Successful long-term survival of patients with trisomy 18 and hepatoblastoma after cardiac surgery.

BACKGROUND

Most patients with trisomy 18 (18T) die of cardiac or other severe anomalies within the first few years. Although surgical intervention for cardiac lesions or other diseases has improved these patients' prognoses in recent years, some survivors face the risk of developing neoplasms, such as hepatoblastoma. However, the treatment strategy remains controversial because of the poor prognosis. We evaluated the merits of aggressive therapeutic interventions for malignant tumors in 18T patients.

METHODS

We encountered five patients who underwent hepatoblastoma treatment within the last 11 years. These patients, four girls and one boy, were aged 10 months to 3 years and 9 months at diagnosis; all had previously undergone cardiac interventions.

RESULTS

Three patients were diagnosed with PRETEXT I, whereas the remaining two were diagnosed with PRETEXT II and IV. Primary resection of the tumor was conducted in three patients with PRETEXT I, followed by chemotherapy in two patients. Open biopsy and neoadjuvant chemotherapy were administered in two patients (PRETEXT II and IV), and subsequent tumor resection could be conducted only in the PRETEXT II patient and not in the PRETEXT IV patient. Four patients who underwent liver resection achieved disease-free survival for 6-10 years postoperatively, and the PRETEXT IV patient did not have tumor regrowth for approximately 2 years and died of another cause. All treatments, including chemotherapy and surgical resection, were administered safely.

CONCLUSIONS

Surgical treatment of hepatoblastoma in 18 T patients can be performed safely after cardiac intervention, after obtaining informed consent regarding the associated risks and benefits.