Brain metastasis of choriocarcinoma in males. Apropos of a case. Review of the literature]

A 22 year-old male patient had a choriocarcinoma of the mediastinum metastatic to the brain, liver, lung and skin. He was treated with five cycles of chemotherapy containing cisplatinum, vinblastine, VP16 and bleomycin and he achieved a partial remission. Then he developed a progressive disease exclusively located to the brain and he died of an intracranial hemorrhage. The autopsy showed the mediastinum and the lung being free of residual trophoblastic tumor. Pure choriocarcinoma is rare in males, but brain metastases are frequently present in this case. Therapeutic guidelines are uncertain, so they must refer to the experience obtained in gestational choriocarcinoma. Two groups of patients are individualized both in male germ cell tumors and in placental choriocarcinoma: one group of patients with brain metastases at diagnosis, with a better prognosis, and one group of patients with brain metastases occurring in the course of the disease, with a poor outcome. The risk of intratumoral hemorrhage is common to all varieties of choriocarcinoma brain metastases and is not lowered by greater effectiveness of the chemotherapy. Brain metastases found at the moment of the choriocarcinoma diagnosis require chemotherapy and radiotherapy and in some selected cases, a surgical removal.     

Malignant Germ Cell Tumors Metastatic to the Brain: A Model for a Curable Neoplasm? The Freiburg Experience and a Review of the Literature

The aim of this study on malignant germ cell tumors metastasizing to the brain is (a) to report our institutional experience, (b) to present three patients surviving for more than seven years, and (c) to review the literature with regard to long-term survival.From 1985 to 2000, 916 consecutive patients were treated with whole brain radiation therapy for brain metastases at our hospital. Eleven patients had cerebral lesions from histologically proven malignant germ cell tumors. Brain metastases were diagnosed at presentation (n = 2), following complete remission (n = 3), or along with extracerebral tumor progression (n = 6). Seven patients had a single brain metastasis. Three patients underwent resection. Eight patients reached the planned total dose of 50Gy. Eight patients had chemotherapy.Median survival was 6.6 months. The long-term survivors all had an isolated cerebral relapse after complete remission, presented with a single brain metastasis, and were treated with resection and whole brain radiation therapy to a total dose of 50Gy. The first patient died from a late relapse 89 months after the diagnosis of brain metastasis, the second patient is well and alive at 95 months. The third patient is currently being treated for a second malignancy originating from the lung. He is alive at 194 months, the longest survival for brain metastases from malignant germ cell tumors ever reported.Altogether, our study demonstrates that advanced extracerebral disease at initial diagnosis and isolated cerebral relapse after complete remission do not preclude long-term survival. Resection and whole brain radiation therapy might result in durable cerebral control with minimal morbidity.     

Extragonadal retroperitoneal germ cell tumor: evidence of origin in the testis.

BACKGROUND: The origin of extragonadal retroperitoneal germ cell tumors remains controversial. Whether they develop primarily in the retroperitoneum or whether they are metastases of a primary testicular tumor has long been debated. PATIENTS AND METHODS: We retrospectively analyzed 26 patients treated as having primary extragonadal retroperitoneal germ cell tumors based upon the findings of testicular palpation by the referring physician. Testicular evaluation was then extended with ultrasonographical and histological examinations. RESULTS: Biopsy of the extragonadal tumor was performed in 25 patients, confirming diagnosis of extragonadal retroperitoneal germ cell tumor. Prior to treatment patients were clinically evaluated by several physicians and the testes were not considered suspicious for testicular cancer. At urological workup, testes were found to be atrophic and/or indurated in 14 (54%) patients, enlarged in one (4%) and unremarkable in 11 (42%). Ultrasound examination of the testes in 20 patients showed pathological findings in all of them. Histology of the testis was available in 25 of 26 patients and revealed active tumor in three, intratubular germ cell neoplasia in four, scar tissue in 12, sclerosis in three, sclerosis and fibrosis in one, and fibrosis alone in two. CONCLUSIONS: So-called primary extragonadal germ cell tumors in the retroperitoneum are very likely a rare or non-existing entity and should be considered as metastases of a viable or burned-out testicular cancer until proven otherwise. All of our patients with histologically examined testes had pathological finding, 76% of which were either viable tumor or scars.     

