2009
DOI: 10.1177/000313480907500203
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Surgical Considerations for the Familial Cancer Syndrome, Neurofibromatosis 1: A Comprehensive Review

Abstract: Neurofibromatosis 1 is one of the more common in heritable disorders that surgeons may encounter. A plethora of systemic associations, both benign and malignant, can affect these patients, and an acute awareness of these associations is essential for proper surgical care. A complete review of this disorder from the surgical perspective follows, highlighting the importance of this awareness. A brief review on the management and follow-up of surgical malignancies associated with this disorder is included.

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Cited by 10 publications
(6 citation statements)
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“…The tumor resection aims to relieve the symptoms. 22,23,31,[34][35][36] This patient had undergone total resection of the tumor. Four years later, the patient presented with FIG.…”
Section: Discussionmentioning
confidence: 99%
“…The tumor resection aims to relieve the symptoms. 22,23,31,[34][35][36] This patient had undergone total resection of the tumor. Four years later, the patient presented with FIG.…”
Section: Discussionmentioning
confidence: 99%
“…Unlike isolated, sporadic neurofibromas, plexiform neurofibromas carry an intrinsically higher risk of malignant degeneration into malignant PNSTs. 69 The "T2 rim" sign is a thin rim of hyperintense signal on T2-weighted sequences, more common in schwannomas than neurofibromas. 67 Intratumoral cysts, or intratumoral cystic degeneration, is an additional finding more commonly identified in schwannomas than in neurofibromas.…”
Section: Neoplasmmentioning
confidence: 99%
“…Complete resection is challenging. 42 Most MPNSTs (50%) arise from neurofibromas in the context of type 1 NF. They represent 5 to 10% of all soft tissue sarcomas.…”
Section: Nerve Sheath Tumorsmentioning
confidence: 99%