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 Table of Contents  
CASE REPORT
Year : 2015  |  Volume : 6  |  Issue : 2  |  Page : 163-165

Solitary neurofibroma of the inferior orbit: A rare occurrence


1 Department of Ophthalmology, Father Muller Medical College, Mangalore, Karnataka, India
2 Department of Radiology, Father Muller Medical College, Mangalore, Karnataka, India
3 Department of Pathology, Father Muller Medical College, Mangalore, Karnataka, India

Date of Web Publication13-Jul-2015

Correspondence Address:
Dr. V Sowmya
Department of Ophthalmology, Father Muller Medical College, Kankanady, Mangalore - 575 002, Karnataka
India
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/0975-9727.160699

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  Abstract 

Solitary neurofibroma in the absence of neurofibromatosis is of rare occurrence and very few have been reported in the literature till now especially in the inferior orbit. A case of solitary neurofibroma of the inferior orbit is presented here. 32 year old male patient presented to us with history of swelling below the left lower lid since 2 years. After the detailed clinical and radiological examination patient underwent anterior orbitotomy and excision of the mass. Histopathological examination confirmed the diagnosis of neurofibroma of the inferior orbit which is of very rare occurrence.

Keywords: Inferior orbit, neurofibroma, solitary


How to cite this article:
Sowmya V, Nazareth NE, Basti RS, Marla N. Solitary neurofibroma of the inferior orbit: A rare occurrence. Muller J Med Sci Res 2015;6:163-5

How to cite this URL:
Sowmya V, Nazareth NE, Basti RS, Marla N. Solitary neurofibroma of the inferior orbit: A rare occurrence. Muller J Med Sci Res [serial online] 2015 [cited 2019 Oct 16];6:163-5. Available from: http://www.mjmsr.net/text.asp?2015/6/2/163/160699


  Introduction Top


Neurofibromas are benign tumors of the nerves characterized by proliferation of nerve cells and Schwann cells along with intervening fibrous components. Commonly associated with neurofibromatosis along with other systemic findings. Occurrence of isolated intraorbital neurofibroma is relatively rare especially in inferior orbit. It accounts for 1% of orbital neoplasms and most commonly they occur in the superior orbit. May mimic haemangioma in radiodiagnostic studies. Purpose of this case report is to emphasise on the occurrence of this rare tumour and its management.


  Case Report Top


A 32-year-old man presented with a large swelling below the left lower lid for the past 2 years. Patient noticed a small swelling accidentally 2 years ago which was painless and gradually progressed in size. There was no history of trauma or similar swellings elsewhere in the body. On examination there was a single firm to hard nodular mobile, non-pulsatile mass measuring 3 × 3 cm below the left lower lid. Skin over the mass was hyperpigmented. There was no associated bruit. Sensation on and around the mass was normal. Exophthalmometry showed a mild proptosis of 2 mm with mild hyperglobus [Figure 1] and [Figure 2]. Extraocular movements were normal. Slit-lamp biomicroscopy examination of the left eye revealed a normal anterior segment. Intraocular pressures were 14 mmHg in both eyes. The fundus examination showed choroidal folds in the left eye. The systemic examination presented with no significant findings. Systemic features of neurofibromatosis like multiple, multifocal neurofibromas, café au lait spots or axillary freckling were absent. The family history was not significant.
Figure 1: Showing swelling below left lower lid

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Figure 2: Showing left mild proptosis and mass

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MRI of the orbit showed a well-defined T1 hypointense T2 isointense mass lesion measuring 29 × 20 × 23 mm in infero-lateral aspect of left orbit. Thin hypointense capsule around the lesion was seen on T2 and FLAIR sequence along with scalloping of inferior orbital margin, globe indentation and mild proptosis arriving at the differential diagnosis of neurofibroma/haemangioma [Figure 3] and [Figure 4]. Ultrasound Doppler was done to look for flow which showed well encapsulated mass lesion of uniform echogenecity in the left inferior orbital extraconal space along with multiple vessels on the surface which showed low resistance arterial flow hinting more towards the possibility of neurofibroma.
Figure 3: MRI showing mass in th left inferolateral part of the orbit

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Figure 4: MRI orbit showing mild proptosis with globe indentation and inferior orbital margin scalloping

