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Year : 2006  |  Volume : 54  |  Issue : 3  |  Page : 206-208

Primary orbital neuroblastoma in a neonate

1 Department of Neurosurgery, Celal Bayar University School of Medicine, Manisa, Turkey
2 Department of Ophthalmology, Celal Bayar University School of Medicine, Manisa, Turkey
3 Department of Pediatrics, Celal Bayar University School of Medicine, Manisa, Turkey
4 Department of Pathology, Celal Bayar University School of Medicine, Manisa, Turkey

Correspondence Address:
Hasan Mirzai
2040 Sok. Pamukkale 4/60 D:67, Mavisehir Izmir, 35540
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/0301-4738.27078

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Neuroblastoma is an undifferentiated malignant tumor of the primitive neuroblasts. Orbital neuroblastoma is typically a metastatic tumor. We describe a two-days-old girl, who presented with a large tumor in her left orbit. Magnetic resonance imaging revealed that the tumor originated from the retrobulbar area, extending into the upper and lateral orbit. She was operated on the fifth day of life. A histopathologic diagnosis of neuroblastoma was made. Medical evaluation including chest roentgenogram, ultrasonography of the abdomen, whole body computerized tomogram and bone scintigraphy showed no evidence of systemic involvement or metastasis. Neuroblastoma should be considered in the differential diagnosis of neonatal orbital tumors.

Keywords: Neonatal tumors, neuroblastoma orbital tumors, primary neuroblastoma.

How to cite this article:
Mirzai H, Baser EF, Tansug N, Nese N, Isisag A. Primary orbital neuroblastoma in a neonate. Indian J Ophthalmol 2006;54:206-8

How to cite this URL:
Mirzai H, Baser EF, Tansug N, Nese N, Isisag A. Primary orbital neuroblastoma in a neonate. Indian J Ophthalmol [serial online] 2006 [cited 2022 Aug 13];54:206-8. Available from: https://www.ijo.in/text.asp?2006/54/3/206/27078

Tumors diagnosed during the first month of life are rare, constituting only 0.5-2% of all childhood neoplasms.[1] Neuroblastoma is among the most common solid tumors of the neonatal tumors.[1],[2],[3],[4],[5],[6] The most frequent sites of primary neonatal neuroblastoma are thoracal, cervical, abdominal and pelvic locations.[1],[2],[7],[8] Only 8% of neuroblastomas first present with an orbital lesion.[9] Primary orbital neuroblastoma has been reported before, in adults.[10],[11]

In this article, we describe a neonate with primary orbital neuroblastoma. To the best of our knowledge, primary orbital neuroblastoma in a neonate has not been reported in the English literature before.

Case Report

A two-days-old girl was admitted to our university hospital with a giant mass in her left eye. The mass was red and firm in appearance and protruded underneath her left upper eyelid, impeding any view of her left globe [Figure - 1]. She had no ecchymosis of the periorbital area. She was otherwise physically normal and showed no signs of any congenital malformations. The patient was the product of an uncomplicated pregnancy and was born at full term by normal delivery.

Magnetic resonance imaging showed a large (42.5 cm) posterolateral solid orbital tumor. The tumor originated from the retrobulbar area, extended into the upper and lateral orbit and then protruded externally, significantly displacing the globe medially and inferiorly. The left globe was distorted due to the neighboring mass, but was otherwise normal. There was no evidence of a mass in the sinuses, nasal cavities or intracranial cavities [Figure - 2].

Because of the serious concern about amblyopia, she was operated on the fifth day of life, by a team of a neurosurgeon and an ophthalmologist. The operation was as follows: Under general anesthesia, the lids were retracted and a lateral canthotomy was performed. With the aid of an operating microscope, first, the top portion protruding between the globe and the upper eyelid was debulked and sent for frozen section. On frozen section examination of the specimen, a malignant small-cell tumor was seen. Dissection was carried down deep into to the retrobulbar area, between the globe and the lateral orbital wall, taking special care to preserve the muscles and the globe. None of these structures were infiltrated by the tumor. The tumor was excised totally. Examination of the left globe after tumor excision revealed no pathologies.

Microscopic examination revealed a tumor consisting of round cells with high mitotic activity, in a neurofibrillary and hemorrhagic background. The chromatin of the cells was moderately condensed. Not infrequently, the tumor cells were forming nodular figures and Homer-Wright pseudorosettes. Some sites showed large areas of necrosis and Touton giant cells. No ganglionic differentation was seen. The tumor cells were immunoreactive for synoptophysin, neuron-specific enolase, chromogranin and focally glial fibrillary acidic protein, while S-100, leukocyte common antigen, neurofilament and desmin were negative. A histopathologic diagnosis of neuroblastoma was made [Figure - 3] a-b-c.

