Lymphatic Leakage after Surgery for Neuroblastoma: A Rare Complication?

Author(s):  
Alexandra Froeba-Pohl ◽  
Jakob Muehling ◽  
Katharina Vill ◽  
Veit Grote ◽  
Tim Komm ◽  
...  

Abstract Introduction Neuroblastoma is the most common extracranial solid tumor in infancy. It is responsible for around 15% of all oncological deaths during childhood. Due to its retroperitoneal location, neuroblastoma is invasively growing directly in and around the lymphatic duct. Consecutively, lymphatic leakage (LL) after surgery for neuroblastoma is a known complication. The purpose of this study is the investigation of frequency and impact of this complication. Material and Methods Between February 2003 and December 2016, 204 patients with neuroblastoma received surgical treatment in our department. A retrospective analysis for macroscopical extent of resection, duration of drainage postsurgery, maximum amount of fluid drained in 24 hours, MYCN amplification status, therapeutic options for LL, follow-up status, and overall survival was performed. Results A total of 40% of patients (82/204) showed LL to some extent. In patients with MYCN amplification, LL was seen significantly more often than in patients without MYCN amplification status (p = 0.019). LL was also significantly correlated with extent of surgery (p = 0.005). Follow-up status and overall survival were significantly inversely associated with LL (p = 0.004 and p = 0.0001). LL was self-limiting in all cases. There was a trend toward shorter duration of LL if either no special therapy was chosen or total parenteral nutrition (TPN) was administered (p = 0.0603). Conclusion We show that LL in neuroblastoma is a common complication of tumor resection and occurring more often than anticipated. Since, in our study cohort, all cases of LL were self-limiting, we question the indication for invasive therapy besides supporting measures.

2021 ◽  
Vol 19 (1) ◽  
Author(s):  
Chun-Kai Liao ◽  
Yueh-Chen Lin ◽  
Yu-Jen Hsu ◽  
Yih-Jong Chern ◽  
Jeng-Fu You ◽  
...  

Abstract Background Although extended colectomy (EC) was recommended for HNPCC patients, previous studies did not show significantly improved overall survival. Immunohistochemical (IHC) stain of mismatch repair (MMR) gene protein expression is now a feasible and reliable test clinically. Therefore, we tried to investigate whether we could use MMR IHC stain to select operation types in HNPCC patients. Patients and methods Between 1995 and 2013, 186 HNPCC patients were collected. Status of MMR protein expression, perioperative clinic-pathological variables and post-operative follow up status were analyzed by multivariate analyses. Results Sixty-five percent (121 of 186) patients of these HNPCC patients demonstrated loss of at least one MMR protein. There were several significant differences existing between deficient MMR (dMMR) and proficient MMR (pMMR) subgroups in terms of clinic-pathological characteristics. With the average follow-up duration of 93.9 months, we observed significantly high risk of developing metachronous CRC between SC and EC subgroups (crude rate 8.5% vs. 0%, p = 0.035). However, no significant difference was observed among the presence of extra-colonic tumors (12.4% vs. 5.8%, p = 0.284). The positive and negative prediction rate of metachronous CRC in dMMR subgroup was 12.8 and 87.2% while 1.9 and 98.1% in the pMMR subgroup. Survival outcomes were significantly affected by MMR status and resection types by multivariate analysis. Significantly better OS in dMMR subgroup (HR = 0.479, 95% CI: 0.257–0.894, p = 0.021) comparing with pMMR subgroup was observed. However, significant improved DFS (HR = 0.367, 95% CI: 0.172–.0787, p = 0.010) but not significant for OS (HR = 0.510, 95% CI: 0.219–1.150, p = 0.103) for EC subgroup compared with SC subgroup. Differences existing among different subgroups by combing extent of resection and MMR status. In dMMR subgroup, SC, compared with EC, demonstrated significantly worse DFS by multivariate analyses (HR = 3.526, 95% CI: 1.346–9.236, p = 0.010) but not for OS (HR = 2.387, 95% CI: 0.788–7.229, p = 0.124), however, no significantly differences of OS and DFS in pMMR subgroup between SC and EC were found. Conclusions Significantly better overall survival and higher rate of metachronous CRC exist in dMMR subgroup of HNPCC patients comparing with pMMR subgroup. Extended colectomy significantly improved DFS and was thus recommended for dMMR subgroup but not pMMR subgroup of HNPCC patients.