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After taking the detailed informed consent patient was taken up for the left orbitotomy under general anaesthesia. Left subciliary incision was put and orbicularis oculi was dissected. Septum identified and opened. On tapping over the eyeball greyish white mass prolapsed out which was separated from the surrounding structures carefully using blunt dissection and mass was sent for histopathology examination [Figure 5]. Wound was closed in layers. Post-operative period was uneventful with normal extraocular movements and sensations. Histopathology showed a tumor covered by a thin fibrous capsule. Tumor composed of elongated spindle cells with pale eosinophilic cytoplasm and wavy nucleus in fibromyxoid stroma with no mitotic activity.Thin and thick walled blood vessels admixed with tumor cells were noted in the stroma [Figure 6]. All these features confirmed the diagnosis of Solitary Neurofibroma of the inferior orbit. No recurrence noticed in the past 1 year follow up [Figure 7].
Figure 5: Excision biopsy specimen removed in toto

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Figure 6: Histopathology showing spindle cells with wavy nucleus and indistict cytoplam. 100x

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Figure 7: Post operative picture showing scar of subciliary incision merging with eyelid crease

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  Discussion Top


The exact incidence of true solitary or isolated neurofibroma in the orbit is not available and it is difficult to obtain due its association with neurofibromatosis; however, it is relatively rare. Only 3 cases of solitary neurofibroma were encountered by Rootman [1] in his series of orbital tumors, occurring in middle-aged persons as a slow-growing solid mass which were present in the superior orbit. Garrity and Henderson [2] had 9 cases, arising usually from the supraorbital frontal branch of the trigeminal nerve. Rose and Wright [3] showed a 93% incidence of benign neurilemmoma or neurofibroma among orbital peripheral nerve sheath tumors. They defined a family history of systemic neurofibromatosis in one-quarter of patients with solitary neurofibroma. These authors described postoperative sensory deficit in 72% of patients with neurofibroma. Shields et al. [4] reported a patient who had no manifestations of neurofibromatosis with 3 separate right orbital lesions. Alkatan [5] reported a solitary neurofibroma in the superomedail orbit with posterior extension. All these authors reported the occurrence of solitary neurofibroma in the superior orbit. To the best of our knowledge only two cases of solitary neurofibroma of the inferior orbit are reported till date in the literature. [6],[7]


  Conclusion Top


Solitary neurofibromas are rare, but if untreated they can lead to devastating complications. They should be removed intact. The present case is unusual in a few aspects, as the site of neurofibroma in the inferior orbit, which is uncommon and no preoperative or postoperative sensory deficit noted. Preoperative diagnosis became easier with the availability of MRI and Doppler ultrasonography. Rarity of the tumor makes it often difficult for an ophthalmologist to diagnose it on a presumptive basis. Imaging and early surgical intervention may be the only way to arrive at a definitive diagnosis for such orbital mass lesions and give excellent post-operative result if intervened earlier.

 
  References Top

1.
Neoplasia RJ. In: Diseases of the Orbit: A Multidisciplinary Approach. 2 nd ed. Philadelphia: Lippincott Williams & Wilkins; 2003. p. 242-9.  Back to cited text no. 1
    
2.
Garrity JA, Henderson JW. Tumors of peripheral nerve sheath origin. In: Garrity JA, Henderson JW, editors. Henderson′s Orbital Tumors, 4 th ed. Philadelphia: Lippincott Williams & Wilkins; 2007. p. 221-8.  Back to cited text no. 2
    
3.
Rose GE, Wright JE. Isolated peripheral nerve sheath tumors of the orbit. Eye (Lond) 1991;5:668-73.  Back to cited text no. 3
    
4.
Shields JA, Shields CL, Lieb WE, Eagle RC. Multiple orbital neurofibromas unassociated with von Recklinghausen′s disease. Arch Ophthalmol 1990;108:80-3.  Back to cited text no. 4
    
5.
Alkatan HM. Solitary neurofibroma in the absence of neurofibromatosis. Can J Ophthalmol 2007;42:628-9.  Back to cited text no. 5
    
6.
Della Rocca RC, Roen J, Labay GR, Bedrossian E. Isolated neurofibroma of the orbit. Ophthalmic Surg 1985;16:634-8.  Back to cited text no. 6
    
7.
Berney C, Spahn B, Oberhansli C, Uffer S, Borruat FX. Multiple intraorbital neurofibromas: A rare cause of proptosis. Klin Monbl Augenheilkd 2004;221:418-20.  Back to cited text no. 7
    


    Figures

  [Figure 1], [Figure 2], [Figure 3], [Figure 4], [Figure 5], [Figure 6], [Figure 7]



 

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Abstract
Introduction
Case Report
Discussion
Conclusion
References
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