Subsequent medical evaluation including chest roentgenogram, ultrasonography of the abdomen, whole body computed tomography and bone scintigraphy showed no evidence of systemic involvement or metastasis. The tumor was diagnosed as a primary orbital neuroblastoma and the patient was referred to the pediatric oncology department for further follow-up and treatment.

  Discussion Top

Neuroblastoma is an undifferentiated malignant tumor of the primitive neuroblasts, which may be metastatic to the orbit. It presents as the second most common orbital tumor in children, after rhabdomyosarcoma. Rarely, do neuroblastomas represent primary lesions in the orbit, where they may arise from the ciliary ganglion.[9]

This tumor accounts for 8% of all childhood cancers and is the most common extracranial solid tumor of childhood.[8] The mean age at presentation is 2 years old. Orbital neuroblastoma classically occurs as a metastasis in children, presenting typically with rapid progression of proptosis and periorbital ecchymosis.[12]

Commonly, there is bone destruction, particularly of the lateral orbital wall. Ten to forty percent of systemic neuroblastomas result in orbital metastasis, on an average of 3 months after diagnosis. Forty percent of orbital metastases are bilateral. Other possible ophthalmic manifestations of metastatic neuroblastoma are Horner's syndrome, papilledema, retinal striae, anisocoria, nystagmus and cranial nerve paralysis. About 90% of orbital lesions originate from the abdomen.[8],[9],[12]

The clinical, surgical, radiologic and histologic features of our patient supported the diagnosis of a primary orbital neuroblastoma. The lack of systemic symptoms along with normal findings from physical, laboratory and radiologic examinations, ruled out a diagnosis of metastatic orbital neuroblastoma.

Histopathologically, other small round-cell tumors, especially non-Hodgkin lymphomas and rhabdomyosarcomas, must be taken into consideration for differential diagnosis of a neuroblastoma. The presence of Homer-Wright pseudorosettes in a neurofibrillary background and the absence of rhabdomyoblasts allowed us to make a diagnosis of neuroblastoma. In addition, immunohistochemistry revealed that tumoral cells were positive for neuronal markers, i.e., synaptopysin, neuron-specific enolase and chromogranin and negative for leukocyte common antigen and desmin, ruling out lymphoma and rhabdomyosarcoma with certainty.

In conclusion, we believe that the neuroblastoma in this newborn originated from the ciliary ganglion and that neuroblastoma should be considered in the differential diagnosis of neonatal orbital tumors.

  References Top

Moppett J, Haddadin I, Foot AB. On behalf of the United Kingdom Children's Cancer Study Group. Neonatal neuroblastoma. Arch Dis Child Fetal Neonatal Ed 1999; 81:F134-7.  Back to cited text no. 1
Halperin EC. Neonatal neoplasms. Int J Radiat Oncol Biol Phys 2000;47:171-8.  Back to cited text no. 2
Rao S, Azmy A, Carachi R. Neonatal tumors: A single-centre experience. Pediatr Surg Int 2002;18:306-9.  Back to cited text no. 3
Borch K, Jacobsen T, Olsen JH, Hirsch F, Hertz H. Neonatal cancer in Denmark 1943-1985. Pediatr Hematol Oncol 1992;9:209-16.   Back to cited text no. 4
Parkes SE, Muir KR, Southern L, Cameron AH, Darbyshire PJ, Stevens MC. Neonatal tumors: A thirty-year population-based study. Med Pediatr Oncol 1994;22:309-17.  Back to cited text no. 5
Vasilatou-Kosmidis H. Cancer in neonates and infants. Med Pediatr Oncol 2003;41:7-9.  Back to cited text no. 6
Hadley GP, Govender D, Landers G. Malignant solid tumours in neonates: An African perspective. Pediatr Surg Int 2002;18:653-7.  Back to cited text no. 7
Belgaumi AF, Kauffman WM, Jenkins JJ, Cordoba J, Bowman LC, Santana VM, et al . Blindness in children with neuroblastoma. Cancer 1997;80:1997-2004.  Back to cited text no. 8
Dutton JJ. Orbital Diseases. In : Yanoff M, Duker JS, editors. Ophthalmology. Mosby: St. Louis, MO; 2004. p. 729-43.   Back to cited text no. 9
Bullock JD, Goldberg SH, Rakes SM, Felder DS, Connelly PJ. Primary orbital neuroblastoma. Arch Ophtalmol 1989;107:1031-3.  Back to cited text no. 10
Jakobiec FA, Klepach GL, Crissman JD, Spoor TC. Primary differentiated neuroblastoma of the orbit. Ophthalmology 1987;94:255-66.  Back to cited text no. 11
Musarella MA, Chan HS, DeBoer G, Gallie BL. Ocular involvement in neuroblastoma: prognostic implications. Ophthalmology 1984;91:936-40.  Back to cited text no. 12


  [Figure - 1], [Figure - 2], [Figure - 3]

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