Klinefelter's syndrome and extragonadal germ cell tumors

A 25-year-old man presented with diffuse metastatic pure choriocarcinoma, thyrotoxicosis, and cardiac tamponade. No discernable testicular primary tumor was found. The patient's peripheral blood karyotype was 47, XXY and phenotypic features of Klinefelter's syndrome were present. The patient was treated with aggressive combination chemotherapy followed by salvage surgery and remains in complete remission 3 years after diagnosis. Pure choriocarcinoma, although rare as a primary testicular neoplasm, accounts for 15% of extragonadal germ cell tumors in general and 30% of germ cell tumors in patients with Klinefelter's syndrome. Historically, the diagnosis of pure choriocarcinoma has been thought to convey a very poor prognosis. The occurrence of hyperthyroidism is unique to tumors containing choriocarcinomatous elements and the management of this disorder is discussed. Treatment of extragonadal germ cell tumors is also discussed with special reference to the roles of combination chemotherapy and salvage surgery.     

Primary retroperitoneal pure choriocarcinoma. Two long-term complete responders from a rare fatal disease

Extragonadal germ cell tumors may have a worse prognosis than germ cell tumors of gonadal origin, even when tumor bulk and extent of dissemination are equal. Primary retroperitoneal pure choriocarcinoma is one of the least common subgroups of the extragonadal germ cell tumors and has previously appeared to have the worst prognosis. Two new case reports on the treatment and follow-up of two patients with primary retroperitoneal pure choriocarcinoma are discussed. Both patients are disease-free at 24 and 81 months, respectively. After aggressive primary multiagent drug therapy for primary retroperitoneal pure choriocarcinoma, a favorable prognosis may be anticipated.     

Extragonadal germ cell tumors. Clinicopathologic findings and treatment experience in 12 patients

In patients with primary germ cell tumors, treatment with combination chemotherapy followed by surgical debulking of residual tissue usually produces favorable results. The best treatment for patients with extragonadal germ cell tumors (EGCT) remains a problem. In our series of 12 patients, important clinical features were related to the site of bulky tumor, and all patients exhibited sharply elevated levels of lactate dehydrogenase (LDH), beta subunit human chorionic gonadotropin (beta-HCG), and/or alpha-fetoprotein (AFP). Each patient was treated with systemic chemotherapy, and ten were treated with the same combination chemotherapy--cyclophosphamide, actinomycin, vinblastine, bleomycin, and cisplatin (VAB) alternating with VP-16 and vincristine (VV). Of these ten patients, five died of progressive disease, three of whom had brain metastases. The other five are alive and clinically free of disease. The addition of VP-16 and vincristine did not improve responses. Advanced disease at presentation contributes to the poorer prognosis for these patients. Earlier diagnosis and surgical debulking may improve the long-term survival of patients with this disease.     

Hematologic disorders associated with primary mediastinal nonseminomatous germ cell tumors

