NEUROBLASTOMA of the adrenal gland with its subsequent metastases is a serious disease which occurs almost entirely in children. It is of great interest to ophthalmologists because of its metastasis to the orbit, with resultant proptosis, discoloration of the lids and loss of vision; its rarity; its rapidly fatal termination, and its insidiousness and concealment of the primary lesion, causing difficulty in diagnosis in the early stages. The orbit is invariably involved in the metastases, and frequently it is the eye which gives the first noticeable indication of the disease.
Less than 4 cases a year have been recorded since Vinchow1 discovered his case in 1864, the total number reported up to the end of 1940 being only 291.
The clinical course in cases of this disease is almost universally rapid, progressing to fatal termination. The average duration of the symptoms and signs is two to five months. The
COX RA. PROPTOSIS DUE TO NEUROBLASTOMA OF THE ADRENAL CORTEX (HUTCHINSON'S SYNDROME)Report of a Case. Arch Ophthalmol. 1948;39(6):731-738. doi:10.1001/archopht.1948.00900020741002