Spontaneously Regressed Probable Retinoblastoma | Spontaneous Remission Database

Abstract

A 5-year-old girl with a clinical picture of spontaneously regressed probable retinoblastoma is presented along with color photographs of the lesion. The cause of the spontaneous regression remains a mystery. One possible factor may be that the tumor outgrows its own blood supply and thereby becomes necrotic, perhaps with toxic products causing further destruction of the tumor cells. The process could arrest and eliminate the growing tumor. On the other hand, other systemic factors such as immune responses by the host may be involved since Meller (American Journal of Ophthalmology 32 (1915) 193-199) observed a simultaneous regression at three separate sites in the same eye.

Case Details

Personal Characteristics

A 5-year-old girl, physicians daughter, in excellent health. No complicating factors during maternal pregnancy; the child was a full term infant with no obvious serious illnesses occurring during childhood.

Clinical Characteristics

The vision in her right eye was reduced over the left. All the pathologic findings were limited to her right fundus. The vitreous was completely clear. The optic nerve was of good color and sharply delineated. The foveal reflex was normally developed. About one-half disc diameter above the fovea and above the optic nerve was a large lesion measuring about 4 millimeters in diameter. In the center was a honeycombed mass with cottage cheese texture. The lesion was surrounded by some translucent, grayish tissue remnants. Through these and at the edge a pigment disturbance in the retinal pigment epithelium and choroid could be seen. No other tumors or pathologic findings were visible on scleral indentation in either eye.

Remission Characteristics

The lesion itself looks very much like a retinoblastoma which has been treated and cured, but this patient received no treatment. The presence of a lesion which looks like retinoblastoma with no obvious activity suggests that this probably is a rare case of a spontaneously regressed retinoblastoma.

Treatment & Mechanisms

Proposed Remission Mechanisms

One possible factor may be that the tumor outgrows its own blood supply and thereby becomes necrotic, perhaps with toxic products causing further destruction of the tumor cells. The process could arrest and eliminate the growing tumor. On the other hand, other systemic factors such as immune responses by the host may be involved.

Additional Notes

The differential diagnosis included retinoblastoma, Coats Disease, and perhaps larval granulomatosis. Because of the characteristic cottage cheese appearance, the most likely clinical diagnosis was retinoblastoma. Against the diagnosis of retinoblastoma were the following factors: The average age at which the active tumor is usually first seen is 13 months. Although initial discovery of retinoblastoma does occur in older children and even adults, these cases are rare. In addition, there were no other lesions besides that visible in the macular area in either eye. There was no family history of retinoblastoma and no involvement of the retinas of either parent nor of a 3-month-old brother. The lesion seemed inactive; there was no pinkish or fleshy active-looking tumor present here.