|Year : 2014 | Volume
| Issue : 2 | Page : 103-105
Neurothekoma of orbit
Nitin V Trivedi1, Girish P Nehete2, Poorti G Nehete3
1 Professor, Oculoplasty Clinic, Nagri Eye Hospital, Ahmedabad, Gujarat, India
2 Oculoplasty Fellow, Pushpa Eye Hospital, Nasik, Maharashtra, India
3 Mahatme Eye Hospital and Eye Bank, Nagpur, Maharashtra, India
|Date of Submission||16-May-2013|
|Date of Acceptance||05-Dec-2013|
|Date of Web Publication||11-Apr-2014|
Nitin V Trivedi
Avataran Nursing Home, 49-A, Lad Society, Sandesh Press Road, Vastrapur, Ahmedabad - 380 054, Gujarat
Source of Support: None, Conflict of Interest: None
Myxoid neurothekeoma, also known as "dermal nerve sheath myxoma", is a rare tumor of presumed nerve sheath origin, which affects adolescents and young adults. A middle-aged woman came with the complaint of inferior displacement of left eyeball for 4 years. She was diagnosed previously as pseudotumor and treated with systemic steroids twice before, following which symptoms were relieved for short duration. There is a history of ptosis surgery done in childhood. Orbit examination showed firm, nonmovable, nonreducible mass below superior orbital margin. Computed tomography scan of orbit showed a round well-circumscribed, isodense mass located in the suprabulbar extraconal space. Surgical excision of mass through anterior orbitotomy was performed. One large size nodule with rest small daughter nodules was removed. Histopathological examination revealed myxoid lobules and fascicles of spindle cells with no atypia, suggestive of nerve sheath myxoma. It is so far rarest tumor in the orbit.
Keywords: Myxoid neurothekeoma, orbitotomy, pseudotumor
|How to cite this article:|
Trivedi NV, Nehete GP, Nehete PG. Neurothekoma of orbit. J Clin Ophthalmol Res 2014;2:103-5
Isolated orbital myxoma is a rarest entity in ocular oncology literature.  Myxomas can cause gradually progressive proptosis over a long period and occasional acute exacerbation can occur due to myxomatous degeneration which can mimic as a pseudotumor. Here, we are reporting a case of isolated orbital nerve sheath myxoma that is neurothekeoma. Our purpose to report this case as seen rarely in ophthalmic practice. So, one can keep as a differential diagnosis for gradually progressive proptosis.
| Case Report|| |
A 41-year-old woman referred to our oculoplasty clinic with complaints of inferior displacement of left eyeball for 4 years. She had noticed gradual progressive displacement of eyeball over these years occasionally accompanied with pain, redness, and watering. She was diagnosed previously as pseudotumor by private ophthalmologist and treated with systemic steroids twice before, following which she had shown symptomatic improvement. Also, there was a history of same eye ptosis surgery done in childhood and postoperatively she had developed exposure keratitis, which was treated accordingly.
On presentation, both eye unaided visual acuity was 20/20. Ocular movements were normal in both eyes. Intraocular pressure in both eyes was 18 mm Hg with Goldmann applanation tonometer. Slit lamp examination of anterior segment of both eyes was unremarkable. No evidence of ptosis was noted.
Orbit examination showed nonaxial inferior proptosis of left eyeball [Figure 1]. Vertical displacement, that is, corneal reflex from eyebrow was 10 mm more than fellow eye, while corneal reflex from midline was same in both eyes. On palpation, the mass was firm in consistency and nonreducible on retropulsion.
Magnetic resonance imaging of orbit done 2 years back showed hyperintense circumscribed mass with similar small adjacent masses in suprabulbar region of left orbit [Figure 2].
|Figure 2: Magnetic resonance imaging of orbit showing hyperintense circumscribed mass in suprabulbar region|
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Computed tomography scan of orbit at the time of presentation to us, showed a round well-circumscribed, isodense, minimally enhancing mass lesion. The mass was located in the suprabulbar extraconal space. A small round calcification was seen within the mass [Figure 3].
|Figure 3: Computed tomography or orbit showing isodense circumscribed mass in superior compartment|
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General examination of patient was unremarkable. There was no evidence of any skin pigmentation. Complete blood counts were within normal range. Electrocardiogram was normal.
Anterior orbitotomy through lid crease incision was performed under general anesthesia. As the dissection went on, orbital fat was retracted back and a mass of size 3 × 2.5 cm, firm in consistency was removed en bloc. Following which few small grape-like nodules were removed. Excess skin was trimmed and the incision was closed.
Gross examination showed a multinodular well-encapsulated mass measuring 3.0 × 2.5 × 1.2 cm. Cut surface was shiny and myxoid. Few nodules, separate from the main mass of size 0.6-1.0 cm, were seen [Figure 4].
Microscopic examination revealed myxoid lobules and fascicles of spindle cells. Lobules were separated by fibrous stroma. There was no evidence of atypical cells [Figure 5], [Figure 6], [Figure 7].
|Figure 5: Histopathology examination slide of the tumor showing myxoid lobules and fascicles of spindle cells (H&E Stain at 10×)|
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Histopathology report was consistent with nerve sheath myxoma (neurothekeoma).
Postoperatively, proptosis reduced as corneal reflex from eyebrow was 4 mm as compared with fellow eye. Rest anterior segment was normal.
| Discussion|| |
Myxoma is an uncommon benign tumor of mesenchymal origin, rarely found in medical literature. Fuchs in 1914 is credited with the first report of a myxoma of the orbit in a 40-year-old woman who had increasing proptosis of 7 years' duration. So far, only nine cases of myxoma of the orbit have been reported. ,,,,,,, Their most common location of occurrence is left atrium of the heart. In the head, mostly reported in mandible and maxillary sinus. Isolated orbital myxoma is very rare as in our case.
In these patients with orbital myxomas, it is essential to look for skin and mucous membrane pigmentation as it may be associated with Carney syndrome. Carney syndrome is characterized by spotted pigmentation of skin and mucous membrane, myxoma, endocrine overactivity, and schwannoma. The spotted pigmentation of skin is the first sign of syndrome. This condition may be associated with atrial myxoma, so one can diagnose it in advance.  In our case, general examination of patient was normal.
Myxomas are generally slow-growing masses. There may occasionally a period of accelerated growth, due to myxoid degeneration in these tumors. They may appear encapsulated owing to compression and condensation of the surrounding tissues, but they lack a true capsule and are locally infiltrative. Microscopically, stellate or spindle-shaped cells with small, pyknotic nuclei, are seen in an abundant myxoid or mucoid stroma rich in hyaluronidase. 
The tumor in our case presented in 4 th decade of life. The tumor showed increase in size in last 4 years, most probably due to myxoid degeneration. History of ptosis surgery suggests that it might be of inflammatory etiology. The tumor also showed some response to steroids as patient improved symptomatically before for short duration. Complete excision is the treatment of choice. In literature till date, there is only one case of recurrence of myxoma described.  In our case, there is no evidence of recurrence till 3 years of follow-up period.
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[Figure 1], [Figure 2], [Figure 3], [Figure 4], [Figure 5], [Figure 6], [Figure 7]