Re-evaluating surgery and re-irradiation for locally recurrent pediatric ependymoma – a multi-institutional study

Abstract Background The goal of this study was to evaluate extent of surgical resection, and timing and volume of re-irradiation, on survival for children with locally recurrent ependymoma. Methods Children with locally recurrent ependymoma treated with a second course of fractionated radiotherapy (RT2) from six North American cancer centres were reviewed. The index time was from the start of RT2 unless otherwise stated. Results 35 patients were included in the study. The median doses for first radiation (RT1) and RT2 were 55.8 and 54 Gy, respectively. Median follow-up time was 5.6 years. Median overall survival (OS) for all patients from RT2 was 65 months. Gross total resection (GTR) was performed in 46% and 66% of patients prior to RT1 and RT2, respectively. GTR prior to RT2 was independently associated with improved progression free survival (PFS) for all patients (HR 0.41, p = 0.04), with an OS benefit (HR 0.26, p = 0.03) for infratentorial tumours. Median PFS was superior with craniospinal irradiation (CSI) RT2 (not reached) compared to focal RT2 (56.9 months; log-rank p = 0.03). All distant failures (except one) occurred after focal RT2. Local failures after focal RT2 were predominantly in patients with less than GTR pre-RT2. Conclusions Patients with locally recurrent pediatric ependymoma should be considered for re-treatment with repeat maximal safe resection (ideally GTR) and CSI re-irradiation, with careful discussion of the potential side effects of these treatments.

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Treatment of Angiosarcoma with Pazopanib and Paclitaxel: Results of the EVA (Evaluation of Votrient® in Angiosarcoma) Phase II Trial of the German Interdisciplinary Sarcoma Group (GISG-06)

Cancers ◽

10.3390/cancers13061223 ◽

2021 ◽

Vol 13 (6) ◽

pp. 1223

Author(s):

Daniel Pink ◽

Dimosthenis Andreou ◽

Sebastian Bauer ◽

Thomas Brodowicz ◽

Bernd Kasper ◽

...

Keyword(s):

Immune Checkpoint Inhibitors ◽

Median Overall Survival ◽

Phase Ii Trial ◽

Checkpoint Inhibitors ◽

Progression Free Survival ◽

Free Survival ◽

Safety And Efficacy ◽

Future Studies ◽

Entire Cohort

We aimed to evaluate the efficacy and toxicity of paclitaxel combined with pazopanib in advanced angiosarcoma (AS). The primary end point was progression-free survival (PFS) rate at six months (PFSR6). Planned accrual was 44 patients in order to detect a PFSR6 of >55%, with an interim futility analysis of the first 14 patients. The study did not meet its predetermined interim target of 6/14 patients progression-free at 6 months. At the time of this finding, 26 patients had been enrolled between July 2014 and April 2016, resulting in an overrunning of 12 patients. After a median follow-up of 9.5 (IQR 7.7–15.4) months, PFSR6 amounted to 46%. Two patients had a complete and seven patients a partial response. Patients with superficial AS had a significantly higher PFSR6 (61% vs. 13%, p = 0.0247) and PFS (11.3 vs. 2.7 months, p < 0.0001) compared to patients with visceral AS. The median overall survival in the entire cohort was 21.6 months. A total of 10 drug-related serious adverse effects were reported in 5 patients, including a fatal hepatic failure. Although our study did not meet its primary endpoint, the median PFS of 11.6 months in patients with superficial AS appears to be promising. Taking recent reports into consideration, future studies should evaluate the safety and efficacy of VEGFR and immune checkpoint inhibitors with or without paclitaxel in a randomized, multiarm setting.

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Evolution of Surgical Approaches in the Treatment of Petroclival Meningiomas: A Retrospective Review

Operative Neurosurgery ◽

10.1227/01.neu.0000303218.61230.39 ◽

2007 ◽

Vol 61 (suppl_5) ◽

pp. ONS202-ONS211 ◽

Cited By ~ 36

Author(s):

Nicholas C. Bambakidis ◽

U. Kumar Kakarla ◽

Louis J. Kim ◽

Peter Nakaji ◽

Randall W. Porter ◽

...