BACKGROUND. The association between primary germ cell tumors of the mediastinum (the space between the lung pleura that contains the heart and other chest viscera) and hematologic malignancies has been described by retrospective analysis of patients treated at individual clinical centers. To better characterize the risk of hematologic disorders in patients with extragonadal germ cell tumors and to describe the clinical and biologic features of the disorders, we studied an unselected population in a large, international, multicenter database. METHODS. Six hundred thirty-five patients treated at 11 centers in the United States and Europe from 1975 through 1996 were evaluated retrospectively. RESULTS. A hematologic disorder was observed in 17 patients with germ cell tumors. All cases developed among the 287 patients with primary mediastinal nonseminomatous germ cell tumors, giving an incidence rate in this group of 2.0% (95% confidence interval [CI] = 1.1%-3.1%) per year over a median follow-up time of 3 years. The risk of developing hematologic disorders was statistically significantly increased in patients with primary mediastinal nonseminomatous germ cell tumors in comparison with the age-matched general population (standardized incidence ratio = 250; 95% CI = 140-405). The median time to onset of hematologic neoplasia was 6 months (range, 0-47 months), and the median survival after diagnosis of the hematologic disorder was 5 months (range, 0-16 months) (two-sided P<.0001, comparing survival from the time of diagnosis of the germ cell tumor of patients with and without hematologic disorders). CONCLUSION. In our study, approximately one in 17 patients with primary mediastinal nonseminomatous germ cell tumors was affected by a hematologic disorder, whereas no cases were seen among 334 patients with other extragonadal germ cell tumors. The hematologic disorder had a statistically significant impact on prognosis, with none of the 17 reported patients surviving for more than 2 years.     

Neurological complications of malignant germ cell tumors of testis: biology of brain metastases (I).

Central nervous system metastases are a common complication of disseminated germ cell tumors of the testis. They occurred in 16% of 242 patients treated and in 25% of the patients who died in our VAB chemotherapy series. Pulmonary metastases preceded or coincided with the development of brain metastases. The frequency of brain metastases differed with the histology of the primary tumor. They occurred in 13% of pure embryonal carcinomas, 18% of mixed tumors containing embryonal or choriocarcinoma elements, and 83% of pure choriocarcinomas. Embryonal carcinoma and choriocarcinoma were the principle histologies found in brain metastases. Characteristically, pure choriocarcinoma deposits in the brain were multiple (8/9) and cerebellar involvement was common (5/9). Pure embryonal carcinoma CNS metastases were typically single (6/8) or very few and cerebellar involvement was not observed. The interval from the diagnosis of malignancy to the diagnosis of brain metastases was longer for embryonal carcinoma than for pure choriocarcinoma (23 mos. vs. 6.5 mos.). Survival following the diagnosis of brain metastases was poor. There was a tendency toward longer survival for histologically pure embryonal carcinoma deposits in the brain than for the pure choriocarcinomas (6.5 mos. vs. 1 mo.).     

Germ cell tumor of the colon with an adenocarcinomatous component

A case of combined germ cell tumor and adenocarcinoma that arose in the colon of a 62-year-old man is described. The clinical and pathological findings are presented. The patient had widespread metastases at diagnosis and poor prognosis after operation (right hemicolectomy) was performed in spite of receiving chemotherapy. Pathologically, the germ cell tumor was composed of a yolk sac tumor and choriocarcinoma. Further, all the metastatic lesions showed a germ cell phenotype. An extragonadal germ cell tumor is extremely rare. To our knowledge, only a few cases have been reported in the English-language literature. Our present report will contribute to the understanding of the characteristics of this rare neoplasm.     

Extragonadal and poor risk nonseminomatous germ cell tumors. Survival and prognostic features.

