Literature ReviewPitfalls in Diagnosis and Management of Testicular Choriocarcinoma Metastatic to the Brain: Report of 2 Cases and Review of Literature
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.
Section snippets
Methods
Medline was searched for all publications in the past 40 years including the terms “testicular choriocarcinoma” and “cerebral metastasis” or “brain metastasis.” Articles were included for analysis only if they included cases with histologically proven testicular choriocarcinoma. Articles that included patients with tumors classified as a mix of choriocarcinoma and other GCT subtypes were excluded because these are known to behave differently than pure choriocarcinoma. Articles without a version
Results
The initial search yielded 38 papers that reported a total of 49 cases. Fifteen papers were excluded because they only reported on cases in which the histologic diagnosis was a mix of choriocarcinoma and other GCT subtypes. Two papers were excluded because they did not provide information on patient outcome. Eight papers were excluded because they were not available in English.
A total of 15 cases from the literature and our own 2 previously described cases were included in the analysis (Table 1
Discussion
Choriocarcinoma is a malignant germ cell tumor that arises from trophoblastic tissue, as well as germinal epithelium of placenta, ovaries, and testes. It is characterized by early hematogenous spread to the lungs, liver, kidney, and brain.6, 21 Similar to the 2 cases we have presented, the tumor has frequently already metastasized at the time of diagnosis. Choriocarcinoma metastases are frequently hemorrhagic and carry a poor prognosis.22 Although the brain is not an uncommon site for
Conclusion
The 2 cases we have presented along with our review of the literature illustrate the propensity for cerebral metastases of pure testicular choriocarcinoma to present with acute hemorrhage and lead to poor outcomes when not accurately diagnosed and surgically intervened upon early. The presentation of hemorrhagic choriocarcinoma may initially mimic that of an occult vascular malformation. Accurate diagnosis and prompt neurosurgical intervention greatly improve the short-term outcome.
References (36)
Nonseminomatous germ cell tumors of the testis: current concepts and controversies
Urology
(1994)- et al.
Delayed effects of whole brain radiotherapy in germ cell tumor patients with central nervous system metastases
Int J Radiat Oncol Biol Phys
(2008) - et al.
Abnormal seminiferous tubule cells associated with choriocarcinoma: immunoperoxidase study
Urology
(1979) - et al.
Brain metastasis of choriocarcinoma
Surg Neurol
(1982) - et al.
Implications for the pathogenesis of aneurysm formation: metastatic choriocarcinoma with spontaneous splenic rupture. Case report and a review
Surg Neurol
(1992) - et al.
Pathogenesis of gestational trophoblastic neoplasms
Pathobiol Annu
(1981) Intracranial hemorrhage caused by metastatic tumors
Neurology
(1977)- et al.
Central nervous system metastases of choriocarcinoma. 23 years' experience at Charing Cross Hospital
Cancer
(1983) - et al.
Metastatic choriocarcinoma with neoplastic aneurysms cured by aneurysm resection and chemotherapy. Case report
J Neurosurg
(1994) - et al.
Intracranial vascular complications of choriocarcinoma
Neurosurgery
(1978)
Upper gastrointestinal bleeding as an initial manifestation of metastasis secondary to choriocarcinoma
Turk J Gastroenterol
Pericardial tamponade caused by tumor hemorrhage—a rare complication of metastatic testicular choriocarcinoma
Pathol Oncol Res
Metastatic choriocarcinoma induced separate simultaneous intracerebral haemorrhages: a very rare occurrence and its novel association with Klinefelter syndrome
BMJ Case Rep
Metastatic testicular choriocarcinoma: a rare cause of upper GI bleeding
ACG Case Rep J
Hemorrhagic collision metastasis in a cerebral arteriovenous malformation
J Neurointerv Surg
Clinical reasoning: a 25-year-old man with headaches and collapse
Neurology
Neurological picture. Metastatic choriocarcinoma
J Neurol Neurosurg Psychiatry
Brain metastases in children and adolescents with extracranial germ cell tumor—data of the MAHO/MAKEI-registry
Klin Padiatr
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Conflict of interest statement: This research did not receive any specific grant from funding agencies in the public, commercial, or not-for-profit sectors.