Abstract:
:A malignant peripheral nerve sheath tumor (MPNST) is defined as any malignant tumor that develops or differentiates from cells in the peripheral nerve sheath. This tumor is commonly associated with neurofibromatosis type 1 (NF1) and previous radiotherapy treatment. Primary intraosseous MPNSTs are extremely rare and a case of the lumbar spine in a patient without NF1 is reported in the present study, with a review of the intraosseous MPNST literature. A 45-year-old female presented with a 1-month history of severe lower back pain and pain radiating to the left leg. A total en bloc spondylectomy of L4 was performed. The postoperative histopathological diagnosis was MPNST with deletion of NF1, confirmed by dual-color fluorescence in situ hybridization (FISH) analysis. The tumor recurred 1 month following the surgery. Although adjuvant chemotherapy was administered, the patient succumbed due to intramedullary dissemination and carcinomatous meningitis 8 months following the initial consultation. NF1 deletion by FISH analysis may be particularly useful in distinguishing MPNST from other high-grade malignancies with overlapping morphological features.
journal_name
Oncol Lettjournal_title
Oncology lettersauthors
Suzuki K,Yasuda T,Hori T,Watanabe K,Kanamori M,Kimura Tdoi
10.3892/ol.2014.1987subject
Has Abstractpub_date
2014-06-01 00:00:00pages
1965-1969issue
6eissn
1792-1074issn
1792-1082pii
ol-07-06-1965journal_volume
7pub_type
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