Neurofibrosarcomas of the upper extremity

Rogalski, Roger P.; Louis, Dean S.

doi:10.1016/s0363-5023(10)80153-4

Cited by 9 publications

(8 citation statements)

References 11 publications

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“…However, though pain, neurological deficits, and growing masses are the main clinical indicators of malignancy, they are also presenting symptoms in some patients with benign plexiform neurofibromas, making it difficult to differentiate on clinical grounds only, between malignant and benign lesions [7, 10, 22, 23]. In the current study, the trunk was the commonest involved anatomic site (42%), a finding reported in some studies [24].…”

Section: Discussionsupporting

confidence: 51%

“…While this may be impossible in anatomically centrally located sites, unfortunately, surgery is the only modality of therapy that can achieve cure [22, 27, 28]. Limb-sparing excision with chemoradiotherapy in combination or separately is increasingly favored.…”

Section: Discussionmentioning

confidence: 99%

“…Limb-sparing excision with chemoradiotherapy in combination or separately is increasingly favored. The role of chemotherapy for MPNSTs is not yet defined, although a favorable response in both children and adults has been reported [1, 7, 12, 22]. Local recurrence of MPNST is high, even with multimodal therapy, reported to be between 30% to 65% [27, 28].…”

Section: Discussionmentioning

confidence: 99%

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Malignant Peripheral Nerve Sheath Tumors in Africa: A Clinicopathological Study

Nthumba

Juma

2011

ISRN Surgery

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Introduction. Malignant peripheral nerve sheath tumors (MPNSTs) are rare, aggressive soft tissue sarcomas associated with poor prognosis, that most commonly affect patients aged 20 to 50 years, but have also been reported in children. There is little reported in literature on these tumors in Africa. Materials and Methods. A search of the hospital pathology database between 1992 and 2008 revealed 333 nerve sheath tumors, of which 31 were MPNSTs. Four representative case reports are presented. Discussion. MNPSTs have rarely been reported from sub-Saharan Africa; in this study, they constituted 9.3% of all nerve sheath tumors. The trunk (42%) and limbs (45%) were the most frequently affected anatomical sites. Late presentation of malignant lesions in this environment is exemplified by the four case presentations patients. Conclusions. This report confirms observations from studies on MPNSTs from other environments. Anatomically centrally located MPNSTs may have a higher incidence in sub-Saharan Africa than in the West. Because NF1-associated MPNSTs are difficult to diagnose clinically, and because surgery is the only mode of therapy that offers a complete cure, a lifetime follow-up is important, as this would enable diagnosis of early lesions amenable to surgical extirpation.

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Section: Discussionsupporting

confidence: 51%

Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

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Malignant Peripheral Nerve Sheath Tumors in Africa: A Clinicopathological Study

Nthumba

Juma

2011

ISRN Surgery

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“…MPNST of the upper limb is a rare and life-threatening complication known in patients with and without NF1 [ 41 ], [ 42 ], [ 43 ]. MPNST contribute significantly to reduced life expectancy in NF1 [ 44 ].…”

Section: Resultsmentioning

confidence: 99%

Peripheral nerve sheath tumors of the upper extremity and hand in patients with neurofibromatosis type 1: topography of tumors and evaluation of surgical treatment in 62 patients

Friedrich

Diekmeier

2017

GMS Interdisciplinary Plastic and Reconstructive Surgery DGPW; 6:Doc15

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“…50,53,57 Metastasis is by the way of haematogenous spread although perineural extension and embolization of lymphatics may also occur. Radiotherapy may be helpful in controlling local disease.…”

Section: Managementmentioning

confidence: 99%