scholarly journals Fascicle Sparing Capsular Resections of Atypical Neurofibromas in Neurofibromatosis 1

Neurosurgery ◽  
2019 ◽  
Vol 66 (Supplement_1) ◽  
Author(s):  
Charlie Nelson ◽  
Eva Dombi ◽  
Jared S Rosenblum ◽  
Markku Miettenen ◽  
Tanya Lehky ◽  
...  

Abstract INTRODUCTION Neurofibromatosis type 1 (NF1) patients are predisposed to neurofibromas (NF), which can progress to premalignant atypical neurofibromas (ANF) and malignant peripheral nerve sheath tumors (MPNST). Subtotal resection of ANF may prevent metastases and deaths, but local recurrences require reoperation. Here, we assess the surgical morbidity associated with gross total, extracapsular resection of targeted ANF nodules identified via serial volumetric magnetic resonance imaging (MRI) and 18F-FDG-PET imaging. METHODS We retrospectively analyzed the clinical outcomes of 11 NF1 patients following 16 NF surgical resections of 21 tumors at the NIH Clinical Center between 2008 and 2018. Preoperative volumetric growth rates and 18F-FDG-PET SUVMAX of the target lesions were measured and assessed in tandem with postoperative complications, histopathological classification of the resected tumors, and surgical margins through Dunnett's multiple comparison tests and t-tests. RESULTS Preoperatively, 13 of the 14 (93%) sets of serial preoperative MRI scans showed rapid growth ( = 20% increase in volume per year), and 10 of the 11 (91%) 18F-FDG-PET scans indicated increased positron emission tomography (PET) avidity (median SUVMax = 6.45). Gross total, extracapsular resections of the targeted neurofibroma nodules were annotated by the surgeon in all 16 (100%) surgeries, and most surgeries (n = 14, 88%) resulted in no persistent postoperative parent nerve-related complications. SUVMax was significantly greater in the ANF (6.51 ± 0.83, P = .0042) and low grade MPNST (13.8, P = .0001) strata than in the benign NF (1.9) stratum. To date, none of the resected NF have recurred. CONCLUSION This study confirms that the combination of increased 18F-FDG-PET SUVMax, rapid growth, and pain can serve as reliable indicators of atypical transformation and the need for surgical intervention. We also demonstrate the ability to achieve safe, fascicle-sparing gross total, extracapsular resection of ANF using intraoperative nerve stimulation, histological verification, and continued monitoring for tumor recurrence.

2020 ◽  
Vol 133 (5) ◽  
pp. 1516-1526 ◽  
Author(s):  
Charlie N. Nelson ◽  
Eva Dombi ◽  
Jared S. Rosenblum ◽  
Markku M. Miettinen ◽  
Tanya J. Lehky ◽  
...  

OBJECTIVEPatients with neurofibromatosis type 1 (NF1) are predisposed to visceral neurofibromas, some of which can progress to premalignant atypical neurofibromas (ANFs) and malignant peripheral nerve sheath tumors (MPNSTs). Though subtotal resection of ANF may prevent malignant transformation and thus deaths with no neural complications, local recurrences require reoperation. The aim of this study was to assess the surgical morbidity associated with marginal resection of targeted ANF nodules identified via preoperative serial volumetric MRI and 18F-FDG-PET imaging.METHODSThe authors analyzed clinical outcomes of 16 NF resections of 21 tumors in 11 NF1 patients treated at the NIH Clinical Center between 2008 and 2018. Preoperative volumetric growth rates and 18F-FDG-PET SUVMax (maximum standardized uptake value within the tumor) of the target lesions and any electromyographic or nerve conduction velocity abnormalities of the parent nerves were measured and assessed in tandem with postoperative complications, histopathological classification of the resected tumors, and surgical margins through Dunnett’s multiple comparisons test and t-test. The surgical approach for safe marginal resection of ANF was also described.RESULTSEleven consecutive NF1 patients (4 male, 7 female; median age 18.5 years) underwent 16 surgical procedures for marginal resections of 21 tumors. Preoperatively, 13 of the 14 (93%) sets of serial MRI studies and 10 of the 11 (91%) 18F-FDG-PET scans showed rapid growth (≥ 20% increase in volume per year) and avidity (SUVMax ≥ 3.5) of the identified tumor, respectively (median tumor size 48.7 cm3; median growth rate 92% per year; median SUVMax 6.45). Most surgeries (n = 14, 88%) resulted in no persistent postoperative parent nerve–related complications, and to date, none of the resected tumors have recurred. The median length of postoperative follow-up has been 2.45 years (range 0.00–10.39 years). Histopathological analysis confirmed significantly greater SUVMax among the ANFs (6.51 ± 0.83, p = 0.0042) and low-grade MPNSTs (13.8, p = 0.0001) than in benign neurofibromas (1.9).CONCLUSIONSThis report evaluates the utility of serial imaging (MRI and 18F-FDG-PET SUVMax) to successfully detect ANF and demonstrates that safe, fascicle-sparing gross-total, extracapsular resection of ANF is possible with the use of intraoperative nerve stimulation and microdissection of nerve fascicles.


