) were alive with disease (AWD) at a mean follow-up of 29months, 19 (40%) showed no evidence of disease (NED) at a mean follow-up of 80months. Early complications were related to unexpected neurological damage, observed in 12 patients (25%): in 5 cases, the deficit resolved in a mean 6months; in the others, no or only partial recovery was observed. Two implants (4%) developed infection: 1 was treated with antibiotic therapy, and the other required implant revision. One implant showing cement extrusion was revised. In 3 patients (6%), radiography showed a radiolucent halo around the stem (2 humeral, 1 ulnar); no measures were taken, as the patients were completely asymptomatic at every follow-up. In 3 patients (6%), partial resorption of the allograft was observed on X-ray, but remained unchanged at last follow-up, without pain or functional impairment. Seven local recurrences (15%) were observed, at a mean 16months after surgery; 5 were treated by resection and/or radiotherapy, and 2 by amputation. Mean functional scores on MEPS and MSTS were respectively 84% and 22/30 (73%). ConclusionsElbow prostheses provided better function in primary than in metastatic tumor. Elbow prosthesis reconstruction after tumor resection is a viable option both for primary and secondary bone neoplasms. Type of studyTherapeutic. Level of evidenceIV, retrospective study.

Total elbow arthroplasty for primary and metastatic tumor

BackgroundProstheses can be used in elbow reconstruction in both primary and metastatic lesions. Several authors have reported their experience with different types of implant, but not with modular prostheses. HypothesisLimb salvage using an elbow prosthesis is effective in obtaining good functional results and reliable local tumor control. Material and methodsForty-seven patients treated at the Rizzoli Institute for elbow neoplasm from 1990 to 2012 were evaluated. There were 30 primary tumors (64%), 24 bone tumors and 6 soft tissue sarcomas, and 17 bone metastases. Elbow reconstruction used a modular prosthesis in 25 patients and a standard prosthesis in 22. Reconstruction was primary in 30 patients and secondary in 17. ResultsAt last control, 15 (32%) were dead of disease (DOD) at a mean follow-up of 35 months, 12 (25%) were alive with disease (AWD) at a mean follow-up of 29 months, 19 (40%) showed no evidence of disease (NED) at a mean follow-up of 80 months. Early complications were related to unexpected neurological damage, observed in 12 patients (25%): in 5 cases the deficit resolved in a mean 6 months; in the others, no or only partial recovery was observed. Two implants (4%) developed infection: 1 was treated with antibiotic therapy, and the other required implant revision. One implant showing cement extrusion was revised. In 3 patients (6%) radiography showed a radiolucent halo around the stem (2 humeral, 1 ulnar); no measures were taken, as the patients were completely asymptomatic at every follow-up. In 3 patients (6%) partial resorption of the allograft was observed on X-ray, but remained unchanged at last follow-up, without pain or functional impairment. Seven local recurrences (15%) were observed, at a mean of 16 months after surgery; 5 were treated by resection and/or radiotherapy, and 2 by amputation. Mean functional scores on MEPS and MSTS were respectively 84% and 22/30 (73%). ConclusionsElbow prostheses provided better function in primary than in metastatic tumor. Elbow prosthesis reconstruction after tumor resection is a viable option both for primary and secondary bone neoplasms. Type of studyTherapeutic. Level of evidenceIV, retrospective study.

SU‐E‐J‐258: Prediction of Cervical Cancer Treatment Response Using Radiomics Features Based On F18‐FDG Uptake in PET Images

Purpose:Radiomics have shown potential for predicting treatment outcomes in several body sites. This study investigated the correlation between PET Radiomics features and treatment response of cervical cancer outcomes.Methods:our dataset consisted of a cohort of 79 patients diagnosed with cervical cancer, FIGO stage IB‐IVA, age range 25–86 years, (median age at diagnosis: 50 years) all treated between: 2009–14 with external beam radiation therapy to a dose range between: 45–50.4 Gy (median= 45 Gy), concurrent cisplatin chemotherapy and MRI‐based brachytherapy to a dose of 20–30 Gy (median= 28 Gy). Metabolic Tumor Volume (MTV) in patient's primary site was delineated on pretreatment PET/CT by two board certified Radiation Oncologists. The features extracted from each patient's volume were: 26 Co‐occurrence matrix (COM) Feature, 11 Run‐Length Matrix (RLM), 11 Gray Level Size Zone Matrix (GLSZM) and 33 Intensity‐based features (IBF). The treatment outcome was divided based on the last follow up status into three classes: No Evidence of Disease (NED), Alive with Disease (AWD) and Dead of Disease (DOD). The ability for the radiomics features to differentiate between the 3 treatments outcome categories were assessed by One‐Way ANOVA test with p‐value &lt; 0.05 was to be statistically significant. The results from the analysis were compared with the ones obtained previously for standard Uptake Value (SUV).Results:Based on patients last clinical follow‐up; 52 showed NED, 17 AWD and 10 DOD. Radiomics Features were able to classify the patients based on their treatment response. A parallel analysis was done for SUV measurements for comparison.Conclusion:Radiomics features were able to differentiate between the three different classes of treatment outcomes. However, most of the features were only able to differentiate between NED and DOD class. Also, The ability or radiomics features to differentiate types of response were more significant than SUV.