Keyword(s):

Survival Rates ◽

Progression Free Survival ◽

Surgical Approaches ◽

Single Institution ◽

Short Term ◽

Total Resection ◽

Free Survival ◽

Petroclival Meningioma ◽

Petroclival Meningiomas

Abstract Objective: We examined the surgical approaches used at a single institution to treat petroclival meningioma and evaluated changes in method utilization over time. Methods: Craniotomies performed to treat petroclival meningioma between September of 1994 and July of 2005 were examined retrospectively. We reviewed 46 patients (mean follow-up, 3.6 yr). Techniques included combined petrosal or transcochlear approaches (15% of patients), retrosigmoid craniotomies with or without some degree of petrosectomy (59% of patients), orbitozygomatic craniotomies (7% of patients), and combined orbitozygomatic-retrosigmoid approaches (19% of patients). In 18 patients, the tumor extended supratentorially. Overall, the rate of gross total resection was 43%. Seven patients demonstrated progression over a mean of 5.9 years. No patients died. At 36 months, the progression-free survival rate for patients treated without petrosal approaches was 96%. Of 14 patients treated with stereotactic radiosurgery, none developed progression. Conclusion: Over the study period, a diminishing proportion of patients with petroclival meningioma were treated using petrosal approaches. Utilization of the orbitozygomatic and retrosigmoid approaches alone or in combination provided a viable alternative to petrosal approaches for treatment of petroclival meningioma. Regardless of approach, progression-free survival rates were excellent over short-term follow-up period.

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Clinical Presentation, Natural History, and Therapeutic Approach in Patients with Solitary Fibrous Tumor: A Retrospective Analysis

Sarcoma ◽

10.1155/2020/1385978 ◽

2020 ◽

Vol 2020 ◽

pp. 1-9 ◽

Cited By ~ 2

Author(s):

P. Schöffski ◽

I. Timmermans ◽

D. Hompes ◽

M. Stas ◽

F. Sinnaeve ◽

...

Keyword(s):

Solitary Fibrous Tumor ◽

Systemic Therapy ◽

Median Overall Survival ◽

Progression Free Survival ◽

Response Rates ◽

Free Survival ◽

Metachronous Metastasis ◽

Third Line ◽

Fibrous Tumor

Background. Solitary fibrous tumor (SFT) is a rare variant of soft tissue sarcoma (STS). Materials and Methods. We reviewed SFT patients (pts) treated at our institution between 12/1990 and 09/2017. Results. We identified 94 pts with a median follow-up (mFU) of 4.7 years (range: 0.1–21.53). Primary sites were the chest (33%), abdomen (21.3%), brain (12.8%), and extremities (9.6%); 6.4% of pts presented with synchronous metastasis. Median overall survival (mOS) from the first diagnosis was 56.0 months (m) (0.3–258.3). Doege–Potter syndrome was seen in 2.1% of pts. Primary resection was performed in 86 pts (91.5%). Median progression-free survival was 34.1 m (1.0–157.1), and 43% of pts stayed SFT-free during FU. Local recurrence occurred in 26.7% after a mFU of 35.5 m (1.0–153.8), associated with an OS of 45.1 m (4.7–118.2). Metachronous metastasis occurred in 30.2% after a mFU of 36.0 m (0.1–157.1). OS in metastatic pts was 19.0 m (0.3–149.0). Systemic therapy was given to 26 pts (27.7%) with inoperable/metastatic disease. The most common (57.7%) upfront therapy was doxorubicin, achieving responses in 13.3% of pts with a PFS of 4.8 m (0.4–23.8). In second line, pts were treated with ifosfamide or pazopanib, the latter achieving the highest response rates. Third-line treatment was heterogeneous. Conclusion. SFT is an orphan malignancy with a highly variable clinical course and a considerable risk of local failure and metachronous metastasis. Surgery is the only curative option; palliative systemic therapy is used in inoperable/metastatic cases but achieves low response rates. The highest response rates are seen with pazopanib in second/third line.

