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      Unusual Evolution of Plexiform Neurofibroma in the Scalp: A Case Report

      case-report

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          Abstract

          The neurofibromatosis is a frequent and polymorphic genetic disorder. The severity is related to the complications. The degeneration of neurofibroma is a very rare complication of neurofibromatosis. In the literature, a few cases of solitary neurofibroma, which turned into a malignant tumor were reported. In our case, we described a very rare clinical case of neurofibrosarcoma in the scalp, and surgical treatment.

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          Clinical and genetic aspects of neurofibromatosis 1.

          Eric Friedman, G K Jett (2009)
          Neurofibromatosis 1 is an autosomal dominant disorder characterized by multiple café-au-lait spots, axillary and inguinal freckling, multiple cutaneous neurofibromas, and iris Lisch nodules. Learning disabilities are present in at least 50% of individuals with neurofibromatosis 1. Less common but potentially more serious manifestations include plexiform neurofibromas, optic nerve and other central nervous system gliomas, malignant peripheral nerve sheath tumors, scoliosis, tibial dysplasia, and vasculopathy. The diagnosis of neurofibromatosis 1 is usually based on clinical findings. Neurofibromatosis 1, one of the most common Mendelian disorders, is caused by heterozygous mutations of the NF1 gene. Almost one half of all affected individuals have de novo mutations. Molecular genetic testing is available clinically but is infrequently needed for diagnosis. Disease management includes referral to specialists for treatment of complications involving the eye, central or peripheral nervous system, cardiovascular system, spine, or long bones. Surgery to remove both benign and malignant tumors or to correct skeletal manifestations is sometimes warranted. Annual physical examination by a physician familiar with the disorder is recommended. Other recommendations include ophthalmologic examinations annually in children and less frequently in adults, regular developmental assessment in children, regular blood pressure monitoring, and magnetic resonance imaging for follow-up of clinically suspected intracranial and other internal tumors.
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            Survival meta-analyses for >1800 malignant peripheral nerve sheath tumor patients with and without neurofibromatosis type 1

            Matthias Kolberg, Maren Høland, Trude Ågesen … (2012)
            There are conflicting reports as to whether malignant peripheral nerve sheath tumor (MPNST) patients with neurofibromatosis type 1 (NF1) have worse prognosis than non-NF1 MPNST patients. Large clinical studies to address this problem are lacking due to the rareness of MPNST. We have performed meta-analyses testing the effect of NF1 status on MPNST survival based on publications from the last 50 years, including only nonoverlapping patients reported from each institution. In addition, we analyzed survival characteristics for 179 MPNST patients from 3 European sarcoma centers. The meta-analyses including data from a total of 48 studies and >1800 patients revealed a significantly higher odds ratio for overall survival (OROS) and disease-specific survival (ORDSS) in the non-NF1 group (OROS = 1.75, 95% confidence interval [CI] = 1.28–2.39, and ORDSS = 1.68, 95% CI = 1.18–2.40). However, in studies published in the last decade, survival in the 2 patient groups has been converging, as especially the NF1 group has shown improved prognosis. For our own MPNST patients, NF1 status had no effect on overall or disease-specific survival. The compiled literature from 1963 to the present indicates a significantly worse outcome of MPNST in patients with NF1 syndrome compared with non-NF1 patients. However, survival for the NF1 patients has improved in the last decade, and the survival difference is diminishing. These observations support the hypothesis that MPNSTs arising in NF1 and non-NF1 patients are not different per se. Consequently, we suggest that the choice of treatment for MPNST should be independent of NF1 status.
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              Von Recklinghausen neurofibromatosis. A clinical and population study in south-east Wales.

              D. A. S. Compston, S Huson, Johnathan Harper (1988)
              A population-based study in south-east Wales (population 668,100) identified 135 patients with von Recklinghausen neurofibromatosis (prevalence 20/10(5]. In addition to multiple café-au-lait spots and/or dermal neurofibromas, freckling was present in the axilla (67%), groin (44%) or submammary areas (29% of adult females). Although not a criterion for diagnosis, Lisch nodules were almost invariably present in the iris (93% of patients overall; 96% of those aged greater than or equal to 20 yrs). The complications of von Recklinghausen neurofibromatosis in this cohort (n = 135 unless stated) were plexiform neurofibromas (40/125), severe mental retardation (1), epilepsy (6), optic glioma (2), spinal neurofibroma (2), aqueduct stenosis (2), meningioangiomatosis (1), scoliosis requiring surgery (6), pseudoarthrosis (3), delayed puberty (2), visceral and endocrine tumours (6), and congenital glaucoma (1). There were no cases of acoustic neuroma. Considering all living family members aged greater than or equal to 18 yrs, together with their deceased relatives, the frequency of CNS and malignant tumours related to the disease was 4.4-5.2%. Uncomplicated von Recklinghausen neurofibromatosis is disfiguring but not a major cause of morbidity. The management of the disease relates to its complications which can be divided into three categories: those which occur in childhood and cause lifelong morbidity (moderate-severe mental handicap, facial plexiform neurofibromas, orthopaedic), those which can occur at any time but are 'treatable' (benign disorders of the nervous system, visceral and endocrine tumours, renal artery stenosis), and malignant or CNS tumours. The combined frequency for each category based on this survey was 12%, 16% and 4.4-5.2%, respectively.
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                Author and article information

                Journal
                Journal ID (nlm-ta): World J Plast Surg
                Journal ID (iso-abbrev): World J Plast Surg
                Journal ID (publisher-id): WJPS
                Title: World Journal of Plastic Surgery
                Publisher: Iranian Society for Plastic Surgeons (Tehran, Iran )
                ISSN (Print): 2228-7914
                ISSN (Electronic): 2252-0724
                Publication date (Print): January 2018
                Volume: 7
                Issue: 1
                Pages: 128-131
                Affiliations
                [1]National Center of Burns and Plastic Surgery, University Hospital Ibn Rochd, Casablanca, Morocco
                Author notes
                [* ]Corresponding Author: Amine Rafik, National Center of Burns and Plastic Surgery, University Hospital Ibn Rochd, Casablanca, Morocco. E-mail: aminerafik8@gmail.com
                Article
                Publisher ID: wjps-7-128
                PMC ID: 5890378
                SO-VID: ab318b76-31be-4459-aa4a-2dc24a969435
                License:

                This is an Open Access article distributed under the terms of the Creative Commons Attribution License, ( http://creativecommons.org/licenses/by/3.0/) which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.

                History
                Date received : 17 May 2016
                Date revision received : 11 September 2017
                Date accepted : 2 November 2017
                Categories
                Subject: Case Report

                Keywords: neurofibroma,neurofibromatosis,scalp,schwannoma,surgery
                Data availability:
                Keywords: neurofibroma, neurofibromatosis, scalp, schwannoma, surgery

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