Cases reported "Germinoma"

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1/62. De novo development of a cavernous malformation of the spinal cord following spinal axis radiation. Case report.

    Analysis of recent reports has suggested that cavernous malformations (CMs) of the brain may have an acquired pathogenesis and a dynamic pathophysiological composition, with documented appearance of new lesions in familial cases and following radiotherapy. The authors report the first case of demonstrated de novo formation of an intramedullary CM following spinal radiation therapy. A 17 year-old boy presented with diabetes insipidus and delayed puberty. Evaluation of endocrine levels revealed hypopituitarism, and magnetic resonance (MR) imaging demonstrated an infundibular mass. The patient underwent a pterional craniotomy and removal of an infundibular germinoma. The MR image of the spine demonstrated normal results. The patient received craniospinal radiation therapy and did well. He presented 5 years later with acute onset of back pain, lower-extremity weakness and numbness, and difficulty with urination. An MR image obtained of the spine revealed an intramedullary T-7 lesion; its signal characteristics were consistent with a CM. The patient was initially managed conservatively but developed progressive myelopathy and partial brown-sequard syndrome. Although he received high-dose steroids and bed rest, his symptoms worsened. He underwent a costotransversectomy and excision of a hemorrhagic vascular lesion via an anterolateral myelotomy. Pathological examination confirmed features of a CM. The patient has done well and was walking without assistance within 4 weeks of surgery. De novo genesis of CMs may be associated with prior radiation therapy to the spinal cord.
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2/62. Intracranial germ cell tumors in children with and without down syndrome.

    PURPOSE: Two Chinese children with down syndrome affected by intracranial germ cell tumors are described. Because they represent two of eight affected patients in the current series from 1990 to 1996, it is postulated that such occurrence may be more than a coincidental event. patients AND methods: Two children with down syndrome developed germ cell tumors in atypical intracranial sites that affected basal ganglion and cerebellum. The pathology showed germinoma and yolk sac tumor, respectively. These were treated by radical surgical resection and chemotherapy with cisplatin, etoposide, and bleomycin, but without radiotherapy. RESULTS: One patient survived 3 years without radiologic evidence of tumor. The other died from infective complications caused by severe myelosuppression after chemotherapy. CONCLUSIONS: Subtle neurologic manifestations in developmentally handicapped patients with intracranial space-occupying lesions could result in delayed diagnosis. Children with down syndrome suffering from brain tumors may have a higher chance for germ cell tumors. Assay for alpha-fetoprotein and beta-human chorionic gonadotrophin could hasten diagnosis in some cases. This observation and review of literature suggest an increased risk of developing intracranial germ cell tumors in subjects with down syndrome.
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3/62. Mixed germ cell tumor presenting as intratumoral hemorrhage: report of a case originated from the pineal region.

    A 17-year-old male patient was brought to our clinic because of sudden onset of headache, vomiting, followed by transient loss of consciousness during a strenuous exercise. Neurologic examinations revealed that the patient had severe sensorimotor and brain stem dysfunction. Examinations of cranial CT and MR imaging showed a huge heterogeneously enhanced tumor originated from the pineal region with tumoral hemorrhage. The tumor markers were found to be high in AFP but not the beta-HCG and CEA. A clinical diagnosis highly suggestive of germ cell tumor was made. Prior to the planned emergency radiation therapy, he received an external ventricular drainage (EVD) and open biopsy of the tumor. Due to a postoperative complication of cerebellar hemorrhage observed 8 hours later, another maneuver was therefore required to extirpate the pineal tumor and cerebellar hematoma. The histological diagnosis proved to be a mixed germ cell tumor with tumoral hemorrhage. Spontaneous intratumoral hemorrhage in germ cell tumor of the pineal region is rare, probably due to compromised venous circulation within the tumor. The bleeding propensity, which may contribute to the formation of cerebellar hematoma, warrants a special attention when a biopsy procedure is to be performed.
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4/62. Treatment of intracranial nongerminomatous germ-cell tumor by high-dose chemotherapy and autologous stem-cell rescue.

