Introduction: Intracranial malignant teratoma is an exceptionally rare disease with only sporadic surgically treated cases reported to-date. We aim to characterize the role of surgery in survival of these patients in a population based study.
Methods: We utilized the Surveillance, Epidemiology, and End Results(SEER) database for this study. Histologically diagnosed primary intracranial malignant teratomas, teratocarinomas or teratoma with malignant transformation classified by International Classification of Diseases for Oncology (ICD-O) were included. Surgical treatment was subdivided into no surgery, partial resection, gross/total resection(GTR) or unknown. Other characteristics including age, gender, race, tumor characteristics, postoperative radiation were also retrieved from the database. Multivariate accelerated failure time(AFT) regression was performed to determine the effect of surgical resection on survival when accounting for other confounding variables.
Results: A total of 86 patients with primary intracranial malignant teratomas were included. Average age of all patients was 10.9 +/- 14.2 years, with 57(66.3%) male. Patient race was: White(81.4%), Black(5.8%), and Others(12.8%). Twenty-nine patients(33.7%) had tumors primarily located in the pineal/pituitary gland, and the rest were located in other intracerebral locations. Seventy-three(84.9%) patients underwent surgical resection, with 40(54.8%) achieving gross/total resection, and 26 patients(30.2%) received post-operative radiotherapy. Cumulative 3-year survival was 72.5%, with 21.5% in no surgery, 68.8% in partial resection and 89.8% in gross/total resection. Multivariate AFT regression showed both partial resection (HR:0.14, p=0.001) and gross/total resection(HR:0.04, p<0.001) were associated with prolonged survival of patients. Postoperative radiosurgery did not show survival benefit(HR:0.61, p=0.423). Longer survival was observed for lesions in pineal/pituitary glands(HR:0.19, p=0.009). No effect on survival was observed for tumor size or tumor stage.
Conclusions: The overall prognosis of untreated malignant intracranial teratoma is poor. Surgical resection, especially gross/total resection, provides significant survival benefit in patients with intracranial malignant teratomas. Therefore, surgery should be recommended for these patients with a favorable treatment risk profile.
Patient Care: Teratomas are mostly skull-based tumors that are benign. Malignant teratomas are exceptionally rare with unknown disease progression. Only sporadic case reports are reported to date. We utilized a population-based prospective database and illustrated the survival of patients with malignant teratomas. We found that the 3-year cumulative survival is 72.%. Additionally, we confirmed the role of surgical resection, particularly GTR, in prolonging survival in these patients, with a 80% reduction in teratoma-related death. Provided that no reliable evidence exists to-date for these tumors, this study may serve as the foundational evidence to support surgical resection in these cases.
Learning Objectives: By the conclusion of this session, participants should be able to:
1) Understand that untreated intracranial malignant teratomas have a poor prognosis
2) Learn that surgical resection provides survival benefits for these patients
3) Learn that malignant teratoma in the pineal/pituitary glands have a better prognosis than other locations