ESPE Abstracts (2016) 86 P-P1-42

Acute Lysis of a Giant Pediatric Adrenal Cortical Carcinoma Following One Dose of op'DDD

Emmanuelle Mottea, Anya Rothenbuhlera, Philippe Durandb & Pierre Bougnèresa


aPediatric Endocrinology, Bicêtre, France; bPediatric Intensive Care, Bicêtre, France


Background: op’DDD can be used in adrenal cortical carcinoma (ACC) i) when surgery is impossible ii) or as an adjunct to surgery when local extension or metastases are present.

Objectives and hypotheses: To report the unexpected spectacular effects of op’DDD in an unoperable ACC.

Patient and results: A 3-year-old black African girl presented in poor shape with a 2-year history of pubic hair, clitoromegaly, abdominal distension. The CT scan revealed a giant ACC (28 cm×25 cm) that compressed the right kidney, inferior vena cava, diaphragm, right lung, right hepatic lobe, with no metastases. Serum testosterone was 27 ng/ml, SDHA 4270 ng/ml. Given the risk of a primary surgery, surgeons refused to attempt tumorectomy, thus op’DDD treatment was attempted. Twelve hours after a first 500 mg dose of op’DDD, the child experienced acute intense abdominal pain and oliguria due to a typical lysis tumor syndrome with hyperkaliemia (9.4 mmol/l), hyperphosphatemia (2.8 mmol/l), hyperuricemia (572 μmol/l) and high LDL (843 UI/l). The patient went into intensive care for hemofiltration and recovered. op’DDD treatment was maintained under cover of hyperhydration and uricolytic drugs. One week after the acute lysis, the CT scan showed that the tumor had shrinked while large zones of necrobiosis had appeared. Serum testosterone was 0.65 ng/ml and SDHEA 268 ng/ml. Six weeks later, a 14-h surgical intervention (Pr H. Martelli and S. Branchereau) allowed the complete exercise of a 1.5 kg tumor. One month later, the CT scan showed no tumoral remnants.

Conclusion: The induction of an acute lysis of ACC by op’DDD has not been reported before.

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