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Conversion Chemotherapy With a Modified FLOX Regimen for Borderline or Unresectable Liver Metastases From Colorectal Cancer: An Alternative for Limited-Resources Settings

Journal of Global Oncology ◽

10.1200/jgo.19.00180 ◽

2019 ◽

pp. 1-6

Author(s):

Renata Colombo Bonadio ◽

Paulo Henrique Amor Divino ◽

Jorge Santiago Madero Obando ◽

Karolina Cayres Alvino Lima ◽

Débora Zachello Recchimuzzi ◽

...

Keyword(s):

Colorectal Cancer ◽

Liver Metastases ◽

Median Overall Survival ◽

Low Cost ◽

Progression Free Survival ◽

Hazard Ratio ◽

R0 Resection ◽

Free Survival ◽

Unresectable Liver Metastases

PURPOSE Conversion chemotherapy is often used for borderline or unresectable (B/U) liver metastases from colorectal cancer (CRC) with the aim of achieving resectability. Although intensive and costly regimens are often used, the best regimen in this scenario remains unclear. We aimed to evaluate the outcomes of patients with B/U liver metastases from CRC treated with conversion chemotherapy with the modified fluorouracil, leucovorin, and oxaliplatin (mFLOX) regimen followed by metastasectomy. METHODS We performed a single-center retrospective analysis of patients with B/U liver metastases from CRC treated with chemotherapy with the mFLOX regimen followed by surgery. B/U disease was defined as at least one of the following: more than four lesions, involvement of hepatic artery or portal vein, or involvement of biliary structure. RESULTS Fifty-four consecutive patients who met our criteria for B/U liver metastases were evaluated. Thirty-five patients (64%) had more than four liver lesions, 16 (29%) had key vascular structure involvement, and 16 (29%) had biliary involvement. After chemotherapy, all patients had surgery and 42 (77%) had R0 resection. After a median follow-up of 37.2 months, median progression-free survival (PFS) was 16.9 months and median overall survival (OS) was 68.3 months. R1-R2 resections were associated with worse PFS and OS compared with R0 resection (PFS: hazard ratio, 2.65; P = .007; OS: hazard ratio, 2.90; P = .014). CONCLUSION Treatment of B/U liver metastases from CRC with conversion chemotherapy using mFLOX regimen followed by surgical resection was associated with a high R0 resection rate and favorable survival outcomes. On the basis of our results, we consider mFLOX a low-cost option for conversion chemotherapy among other options that have been proposed.

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An update on BREAK-3, a phase III, randomized trial: Dabrafenib (DAB) versus dacarbazine (DTIC) in patients with BRAF V600E-positive mutation metastatic melanoma (MM).

Journal of Clinical Oncology ◽

10.1200/jco.2013.31.15_suppl.9013 ◽

2013 ◽

Vol 31 (15_suppl) ◽

pp. 9013-9013 ◽

Cited By ~ 45

Author(s):

Axel Hauschild ◽

Jean Jacques Grob ◽

Lev V. Demidov ◽

Thomas Jouary ◽

Ralf Gutzmer ◽

...

Keyword(s):

