Plexiform Neurofibroma of Nasal Tip

Abstract

Neurogenic tumor is the name given to any tumor that arises from the nerve tissue or its coverings. Neurogenic tumors of Sino-nasal cavity are a very rare entity. The most common types are Schwannomas and Neurofibromas and the plexiform subtype is one form of these neuroendocrine tumors. We report the case series of two such cases of a plexiform neurofibroma of the nasal tip, which were excised via an open rhinoplasty approach.

Diagnostic and treatment challenges in neurofibroma concerning the external nose

“…35 The deeper, infiltrative portion is diffusely hypoechoic, in contrast to subcutaneous tissue. 33 In this review, four patients (30.76 per cent) underwent biopsy before definitive surgery, 3,15,17 and the results were inconclusive. Biopsy findings may indicate the benign neurogenic nature of the tumour; however, a definitive diagnosis cannot often be reached.…”

“…18 Involvement of the nasal tip 3,15,17 was identified in three patients in this review, for which the treatment remains more challenging because of its prominent position, thick and less flexible skin, more sebaceous glands, and consequent higher chance of recurrence. 15,48,51–54 Bartolo et al . found that even after surgery, the nasal tip may stay bulky and need revision surgery.…”

“…These included 11 case reports, and 1 case series including 2 patients (Table 1). 3,[12][13][14][15][16][17][18][19][20][21][22] No previous reviews or case series were identified. The mean age of presentation was 31 years (range, 0.25-72 years); 61.5 per cent of patients were male and 38.5 per cent were female (Table 2).…”

“…performed CT, MRI and diethylene triamine penta-acetic acid cisternography in their patient to rule out meningocele. 12 Others opted for MRI, 3,15,22 ultrasound 13 and contrast-enhanced CT. 19,22 Gupta et al . performed FNAC in their report, which was suggestive of benign peripheral nerve sheath tumour.…”

“…44 In this review, variants were not mentioned for 46.15 per cent of patients. [12][13][14]17,18,20 Three had plexiform, 3,15 two had solitary, 19,22 one had diffuse 21 and one had myxoid 16 variants. The cause of solitary neurofibroma is unknown, although Marocchio 45 and da Rosa et al 46 have reported it as a hamartomatous malformation or caused by trauma.…”