    Nongerminomatous germ-cell tumor (NGGCT) in the central nervous system (CNS) is still highly lethal. The present study evaluated the outcome of high-dose chemotherapy followed by autologous stem-cell rescue (ASCR). The patients included three cases of choriocarcinoma, two cases of embryonal carcinoma and one case of yolk sac carcinoma. High-dose cisplatin (200 mg/m2), etoposide (1250 mg/m2) and ACNU (150 mg/m2) were administrated in combination with ASCR to patients at complete remission as a result of surgical removal, irradiation, and from four to seven courses of induction chemotherapy. All the patients treated with this therapy were alive from one to seven years after the diagnosis, living with good performance status. The patients have not required any additional treatments after ASCR. The myelosuppression period, characterized by fewer than 500/microl peripheral neutrophils, ranged from 8 to 15 days (median, 11.5 days). Within seven days of ASCR, high fever was found in four patients. Although mild liver dysfunction was found in all patients, renal dysfunction was not observed. hearing disturbance was found in 50% of the patients. This treatment regime will improve long-term survival for patients with NGGCT.
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keywords = central nervous system, nervous system
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5/62. Mixed germ cell tumour of the pineal region: a case report.

    An intracranial mixed germ cell tumour with germinoma and teratoma components is reported. The patient presented with parinaud's syndrome and precocious puberty. The treatment involved partial surgical debulking followed by whole brain radiotherapy (4500 cGY in 25 fraction over 5 weeks) and chemotherapy (consisting of cisplatin and etoposide). Post treatment MRI showed no residual lesion. The controversies in the management are discussed.
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6/62. Facilitation of ipsilateral motor pathways during recovery from hemiplegia in two adolescent patients.

    In two hemiplegic patients with acquired cerebral lesions, transcranial magnetic stimulation (TMS) was carried out to examine the contribution of the ipsilateral motor pathways to recovery from hemiplegia. A 13-year-old girl (patient 1) had acute hemiplegia due to a rupture of an arteriovenous malformation, and a 13-year-old boy (patient 2) had subacute hemiplegia due to a brain tumour. They showed complete upper limb palsy but recovered after therapy; patient 1 had slightly disabled motor function of the arm, and patient 2 recovered completely. Motor evoked potentials (MEPs) were recorded from the biceps brachii muscles on both sides. The MEPs of the paretic biceps were only elicited by TMS of the intact hemisphere at the beginning of recovery from hemiplegia, but not by TMS of the affected hemisphere. The MEP amplitudes increased and cortical representation areas for the paretic biceps by TMS were enlarged temporarily during recovery. They regressed in patient 1 and MEPs were not evoked at all in patient 2 after recovery. Conversely, MEPs were obtained by TMS of the affected hemisphere after recovery in both patients. These data indicate that ipsilateral motor pathways play a role in recovery from hemiplegia, especially at the beginning, and become inactivated when the contralateral motor pathways recover.
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7/62. recurrence of sellar and suprasellar tumors in children treated with hGH--relation to immunohistochemical study on GH receptor.

    PURPOSE: GH replacement therapy is required in the majority of children with GH deficiency after treatment of sellar and suprasellar tumors. Owing to the high cell proliferative ability of human GH (hGH), its influence on tumor recurrence has been debated. We retrospectively studied the immunohistochemical expression of the GH receptor in various tumor tissues, in order to investigate the relation between tumor recurrence and hGH replacement. methods: GH replacement therapy was performed in 25 patients (8 boys and 17 girls) after the treatment. Tumor recurrence was noted in 4 patients (craniopharyngioma: 2 patients, pilocytic astrocytoma and germinoma: 1 each). Immunohistochemical study of GH receptor in tumor tissue was carried out in those recurrent and recurrence-free cases, by using MAb 263 as a primary antibody. RESULTS: Two patients with recurrent craniopharyngioma were positive for MAb 263, but 1 recurrence-free patient was negative. patients with pilocytic astrocytoma (recurrent and recurrence-free: 1 each) were all positive. Five patients with germinoma (1 with recurrence and 4 without recurrence) were all negative. CONCLUSION: In the patients with craniopharyngioma treated with GH, a positive immunohistochemical expression of GH receptor in tumor tissue may indicate a high probability of recurrence. In our cases, GH receptor was positive in astrocytomas and negative in germinomas, with or without recurrence. It is therefore speculated that each brain tumor may have its specificity in GH receptor expression.
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8/62. Delayed resolution of intracranial germinoma after radiotherapy: a preliminary study of the correlation between histology and magnetic resonance imaging.

