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1/48. radiation-induced brain calcification: paradoxical high signal intensity in T1-weighted MR images.

    BACKGROUND: Irradiation to the central nervous system (CNS) in childhood is known to induce cerebral calcification after a latent period. Calcification has been generally found to show nil or a reduction in signal intensity in magnetic resonance (MR) images. However, we have studied three patients with radiation-induced brain calcification, who manifested increased signal intensity on T1-weighted MR images. METHOD: Three girls had each been diagnosed as having a suprasellar germ cell tumour and were treated with conventional fractionated radiotherapy in their childhood. In one case, chemotherapy was given prior to the CNS irradiation. FINDINGS: All three patients survived their disease, and a follow-up CT scan revealed calcification in the brain, which has shown an increased signal intensity in the T1-weighted images of MR. INTERPRETATION: Cerebral calcification may be presented as a high signal intensity in the T1-weighted MR images. This may be explained by a surface-relaxation effect by the calcium salt particle, precipitated in the brain due to radiation-induced mineralising microangiopathy.
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ranking = 1
keywords = cell tumour
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2/48. Disseminated choriocarcinoma in infancy is curable by chemotherapy and delayed tumour resection.

    Infantile choriocarcinoma has a poor prognosis with only 2 surviving children reported in the literature. 2 additional successfully treated children are presented. 2 infants (age 3 and 4 months at diagnosis) suffering from rapidly progressive choriocarcinoma with widespread haematogenous metastases involving the liver were treated according to the cooperative germ cell tumour treatment protocol (MAKEI 96) of the German Society of Pediatric Oncology and hematology (GPOH). PEI-chemotherapy (cisplatin, etoposide, ifosfamide; no ifosfamide before the age of 4 months) was combined with delayed tumour resection. Treatment resulted in sustained remission in both children (event-free survival 42 and 40 months). interphase fluorescent in situ hybridisation (FISH) analysis of the paraffin-embedded tumour sample from case one revealed four to eight copies of chromosomes X, 1 and 17 and two Y chromosomes. Hybridisation with sub-telomere and centromere specific probes for chromosome 1 displayed an imbalance between the short and long arms of chromosome 1. In the tumour cells from case 2, only a polysomy of chromosome X could be proven, other aberrations were not analysed in this case for technical reasons.
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ranking = 1
keywords = cell tumour
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3/48. Unusual intrathoracic location of a primary germ cell tumour.

    The primary location of non-metastatic germ cell tumours of the chest is the anterior mediastinal compartment. Germ cell tumour arising from lung parenchyma is one of the rarest conditions in human and only a few cases of choriocarcinomas and yolk sac tumour have been reported to date. Here we report a case of intrapulmonary mixed type germ cell tumour, containing embryonal carcinoma, choriocarcinoma and yolk sac tumour elements. diagnosis of the lesion was achieved by open thoracotomy and bulk of the tumour was resected by right upper lobectomy.
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ranking = 7
keywords = cell tumour
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4/48. Mixed malignant germ cell tumour of the lateral ventricle in an 8-month-old girl: case report and review of the literature.

    CASE REPORT: We report a huge intracerebral malignant germ cell tumour (GCT) which appeared in the lateral ventricles of an 8-month-old girl. Due to extensive tumour vascularisation only partial resection was achieved. histology revealed an embryonal carcinoma mixed with a teratoma. The MIB-1 staining index was >20%. Chemotherapy induced a marked regression of the tumour. After chemotherapy complete resection of the tumour remnant was easily achieved. histology showed only mesenchymal differentiated tumour tissue and the embryonal carcinoma could no longer be detected. More than 2 years after the second operation and 31 months after diagnosis the child remains tumour-free. CONCLUSION: The majority of cranial mixed malignant GCTs affects patients older than 4 years of age. To our knowledge this is the youngest patient in whom an intracranial malignant GCT containing an embryonal carcinoma has been diagnosed and successfully treated.
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ranking = 5
keywords = cell tumour
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5/48. Management of spinal cord and cauda equina compression secondary to epidural metastatic disease in adults with malignant germ cell tumours.