Median Overall Survival ◽

Braf Inhibitor ◽

Progression Free Survival ◽

Braf V600e ◽

Phase Iii ◽

Primary Analysis ◽

Free Survival ◽

Independent Review ◽

Clinical Trial Information

9013 Background: Dabrafenib is a selective BRAF inhibitor with demonstrated efficacy in BRAF V600E-positive mutation in MM. The primary analysis of BREAK-3 (NCT01227889) compared progression-free survival (PFS) in patients (pts) with BRAF V600E-positive mutation MM treated with dabrafenib or DTIC. Methods: Median PFS for dabrafenib of 5.1 months (mo) and study methods were previously described (Hauschild A, et al. Lancet. 2012,380:358–365). Independent review ended at the primary analysis. PFS was updated in Jun 2012 at median follow-up of 10.5 mo for dabrafenib (67% of PFS events), and 9.9 mo for DTIC. Median overall survival (OS) was not reached, so another analysis of OS and safety was performed with data as of Dec 2012, at which time the median follow-up was 15.2 (dabrafenib) and 12.7 (DTIC) mo. PFS of subjects who crossed over was also evaluated at that time. Results: PFS hazard ratio was 0.37 [95% CI; 0.23, 0.57]; median PFS was 6.9 mo dabrafenib and 2.7 mo DTIC. In Dec 2012, 36/63 DTIC pts crossed over; median PFS was 4.3 [95% CI; 4.1, 6.1] mos. OS is presented in the Table.The four most common adverse events (AE) on the dabrafenib arm were hyperkeratosis (39%), headache (35%), arthralgia (35%), and pyrexia (32%). Serious AEs ≥ 5% on the dabrafenib arm included cutaneous squamous cell carcinoma/keratoacanthoma (10%) and pyrexia (5%). Conclusions: Longer follow-up confirms the benefits of dabrafenib on PFS and response rate. Median OS in the dabrafenib arm was over 18 mo and over 15 mo in the DTIC arm. OS results are confounded by crossover of DTIC pts to dabrafenib and likely by subsequent therapy after progression. The effects of subsequent therapy results will be investigated. The safety profile had no significant changes. Clinical trial information: NCT01227889. [Table: see text]

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Trecc: Re-challenge therapy with anti-EGFR in metastatic colorectal adenocarcinoma (mCRC).

Journal of Clinical Oncology ◽

10.1200/jco.2019.37.4_suppl.683 ◽

2019 ◽

Vol 37 (4_suppl) ◽

pp. 683-683

Author(s):

Amanda Karani ◽

Tiago Felismino ◽

Lara Azevedo Diniz ◽

Mariana Petaccia Macedo ◽

Larissa Machado ◽

...

Keyword(s):

Prognostic Factors ◽

Median Overall Survival ◽

Colorectal Adenocarcinoma ◽

Univariate Analysis ◽

Objective Response ◽

Progression Free Survival ◽

Resistance Mechanisms ◽

Free Survival ◽

Free Interval

683 Background: Anti-EGFR plus chemotherapy (CT) promotes high response rates (RR) and median overall survival (OS) surpasses 30 months in RASwt/BRAFwt mCRC. After disease progression (PD), resistance mechanisms have been described. The aim of our study was to evaluate efficacy of anti-EGFR re-challenge (TRECC). Methods: We retrospectively analyzed a cohort of patients (pts) with mCRC. All pts had received anti-EGFR plus CT and were discontinued for different reasons. During the treatment, there was re-challenge with an anti-EGFR + CT. We aimed to evaluate progression-free survival (PFS) and OS after re-challenge and prognostic factors associated with PFS. Results: Sixty eight pts met the study criteria. Median follow-up after re-challenge was 39.3m. Discontinuation after first exposure was 25% due to PD; 75% for other reasons. Median anti-EGFR free interval was 10.5m. At re-challenge, main CT regime was: FOLFIRI 58.8%, Cetuximab and Panitumumab were used in 59 and 9 pts respectively. mPFS after re-challenge was 6.6m; mOS was 24.4m. Objective response rate (CR + PR) at re-challenge was 42.6%. In an univariate analysis, adverse prognostic factors related to PFS were: absence of objective response at 1st EGFR exposure (HR 2.12, CI:1.20-3.74 p = 0.009); PD as reason for 1st discontinuation (HR 3.44, CI:1.88-6.29 p < 0.0001); re-challenge at fourth or later lines (HR 2,51, CI:1.49-4.23 p = 0.001); panitumumab use (HR 2.26 CI:1.18-5.54 p = 0.017). In a multivariate model, only PD as reason for 1st discontinuation remained statistically significant (HR = 2.63, CI:1.14-6.03 p = 0.022). mPFS was 3.3m and 8.4m and mOS was 7,5m and 33,4m in patients with PD as reason for 1st discontinuation and other reasons respectively. Conclusions: Re-challenge therapy is commonly used due to paucity of effective lines of treament for mCRC. In our analysis, pts that stopped 1st anti-EGFR therapy due to PD have shorter survival, suggesting these pts do not benefit from TRECC. However, interruption due to treatment holiday after PR/CR resulted in longer PFS. In conclusion, for a selected group of pts, TRECC could be considered a strategy of treatment. Due to the limited number of pts, our data should be evaluated in a prospective cohort of patients.