2020 ◽  
Vol 25 (1) ◽  
pp. 30-36
Author(s):  
Soliman Oushy ◽  
Avital Perry ◽  
Christopher S. Graffeo ◽  
Aditya Raghunathan ◽  
Lucas P. Carlstrom ◽  
...  

OBJECTIVEGanglioglioma is a low-grade central nervous system neoplasm with a pediatric predominance, accounting for 10% of all brain tumors in children. Gangliogliomas of the cervicomedullary junction (GGCMJs) and brainstem (GGBSs) present a host of management challenges, including a significant risk of surgical morbidity. At present, understanding of the prognostic factors—including BRAF V600E status—is incomplete. Here, the authors report a single-institution GGCMJ and GGBS experience and review the pertinent literature.METHODSA prospectively maintained neurosurgical database at a large tertiary care academic referral center was retrospectively queried for cases of GGCMJ pathologically confirmed in the period from 1995 to 2015; appropriate cases were defined by diagnosis codes and keywords. Secondary supplemental chart review was conducted to confirm or capture relevant data. The primary study outcome was treatment failure as defined by evidence of radiographic recurrence or progression and/or clinical or functional decline. A review of the literature was conducted as well.RESULTSFive neurosurgically managed GGBS patients were identified, and the neoplasms in 4 were classified as GGCMJ. All 5 patients were younger than 18 years old (median 15 years, range 4–16 years) and 3 (60%) were female. One patient underwent gross-total resection, 2 underwent aggressive subtotal resection (STR), and 2 underwent stereotactic biopsy only. All patients who had undergone STR or biopsy required repeat resection for tumor control or progression. Progressive disease was treated with radiotherapy in 2 patients, chemotherapy in 2, and chemoradiotherapy alone in 1. Immunostaining for BRAF V600E was positive in 3 patients (60%). All 5 patients experienced at least one major complication, including wound infection, foot drop, hemiparesis, quadriparesis, cranial neuropathy, C2–3 subluxation, syringomyelia, hydrocephalus, aspiration, and coma. Overall mortality was 20%, with 1 death observed over 11 years of follow-up.CONCLUSIONSGGBS and GGCMJ are rare, benign posterior fossa tumors that carry significant perioperative morbidity. Contemporary management strategies are heterogeneous and include combinations of resection, radiotherapy, and chemotherapy. The BRAF V600E mutation is frequently observed in GGBS and GGCMJ and appears to have both prognostic and therapeutic significance with targeted biological agents.


2016 ◽  
Vol 45 (8) ◽  
pp. 1097-1105 ◽  
Author(s):  
Stephen M. Broski ◽  
Geoffrey B. Johnson ◽  
Benjamin M. Howe ◽  
Mark A. Nathan ◽  
Doris E. Wenger ◽  
...  

2020 ◽  
Author(s):  
P Melcher ◽  
J Henkelmann ◽  
S Purz ◽  
R Henkelmann ◽  
P Hepp ◽  
...  
Keyword(s):  
Fdg Pet ◽  

VASA ◽  
2011 ◽  
Vol 40 (5) ◽  
pp. 418-421 ◽  
Author(s):  
Goudard ◽  
Pierret ◽  
Dusaud ◽  
Falzone ◽  
Tourtier ◽  
...  