    AIM: To review the management and clinical outcome of 10 patients, presenting to a single centre with symptoms and signs of spinal cord or cauda equina compression secondary to epidural metastatic disease from a testicular germ cell cancer. methods: Clinical data regarding presenting history, physical examination, staging investigations, treatment and clinical outcome were retrospectively obtained from patient records. RESULTS: Eight patients exhibited neurological deficits at the time of initial presentation of germ cell cancer or as a first manifestation of relapse following dog leg irradiation. Four of these cases were managed with chemotherapy alone, with excellent neurological recovery, whilst four underwent decompressive laminectomy--in three cases prior to referral and in one case after commencing chemotherapy. Five of the eight patients relapsed. Four required further chemotherapy (high dose in two cases). The remaining patient underwent thoracic surgery, with resection of teratoma differentiated. Six of the eight patients are currently alive and disease free. Two patients had chemorefractory disease and died, though one was treated in the pre-cisplatin era. Two patients presented with cord compression as a feature of disease relapse following chemotherapy, and were managed with radiotherapy alone in an attempt to achieve local disease control and limit neurological dysfunction. However, both subsequently died with progressive disease. CONCLUSION: Epidural spinal cord or cauda equina compression is a rare complication of metastatic germ cell cancer, which can be successfully managed in chemo-naive patients with good neurological outcome.
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ranking = 4
keywords = cell tumour
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6/48. Intracranial germ cell tumours: I. Experience with platinum based chemotherapy and implications for curative chemoradiotherapy.

    The results of treatment with platinum based combination chemotherapy in ten patients with intracranial germ cell tumours (GCT) are presented. Two patients, treated for relapse within the central nervous system (CNS), attained partial responses of short duration. One patient with systemic relapse was successfully salvaged with chemotherapy. Seven patients received primary chemotherapy, six of whom received a 'CNS friendly' regimen consisting of vincristine, etoposide, carboplatin (VEJ) prior to craniospinal axis (CSA) irradiation. Three complete and three partial responses, and one patient with stable disease, were seen prior to irradiation. All seven patients are alive and remain disease-free at a median time of 12 months after treatment. Current treatment policy for germinomas attaining complete response to two courses of VEJ is a lowered CSA dose prescription, while non-germinomatous germ cell tumours (NGGCT) receive standard total dose CSA irradiation.
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ranking = 6
keywords = cell tumour
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7/48. The association of germ cell tumours of the testis with sarcoid-like processes.

    Sarcoid-type pulmonary lymphadenopathy associated with testicular cancer is a rare condition which has been previously reported in only 14 cases. Earlier case reports have failed to distinguish between generalized sarcoidosis as opposed to a local granulomatous reaction to tumour. We describe a further 8 cases of the association and provide strong supportive evidence for systemic sarcoidosis in 5 of our patients. In 3 of our patients with systemic sarcoidosis there was coexisting testicular cancer requiring additional treatment. We therefore advise caution in the interpretation of the clinical and histological findings in these patients, and recommend thorough investigation of all such cases.
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ranking = 4
keywords = cell tumour
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8/48. Malignant head and neck germ cell tumours.

    Malignant germ cell tumours of the head and neck are extremely rare. Their behaviour, despite aggressive surgical and adjuvant therapy, is relentless with most cases resulting in a fatal outcome. In this paper we present a case of a cervical embryonal carcinoma and review the presentation, clinical course and treatment of malignant germ cell tumours.
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ranking = 6
keywords = cell tumour
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9/48. Rhabdomyosarcomas developing in association with mediastinal germ cell tumours.

    Two mediastinal rhabdomyosarcomas that arose in association with germ cell tumours are reported. One presented as a small component of a mixed germ cell tumour with yolk sac and immature teratomatous elements. The other appeared as a large mass 4 months after diagnosis of a yolk sac carcinoma that had been treated with chemotherapy. The first patient was alive and free of disease 7 years later, whereas the second died of tumour 3 months post-operatively. The proportion of rhabdomyosarcoma within the germ cell tumours appears to have influenced the prognosis of these patients. This observation emphasizes the necessity of performing a thorough search for sarcomatous elements and quantifying their relative proportion in germ cell tumours of the mediastinum.
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ranking = 8
keywords = cell tumour
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10/48. Primary malignant retroperitoneal germ cell tumour presenting with accelerated hypertension.

    A 42 year old man presented with accelerated hypertension and an abdominal mass that proved to be a primary malignant retroperitoneal germ cell tumour involving renal vessels. The hypertension resolved with chemotherapy. To our knowledge this is the first case of a primary malignant retroperitoneal germ cell tumour to present with accelerated hypertension.
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ranking = 6
keywords = cell tumour
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