Special Pattern of Widespread Neuroblastoma with a Favourable Prognosis

Case Details

Personal Characteristics

A five-month-old boy was well but was noted to have an abdominal mass on a routine “well-baby” examination in september 1962. He was alert and robust

Clinical Characteristics

The grossly enlarged liver extended to a point 3 centimeters above the inguinal ligament. Chest x-ray normal; intravenous urography showed no displacement or distortion of the collecting systems; no calcification in the enlarged liver nor in the suprarenal regions. Bone marrow not aspirated. Total urinary vanillylmandelic acid content in a twenty-four hour sample was raised (40.7 milligrams, compared with normal twenty-four hour excretion for the age of 0.1-1.3 milligrams).

Remission Characteristics

The boy grew and developed normally. The liver regressed in size and was not palpable one year after operation. Eighteen months after initial diagnosis liver biopsy showed normal hepatic tissue only. The vanillylmandelic acid level was normal. When last seen (1970) he was a normal, healthy eight-year-old boy with no evidence of disease.

Treatment & Mechanisms

Proposed Remission Mechanisms

Not discussed

Clinical Treatment

At laparotomy, irregular nodules throughout the liver and a non-encapsulated tumour arising from the right adrenal extending through the diaphragm into the posterior mediastinum were found. The abdominal portion of the tumour was removed and a biopsy taken from the liver; histologically, both were neuroblastoma. No further treatment was administered.

Additional Notes

Patients who fit the syndrome can have widespread disease in the liver, skin, and bone-marrow, or any combination of these. The primary tumour in some may be relatively small. Twenty-one of 25 such patients (84%) survived for two years or longer. Radiation therapy and chemotherapy may not be necessary in the management of certain children with this syndrome.