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Clinical Effectiveness and Potential Survival Benefit of US-Guided High-Intensity Focused Ultrasound Therapy in Patients with Advanced-Stage Pancreatic Cancer

Ultraschall in der Medizin - European Journal of Ultrasound ◽

10.1055/a-0591-3386 ◽

2018 ◽

Vol 40 (05) ◽

pp. 625-637 ◽

Cited By ~ 8

Author(s):

Milka Marinova ◽

Hannah C. Huxold ◽

Jana Henseler ◽

Martin Mücke ◽

Rupert Conrad ◽

...

Keyword(s):

Pancreatic Cancer ◽

Survival Benefit ◽

Median Overall Survival ◽

Focused Ultrasound ◽

Tumor Volume ◽

Progression Free Survival ◽

Clinical Effectiveness ◽

High Intensity Focused Ultrasound ◽

Free Survival

Abstract Purpose Pancreatic cancer (PaC) is a life-limiting tumor with a wide range of incapacitating symptoms such as cancer pain in more than 80 % of patients. This prospective interventional study addresses the clinical effectiveness of ultrasound-guided high-intensity focused ultrasound (HIFU) treatment for patients with advanced-stage PaC, including pain perception, tumor size and survival benefit. Materials and Methods 50 patients with late-stage PaC underwent HIFU. Clinical assessment included evaluation of tumor volume by imaging and pain burden (pain severity, pain sensation, interference with daily activities) using the Brief Pain Inventory at baseline and follow-up. Median overall survival, progression-free survival and time to local progression were estimated using Kaplan-Meier analysis. Results In 84 % of patients, significant early relief of cancer-induced abdominal pain was achieved by HIFU independent of metastatic status; it persisted during follow-up. Tumor volume reduction was 37.8 ± 18.1 % after 6 weeks and 57.9 ± 25.9 % after 6 months. 21 % of HIFU-treated patients had local tumor progression with a median time of 14.4 months from intervention. The median overall survival and progression-free survival were 16.2 and 16.9 months from diagnosis and 8.3 and 6.8 months from intervention. Conclusion In patients with advanced pancreatic cancer and otherwise limited treatment options, HIFU resulted in significant early and long-lasting pain relief and tumor size reduction over time independent of metastatic status. Clinical data suggest an additional potential survival benefit.

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Very High Efficiency of ICE (Ifosfamide-Carboplatin-Etoposide) in Relapse/Refractory (R/R) Primary Central Nervous System (PCNSL) and Vitreo-Retinal (VRL) Non Hodgkin Lymphoma. a LOC Network Multicenter Retrospective Study on 58 Cases

Blood ◽

10.1182/blood.v126.23.1524.1524 ◽

2015 ◽

Vol 126 (23) ◽

pp. 1524-1524 ◽

Cited By ~ 2

Author(s):

Sylvain Choquet ◽

Adrien Grenier ◽

Caroline Houillier ◽

Carole Soussain ◽

Marie Pierre Moles ◽

...