One hundred forty-nine patients with poor risk nonseminomatous germ cell tumors (NSGCT) treated between 1975 and 1988 were studied. Patients were considered poor risk if they had an extragonadal primary site or testicular NSGCT with low predicted probability of achieving a complete response (CR). Primary sites were the testis (99 patients), retroperitoneum (18 patients), and mediastinum (32 patients). Patients with mediastinal NSGCT had lower human chorionic gonadotropin (HCG) (P less than 0.0001) and lactate dehydrogenase (LDH) levels (P less than 0.0001), and more frequent yolk sac elements (P = 0.002). CR rates were 38% for mediastinal, 61% for retroperitoneal, and 38% for testicular primary sites. Mediastinal NSGCT patients more frequently required resection of residual malignancy to attain a CR (6 of 12). Mediastinal NSGCT had the worst event-free survival (P = 0.02). Cox regression analysis identified brain or liver metastases as the most important predictor of event-free survival in poor risk patients. Retroperitoneal NSGCT often have a poor outcome due to advanced presentation, but the likelihood of a CR to therapy can be predicted using criteria applicable to testicular primary tumors. Therefore, not all retroperitoneal NSGCT are poor risk, and retroperitoneal tumors are probably of occult testicular origin. Mediastinal NSGCT have distinct clinical and pathologic features, do not respond as well to chemotherapy, relapse more frequently, and have the worst survival. The likelihood of a CR cannot be predicted using criteria developed for primary testicular tumors, suggesting that mediastinal primary NSGCT is a distinct clinical entity.     

VAB-5 combination chemotherapy in prognostically poor risk patients with germ cell tumors

Forty-four patients with nonseminomatous germ cell tumors with "poor prognostic features" were entered on the VAB-5 regimen and 38 are evaluable. VAB-5 represents an intensified version of the VAB-4 protocol. Poor prognostic features were considered to be bulky metastases ( greater than 5 cm in diameter), palpable retroperitoneal disease, liver metastases, brain metastases, involvement of two or more parenchymal organs, pure choriocarcinoma, alpha fetoprotein or human chorionic gonadotropin serum levels over 1000 ng/ml, lactic acid dehydrogenase serum levels over 400 mg/dl, and failure to prior chemotherapy. Eighteen of 38 evaluable patients became free of neoplasm, 11 with chemotherapy alone, and seven with combined chemotherapy and surgery. Fourteen of 18 complete responders remain alive and free of disease with a median follow-up of 50 months. Complete remission with testis tumor occurred in 13/15 without and 5/15 with prior chemotherapy and in none of eight patients with primary extragonadal germ cell tumors. Thirty-one patients received antibiotics when they developed fever during myelosuppression. Ten patients developed transient serum creatinine levels over 2 mg/dl after cis-platinum and one required hemodialysis with subsequent recovery. All patients had severe mucositis after induction. An apparent improvement of results over prior VAB protocols in patients with poor prognostic features was compromised by significant increases in toxicity and such patients require special study to improve cure rates.     

Chemotherapy of extragonadal germ cell tumors

Forty-nine patients with histologically proven germ cell tumors arising in extragonadal sites were retrospectively reviewed. Included in the review were an additional seven patients with undifferentiated tumors with a pathologic appearance compatible with that of a germ cell tumor and elevated levels of serum biomarkers (beta subunit of human chorionic gonadotropin [beta-HCG] +/- alpha-fetoprotein [AFP]. Nineteen patients had a pure seminoma arising in an extragonadal site, whereas 30 patients had nonseminomatous germ cell tumors. Seven patients had primary undifferentiated tumors with elevated levels of serum biomarkers. Sixteen (84%) of the 19 patients with pure extragonadal seminomas with normal levels of serum AFP are alive and free of disease. Eighteen of these 19 patients received platinum-containing regimens and four had received prior chemotherapy that failed. Of the patients with nonseminomatous germ cell tumors, 12 (40%) of the 30 are alive and free of disease with vinblastine/bleomycin +/- cisplatin (13 patients) or CISCAII (cisplatin, cyclophosphamide, and doxorubicin) (nine patients) alternating CISCAII/VBIV (eight patients) chemotherapy. None of the seven patients with undifferentiated germ cell tumors are alive and free of disease. Three of the five patients with pure anterior mediastinal endodermal sinus tumors treated with chemotherapy remain alive and free of disease. Of the seven patients with choriocarcinomas arising in extragonadal sites, three are alive and free of disease. A classification for patients with extragonadal germ cell tumors incorporating site of origin, histology, and likelihood of being truly extragonadal is proposed. The implications of this classification are discussed.     

