Congenital giant craniopharyngioma.

PURPOSE

Congenital intracranial tumors are extremely rare and the most common is teratoma. Craniopharyngioma is a rare neonatal tumor with only eight cases reported. The management of this tumor in the neonatal period is still controversial, with the best results obtained when radical resection is performed. We present the case of a patient who received the diagnosis of a suprasellar tumor during the prenatal period and reviewed literature regarding the management.

METHODS

We report a case of neonatal craniopharyngioma treated surgically.

RESULTS

The routine ultrasound at 29 weeks of gestation showed a suprasellar echogenic image measuring 44 mm in diameter with polyhydramnios and macrocephaly. The patient was born at 38 weeks of gestation and underwent a surgical treatment on its 32nd day of life and the excision of almost 80 % of the lesion was achieved. He developed a subdural hygroma and on the 51st day of life, a subduroperitoneal shunt was installed to treat it. This patient died at 8 months of life due to complications of a shunt infection.

CONCLUSION

The present case is the ninth diagnosed during the prenatal period and the literature is controversial on the management of this rare tumor. The complete excision of the lesion using the microsurgical technique is the gold standard treatment for these patients; however, there are many factors that limit this approach in neonates. Usually, the resected tumors were smaller than 6 cm. Tumors larger than 8 cm have a worse prognosis, with a short survival time.