Neurosurgery ◽  
2011 ◽  
Vol 70 (5) ◽  
pp. 1081-1094 ◽  
Author(s):  
Miran Skrap ◽  
Massimo Mondani ◽  
Barbara Tomasino ◽  
Luca Weis ◽  
Riccardo Budai ◽  
...  

Abstract BACKGROUND: Despite intraoperative technical improvements, the insula remains a challenging area for surgery because of its critical relationships with vascular and neurophysiological functional structures. OBJECTIVE: To retrospectively investigate the morbidity profile in insular nonenhancing gliomas, with special emphasis on volumetric analysis of tumoral resection. METHODS: From 2000 to 2010, 66 patients underwent surgery. All surgical procedures were conducted under cortical-subcortical stimulation and neurophysiological monitoring. Volumetric scan analysis was applied on T2-weighted magnetic resonance images (MRIs) to establish preoperative and postoperative tumoral volume. RESULTS: The median preoperative tumor volume was 108 cm3. The median extent of resection was 80%. The median follow-up was 4.3 years. An immediate postoperative worsening was detected in 33.4% of cases; a definitive worsening resulted in 6% of cases. Patients with extent of resection of > 90% had an estimated 5-year overall survival rate of 92%, whereas those with extent of resection between 70% and 90% had a 5-year overall survival rate of 82% (P < .001). The difference between preoperative tumoral volumes on T2-weighted MRI and on postcontrast T1-weighted MRI ([T2 − T1] MRI volume) was computed to evaluate the role of the diffusive tumoral growing pattern on overall survival. Patients with preoperative volumetric difference < 30 cm3 demonstrated a 5-year overall survival rate of 92%, whereas those with a difference of > 30 cm3 had a 5-year overall survival rate of 57% (P = .02). CONCLUSION: With intraoperative cortico-subcortical mapping and neurophysiological monitoring, a major resection is possible with an acceptable risk and a significant result in the follow-up.


Author(s):  
Arad Iranmehr ◽  
Mostafa Esmaeilnia ◽  
Khashayar Afshari ◽  
Seyed Mousa Sadrehosseini ◽  
Azin Tabari ◽  
...  

Abstract Background Recently the endoscopic endonasal surgery (EES) has been introduced as a modality for the treatment of patients with craniopharyngiomas. In this study, we describe our initial experience in treatment of 29 patients with craniopharyngiomas using this approach. Methods Twenty-nine consecutive patients with craniopharyngiomas who had undergone EES in a 5-year period were studied retrospectively. Patients underwent preoperative and postoperative endocrinologic and ophthalmologic evaluations. Radiologic characteristics of tumors and extent of resection were determined. The recurrence and complications were evaluated. Results Pituitary and visual dysfunction were observed preoperatively in 89.7 and 86% of patients, respectively. After EES, visual outcome either showed an improvement or else remained unchanged in 92.3% of the cases; however, pituitary function remained unchanged and even got worsened in 34.6% of the cases. Prevalence of diabetes insipidus before and after surgery was 58.6 and 69.2%. The rate of gross total resection was 62%. Moreover, 86.2% of the tumors were almost totally resected (more than 95% of the tumor size resected). After surgery, cerebrospinal fluid (CSF) leak and meningitis occurred in four (13.8%) and two (6.9%) patients, respectively. Perioperative mortality was seen in two of the cases (6.9%). The mean follow-up was 25 months and tumor recurrence was discovered in four patients (15.3%). Conclusion The EES with the goal of maximal and safe tumor resection could be used for the treatment of most craniopharyngiomas. Although the rates of visual improvement and gross tumor resection are high, CSF leak, pituitary dysfunction, and meningitis are serious concerns.


