these tumors are rarely reported in infants . these lesions present as slowly progressive masses that can either compress to the adjacent vital structures or interfere with normal physiology .
we report a case of giant , solitary nf presenting as a progressive mass in parotid region in an infant and review the relevant literature .
an 11-month - old female child was admitted in surgery with a progressively enlarging swelling in the right parotid region of two months duration .
there was a large ( 7  8 cm ) , firm , non - tender mass present in right parotid and infra - temporal region with a normal overlying skin [ figure 1 ] .
there were no external features suggestive of nf-1 / nf-2 like macules , lich nodules .
her hemoglobin was 12 gm % and total leucocyte count and erythrocyte sedimentation rate ( esr ) were 6200 / cm m and 20 respectively .
the routine urine examination , c - reactive protein , and kidney function test were also within normal limits .
routine x - ray and ultrasonography ( usg ) of her chest and abdomen and computed tomography ( ct ) scan of head were normal .
the doppler study of the neck showed a homogenous and hyper - vascular solid mass , situated within the subcutaneous plane of right parotid region .
ct with contrast suggested a large well defined homogenous , enhancing mass reaching up to the lateral pharyngeal wall without infiltrating the surrounding structures [ figure 2 ] .
fine needle aspiration cytology ( fnac ) of the neck was inconclusive . in view of persisting confusion over the diagnosis ,
the tumor was exposed and excised completely with extended blair 's incision [ figure 3 ] . on exploration
the tumor was situated within the subcutaneous plane and the parotid gland was shifted superiorly and well separated from the mass . facial nerve and its branches
the patient is doing well at two - year follow - up . clinical photograph showing large mass in the right parotid region ct scan showing details of the extent of mass and the lesion was enhancing after contrast administration intra - operative photograph well defi ned plane of cleavage and complete excision of the tumor histopathology showing features of neurofi broma ( h & e , 40 )
schwannomas or nfs are most common with a common origin  the schwann cell . in the head and neck , about 25% of all nfs
are found but solitary nfs are rare in this region . generally nfs show autosomal dominant pattern , occurring either due to spontaneous mutation or from the passing of infected gene with complete penetrance and variable expression .
they are well - differentiated , non - encapsulated nerve sheath tumor , composed of shawn cells , perineural - like cells , fibroblasts , and transitional cells and arise within the endo - neurium .
the nerve fibers are incorporated within the tumor therefore can not be separated and resection may sacrifice the nerve of origin .
nfs have to be differentiated from schwannomas and malignant peripheral nerve sheath tumors ( mpnst ) .
schwannomas are encapsulated tumors of nerve sheath and grow eccentrically without infiltrating the nerve and may be dissected out successfully from the nerve .
they have a typical palisade pattern of the nuclei , and tumor cell density is higher compared with that in nfs .
mitosis and a mucoid extra cellular matrix with only scattered tumor cells ( the indicator of malignancy ) are common in schwannoma .
histopathologicaly , type - a tissue of antoni ( the narrow cylinders with tapering ends ) - compact and composed bundles of long bipolar spindle cells with oval or rod - shaped nuclei and type b tissue - a loose texture ; and polymorphism of the tumor cells are highly specific structures of neurilemomas .
nfs are seen to be slowly growing asymptomatic soft tissue masses and often do not have any neurological deficits .
the diagnostic workup includes ruling out the presence of other nfs and any possibility of neurofibromatosis nf1 and nf2 .
the diagnostic criteria for nf1 includes family history , six or more caf - au - lait macules , neurofibromas , lisch nodules , tumor on the optic nerve and skeletal abnormalities , of which two must be present in a patient to be diagnosed as nf1 . the diagnostic criteria for nf2
is bilateral vestibular schwannomas ( vs ) or family history of nf2 ( first degree family relative ) plus unilateral vs < 30 years or any two of the meningioma , glioma , schwannoma and juvenile posterior sub capsular lenticular opacities/ juvenile cortical cataract .
usg is not very specific and both schwannomas and nfs are commonly homogeneous and hypo echoic and produce posterior acoustic enhancement .
ct with contrast in nfs usually shows an ovoid , well - demarcated , homogeneous and iso - attenuation relative to muscle .
heterogeneous contrast enhancement , as demonstrated in the present case , has been described to be typical for nfs .
immuno - histochemically , many of the spindle cells are positive for common neural markers , with patchy staining for cd34 and epithelial membrane antigen .
immuno - reactivity for s-100 protein , neuron - specific enolase , and vimentin is characteristic for nfs .
the transformation of a nf into a mpnst has been observed in 2 - 5% of cases with nf-1 .
to summarize , solitary nfs are rare in infants , especially in head and neck region .
they may mimic parotid tumor and even with careful evaluation preoperative diagnosis may be difficult . the present case is unique in its age of presentation ( 11 months ) , site and size ( about 8  15 cm ) , without any symptoms or neurological deficit and could be managed successfully with good outcome .
 solitary neurofibroma is a rare tumor of the head and neck region . 
 it is more common in viscera , where it is associated with features of nf1 . 
 it occurs most often between the third and fourth decade . 
 these lesions are extremely rare in infants . 
 we report a case of giant , solitary neurofibroma presenting as a progressive mass in parotid region in an infant . 
 this case is unique in its age of presentation ( 11 months ) , site , size ( about 8  15 cm ) without any symptoms or neurological deficit . 
Introduction
Case Report
Discussion