Metastatic testicular cancer and extragonadal germ cell tumor presenting with neurological symptoms

Summary We present two patients with testicular cancer and extragonadal germ cell tumor respectively in whom neurological symptoms due to metastases preceded the correct diagnosis. Testicular cancer and extragonadal germ cell tumor are today curable malignancies even when distant metastases are present at diagnosis. The diagnosis of germ cell tumor should be considered whenever a young man presents with metastases of uncertain origin, and all histological specimens should be revised with the diagnosis of germ cell tumor in mind.     

The abnormal occurrence and the differentiation-dependent distribution of N-acetyl and N-glycolyl species of the ganglioside GM2 in human germ cell tumors. A study with specific monoclonal antibodies

Human primary germ cell tumors were analyzed for the presence of the ganglioside GM2 using three specific monoclonal antibodies which can distinguish the molecular species of the sialic acid moiety: the antibody MK1-16 is specific for N-acetyl GM2, MK2-34 is specific for N-glycolyl GM2, and MK1-17 detects both N-acetyl and N-glycolyl GM2. When the occurrence of the GM2 antigen was tested in 107 cases of human germ cell tumors by the immunohistochemical technique using these antibodies, seminoma was characterized as having the highest frequency of N-acetyl GM2 (89.4%, 42 of 47 cases) among germ cell tumors, followed by embryonal carcinoma (40.0%), and teratocarcinoma (26.6%). Compared with this, yolk sac tumors and choriocarcinoma had a much lower positive incidence of the N-acetyl GM2 antigen. On the other hand, the N-glycolyl GM2 antigen was not found at all in 47 cases of seminoma (0%), and the positive incidence was very low in embryonal carcinoma (6.6%), although considerably higher incidences were obtained with choriocarcinoma (25.0%), yolk sac tumor (22.2%), and teratocarcinoma (13.3%). The presence and molecular species of the GM2 antigens in these human germ cell tumors were also ascertained chemically by the thin-layer chromatography (TLC) immunostaining of the ganglioside fractions prepared from primary germ cell tumors. These results indicate that seminoma specifically contains N-acetyl GM2 and no N-glycolyl GM2, suggesting that N-acetyl GM2 could be a good marker for seminoma. On the other hand, non-seminomatous germ cell tumors were characterized by the presence of N-glycolyl GM2, one of the Hanganutziu-Deicher antigens (H-D antigens). Moreover, the positive occurrence of N-glycolyl GM2 correlated very well with the degree of differentiation of non-seminomatous germ cell tumors, i.e., the differentiated tumors such as yolk sac tumors, choriocarcinoma, and teratocarcinoma had a higher positive incidence of N-glycolyl GM2 type H-D antigen but a lower positive incidence of N-acetyl GM2 when compared with embryonal carcinoma, the most undifferentiated tumors among non-seminomatous germ cell tumors.     

Extragonadal germ cell tumors of the mediastinum and retroperitoneum: results from an international analysis.