2021 ◽  
Vol 12 ◽  
Author(s):  
Dan Cao ◽  
Yong Chen ◽  
Zhengqian Guo ◽  
Yibo Ou ◽  
Jian Chen

Objective: This study aimed to explore the immediate postoperative and long-term outcomes of central neurocytoma (CN) based on 15 years of experience in our institution.Methods: This single-institution study collected data of 43 patients with CN who underwent surgery between 2005 and 2020. We reviewed data of clinical, immediate postoperative outcome, and long-term outcome of patients. More specifically, we divided complications into neurological and regional complications groups.Results: Among the 43 patients with CN who underwent surgery, the transcortical (72.1%) or transcallosal (25.6%) approach was used. There were 18 patients (41.9%) who complained about postoperative neurological complications, including motor weakness (25.6%), memory deficit (18.6%), aphasia (7.0%), and seizure (4.7%). In addition, 18 patients suffered postoperative regional complications such as hydrocephalus (2.3%), hematoma (34.9%), infection (4.7%), and subcutaneous hydrops (2.3%). Only one-quarter of patients had suffered permanent surgical complications. The majority of patients recovered from the deficit and could turn back to normal life. There were no significant differences in the clinical outcomes between transcortical and transcallosal approaches. At a median follow-up of 61.8 months, the 5-year overall survival and progression-free survival were 87.0 and 74.0%, respectively. A multivariate Cox model analysis showed that the extent of resection was not related to progression-free survival. However, the extent of resection was significantly associated with overall survival, and gross total resection decreased the risk of death.Conclusions: Patients with CN show favorable outcomes after surgery. The transcortical and transcallosal approaches have similar postoperative complication rates and long-term follow-up outcomes. In terms of long-term prognosis, maximal safety resection should be the first choice of CN.


Blood ◽  
2006 ◽  
Vol 108 (11) ◽  
pp. 3626-3626
Author(s):  
Alexandra P. Wolanskyj ◽  
Naseema Gangat ◽  
Susan M. Schwager ◽  
Rhett P. Ketterling ◽  
Ayalew Tefferi

Abstract Objectives: We conducted a study to describe the cytogenetic findings as well as clinical correlates and long-term prognostic relevance of abnormal cytogenetics at the time of diagnosis of Essential thrombocythemia (ET), in terms of clinical presentation, disease transformation into more aggressive myeloid disorders, and life expectancy. Patients and Methods: The study cohort consisted of a consecutive group of patients with ET who fulfilled the World Health Organization (WHO) diagnostic criteria, in whom bone marrow biopsy was performed at diagnosis, with interpretable cytogenetic analysis obtained in all cases. Results: A total of 403 patients were studied (median age, 56 years; median follow-up 64 months). The prevalence of abnormal cytogenetics at presentation was 6.7 % (27 of 403). The most common cytogenetic anomalies identified included trisomy 9 (4 patients), trisomy 8 (3 patients) and deletion 20q (3 patients). Parameters at diagnosis that were significantly associated with abnormal cytogenetics included palpable splenomegaly (p=0.03), current tobacco use (p=0.04); venous thrombosis (p= 0.019), extreme thrombocytosis (i.e., platelet count >1500 × 109/L, p = 0.03) and anemia with a hemoglobin of less than 10 g/dl (p=0.02); but did not include Jak2 mutation status, nor advanced age (≥ 60 years). During follow up, patients with abnormal cytogenetics were more likely to experience venous thrombosis (p=0.02) but not shorter survival [figure 1], transformation to AML, MDS or MMM, nor a greater requirement for cytotoxic therapy. Conclusion: Cytogenetic anomalies at presentation are relatively uncommon in ET, and do not predict a greater predilection towards evolution into more aggressive myeloid disorders, nor inferior survival. Fig. 1 Overall Survival According to Normal vs Abnormal Karyotype Fig. 1. Overall Survival According to Normal vs Abnormal Karyotype


2014 ◽  
Vol 120 (4) ◽  
pp. 846-853 ◽  
Author(s):  
Mark E. Oppenlander ◽  
Andrew B. Wolf ◽  
Laura A. Snyder ◽  
Robert Bina ◽  
Jeffrey R. Wilson ◽  
...  

