Intracranial neuroendocrine carcinoma: report of 6 cases and review of the literature

XIONG Ping; TANG Xiao-ping; ZHANG Tao

doi:10.13798/j.issn.1009-153X.2019.03.003

Abstract：Objective To investigate the clinical manifestations, imaging features and pathological features of intracranial neuroendocrine carcinoma in order to improve the diagnosis and treatment of this disease. Methods The clinical data of 6 patients (age, 23~65 years; gender, 4 males and 2 females) with intracranial neuroendocrine carcinomas who were treated in our hospital from 2000 to 2018 were retrospectively analyzed. Of 6 carinomas, 1 was in the left parietal occipital lobe and the fourth ventricle, 1 in the right parietal lobe, 3 in the right frontal lobe and 1 in the cerebellar hemisphere. The space-occupying lesions were found also in the lungs of 4 patients. The surgical resection of the carinomas was performed on all the patients. The carcinomas were preoperatively misdiagnosed as gliomas in all the patients. All the neuroendocrine carcinomas were definitely made by pathological examination. Results Five patients were followed up from 1 week to 12 months after the surgery. One patient with metastatic tumor undergoing postoperative gamma knife treatment died 6 months after the surgery. One patient who did not receive further postoperative radiotherapy and chemotherapy died 3 months after the surgery. Three patients receiving the postoperative gamma knife therapy still survived 12 months after the surgery. One patient lost to be followed up. Conclusions The intracranial neuroendocrine carcinoma is a rare, and highly malignant tumor and the primary intracranial neuroendocrine carcinoma is more rare. Its diagnosis is difficult according to its clinical manifestations and imaging features. The histopathological manifestations are the gold standard of its diagnosis. The surgery and postoperative radiochemotherapy is a main method to treat the carcinoma.