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1/122. Angiosarcoma of the testis.

    A primary angiosarcoma of the testis in a 74-year-old patient was a highly anaplastic epthelioid angiosarcoma, which was positive for endothelial markers immunohistochemically. The tumour was unrelated to testicular germ cell neoplasm; the patient had received no previous radiation or chemotherapy.
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keywords = tumour
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2/122. Spontaneous intralesional haemorrhage in dysembryoplastic neuroepithelial tumours: a series of five cases.

    Five patients with dysembryoplastic neuroepithelial tumour (DNT) showing extensive secondary haemorrhage, a finding not previously associated with these neoplasms, are described. The clinical presentations, neuroimaging findings, and histopathological features of these patients are reviewed. One patient, a previously asymptomatic 12 year old girl, presented with an acute intracerebral haemorrhage into a DNT. A further four young adults with histories of intractable partial and generalised seizures dating from childhood showed significant chronic haemorrhages within DNT, the MRI appearances in one patient giving a false impression of a cavernoma. Histopathology disclosed vascular abnormalities within these tumours which, together with other factors discussed, may have predisposed these tumours to haemorrhage.
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keywords = tumour
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3/122. 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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4/122. Fine needle aspiration cytology of undifferentiated embryonal sarcoma of the liver: a case report.

    The cytological features of a rare case of undifferentiated (embryonal) sarcoma of the liver are presented. The cytology smears showed singly dispersed polygonal and spindle cells as well as loose clusters of cells held together in myxoid material. Neoplastic cells were generally large with round, oval or lobulated nuclei. The cytoplasm was variable in amount with ill-defined borders. Occasional multinucleated cells were also present. Hyaline globules were present on sections of the cell block. Immunohistochemical studies performed showed positivity for vimentin, cytokeratin and alpha-1-antitrypsin (AAT) in the tumour cells.
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keywords = tumour
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5/122. Hepatic undifferentiated (embryonal) sarcoma in an adult: a case report and review of the literature.

    Undifferentiated (embryonal) sarcoma of the liver (USL) is a rare malignant tumour with a poor prognosis. The absence of specific symptoms, the rapid tumour growth, the normality of the common tumour markers, and the consequential delay in the diagnosis often result in significant enlargement of the tumour. To our knowledge, there have been only 42 reported cases of USL in adults worldwide during the 40 years since the clinicopathological entity of USL was defined. We report here a 27-year-old male with USL who has been treated successfully with surgical resection and adjuvant chemotherapy using ifosfamide, adriamycin and cisplatin. Although the prognosis of the disease remains generally poor, long-term survival has been achieved recently in patients with a combination of surgery and multi-agent chemotherapy.
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keywords = tumour
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6/122. 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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keywords = tumour
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7/122. Embryonal sarcoma of the liver mimicking a hydatid cyst in an adult.

    Undifferentiated (embryonal) sarcoma of the liver is a rare malignant mesenchymal tumour. This presents predominantly in late childhood. It carries a dismal prognosis. Surgical resection with or without adjuvant therapy remains the mainstay of treatment. The present report describes a sixteen year old girl who presented with a large hepatic mass mimicking a hydatid cyst: clinically, radiologically and on serology.
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keywords = tumour
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8/122. 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 = 10
keywords = tumour
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9/122. 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 = 10
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10/122. 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 = tumour
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