title: Diagnostic Accuracy of PET/CT-Guided Percutaneous Biopsies for Malignant Peripheral Nerve Sheath Tumors in Neurofibromatosis Type 1 Patients.
abstract: BACKGROUND
  Malignant peripheral nerve sheath tumors (MPNST) are one of the most frequent causes of death in patients with neurofibromatosis type 1 (NF1). Early detection is crucial because complete surgical resection is the only curative treatment. It has been previously reported that an 18F-fluorodeoxyglucose (FDG) positron emission tomography/computed tomography (PET/CT) image with a T/L (Tumor/Liver) SUV max ratio > 1.5 provides a high negative predictive value; however, it is not specific enough to make a NF1-related MPNST diagnosis. A formal proof of malignant transformation from a histological analysis is necessary before surgical excision because the procedure can cause mutilation. The objective of the present work was to investigate the effectiveness of and complications associated with PET/CT-guided percutaneous biopsies for an NF1-related MPNST diagnosis.
  
  
  METHODS
  PET/CT-guided percutaneous biopsy procedures performed on 26 NF1 patients with a clinical suspicion of MPNST and a suspect lesion from a PET/CT scan (T/L SUV max ratio > 1.5) were retrospectively evaluated. The localization of the suspected malignant site was determined using PET/CT. A stereotactic (ultrasonic and CT control) core biopsy technique was used with a local anesthesia.
  
  
  RESULTS
  The first PET/CT-guided percutaneous biopsies enabled a pathological diagnosis for all of the patients (no "inconclusive " results were obtained), and no secondary procedures were needed. Among the 26 patients, the histopathological results from the biopsy were malignant in 17 cases and benign (BPNST with atypical cells) in nine cases. No complications from the diagnostic procedure were observed. A surgical resection was performed in 18 patients (seven benign and 11 malignant biopsies), removing the fine needle biopsy scar. In addition, six locally advanced/metastatic MPNST were treated with chemo/radiotherapy, and two BPNST had no progression after a follow-up of 14 and 39 months, respectively. The PET/CT-guided percutaneous biopsy gave 25 accurate diagnoses and one false negative (BPNST with atypical cells on the biopsy and MPNST on the operated tumor), resulting in a diagnostic accuracy rate of 96%. This false negative case may be explained by the high heterogeneity of the tumor: benign areas were contiguous with the malignant ones and associated with inflammation.
  
  
  CONCLUSIONS
  PET/CT-guided percutaneous biopsies are an effective and relatively non-traumatic procedure for diagnosis of NF1-related MPNST. It is the most reliable approach for early detection of MPNST.
authors.affiliation: Centre Léon Bérard, Lyon, France.
authors.forename: Mehdi
authors.initials: M
authors.lastname: Brahmi
authors.affiliation: Centre Léon Bérard, Lyon, France.
authors.forename: Philippe
authors.initials: P
authors.lastname: Thiesse
authors.affiliation: Centre Léon Bérard, Lyon, France.
authors.forename: Dominique
authors.initials: D
authors.lastname: Ranchere
authors.affiliation: Centre Léon Bérard, Lyon, France.
authors.forename: Thomas
authors.initials: T
authors.lastname: Mognetti
authors.affiliation: Edouard Herriot Hospital Genetic Laboratory, Lyon, France.
authors.forename: Stephane
authors.initials: S
authors.lastname: Pinson
authors.affiliation: Centre Léon Bérard, Lyon, France.
authors.forename: Caroline
authors.initials: C
authors.lastname: Renard
authors.affiliation: Centre Léon Bérard, Lyon, France.
authors.forename: Anne-Valérie
authors.initials: AV
authors.lastname: Decouvelaere
authors.affiliation: Centre Léon Bérard, Lyon, France.
authors.forename: Jean-Yves
authors.initials: JY
authors.lastname: Blay
authors.affiliation: Centre Léon Bérard, Lyon, France.
authors.forename: Patrick
authors.initials: P
authors.lastname: Combemale
mesh_terms: D000293:Adolescent; D000328:Adult; D001706:Biopsy / Q000379:methods; D002471:Cell Transformation, Neoplastic / Q000473:pathology; D005260:Female; D019788:Fluorodeoxyglucose F18 / Q000008:administration & dosage; D006801:Humans; D008297:Male; D008875:Middle Aged; D018317:Nerve Sheath Neoplasms / Q000175:diagnosis* / Q000473:pathology*; D009456:Neurofibromatosis 1 / Q000175:diagnosis* / Q000473:pathology*; D049268:Positron-Emission Tomography / Q000379:methods; D019275:Radiopharmaceuticals / Q000008:administration & dosage; D012189:Retrospective Studies; D014057:Tomography, X-Ray Computed / Q000379:methods; D055815:Young Adult
chemical_list: D019275:Radiopharmaceuticals; D019788:Fluorodeoxyglucose F18