Retroperitoneal Malignant Peripheral Nerve Sheath Tumor: Evaluation With Serial FDG-PET

Abstract

Retroperitoneal malignant peripheral nerve sheath tumor (MPNST), a rare type of neurogenic tumor, was diagnosed in a 14-year-old girl with a history of neurofibromatosis type 1 (NF1). Immunochemistry demonstrated spindle cells positive for S-100 protein. The patient had multiple tumor recurrences and she was evaluated with serial F-18 fluorodeoxyglucose (FDG) positron emission tomography (PET). A tumor in the right iliac wing showed increased FDG uptake on PET. FDG-PET played an important role in therapy planning and subsequent follow up. This case emphasizes the important role FDG-PET could play in the staging, restaging, and posttherapy follow up of MPNST.

18F-fluorodeoxyglucose positron emission tomography/computed tomography in a case of malignant peripheral nerve sheath tumor: An unusual presentation

“…[ 9 ] It thus plays an important role in the staging, restaging and post-therapy follow-up of MPNST in NF1. [ 10 ] A high FDG uptake has been documented in sporadic MPNSTs also, as is well seen in the coronal maximum intensity projection image [ Figure 2 ] in the present case.…”

Utility of 18F-FDG PET with a Semi-Quantitative Index in the Detection of Sarcomatous Transformation in Patients with Neurofibromatosis Type 1

“…For example, Cardona et al [43] reported 100% sensitivity and 83% specificity in a population of 25 patients (of whom five had NF1) with an absolute SUV max cut-off value for malignancy of 1.8. But other recent studies have demonstrated that an absolute SUV max >3 is associated with poor prognosis and is correlated with Ki67 levels [41], [42], [44]; Tsai [45] set the SUV max malignancy threshold at 4. Karabatsou also reached a 4 SUV max cut-off, but this was in a very small set of 9 patients [46].…”

Solitary Lymph Node Metastasis From a Malignant Peripheral Nerve Sheath Tumor of the Thoracic Vertebra Revealed on FDG PET/CT

“…Similar imaging findings in the vertebral column on PET/CT should consider the possibility of lymphoma, 6,7 chordoma, 8 metastasis, 9 epithelioid hemangioma, 10 tenosynovial giant cell tumor, 11 myeloma, 12 and Rosai-Dorfman disease 13 . This case highlights that solitary lytic thoracic vertebral lesion with intense FDG uptake should regard MPNST as a differential diagnosis and that FDG PET/CT has an important role in the initial staging of MPNST 14–16 …”

“…13 This case highlights that solitary lytic thoracic vertebral lesion with intense FDG uptake should regard MPNST as a differential diagnosis and that FDG PET/CT has an important role in the initial staging of MPNST. [14][15][16]…”