2010
DOI: 10.1016/j.jocn.2010.01.055
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Diffuse scalp malignant peripheral nerve sheath tumor with intracranial extension in a patient with neurofibromatosis type 1

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Cited by 12 publications
(10 citation statements)
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“…Primary scalp MPNST is extremely rare, with only 14 cases reported to date in English literature (Table 1) [6-18]. MPNST is considered to be associated with gene mutations, such as loss of the neurofibromatosis 1 gene protein product (neurofibroma) [13] and rearrangements of the p16 (INK4A) gene [12,14]. It has been reported that approximately one-third of MPNSTs arise de novo , whereas nearly 60% of all MPNSTs represent a sarcomatous degeneration of a benign neurofibroma.…”
Section: Discussionmentioning
confidence: 99%
“…Primary scalp MPNST is extremely rare, with only 14 cases reported to date in English literature (Table 1) [6-18]. MPNST is considered to be associated with gene mutations, such as loss of the neurofibromatosis 1 gene protein product (neurofibroma) [13] and rearrangements of the p16 (INK4A) gene [12,14]. It has been reported that approximately one-third of MPNSTs arise de novo , whereas nearly 60% of all MPNSTs represent a sarcomatous degeneration of a benign neurofibroma.…”
Section: Discussionmentioning
confidence: 99%
“…Six patients received adjuvant radiotherapy, and one patient with metastatic disease to the kidney and lungs received neoadjuvant chemotherapy with doxorubicin 16 . In three patients, calvarial destruction with intracranial invasion of the tumor was present at the time of diagnosis 11,12,15 . Two of these patients were alive and disease free after follow‐ups of 6 and 28 months, respectively; for the third patient, no follow‐up information was provided.…”
Section: Discussionmentioning
confidence: 99%
“…In contrast to classic MPNSTs of deep anatomic origin, which are mostly found on the extremities and trunk, more than half of cutaneous MPNSTs are located on the head and neck 7 . Of those, the majority have been found on the scalp, with a total of 10 reported cases 8–16 …”
mentioning
confidence: 99%
“…There was no recurrence at 18 months of follow-up (Table 1). The etiology is still uncertain but MPNSTs are commonly associated with Von Recklinghausen disease in whom gene mutations are found, such as loss of the neurofibromatosis 1 gene and rearrangement of the p16 (INK4A) gene (4,5). It has been reported that 60% of all MPNSTs represent a malignant transformation of a preexisting benign neurofibroma, whereas 30% arise de novo, and approximately 10% occur in patients with history of previous radiation at the tumor site.…”
Section: Case Reportmentioning
confidence: 99%