Surgical resection and adjuvant chemotherapy of familial intracranial choriocarcinoma

Objective To investigate the comprehensive treatment and prognosis of primary intracranial choriocarcinoma of familial tendency. Methods Two whole sisters admitted to the Department of Neurosurgery, Yuquan Hospital, Tsing Hua University in June 2013 and confirmed as primary choriocarcinoma in sellar region by pathology were summarized. Their ages of onset were 9 and 11 respectively. The lesion site was in the sellar region. The preoperative tumor markers suggested abnormally increased β-HCG; their choriocarcinomas were resected after giving a course of ICE chemotherapy. The elder sister was operated via interhemispheric fissure-corpus callosum-between the fornices approach and the younger one was operated via crown cut-frontal anterior longitudinal fissure approach. They underwent regular chemotherapy after surgery and did not undergo radiotherapy. Results The 2 choriocarcinomas achieved near-total resection under the microscope. They all recovered well after surgery and did not have serious complications in saddle regions, and both were pathologically confirmed as choriocarcinoma. The examination before discharge showed that β-HCG turned to negative. They had 5 regular chemotherapies, and they were followed up to now and their choriocarcinomas did not recur. Conclusions Primary intracranial choriocarcinoma is high malignant and it is rare in clinical practice. Its familial predisposition onset is rarely reported. As long as the diagnosis is clear, the use of regular chemotherapy and appropriate surgical approach may achieve a satisfactory efficacy. If it can be studied from genomics, it might be able to achieve a breakthrough on the basis of primary intracranial choriocarcinoma. Key words: Neoplasms, germ cell and embryonal; Choriocarcinoma; Neurosurgical procedures; Chemotherapy