Clinical analysis of 28 cases with pediatric germ cell tumors in pineal region

• Main Authors: Ju⁃lei WANG, Tao HUANG, Qi⁃long TIAN, Jia⁃hua ZHOU, Liang WANG, Yan QU
• Format: Article
• Language: English
• Published: Tianjin Huanhu Hospital 2020-04-01
• Series: Chinese Journal of Contemporary Neurology and Neurosurgery
• Subjects: neoplasms, germ cell and embryonal; pineal gland; child; neurosurgical procedures; chemoradiotherapy
• Online Access: http://www.cjcnn.org/index.php/cjcnn/article/view/2124

Objective To summarize the treatment methods and effects of pediatric germ cell tumors in pineal region. Methods and Results Clinical data of 28 children with primary intracranial pineal region germ cell tumors from March 2012 to December 2019 were analyzed retrospectively. The clinical manifestations include high intracranial pressure symptoms, parinaud symptoms and precocious puberty. Seven patients were diagnosed as germinoma indirectly by diagnostic radiotherapy/chemotherapy, the rest patients were confirmed by pathological examination: 15 mixed germ cell tumors, 3 mature teratomas, 2 malignant teratomas and 1 choriocarcinoma. Managements: 7 cases were continued to complete chemotherapy after being confirmed effective by diagnostic chemotherapy or radiotherapy. Three cases were underwent chemotherapy before operation, ineffectively, and were underwent operation after the tumor progressed, and 18 cases were directly resected. The operative approach was via longitudinal fissure - corpus callosum ⁃ fornix in 15 cases and infratentorial supracerebellar approach in 6 cases. Total resection was performed in 16 cases, subtotal resection in 3 cases and partial resection in 2 cases. The serum tumor markers of all children decreased significantly after surgery, with an average of 69.55% . The average hospital stay was 19.35 d for all. At the time of discharge from hospital, one patient abandoned treatment due to coma, the remaining 27 patients with headache, nausea and vomiting, and other symptoms of high intracranial pressure significantly relieved. There were 10 cases of secondary hydrocephalus, and symptoms of high intracranial pressure recurred. The symptoms disappeared after the shunt. The cases in this group were followed up for an average of 2.53 years, and 7 cases of germinoma had no recurrence. Fifteen cases with mixed germ cell tumors had a complete remission of 8/15; one patient gave up treatment after surgery; 5/15 of relapses, of which 2 recurred in situ, 2 recurred in ventricular dissemination, one recurred in spinal cord disseminated; and one case (1/15) died of tumor progression 23 months after surgery. There were no recurrences in 5 cases with teratoma. One case with choriocarcinoma died of tumor progression 4 months after surgery. Conclusions There are various types of germ cell tumors in the pineal gland in children. Mixed germ cell tumors are more common. Serological tumor markers are of great significance in diagnosis and treatment. Surgery combined with standardized chemoradiotherapy is an important treatment method. The use of multi ⁃ disciplinary diagnosis and treatment mode and individualized precise treatment can significantly extend the survival of patients. DOI：10.3969/j.issn.1672⁃6731.2020.04.010