Large Solitary Neurofibroma of Face in a Paediatric Patient

Abstract

A 10-year-old female child was referred to the Department of Oral and Maxil lofacial Surgery, Krishna Hospital, Karad, Maharashtra, India for evaluation and management of a painless soft tissue swelling over the left side of face. The parents reportedly first noticed the growth when the child was four-year-old and the swelling had gradually increased to its present size. Patient's medical history was non contributory. There was no previous history of trauma. On examination, a sagging subcutaneous swelling was noted, extending from left commissure of mouth to the preauricular region. The lesion was about 4 X 7 cm in size and caused drooping of the left corner of the mouth. The overlying skin showed a localized area of hyper pigmentation [Table/ Fig-1]. It was soft, non-tender and non-pulsatile. On palpation, the lobulated mass gave a so-called 'bag of worm' feeling. There was no neurological disturbance associated with the lesion. Facial nerve weakness and paresthesia over distribution of mental, infraorbital nerve were ruled out. The patient had no similar lesion or area of pigmentation elsewhere on the body. Ultrasonography (USG) of the lesion showed a hypoechoic mass in the subcutaneous plane of buccal space measuring about 7.6 cm cranio-caudally, resting over the buccinator, masseter and orbicularis-oris muscle. Well defined hypoechoic area was noted within the swelling, suggestive of necrotic foci. Multiple dilated channels were seen within the lesion with minimal vascularity on color doppler [ AbSTRACT Neurofibroma is a rare, benign, neurogenic tumour which may present as multiple lesions as a part of neurofibromatosis syndrome or as a solitary mass. This paper reports a case of large solitary neurofibroma of face in a young female patient. Clinical presentation, imaging characteristics and management of the pathology has been described in detail. The paper also reviews English literature for similar cases.Magnetic Resonance Imaging (MRI) showed a well-defined enhancing lesion (on T2 images) involving the subcutaneous soft tissue plane with contour bulge on left side of the face. Lesion was seen encasing the facial vessels and its branches [ Table/Fig 3a,b]. No haemorrhagic or calcification foci were seen within the lesion. Underlying mandible and maxillary bones showed normal morphology. Head and neck angiogram showed the subcutaneous mass being supplied by facial artery. Large, early draining, dilated torturous venous channels were noted within the lesion draining into the external jugular vein on the left side suggestive of shunting of capillary network. The left carotid artery system, including common carotid cervical segment, carotid bifurcation, external and internal carotid artery were normal [Table/ Fig-3c,d].The above radiological features were suggestive of non-encapsulated subcutaneous mass with slow flow vascular channels and the differential diagnosis included neurogenic tumour, haemangioma and lipoma. An incision biopsy, carried out under local anaesthesia revealed stratified squamous epitheli...

Orbital intraconal abducens nerve schwannoma: An interdisciplinary approach for management

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