Opsoclonus Raccoon Eye in Fatal Metastatic Neuroblastoma


Metastatic neuroblastoma with opsoclonus raccoon eye presentation is an extremely rare condition presented in stage IV metastatic condition. Histopathology/ cytology, vanillylmandelic acid (VMA), homovanillic acid (HVA) levels and metaiodobenzylguanidine (MIBG) scanning are mainly contributors for the diagnosis of this disease. We are presenting one year male child with history of protrusion of right eye, swelling in right periauricular region, opsoclonus movements of small amplitude and periorbital ecchymosis ‘raccoon eye’. So far only infinitesimal cases has been reported in the literature and our present case report is an extremely rare case of locally advanced metastatic neuroblastoma, who presented as opsoclonus raccoon eye and despite of the aggressive chemotherapy, didn’t responded to the intended treatment. We attempt a review in the literature for different possibilities of staging and diagnostic management.

Neuroblastoma is an enigmatic malignant neoplasm. In its early stages it can be readily cured with surgery or, in some circumstances, can even spontaneously regress or mature to a benign ganglioneuroma. In the more common advanced stages, the disease is often fatal. It is one of the first malignancies in which molecular biologic assays have influenced treatment and prognosis. The three major cardinal eye signs of neuroblastoma include proptosis, Horner's syndrome and opsoclonus eye movements. Treatment of neuroblastoma involves chemotherapy, surgery, radiation and autologous bone marrow transplantation, which is used as a rescue following marrow-ablative induction chemotherapy, while the patients with ophthalmic involvement requires aggressive multiagent high doses chemotherapy.

Ophthalmic involvement in neuroblastoma is uncommon and has an ominous prognosis. Different investigations like bone marrow aspiration cytology, vanillylmandelic acid (VMA), homovanillic acid (HVA) levels and meta-iodobenzylguanidine (MIBG) scanning should be adopted in practice to diagnose at the early stage. The clinical management presents considerable challenges. Intensive multimodality approach should be tested, incorporating surgery, dose intensive combination chemotherapy etc. It is the first malignancies in which molecular biologic assays have influenced treatment and prognosis, hence future challenges include the development of less toxic therapy for patient with localized disease and new approaches for patients with metastatic disease for the treatment or palliation.

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Jones Carrol
Associate Editor
Journal of Biology and Today's World