Object Despite improvements in the medical and surgical management of patients with glioblastoma, tumor recurrence remains inevitable. For recurrent glioblastoma, however, the clinical value of a second resection remains uncertain. Specifically, what proportion of contrast-enhancing recurrent glioblastoma tissue must be removed to improve overall survival and what is the neurological cost of incremental resection beyond this threshold? Methods The authors identified 170 consecutive patients with recurrent supratentorial glioblastomas treated at the Barrow Neurological Institute from 2001 to 2011. All patients previously had a de novo glioblastoma and following their initial resection received standard temozolomide and fractionated radiotherapy. Results The mean clinical follow-up was 22.6 months and no patient was lost to follow-up. At the time of recurrence, the median preoperative tumor volume was 26.1 cm3. Following re-resection, median postoperative tumor volume was 3.1 cm3, equating to an 87.4% extent of resection (EOR). The median overall survival was 19.0 months, with a median progression-free survival following re-resection of 5.2 months. Using Cox proportional hazards analysis, the variables of age, Karnofsky Performance Scale (KPS) score, and EOR were predictive of survival following repeat resection (p = 0.0001). Interestingly, a significant survival advantage was noted with as little as 80% EOR. Recursive partitioning analysis validated these findings and provided additional risk stratification at the highest levels of EOR. Overall, at 7 days after surgery, a deterioration in the NIH stroke scale score by 1 point or more was observed in 39.1% of patients with EOR ≥ 80% as compared with 16.7% for those with EOR < 80% (p = 0.0049). This disparity in neurological morbidity, however, did not endure beyond 30 days postoperatively (p = 0.1279). Conclusions For recurrent glioblastomas, an improvement in overall survival can be attained beyond an 80% EOR. This survival benefit must be balanced against the risk of neurological morbidity, which does increase with more aggressive cytoreduction, but only in the early postoperative period. Interestingly, this putative EOR threshold closely approximates that reported for newly diagnosed glioblastomas, suggesting that for a subset of patients, the survival benefit of microsurgical resection does not diminish despite biological progression.


2019 ◽  
Vol 2019 ◽  
pp. 1-13 ◽  
Author(s):  
Jianping Hu ◽  
Chunlin Zhang ◽  
Kunpeng Zhu ◽  
Lei Zhang ◽  
Tao Cai ◽  
...  

Purpose. The aim of this study was to assess the treatment-related factors associated with local recurrence and overall survival of patients with osteosarcoma treated with limb-salvage surgery. Patients and Methods. Treatment-related factors were analyzed to evaluate their effects on local recurrence-free survival (LRFS) and overall survival (OS) in 182 patients from 2004 to 2013. Results. The mean length of follow-up was 73.4 ± 34.7 months (median, 68 months; range, 12-173 months), and 63 patients died by the end of the follow-up. The 5-year and 10-year overall survival rates were 68.6 ± 6.6% and 59.4 ± 10.6%, respectively. Univariate analysis showed that treatment-related prognostic factors for overall survival were prolonged symptom intervals >=60 days, biopsy/tumor resection performed by different centers, previous medical history, incomplete preoperative chemotherapy (<8 weeks), and prolonged postoperative interval >21 days. In the multivariate analysis, biopsy/tumor resection performed by different centers, incomplete implementation of planned new adjuvant chemotherapy, and delayed resumption of postoperative chemotherapy (>21 days) were risk factors for poor prognosis; biopsy/tumor resection performed by different centers and tumor necrosis <90% were independent predictors of local recurrence. Conclusion. For localized osteosarcoma treated with limb-salvage surgery, it is necessary to optimize timely standard chemotherapy and to resume postoperative chemotherapy to improve survival rates. Biopsies should be performed at experienced institutions in cases of developing local recurrence.


