Journal of Computer Assisted Tomography 13(2):319-322, March/April © 1989 Raven Press, Ltd.. New York Case Report Computed Tomography and Angiographic Findings in Metastatic Choriocarcinoma Arthur S. Watanabe and Wendy R. K. Smoker Abstract: We report a patient with metastatic gestational choriocarcinoma who presented with headaches, speech difficulties, motor aphasia. facial weakness, hemiparesi and a large intraparenchymal hematoma. She subsequently de- veloped a total of five intraparenchymal hemorrhages. Her clinical presenta- tion posed a diagnostic dilemma and emphasizes the importance of a complete differential diagnosis of multiple intracranial hemorrhages. The features of CNS choriocarcinoma are reviewed. Index Terms: Brain, neoplasms—Brain, hemorrhage—Computed tomography. Gestational choriocarcinoma in the United States is an uncommon malignancy of conception occur- ring in ~1/50,000 term pregnancies and 1/30 hyda- tidiform moles (1). Among all cases of gestation re- lated choriocarcinoma, ~45% occur after a molar pregnancy, 24% after a normal term pregnancy, 25% after a spontaneous abortion, and 5% after an ectopic pregnancy (2). Gestational choriocarcinoma that follows hydatidiform mole usually is readily di- agnosed on the basis of rising or plateauing human chorionic gonadotropin (B-HCG) titers following evacuation of the uterus if the disease is suspected. Gestational choriocarcinoma, however, may present in subtle ways and be more difficult to di- agnose, especially if unsuspected. We report a pa- tient with metastatic gestational choriocarcinoma. The case presented a diagnostic dilemma and em- phasizes the importance of considering all differen- tial possibilities in patients who present with intra- cranial hemorrhage. CASE REPORT A 24-year-old. gravida 3 para 2, woman developed Rh isoimmunization at 19 weeks’ gestation, eventually ne- From the Department of Radiology, University of Utah, 50 North Medical Drive, Salt Lake City, UT 84132, U.S.A. Address correspondence and reprint requests to Dr. W. R. K. Smoker. 319 cessitating three intrauterine transfusions and a cesarean delivery at 35 weeks’ gestation. A 1,250 g placenta deliv- ered spontaneously and exhibited changes of erythroblas- tosis fetalis. Fourteen weeks post partum, the mother began to de- velop headaches and awoke one morning with difficulty in speaking. She presented to a local hospital with transcortical motor aphasia, right central facial weakness. and evolving hemiparesis. Unenhanced CT of the head demonstrated a large left frontal intraparenchymal he- matoma (Fig. 1) and she was admitted with a presumptive diagnosis of an arteriovenous malformation. Four vessel cerebral angiography was performed 10 days following the onset of symptoms. The left carotid injection revealed avascular mass effect due to the he- matoma. In addition, multiple areas of segmental vascular dilatation and narrowing, intraarterial thrombosis, and contrast extravasation were identified and believed to be compatible with a necrotizing vasculitis (Fig. 2). The right carotid injection demonstrated irregularity of a parietooc- cipital branch of the middle cerebral artery with a possi- ble aneurysm but no thrombosis or contrast extravasation (Fig. 3). The posterior circulation was normal. Suspected underlying etiologies at that time included subacute bac- terial endocarditis, polyarteritis nodosa, systemic lupus erythematosis, and a granulomatous vasculitis. Serum analysis demonstrated an antinuclear antibody titer of 1:1,280, rheumatoid factor of >60 IU, and Farr assay of 7%, Repeat CT 12 days after the initial scans and 2 days after angiography demonstrated a new right occipital hemorrhage in the distribution of the abnormal right mid- dle cerebral branch identified angiographically, as well as enlargement of the left frontoparietal hemorrhage (Fig. 4). 320 A. S. WATANABE AND W. R. K. SMOKER FIG. 1. Noncontrast CT scan of the head on the day of the initial hemorrhage demonstrates a large left frontal intrapa- renchymal hematoma. There is some mass effect and sur- rounding edema. Serial CT scans ultimately demonstrated a total of five separate supratentorial intraparenchymal hemorrhages. Her platelet count dropped to a low of 5,000/mm* from an admission value of 441,000/mm?*. Idiopathic thrombo- cytopenic purpura. unresponsive to steroids, was sus- pected and presumed to be an autoimmune phenomenon related to her phenytoin therapy. She underwent emer- gency splenectomy. Gross examination of the spleen revealed a single 1.0 x 1.0 x 0.5 cm gray-white parenchymal nodule. Micro- scopic evaluation of this nodule was reported to demon- strate choriocarcinoma. Evaluation for additional me- tastases. including lungs, liver, kidneys. bladder, pelvis. gastrointestinal tract, and cervix, was negative. Therapy began immediately with whole brain irradia- tion and simultaneous methotrexate. actinomycin D, and cyclophosphamide chemotherapy. Her pretreatment B-HCG titer was 52,728 mIU/ml. Forty-one days later, after the third course of chemotherapy, her B-HCG titer was negative. She received three additional full courses of chemotherapy after the negative B-HCG titer. More than | year later, her B-HCG titers have remained nega- tive and she has made a complete neurologic recovery. DISCUSSION Trophoblastic disease is thought to be due to un- controlled invasion of maternal uterine tissue and J Comput Assist Tomogr, Vol. 13, No. 2, 1989 maternal