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| CASE REPORT |
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| Year : 1995 | Volume
: 43
| Issue : 1 | Page : 29-31 |
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Primitive neuroectodermal tumour of the orbit: A case report
RK Bansal, Amod Gupta
From Department of Ophthalmology, Postgraduate Institute of Medical Education and Research, Chandigarh, India
Correspondence Address:
R K Bansal
Department of Ophthalmology, Postgraduate Institute of Medical Education and Research, Chandigarh 160 012
India
 Source of Support: None, Conflict of Interest: None  | Check |
PMID: 8522367 
How to cite this article:
Bansal R K, Gupta A. Primitive neuroectodermal tumour of the orbit: A case report. Indian J Ophthalmol 1995;43:29-31 |
Melatonic neuroectodermal tumour of infancy is a rare, benign pigmented retinal tumour, which predominantly arises from the maxilla.[1][2][3] It has also been reported to arise from other uncommon sites like mandible,[4] epididymus,[5] thigh,[6] mediastinum,[7] zygoma[2],[3],[8],[9] and temporal bones[2],[8] of the skull. Involvement of the frontal bone is rare.[3],[10] A case of orbital melanotic neuroectodermal tumour of infancy with involvement of zygomatic and frontal bones is reported.
| Case report | |  |
A full-term 8-month-old boy, a product of normal delivery, was noticed by parents to develop a painless swelling on the left temporal region near the lateral canthus at the age of three months. The swelling increased slowly until proptosis developed at the age of 4˝ months. Enucleation of the eyeball with partial removal of the mass through lateral orbitotomy was done elsewhere at the age of 6 months. One month following surgery, a swelling appeared under the upper lid and continued to increase in size until the patient presented to us at the age of 8 months. All the milestones were normal for his age.
On examination, the right eye and adnexa were found to be within normal limits. Examination revealed a bony hard swelling on the left side palpable through the upper lid along its entire length. Superior orbital margin could not be identified separately from the mass [Figure - 1]. Eversion of the upper lid was not possible. There was a linear, vertical, depressed surgical mark on the lateral aspect of orbit [Figure - 2]. The lower lid and lower fornix were normal. There was no globe.
Computed tomogram of the orbit showed a mass of heterogenous density with areas of calcification along the roof of the orbit with frontal bone invasion [Figure - 3]. The sinuses were normal. Total exenteration of the orbit with removal of the internal lamina of the frontal bone including removal of the superior orbital margin was done under general anaesthesia. The mass was hard in consistency and black in colour. Histopathology of the exenterated specimen was consistent with the diagnosis of benign neuroectodermal tumour of infancy [Figure - 4].
Two years after the exenteration, the orbit was fully granulated [Figure - 5] without any clinical evidence of recurrence.
| Discussion | | |
A review of 158 cases by Cutler et al[1] and a report of six cases by Lamping et al[3] show the maxilla to be the most common site for the origin of neuroectodermal tumour of infancy. It has also been reported to arise within and around the orbit in five cases where involvement of the zygometic bone was the commonest[2],[3],[8][9][10] (Table). In one case, it has been shown to involve the temporal bone.[2] Involvement of the frontal bone as a primary site or as secondary invasion from zygoma is rare.[9],[10] In one of the six cases reported by Lamping et al,[3] orbital process of the frontal bone was involved by the tumour and in another case reported by Singh et al11 both zygomatic and frontal bones were involved. Our patient probably had primary involvement of the zygometic bone, as is evident from a history of swelling on the lateral aspect of the orbit to start with. The involvement of the frontal bone either occurred primarily or as a result of direct spread of the tumour from zygoma after the initial surgery, when the tumour was removed partly through lateral approach along with enucleation of the globe.
Orbital involvement in primitive neuroectodermal tumour occurs as a result of spread of the tumour from the adjoining bones, which leads to displacement or proptosis of the globe.[3],[8][9][10] (Table). In five previously reported cases, it was possible to salvage the globe because of early diagnoses and only local excision sufficed. In our case, the globe had already been removed when the patient presented to us and the massive infiltration of the orbit along with bony invasion necessitated exteneration of the orbit.
| References | | |
| 1. | Cutler LS, Choudhary AP, Topazian R. Melanotic neuroectodermal tumour of infancy. An ultrastructural study, literature review and reevaluation. Cancer 48:257-270, 1981.  |
| 2. | Koudstaal J, Oldhoff J, Panders AK, et al. Melanotic neuroectodermal tumour of infancy. Cancer 22:151-161, 1968. |
| 3. | Lamping KA, Albert DM, Lack E, et al. Melanotic neuroectoderm tumour of infancy (retinal anlage tumour). Ophthalmology 92:143-149, 1985. |
| 4. | Kerr DA, Weiss AW. Pigmented ameloblastoma of the mandible. Oral Surg 16:1339-1349; 1963. |
| 5. | Zone RM. Retinal anlage tumour of epididymis. A case report. J Urol 103:106-107, 1963. |
| 6. | Vlachos JD, Papadimitriou CS. Melanotic neuroectodermal tumour of infancy. Report of 2 cases. Pathol Eur 4:152-160, 1969. |
| 7. | Negathau MK, Krishnamurthy KR. Melanoameloplastoma. J Paed Surg 8:977-979, 1973. |
| 8. | Hall WC, O'Day DM, Glick AD. Melanotic neuroectodermal tumour of infancy: An ophthalmic appearance. Arch Ophthalmol 97:922-925, 1979. |
| 9. | Templeton AC. Orbital tumours in African children. Br J Ophthalmol 55:254-261, 1971. |
| 10. | Singh AD, Husson M, Shields CL, et al. Primitive neuroectodermal tumour of the orbit. Arch Ophthalmol 112:217-221, 1994. |
[Figure - 1], [Figure - 2], [Figure - 3], [Figure - 4], [Figure - 5]
[Table - 1]
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