Blood ◽  
2019 ◽  
Vol 134 (Supplement_1) ◽  
pp. 973-973
Author(s):  
Rakesh Dhanya ◽  
Rajat Kumar Agarwal ◽  
Amit Sedai ◽  
Ankita Kumari ◽  
Lalith Parmar ◽  
...  

Introduction: An assessment of morbidity and mortality caused by transfusion dependent thalassemia in India has never really been done despite thalassemia being the most prevalent life threatening non-communicable disorder of childhood. There is little structured understanding identifying the key risk factors feeding into research and policy making for effective management of thalassemia. With an estimated 10,000-12,000 children born with thalassemia each year in India, in addition to increasing focus on early pregnancy targeted screening, it seems critical to increase our understanding of risk factors associated with early mortality and morbidity. This is also relevant to family counselling about management options. Methodology: A retrospective analysis of mortality key risk factors in patients suffering from thalassemia major from 5 thalassemia day care centres in India was carried out. This included a total of 1,087 patients (656 males and 431 females with a median age of 8.6 years) enrolled for care between 1 Jan 2010 - 31 Oct 2018 at these centres. These centres were set up by a non-profit organization in collaboration with blood banks and /hospital facilities with the objective to provide comprehensive thalassemia care; A common web-based application was employed (ThalCare™). This system was used to track information associated with treatment including disease history at enrolment, demographic data and follow-up information. All analyses were performed with R Statistical software (3.5.2). Survival analysis was done from the age at presentation to the centre till October 2018. The reasons for mortality were categorized. Overall survival was also separately analyzed for patients in their 1st,2nd, 3rd or subsequent decades of life. The Cnaan and Ryan approach was used as patients entered and left the study cohort (left censored and right truncated data) and observation began only at enrolment and not at disease onset Results: The median age at enrolment was 5.4 years and the median follow up at the centre was 2.5 years. A total of 86 patients were cured by bone marrow transplantation (BMT), 13 of them moved to other centres for care and 41 patients died during the study period (28 males and 13 females). The median age at death was 15.4 years. Actuarial survival at 26.9 years of age was 50% (Figure 1) and under-five mortality was 7 times higher than the general population. Patients with transfusion-transmitted infections (TTI) had 3.4 times higher risk of death (p=0.031). Serum ferritin &gt;4,000 ng/dL was associated with 4.6 times higher risk of mortality compared to ferritin &lt;1,000 ng/dL (p=0.00063). Hemoglobin drop &gt;2 gm/dL/week had 7.7 times higher mortality risk compared to &lt;1 gm/dL/week (p&lt;0.0001). Social determinants (sex, economic status and distance from centre), splenectomy, age at first transfusion and even cardiac complications were not associated with higher mortality risk. Results are summarized in Table 1. A multivariable analysis of risk factors which emerged as univariately significant showed that Hb drop of &gt; 2 gm/week (hazard ratio 5.58 ,p=0.0007) and lack of attention towards care for possible prevention from TTI (hazard ratio 2.86, p=0.0004) are factors independently associated with high mortality. Table 2 shows that in patients born after the year 2,000 overall survival is 85.2% compared to 29.4% for patients born earlier. Main causes of death were infection, iron overload, TTIs, and alloimmunization; In a quarter of patients the cause of death was unknown (Figure 2). Patients who received more than 4 years of adequate care had more than 66% mortality risk reduction (p&lt;0.0001). Conclusion: Comprehensive care right from an early age at dedicated management centres is key to improving life expectancy of thalassemia patients in India. Optimizing blood transfusion, intensifying chelation and preventing TTIs seem particularly important. Sustained efforts in these areas coupled with increased prevention and access to safe BMT will ease the burden for both families and public healthcare. Disclosures No relevant conflicts of interest to declare.


