Denis R. Benjamin

The role of timing of high-dose cytosine arabinoside intensification and of maintenance therapy in the treatment of children with acute nonlymphocytic leukemia

The Childrens Cancer Study Group instituted a pilot study to investigate the use of high doses of cytosine arabinoside (AraC) in the intensification phase of treatment for acute nonlymphocytic leukemia (ANLL). Patients achieving remission and not eligible for allogeneic bone marrow transplantation were treated with four doses of high‐dose AraC and L‐asparaginase. These drugs were repeated either on or after 28 days (q28 days), after recovery of hematologic parameters (for the first 49 patients entered onto this trial); or after 7 days (q7 days), despite dropping blood counts (for the last 53 patients enrolled). After completing an additional 3 months of intensification therapy, patients were then allocated by physician choice to either discontinue therapy or receive 18 28‐day cycles of maintenance therapy, including the daily administration of 6‐thioguanine. Despite three deaths associated with the toxicity of the aggressive (q7 days) AraC timing, patients receiving this approach demonstrated equal or better disease‐free survival from the end of induction (55% versus 42% actuarially at 3 years [P = 0.52]). Maintenance therapy appeared to play no role in improving outcome for people who received the aggressive timing of AraC cycles. Fifty‐nine percent were alive disease free actuarially at 3 years from the decision to not give maintenance therapy (n = 27) compared with 62% for those receiving maintenance therapy (n = 16; P = 0.49). On the other hand, patients who received the less aggressive AraC intensification timing (q28 days) had an improved survival rate if maintenance therapy was administered (n = 17) (65% versus 39% for patients not receiving maintenance therapy [n = 24] at 3 years [P = 0.07]). Maintenance therapy therefore may not improve outcome in patients receiving aggressive timing of high‐dose AraC but may be important in less intensive postremission regimens in childhood ANLL.

Radiotherapy for locally recurrent infantile pancreatic carcinoma (pancreatoblastoma).

The authors report a case of recurrent paacreatoblastoma in a 3‐year‐old girl who was successfully treated with radiotherapy. The patient had two local tumor recurrences before radiation treatment; the second recurrence was managed with radiation alone. Computerized tomography scan at the completion of radiotherapy showed dramatic tumor regression. Six weeks after radiotherapy was finished exploratory laparotomy was performed; multiple biopsies showed no evidence of tumor. The patient is currently disease‐free 2 years after completion of radiotherapy. This case suggests a role for radiation therapy in the management of children with paacreatoblastoma.

Humoral hypercalcemia due to an occult renal adenoma

Abstract. Humoral hypercalcemia refers to the elevated blood calcium levels caused by neoplasms which release a bone resorptive substance into the circulation. Previously reported infants with malignant and benign solid tumors causing humoral hypercalcemia have presented with large abdominal masses. The case we describe, a hypercalcemic infant due to an occult parathyroid hormone-related protein-containing metanephric adenoma of the kidney, shows that radionuclide bone scanning can be a useful test to identify humoral hypercalcemia. Humoral hypercalcemia stemming from a soft tissue neoplasm should be ruled out, even in the absence of clinical signs of a tumor, if bone scans show generalized uptake in the absence of hypervitaminosis D or radiological signs of bone lesions, and serum parathyroid hormone is low.

C-Reactive Protein and Prealbumin in Suspected Sudden Infant Death Syndrome

This study investigated the levels of C-reactive protein (CRP) and prealbumin in 87 infants suspected of dying from sudden infant death syndrome (SIDS). These proteins change rapidly, within 24 hr, in patients who have an acute phase response, especially a response resulting from bacterial infection. In addition, prealbumin is sensitive to a recent reduction of protein or calorie intake. Eighty patients were determined to have typical SIDS. Only four of these had a minimal increase in CRP (0.8-2 mg/dl), and in none could an explanation be found. Seven patients were uncovered who had a significant infection, four of whom had prominent CRP elevations. Prealbumin was not decreased in the SIDS population. We conclude that there is no evidence that the acute inflammatory response is activated in infants dying of SIDS. An elevation of CRP above 2 mg/dl should prompt additional studies to find the cause. There is also no evidence of a recent, significant decrease in nutrition in these patients.