PURPOSE: To characterize the clinical and biologic features of extragonadal germ cell tumor (EGCT) and to determine the overall outcome with currently available treatment strategies. PATIENTS AND METHODS: Of an unselected population of 635 consecutive patients treated from 1975 through 1996 at 11 cancer centers, 341 patients (54%) had primary mediastinal EGCT, and 283 patients (45%) had retroperitoneal EGCT. Five hundred twenty-four patients (83%) had a nonseminomatous germ cell tumor (GCT), and 104 patients (16%) had a seminomatous histology. RESULTS: After platinum-based induction chemotherapy with or without secondary surgery, 141 patients (49%) with mediastinal nonseminomas (median follow-up, 19 months; range, 1 to 178 months) and 144 patients (63%) with retroperitoneal nonseminoma (median follow-up, 29 months; range, 1 to 203 months) are alive (P =.0006). In contrast, the overall survival rate for patients with a seminomatous EGCT is 88%, with no difference between patients with mediastinal or retroperitoneal tumor location (median follow-up, 49 months; range, 4 to 193 months; respective 70 months; range, 1 to 211 months). A significantly lower progression-free survival rate was found in seminoma patients treated with initial radiotherapy alone compared with chemotherapy. Nonseminomatous histology, presence of nonpulmonary visceral metastases, primary mediastinal GCT location, and elevated beta-human chorionic gonadotropin were independent prognostic factors for shorter survival. Hematologic malignancies (n = 17) occurred without exception in patients with primary mediastinal nonseminoma. Sixteen patients developed a metachronous testicular cancer despite the use of platinum-based chemotherapy. CONCLUSION: Whereas patients with pure seminomatous EGCT histology have a long-term chance of cure of almost 90% irrespective of the primary tumor site, 45% of patients with mediastinal nonseminomas are alive at 5 years. This outcome is clearly inferior compared with patients with nonseminomatous retroperitoneal primary tumors.     

Bilateral testicular germ cell tumors: twenty-year experience at M. D. Anderson Cancer Center

BACKGROUND: The incidence of testicular carcinoma in the United States has increased significantly over the last two decades. Germ cell tumors form the majority of malignant testicular tumors. With advances in diagnosis and therapeutic approaches, germ cell tumors are now highly sensitive to treatment, providing long-term survival. It has been speculated that the incidence of bilateral germ cell tumors may increase due to the improved survival of patients with unilateral germ cell tumors. In this report, the authors present a study of bilateral germ cell tumors of the testis in men who were treated at The University of Texas M. D. Anderson Cancer Center over a 20-year period with emphasis on their incidence, histologic features, and clinical features. METHODS: Between 1978 and 1999, 2431 patients with testicular germ cell tumors were treated at The University of Texas M. D. Anderson Cancer Center. Among these, 24 patients with bilateral germ cell tumors were identified. Clinical records and all available pathology slides of the tumors were reviewed. RESULTS: The overall incidence of bilateral germ cell tumors in the patients with testicular germ cell tumors was 1% (24 of 2431 patients). The incidence was 1.8% (14 of 776 patients) in patients with seminoma and 0.6% (10 of 1655 patients) in patients with nonseminomatous germ cell tumors. Patients with seminoma who were age 相似文献   

A randomized trial of standard chemotherapy v a high-dose chemotherapy regimen in the treatment of poor prognosis nonseminomatous germ-cell tumors

We performed a prospective randomized trial of a high-dose chemotherapy regimen v standard cisplatin-based chemotherapy in poor prognosis nonseminomatous germ-cell cancer patients. The high-dose regimen consisting of twice the standard dose of cisplatin (P), along with vinblastine (Ve), bleomycin (B), and the epipodophylotoxin etoposide (VP-16) (V) (PVeBV) was compared to the classic regimen with normal dose cisplatin, vinblastine, and bleomycin (PVeB). Eligibility criteria included large abdominal masses, liver metastases, multiple pulmonary metastases, brain metastases, marked elevations in serum tumor markers (alpha-fetoprotein greater than 1,000 ng/mL or the beta-subunit of human chorionic gonadotropin greater than 10,000 mIU), unfavorable histology (pure choriocarcinoma), or extragonadal germ-cell tumors. Fifty-two consecutive patients with poor prognostic features were randomized to receive either PVeBV or PVeB. The median follow-up is 4 years. Treatment with the high-dose regimen increased the complete remission rate (88% v 67%, P = .14) and was associated with a lower relapse rate (17% v 41%, P = .2). The median survival of patients receiving standard therapy was 30 months, while the median survival for patients receiving the high-dose regimen has not been reached. Actuarial 5-year survival for patients treated with the high-dose regimen is 78%, compared with 48% for patients receiving standard therapy (two-sided Mantel-Cox test = .06). Disease-free survival was also superior for patients randomized to PVeBV (P = .03). Sixty-eight percent of patients (23 of 34) randomized to PVeBV are alive and continuously disease-free, compared with 33% (six of 18) for PVeB (P = .02). The major difference in toxicity between the high-dose regimen and standard therapy was the severity of myelosuppression and the incidence of severe hearing loss. Ninety-one percent of patients treated with PVeBV had a WBC count less than 1,000/microL, compared with 50% of patients receiving PVeB (P less than .05). Hearing aids were recommended for 12 patients who received PVeBV and two who received PVeB. The increased effectiveness of the PVeBV regimen in poor prognosis germ-cell cancer patients may relate to the double-dose cisplatin, the addition of VP-16, or to a synergistic effect of these two drugs.     

