Skip to main page content
U.S. flag

An official website of the United States government

Dot gov

The .gov means it’s official.
Federal government websites often end in .gov or .mil. Before sharing sensitive information, make sure you’re on a federal government site.

Https

The site is secure.
The https:// ensures that you are connecting to the official website and that any information you provide is encrypted and transmitted securely.

Access keys NCBI Homepage MyNCBI Homepage Main Content Main Navigation
Review
. 2016 Oct-Dec;11(4):295-297.
doi: 10.4103/1817-1745.199469.

Congenital extra calvarial plexiform neurofibroma in occipito-cervical region with Occipital bone defect with neurofibromatosis type 1 and segmental neurofibromatosis: Case report and review of literature

Affiliations
Review

Congenital extra calvarial plexiform neurofibroma in occipito-cervical region with Occipital bone defect with neurofibromatosis type 1 and segmental neurofibromatosis: Case report and review of literature

Vivek Kumar Kankane et al. J Pediatr Neurosci. 2016 Oct-Dec.

Abstract

Plexiform neurofibroma (PNF) of the scalp is an extremely rare lesion reported in association with neurofibromatosis (NF). Occipital location of PNF is even more infrequent; we reported one pediatric case of PNF in occipito-cervical region with multiple small occipital bone defects and associated with NF-1.

Keywords: Neurofibromatosis; plexiform neurofibroma; segmental neurofibromatosis.

PubMed Disclaimer

Conflict of interest statement

There are no conflicts of interest.

Figures

Figure 1
Figure 1
(a) Clinical photographs showing the occipito-cervical swelling. (b) Clinical photographs showing café au lait spots on the left side of upper and lower trunk
Figure 2
Figure 2
T1-weighted with postcontrast sagittal magnetic resonance image shows heterogeneous enhancement lesion in the scalp of the right occipital region and nape of neck
Figure 3
Figure 3
(a and b) Both photographs showing three-dimensional computerized tomography skull showing thing of occipital bone right-sided with multiple bone defects

References

    1. Karwacki MW, Wozniak W. The skeleton abnormalities in patients with neurofibromatosis type 1: Important consequences of abnormal gene function. In: Dionyssiotis Y, editor. Osteoporosis. New York: InTech; 2012. pp. 323–41.
    1. Renshaw A, Borsetti M, Nelson RJ, Orlando A. Massive plexiform neurofibroma with associated meningo-encephalocoele and occipital bone defect presenting as a cervical mass. Br J Plast Surg. 2003;56:514–7. - PubMed
    1. Kurimoto M, Mizumaki Y, Fukuda O, Hayashi N, Kuwayama N, Endo S. Giant plexiform neurofibroma and suboccipital meningocele manifesting as segmental neurofibromatosis. Neurol Med Chir (Tokyo) 2008;48:271–4. - PubMed
    1. Scott M. Massive plexiform neurofibroma of the occipital scalp. Case report. J Neurosurg. 1966;25:81–2. - PubMed
    1. Ohaegbulam SC. “Congenital” plexiform neurofibroma of the occipital scalp. Case report. J Neurosurg. 1977;46:245–7. - PubMed