blood sinuses by trophoblastic tissues. The release of trophoblastic cells into the maternal venous circulation is thought by some to be a normal event. Accordingly, metastatic trophoblas- tic disease and choriocarcinoma are disseminated hematogenously with the most common sites of tu- mor origin and involvement being the uterus and lung. Intracranial metastases adversely affect the prog- nosis of the disease and are the leading cause of death in patients who die of choriocarcinoma. The incidence of CNS metastases has been reported to range between 3 and 28% (1). Clinically the presen- tation is nonspecific and patients may demonstrate headache, nausea, vomiting, hemiparesis, seizures, or neurological deficits. A small percentage of pa- tients demonstrate no symptoms. Half of those who die from intracranial metastases present with CNS disease and the other half are reported to develop CNS metastases after treatment. About 33-76% of patients with brain metastases have multiple le- sions. It has been reported that the location of intracra- nial metastases corresponds to the proportion of ce- rebral blood flow supplying each part of the brain with the majority of metastatic lesions occurring in the parietal lobe followed by the occipital, frontal, and temporal lobes. The brain stem and cerebellum are least frequently involved. A tendency for right hemispheric involvement has been reported. These metastases are noted for their propensity to produce hemorrhage and they present in 50% of the cases as intracerebral hemorrhages due to the tendency of the trophoblast cells to penetrate vas- cular walls (3). Intracranial metastases may be man- ifested as aneurysms, usually involving the middle cerebral artery or its branches. These aneurysms are usually solitary although occasionally they may be multiple. About 20% of patients with lung metastases also have CNS lesions. Many studies have reported that 95-99% of patients with brain metastases also have pulmonary metastases since the source of the brain lesions is thought to be the lungs. The average num- ber of metastatic sites in patients with intracranial involvement is four, with liver, kidney, gastrointes- tinal tract, spleen, and peritoneum frequently in- volved sites. Intracranial choriocarcinoma has been reported in one patient who had a possible genital source of metastatic disease, without evidence of trophoblastic disease removed prior to the clinical evidence of the metastatic process (3). Another pa- tient has been reported whose original tumor was thought to have disappeared and who subsequently died of metastatic disease (3). Primary CNS choriocarcinoma, although rare, has also been reported (4). It occurs predominantly in the pineal region or anterior third ventricular re- METASTATIC CHORIOCARCINOMA OF THE BRAIN 321 gion in a younger population (mean age 12 years). well recognized in the obstetrical literature, it is Males are more commonly affected, usually pre- poorly reported in the radiological literature and not senting with symptoms of precocious puberty (4.5). well emphasized in standard major cranial CT text- Although metastatic CNS choriocarcinoma is books (6,7). This case illustrates the importance of FIG. 2. a-c: Lateral views of the left carotid injection at 2, 3, and 6 s, respectively, reveal avascular mass effect due to the hematoma with depression of the Sylvian triangle, multiple areas of segmental vascular dilatation and nar- rowing, intraarterial thrombosis (arrows), and contrast ex- travasation (arrowheads). J Comput Assist Tomogr, Vol. 13, No. 2, 1989 322 A. S. WATANABE AND W. R. K. SMOKER FIG. 3. a, b; Lateral views of the right carotid injection demonstrate irregularity of a parietooccipital branch of the middle cerebral artery (arrowheads) with a possible aneurysm (arrows) without thrombosis or contrast extravasation [(b) is a magnifi- cation of the vessel identified in (a)]. considering all differential possibilities when a pa- tient presents with multiple intracranial hemor- rhagic lesions. In a young woman who has had a recent pregnancy, even without clinical suspicion of trophoblastic disease, and who presents with sub- arachnoid or intracerebral hemorrhage, choriocar- cinoma should be considered. REFERENCES 1. Athanassiou A, Begent RJH, Newlands ES, et al. Central nervous system metastases of choriocarcinoma. 23 years’ experience at Charing Cross Hospital. Cancer 1983;52:1728— 35, 2. Seigle JM, Caputy AJ, Manz HJ, Wheeler C, Fox JL. Mul- tiple oncotic intracranial aneurysms and cardiac metastasis from choriocarcinoma: case report and review of the liter- ature. Neurosurgery 1987;20:39-42. 3. van den Doel EMH, van Merrienboer FJJM, Tuleken CAF. Cerebral hemorrhage from unsuspected choriocarcinoma. Clin Neurol Neurosurg 1985;87:287-90. 4. Bjornsson J, Scheithauer BW, Leech RW. Primary intra- cranial choriocarcinoma: a case report. Clin Neuropathol 1986;5:242-S. §, Krishna Rao CVG, Govinadan S. Case report. Intracranial choriocarcinoma. J Comput Assist Tomogr 1979;3:400-4. FIG. 4. Repeat CT 12 days after the initial scan demonstrates 6. Lee SH, Rao KCVG. Cranial computed tomography. New a new right parietooccipital hemorrhage (dots) and enlarge- York: McGraw-Hill, 1983. ment of the left frontoparietal hemorrhage with increased 7. Haughton AL, Williams VM. Cranial computed tomogra- edema and mass effect. phy. A comprehensive text. St. Louis: CV Mosby, 1985. J Comput Assist Tomogr, Vol. 13, No. 2, 1989