Persistent blood flow in aneurysmal sac after bypass-exclusion is well documented in the literature. Aneurysm enlargement, local compressive symptoms and even sac rupture are commonly described complications. Late secondary infection of popliteal artery aneurysm (PAA) following ligation and venous bypass is exceptional. We report the case of late PAA infection six years after bypass-exclusion in a 75 year-old man which was diagnosed by 18F-FDG PET/CT. The patient was successfully treated by aneurysm resection and antibiotics. The diagnosis of popliteal aneurysm infection is often clinical, echographic and sonographic, but computed tomography scan can be false negative in chronic low-grade infection. 18F-FDG PET/CT is able to accurately diagnose and localize infection with high sensibility and specificity.


2008 ◽  
Vol 63 (3) ◽  
pp. 555-559 ◽  
Author(s):  
Mehmet Emin Kalender ◽  
Alper Sevinc ◽  
Mustafa Yilmaz ◽  
Coskun Ozsarac ◽  
Celalettin Camci

2013 ◽  
Vol 38 (7) ◽  
pp. 495-500 ◽  
Author(s):  
Koen Mertens ◽  
Marjan Acou ◽  
Jel Van Hauwe ◽  
Ine De Ruyck ◽  
Caroline Van den Broecke ◽  
...  

2021 ◽  
Vol 10 (2) ◽  
pp. 361
Author(s):  
Filipe Montes de Jesus ◽  
Vibeke Vergote ◽  
Walter Noordzij ◽  
Daan Dierickx ◽  
Rudi Dierckx ◽  
...  

Background: Post-transplant lymphoproliferative disorder (PTLD) is a complication of organ transplantation classified according to the WHO as nondestructive, polymorphic, monomorphic, and classic Hodgkin Lymphoma subtypes. In this retrospective study, we investigated the potential of semi-quantitative 2-[18F]fluoro-2-deoxy-D-glucose ([18F]FDG) PET/computed tomography (CT)-based parameters to differentiate between the PTLD morphological subtypes. Methods: 96 patients with histopathologically confirmed PTLD and baseline [18F]FDG PET/CT between 2009 and 2019 were included. Extracted semi-quantitative measurements included: Maximum, peak, and mean standardized uptake value (SUVmax, SUVpeak, and SUVmean). Results: Median SUVs were highest for monomorphic PTLD followed by polymorphic and nondestructive subtypes. The median SUVpeak at the biopsy site was significantly higher in monomorphic PTLD (17.8, interquartile range (IQR):16) than in polymorphic subtypes (9.8, IQR:13.4) and nondestructive (4.1, IQR:6.1) (p = 0.04 and p ≤ 0.01, respectively). An SUVpeak ≥ 24.8 was always indicative of a monomorphic PTLD in our dataset. Nevertheless, there was a considerable overlap in SUV across the different morphologies. Conclusion: The median SUVpeak at the biopsy site was significantly higher in monomorphic PTLD than polymorphic and nondestructive subtypes. However, due to significant SUV overlap across the different subtypes, these values may only serve as an indication of PTLD morphology, and SUV-based parameters cannot replace histopathological classification.


2020 ◽  
Author(s):  
Antoni Mestre-Fusco ◽  
Mònica Velasco-Nuño ◽  
Montserrat Negre-Busó ◽  
Núria Ferran ◽  
Sergi Juanpere ◽  
...  

Abstract A 66-year-old male patient, with chronic respiratory disease and right colon resection in March 2017 for colon low-grade adenocarcinoma was assessed for recurrence suggested by elevated levels of tumor marker and no evidence of oncological disease by CT scan. 18F-FDG PET/CT showed bilateral multiple peripheral FDG-avid foci that matched with a peripheral predominant ground-glass opacities (GGOs) observed in lower lobes and multiple FDG-positive enlarged lymph nodes were also identified in the mediastinum. Patient was hospitalized in March 14th 2020, one day after PET/CT scan, with cough, wheezing and fever, and was treated with anti-inflammatory drugs. A first SARS-CoV-2 RT-PCR in March 15th resulted as negative and patient was treated with antibiotic therapy lead to an improvement of respiratory symptoms. PET/CT scan was interpreted as a pneumonia foci. A deteriorating patient condition was observed, with respiratory symptomatology progression, fatigue and D-Dimer elevation and a new RT-PCR resulted positive. A week after PET/CT scan, hospitalization in intensive care unit was necessary for rapidly disease progression and severe respiratory distress syndrome and patient died four days later.


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