Keyword(s):

Median Overall Survival ◽

High Efficiency ◽

Progression Free Survival ◽

High Dose ◽

Free Survival ◽

Non Hodgkin Lymphoma ◽

Grade 3 ◽

Very High ◽

Made In

Abstract Background: R/R PCNSL and VRL carry a very poor prognosis (i.e. 4,5 months, Jahnke, J neuro-oncol 2006)). Autologous stem-cell transplantation (ASCT) is effective in this setting but essentially if the disease is in good response (CR and PR). Since recent recommendations for first line treatment recommend high dose Mtx and AraC, new salvage chemotherapies must use other drugs. ICE regimen contain three drugs known to pass the blood-brain barrier and is validated in R/R systemic lymphoma but not in PCNSL/VRL. Methods: From June 2010 to May 2015, all relapse/refractory PCNSL and VRL already treated by Mtx and AraC in 5 departments of haematology or neuro-oncology in France, where treated by ICE: ifosfamide (5g/m² at day 2), carboplatine (AUC 5 at day 2) and etoposide (100mg/m²/d days 1 to 3) every 3 to 4 weeks. ASCT with thiotepa-busulfan-cyclophosphamide was proposed when possible. Results: Fifty-eight patients have been treated, 25 females and 33 males, median age was 64 [28-84]. Eight received rituximab at day 1 of each cycle. Twelve where refractory, 46 in relapse, with a mean progression free survival (PFS) of 159 days [0-1763] before ICE. Thirty patients (51%) were in second line, 12 (21%) in third, 4 in fourth. Localizations where CNS in 32 cases, CNS + spinal fluid (SF) in 11, CNS + VR in 2, VRL in 9, SF in 3 and CNS+VR+SF in 1. Patients received a median of 3 cycles and 25 needed a dose reduction. Grade 3/4 WHO toxicities where: 29% neutropenic fever, 50% anemia, 69% neutropenia, 78% thrombocytopenia and 3 neurological reversible complications. ASCT have been made in 19 patients (33%): 13 in CR, 4 in PR, 2 in progression. Response rate is 70% (48% CR and 22% PR). With a mean follow-up of 670 days, 25/41 patients in response relapsed (only 7/19 after ASCT), median PFS is 133 days (NR for ASCT), 35 patients died (30 by progression, 4 by sepsis and one by sudden heart arrest in PR). Median Overall survival (OS) was 238 days for all patients but was not reached in case of ASCT, mean follow up for surviving ASCT is 856 days [278-1628]). Conclusion: ICE regimen is very effective in relapse/refractory PCNSL and LVR heavily treated by high dose Mtx and AraC, with a manageable toxicity. If ASCT is performed, OS is very high and relapses rare. ICE + ASCT can represent a new standard in this setting. Disclosures Leblond: Roche: Consultancy, Honoraria, Other: Travel, Accommodations, Expenses, Speakers Bureau; Janssen: Consultancy, Honoraria, Speakers Bureau; GSK: Consultancy, Honoraria, Speakers Bureau; Gilead: Consultancy, Honoraria, Speakers Bureau; Mundipharma: Honoraria.

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Alternating versus continuous “FOLFIRI” in advanced colorectal cancer (ACC): A randomized “GISCAD” trial

Journal of Clinical Oncology ◽

10.1200/jco.2006.24.18_suppl.3505 ◽

2006 ◽

Vol 24 (18_suppl) ◽

pp. 3505-3505 ◽

Cited By ~ 24

Author(s):

R. Labianca ◽

I. Floriani ◽

E. Cortesi ◽

L. Isa ◽

A. Zaniboni ◽

...