Epidemiology of Brain Metastases

Brain metastases are one of the most common neurologic complications of cancer. The incidence is 9%–17% based on various studies, although the exact incidence is thought to be higher. The incidence is increasing with the availability of improved imaging techniques which aid early diagnosis, and effective systemic treatment regimens which prolong life, thus allowing cancer to disseminate to the brain. Lung cancer, breast cancer, and melanoma are the most frequent to develop brain metastases, and account for 67%–80% of all cancers. Most patients with brain metastases have synchronous extracerebral metastases. Some patients present with no known primary cancer diagnosis. In children, brain metastases are rare; germ cell tumors, sarcomas, and neuroblastoma are the common offenders.     

结直肠癌脑转移瘤的单次SRS与多分次SRT治疗比较研究

目的:对比多分次立体定向放射治疗(SRT)及单次立体定向放射外科(SRS)治疗结直肠癌脑实质转移瘤的疗效及不良反应。方法:检索上海市抗癌协会脑转移瘤专业委员会数据库纳入的结直肠癌脑实质转移瘤患者98例,其中行SRT者46例,行SRS者52例。分析两组患者的临床特征并比较两组的局部肿瘤控制时间、中位生存期、放射性脑损伤发...     

Chemotherapy trials in recurrent primary intracranial germ cell tumors

Summary Gonadal germ cell tumors respond favorably to chemotherapy either at diagnosis or when they recur. Histologically similar tumors may arise in the CNS usually in the pineal or suprasellar regions. Although radiation therapy may produce a 5 year disease-free survival in excess of 60% in localized pure germinoma, gern cell tumors of other histology tend to recur. We have conducted 14 chemotherapy trials in 8 patients with recurrent CNS germ cell tumors using 3 different single agent and 2 multi-agent chemotherapy regimens. The histologic diagnoses of the patients were germinoma (4), endodermal sinus tumor (2), embryonal carcinoma (1), and mixed tumor — germinoma plus choriocarcinoma (1). There were 7 males and 1 female with a median age of 13 years. The primary tumor arose in the pineal region in 6 and was multicentric in 2. Seven patients had local recurrences and one developed an initial recurrence in the spinal canal. Three patients had CNS metastases at relapse and 2 had systemic metastases. Objective responses were documented in 7 of 14 trials (50%). Responses were observed with cyclophosphamide (80 mg/kg) in 3 of 4 patients for 2⁺, 3, and 5 mos, cisplatin (120 mg/m²) in 1 of 2 patients for 2⁺ mos, and the VAB 6 protocol (vinblastine, bleomycin, cyclophosphamide, actinomycin-d, cisplatin) in 3 of 5 patients for 5, 8, and 18 mos. The median duration of response was 5 mos. (2¹-18). High doses of single chemotherapy agents such as cyclophosphamide and cisplatin as well the VAB6 regimen have definite activity in recurrent CNS germ cell tumors, especially germinoma. Good palliation may be achieved with chemotherapy alone with acceptable morbidity. Adjuvant chemotherapy should be considered in patients with newly diagnosed primary intracranial germ cell tumors whose tumors are considered unlikely to be permanently controlled with radiation alone.