Malignant peripheral nerve sheath tumors in the setting of segmental neurofibromatosis

Schwarz, Jacob; Belzberg, Allan J.

doi:10.3171/jns.2000.92.2.0342

Cited by 43 publications

(31 citation statements)

References 40 publications

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“…Likewise, Lisch nodules may also be present but only when the involved dermatome includes the eye. 12 Roth, et al, 11 have subdivided segmental NF into four subtypes: 1) true segmental NF, which strictly adheres to Riccardi's criteria; 2) localized cases with underlying deep systemic involvement; 3) hereditary segmental-localized cases with genetic transmission of the disease or with an increased susceptibility to somatic mutation at the locus of the disease; and 4) bilateral segmental NF.…”

Section: Discussionmentioning

confidence: 98%

Segmental neurofibromatosis—induced spinal cord compression

Muthukumar

2001

Journal of Neurosurgery: Spine

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✓ Spinal segmental neurofibromatosis (NF) is a rare entity. To date, patients in reported cases of segmental NF (or NF5) have harbored neurofibromas involving the peripheral nerves only. The author reports a rare case of segmental NF that caused spinal cord compression in a 40-year-old woman who presented with a 6-month history of intercostal neuralgia. Examination revealed mild lower-extremity weakness and dysesthesia in the right-sided T-9 dermatome. Magnetic resonance imaging revealed three neurofibromas involving the T-9 region, which were excised, and the patient's neuralgic pain was resolved postoperatively. Traditionally, it has been believed that segmental NF involved only the peripheral nerves. The present case illustrates that although rare, spinal cord compression can also occur in patients with segmental NF.

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Section: Discussionmentioning

confidence: 98%

Segmental neurofibromatosis—induced spinal cord compression

Muthukumar

2001

Journal of Neurosurgery: Spine

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“…4,14,16,20) Surgery to remove a giant plexiform neurofibroma is indicated by pain, neurological impairment, disfigurement, compromise of surrounding structures, or suspicion of malignancy. 1,3,7,13,20,22,23) In the present case, biopsy was performed under suspicion of a malignant tumor. However, substantial bleeding occurred during the procedure and the histological findings showed numerous abnormally dilated vessels.…”

Section: Discussionmentioning

confidence: 99%

Giant Plexiform Neurofibroma and Suboccipital Meningocele Manifesting as Segmental Neurofibromatosis -Case Report-

Kurimoto

Mizumaki

Fukuda

et al. 2008

Neurol. Med. Chir.(Tokyo)

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Fig. 1 Photographs of the patient's shoulder demonstrating a giant nevus spilus (A) and numerous caf áe-au-lait spots on the right side of the neck (B). AbstractA 34-year-old woman presented with segmental neurofibromatosis manifesting as a soft lump with a large caf áe-au-lait macule on her occipital region and neck. Magnetic resonance imaging showed a thick skin tumor in the occipital region and posterior neck, and a suboccipital meningocele which seemed to have no association with her symptoms. Biopsy lead to a histological diagnosis of giant plexiform neurofibroma. During biopsy, massive local bleeding occurred and hemostasis was achieved by electrocautery and meticulous suture ligation. The postoperative course was uneventful and observation was continued for both the giant plexiform neurofibroma and the meningocele.

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“…38 Because surgical debulking is currently the main treatment option for NF1 tumors, there is a pressing need for consistent, clinically relevant models of NF1 MPNST. In fact, xenograft models have already been used to study therapeutic effects in intraperitoneal and subcutaneous model systems.…”

Section: Discussionmentioning

confidence: 99%

MicroRNA Inhibitors as Anticancer Therapies

Hammond¹

2007

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Public reporting burden for this collection of information is estimated to average 1 hour per response, including the time for reviewing instructions, searching existing data sources, gathering and maintaining the data needed, and completing and reviewing this collection of information. Send comments regarding this burden estimate or any other aspect of this collection of information, including suggestions for reducing this burden to Department of Defense, Washington Headquarters Services, Directorate for Information Operations and Reports (0704-0188), 1215 Jefferson Davis Highway, Suite 1204, Arlington, VA 22202-4302. Respondents should be aware that notwithstanding any other provision of law, no person shall be subject to any penalty for failing to comply with a collection of information if it does not display a currently valid OMB control number. PLEASE DO NOT RETURN YOUR FORM TO THE ABOVE ADDRESS. (From -To) REPORT DATE (DD-MM-YYYY) 01-04-2007 REPORT TYPE Revised Final DATES COVERED PERFORMING ORGANIZATION NAME(S) AND ADDRESS(ES) 8. PERFORMING ORGANIZATION REPORT NUMBERUniversity of Florida Gainesville, FL 32610 SPONSORING / MONITORING AGENCY NAME(S) AND ADDRESS(ES) 10. SPONSOR/MONITOR'S ACRONYM(S) U.S. Army Medical Research and Materiel Command Fort Detrick, Maryland 21702-5012 SPONSOR/MONITOR'S REPORT NUMBER(S) DISTRIBUTION / AVAILABILITY STATEMENTApproved for Public Release; Distribution Unlimited SUPPLEMENTARY NOTES ABSTRACTThe goal of this project is to specify how anti-angiogenic approaches can be effectively applied to NF1 tumors. Invivo and in vitro models were used to firmly conclude that Nf1 haploinsufficiency in endothelial cells results inexaggerated proliferation and angiogenesis in response to key pro-angiogenic factors. Results implicate these growthfactor pathways as potential targets for therapeutic agents. In addition, endostatin was found to be a potent inhibitorof Nf1+/-endothelial cell migration in vitro, suggesting endostatin may be an effective antiangiogenic agent forreducing NF1 tumor growth. Two intraneural xenograft models of NF1 peripheral nerve sheath tumors weredeveloped and characterized. Tumor growth and vascularity of NF1 tumor xenografts was quantified by advancedMRI, gadolinium permeability and dynamic contrast enhancement that match results obtained by conventionalhistological measurements. Several methods to deliver endostatin in vivo were tested and several difficulties wereencountered. Finally, cell factories made by alginate-encapsulation of 293 cells transfected with AAV-endostatinwere developed and are being refined to deliver consistent, high-dose systemic levels of endostatin. The effects ofsystemic endostatin on NF1 xenograft tumor growth will be completed in a no-cost extension of this project. SUBJECT TERMSCancer biology, angiogenesis, xenograft, gene therapy, anti-angiogenic therapy, MRI INTRODUCTIONNeurofibromatosis type 1 (NF1) is a common genetic disease with a wide variety of features which primarily involve the nervous system and related tissues. NF...

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Malignant peripheral nerve sheath tumors in the setting of segmental neurofibromatosis

Cited by 43 publications

References 40 publications

Segmental neurofibromatosis—induced spinal cord compression

Segmental neurofibromatosis—induced spinal cord compression

Giant Plexiform Neurofibroma and Suboccipital Meningocele Manifesting as Segmental Neurofibromatosis -Case Report-

MicroRNA Inhibitors as Anticancer Therapies

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