Pitfalls in Diagnosis and Management of Testicular Choriocarcinoma Metastatic to the Brain: Report of 2 Cases and Review of Literature

Introduction

Pure choriocarcinoma of the testes is a rare, aggressive germ cell tumor that can metastasize to the brain. Although its prognosis has improved with the development of cisplatin-based chemotherapy regimens, cerebral metastases are prone to hemorrhage and associated with high morbidity. Here, we present 2 cases of testicular choriocarcinoma with cerebral metastasis and discuss potential pitfalls in their diagnosis and management. We also review cases in the literature that feature these rare lesions.

Methods

Medline was searched for all publications including the terms “testicular choriocarcinoma” and “cerebral metastasis” or “brain metastasis.” Articles that included patients with tumors classified as a mix of choriocarcinoma and other germ cell tumor subtypes were excluded.

Results

A total of 15 cases from the literature and our own 2 cases were included in the analysis. The mean age at presentation was 25.5 years. Neurologic symptoms accounted for the initial presentation of 9 patients (60%). Outcomes were predominantly poor, with 10 patients (67%) expiring shortly after their initial diagnosis. Three of these deaths were related to mass effect from metastasis-related hemorrhages. Two patients underwent emergent decompressive craniectomies, and both died from cerebral herniation.

Conclusion

The potentially catastrophic nature of choriocarcinoma-related cerebral hemorrhages underscores the need for prompt, accurate diagnosis and aggressive surgical management of these lesions. Their highly vascular nature and lack of findings on cerebral angiography may cause them to be confused with occult vascular malformations.

Introduction

Choriocarcinoma is a highly aggressive, vascular germ cell tumor (GCT) that presents in young males as testicular cancer. It is prone to early hematogenous and lymphatic spread, frequently metastasizing to the central nervous system (CNS), lung, liver, lymph nodes, and bones.¹ As a result, the disease is often not diagnosed until an advanced stage and the initial symptoms are often related to metastases. Cerebral metastases have varied presentations, ranging from asymptomatic or nonspecific, to focal deficits, to depressed mental status and impending herniation from hemorrhage.

Approximately 10% of choriocarcinoma metastasizes to the brain.² When this occurs, tumor hemorrhage is a major cause of morbidity.³ Numerous types of hemorrhage have been associated with choriocarcinoma including intratumoral and peritumoral hemorrhage, subarachnoid hemorrhage, intracerebral hematoma, subdural hematoma, and embolic vascular occlusion.⁴ Hemorrhage can occur when tumor cells invade vessel walls and cause intratumoral hemorrhage or partially invade vessel walls and develop an aneurysm.5, 6

Pure choriocarcinoma is rare, representing only 1% of testicular malignancies. As such, only a limited number of case reports documenting the clinical presentations and outcomes of its metastasis to the brain exist. Here, we present 2 cases of testicular choriocarcinoma and brain metastasis with divergent clinical courses. We include these cases with a systematic review of those described in the literature to provide an overview of the disease's presentation and treatment with an emphasis on potential pitfalls in diagnosis and management.