    A central nervous system germinoma is curable in most cases by radiotherapy, and most of the tumor mass disappears promptly after 20-30 doses. However, some germinomas take a longer period to vanish completely from magnetic resonance imaging (MRI) or computed tomography (CT) scans. In such cases, the tumor may contain components such as teratoma. The aim of this study was to clarify the nature of the persistence of some germinomas. Five cases of histologically verified germinoma in which radiotherapy was performed to treat residual tumor were selected. The doses of focal radiotherapy and whole brain radiotherapy were 10-20 Gy and 20-34 Gy, respectively. In these cases, correlation was made between the degree of persistence of the tumor when assessed by MRI and the amount of interstitial content, as determined by histology. The histological evaluation, using hematoxylin-eosin stain, silver impregnation and Azan staining was carried out independently of clinical information. The tumor vanished soon after radiotherapy in three cases, but 3-15 months passed before the tumor completely vanished from the MRI scans in the other two cases. The histology of the cases in which the tumor disappeared rapidly was predominantly of large tumor cells and only small amounts of reticulin. However, in the persistent tumors, large amounts of reticulin and vascular components were present. Thus, it is proposed that tumors with a large parenchymal component disappear soon after radiotherapy, whereas tumors composed mainly of interstitial component persist. Long-standing enhancement seen on MRI or CT scans of patients with an intracranial germinoma is indicative of a large amount of interstitial component in the tumor.
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ranking = 3.647338299653
keywords = central nervous system, brain, nervous system
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9/62. Recurrent germinoma in the optic nerve: report of two cases.

    OBJECTIVE AND IMPORTANCE: Intracranial germinomas often disseminate via the ventricular and subarachnoid pathways, but seeding to the perioptic arachnoid space is extremely unusual. We report two cases of recurrent germinoma seeding in the optic nerve. CLINICAL PRESENTATION: Two men with pure germinoma were initially treated with three cycles of a three-drug regimen of bleomycin, etoposide, and cisplatin, and a complete response was achieved. Patient 1 experienced ventricle wall dissemination 10 months after undergoing the initial treatment and was successfully treated with three cycles of carboplatin and etoposide and then by 24-Gy whole-ventricle radiation. Twelve months later, he complained of progressive visual acuity loss, and magnetic resonance imaging demonstrated bilateral enhancement of the optic nerves. Patient 2 also experienced ventricle wall dissemination 3 months after undergoing the initial chemotherapy, but he exhibited a complete response after undergoing 24-Gy whole-ventricle radiation. Two years later, he complained of progressive visual acuity loss. magnetic resonance imaging demonstrated bilateral enhancement of the optic nerves and cerebellar hemispheres. INTERVENTION: None of the locations of recurrence were included in the irradiation field, whereas there was no recurrence within the radiation field. Complete responses were obtained with three cycles of a three-drug regimen of ifosfamide, cisplatin, and etoposide and then by 24-Gy whole-brain radiation that included the bilateral optic nerves. The visual acuity of each patient improved slightly. CONCLUSION: Delayed seeding in the optic nerve may result from germinoma cells that remain dormant, so they cannot be destroyed by chemotherapy regimens alone.
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10/62. Advanced non-seminomatous germ cell cancer of the testis with brain metastases: feasibility of additional brain irradiation and whole body hyperthermia plus chemotherapy.

    patients with brain metastases in disseminated non-seminomatous germ cell cancer of the testis are treated by combined modality, e.g., cisplatin-containing chemotherapy, whole brain irradiation and/or surgical excision. However, cure rates of patients refractory to that standard treatment are low (5-year survival rate <30%). Preclinical data on the use of hyperthermia combined with selected cytotoxic drugs clearly show increased tumor cell killing compared to chemotherapy alone with no increase in toxicity to normal tissue. These results are consistent with the concept that whole body hyperthermia (WBH) at 41.8 degrees C is non-myelosuppressive and can potentiate the tumoricidal effects of specific chemotherapeutic agents, thus improving the therapeutic index. We report on a patient with embryonal testicular cancer presenting with lung, liver and brain metastases who initially underwent orchiectomy, whole brain irradiation and cisplatin-containing chemotherapy. Restaging revealed minor regression of brain and lung metastases and no change of liver metastases. However, beta-HCG values dropped from initial 400000 mIU/ml to 12 mIU/ml with a normal alpha-fetoprotein all the time. Then, two cycles of whole body hyperthermia (WBH) plus chemotherapy were performed, followed by one cycle of chemotherapy without WBH. radiotherapy, WBH and chemotherapy were well tolerated, especially no neurologic sequelae occurred. After more than 5 years of follow-up, the patient is still alive and disease-free. WBH plus chemotherapy seems to be feasible and may contribute to long-term survival in patients with advanced stages of non-seminomatous germ cell cancer refractory to standard treatment.
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