2017 ◽  
Vol 35 (2) ◽  
pp. 208-216 ◽  
Author(s):  
Daniel von Allmen ◽  
Andrew M. Davidoff ◽  
Wendy B. London ◽  
Collin Van Ryn ◽  
Daphne A. Haas-Kogan ◽  
...  

Purpose This analysis of patients in the Children’s Oncology Group A3973 study evaluated the impact of extent of primary tumor resection on local progression and survival and assessed concordance between clinical and central imaging review–based assessments of resection extent. Patients and Methods The analytic cohort (n = 220) included patients who had both central surgery review and resection of the primary tumor site. For this analysis, resection categories of < 90% and ≥ 90% were used, with data on resection extent derived from operating surgeons’ assessments (all patients), as well as blinded central imaging review of computed tomography scans for a subset of 84 patients; assessment results were compared for concordance. Treatment outcomes included event-free survival (EFS), overall survival (OS), and cumulative incidence of local progression (CILP). Results Surgeon-assessed extent of resection was ≥ 90% in 154 (70%) patients and < 90% in 66 (30%). Five-year EFS, OS, and CILP (± SE) were 43.5% ± 3.7%, 54.9% ± 3.7%, and 11.9% ± 2.2%, respectively. EFS was higher with ≥ 90% resection (45.9% ± 4.3%) than with < 90% resection (37.9% ± 7.2%; P = .04). Lower CILP ( P = .01) was associated with ≥ 90% resection (8.5% ± 2.3%) compared with < 90% resection (19.8% ± 5.0%). On multivariable analysis, ≥ 90% resection was associated with longer EFS after adjustment for MYCN amplification or diploidy but had no significant effect on OS. Concordance between surgeons’ assessments of resection extent and central image–guided review was low, with agreement of 63% (< 90% v ≥ 90%; simple κ = −0.0301). Conclusion Despite discordance between clinical assessment of resection extent and assessment via central imaging review, a surgeon-assessed resection extent ≥ 90% was associated with significantly better EFS and lower CILP. Improving OS, however, remains a challenge in this disease. These findings support continued attempts at ≥ 90% resection of the primary tumor in high-risk neuroblastoma.


2020 ◽  
Vol 11 ◽  
pp. 414
Author(s):  
Chi-Man Yip ◽  
Shu-Shong Hsu ◽  
Wei-Chuan Liao ◽  
Szu-Hao Liu ◽  
Yung-Shang Lin ◽  
...  

Background: Intracranial solitary fibrous tumor/hemangiopericytoma (HPC) is a rare and aggressive tumor. We conducted this retrospective study to investigate the outcome of patients after treatment, the efficacy of postoperative adjuvant radiotherapy, and the factors not conducive to total resection. Methods: We conducted a retrospective review of the medical records of patients harboring fresh intracranial solitary fibrous tumor/HPC treated from January 2009 to December 2019 in our hospital. We reviewed their clinical presentations, radiologic appearances, tumor size and location, extent of resection, estimate intraoperative blood loss, treatment modalities and results, and duration of follow-up. Results: There were seven consecutive patients (three males and four females). The ages of the patients at the time of diagnosis ranged from 35 to 77 years (mean: 52.86 years). Five patients (71.43%) got tumor bigger than 5 cm in dimension and only 1 patient (14.29%) underwent gross total tumor resection in the first operation without complication. Five patients (71.43%) underwent postoperative adjuvant radiotherapy. Follow-up period ranged from 4.24 to 123.55 months and the median follow-up period was 91.36 months. Three patients had favorable outcome with Glasgow Outcome Scale (GOS) equal to 4; four patients had unfavorable outcome with GOS equal to 2 or 3. No mortality was happened. Conclusion: Gross total tumor resection in the initial surgery is very important to achieve a better outcome. Massive intraoperative bleeding and venous sinus or major vessels adjoining are factors not conducive to total resection. Radiotherapy can be administered as adjuvant therapy for cases showing an aggressive phenotype or not treated with gross total resection.


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