Keyword(s):

Overall Survival ◽

Median Overall Survival ◽

Experimental Studies ◽

Progression Free Survival ◽

Continuous Treatment ◽

Who Grade ◽

Free Survival ◽

Alternating Chemotherapy ◽

Grade 3

3505 Background: In ACC “FOLFIRI” is one of the standard regimens and is able to obtain about 40% response rate (RR) with an overall survival (OS) of 17–18 months. Experimental studies (Sobrero, 2000) indicate that an alternating chemotherapy could delay the appearance of cell resistance and reduce the therapeutic load for patients (pts). Methods: In order to evaluate whether intermittent “FOLFIRI” (CPT-11: 180 mg/sqm d1 + l-folinic acid -FA: 100 mg/sqm in 2 hr + 5fluorouracil-5FU: 400 mg/sqm bolus + 600 mg/sqm 22 hr infusion, d 1 and 2 every 2 weeks, for 2 months on and 2 months off) (arm A) was at least as effective as continuous “FOLFIRI” (same regimen, every month) (arm B), until progression in both arms, 336 pts from 27 Centers were randomised from 7/2001 to 6/2005. The characteristics of pts were: median age 64 years (r 29–75), males 214 (63%), PS 0: 222 (66%), liver mets only 166 (49%), multiple mts including liver 80 (24%). Results: RR was 29% in arm A and 35% in arm B, with a median progression-free survival (PFS) of 8.8 and 7.3 months respectively (HR = 1.00, 95% CI: 0.74 - 1.36). At a median follow-up of 27 months, median overall survival (OS), the primary endpoint of the trial, was 16.9 months in arm A and 17.6 in arm B (HR = 1.11, 95% CI: 0.83 - 1.48). Toxicity was acceptable and similar in the 2 arms (WHO grade 3–4 toxicity: neutropenia in 12% pts, diarrhoea in 11%, nausea/vomiting in 4% and fatigue in 3%). Conclusions: Our results demonstrate that alternating “FOLFIRI” obtains the same survival as a continuous treatment, thus reducing the discomfort to pts and the economic costs. No significant financial relationships to disclose.

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The Long-term Outcome After Resection of Intraspinal Nerve Sheath Tumors

Neurosurgery ◽

10.1227/neu.0000000000000890 ◽

2015 ◽

Vol 77 (4) ◽

pp. 585-593 ◽

Cited By ~ 10

Author(s):

Charlotte Marie Halvorsen ◽

Pål Rønning ◽

John Hald ◽

Tom Børge Johannesen ◽

Frode Kolstad ◽

...

Keyword(s):

Clinical Outcome ◽

Progression Free Survival ◽

Gross Total Resection ◽

Subtotal Resection ◽

Total Resection ◽

Free Survival ◽

Nerve Sheath Tumors ◽

Nerve Sheath

Abstract BACKGROUND: The existing literature on recurrence rates and long-term clinical outcome after resection of intraspinal nerve sheath tumors is limited. OBJECTIVE: To evaluate progression-free survival, overall survival, and long-term clinical outcome in a consecutive series of 131 patients with symptomatic intraspinal nerve sheath tumors. METHODS: Medical charts were retrospectively reviewed. Surviving patients voluntarily participated in a clinical history and physical examination that focused on neurological function and current tumor status. RESULTS: Follow-up data are 100% complete; median follow-up time was 6.1 years. All patients (100%) had surgery as the first line of treatment; gross total resection was performed in 112 patients (85.5%) and subtotal resection in 19 patients (14.5%). Five-year progression-free survival was 89%. The following risk factors for recurrence were identified: neurofibroma, malignant peripheral nerve sheath tumor, subtotal resection, neurofibromatoses/schwannomatosis, and advancing age at diagnosis. More than 95% of patients had neurological function compatible with an independent life at follow-up. The rate of tumor recurrence in nonneurofibromatosis patients undergoing total resection of a single schwannoma was 3% (3/93), in comparison with a recurrence rate of 32% (12/38) in the remaining patients. CONCLUSION: Gross total resection is the gold standard treatment for patients with intraspinal nerve sheath tumors. In a time of limited health care resources, we recommend that follow-up be focused on the subgroup of patients with a high risk of recurrence. The benefit of long-term, yearly magnetic resonance imaging follow-up with respect to recurrence in nonneurofibromatosis patients undergoing gross total resection of a single schwannoma is, in our opinion, questionable.

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