Cases reported "Hemangiopericytoma"

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1/109. Congenital nasal hemangiopericytoma: intrauterine, intraoperative, and histologic findings.

    hemangiopericytoma is a rare tumor of mesenchymal origin. To date, 91 cases of nasal or paranasal hemangiopericytoma and 59 congenital hemangiopericytomas have been reported in the literature. A congenital hemangiopericytoma arising from the nasal cavity and skull base has not yet been described. We report a case of a male newborn with a highly vascular nasal tumor diagnosed by in utero sonography with three-dimensional surface reconstruction. The tumor extended to the right anterior skull base, the right nasal cavity, and the right side of the nasal pyramid. A complete resection by neodymium:yttrium-aluminum-garnet-potassium titanyl phosphate ("Nd:YAG-KTP") laser was performed on the day of cesarean section at 33 weeks' gestation. The tumor was diagnosed as hemangiopericytoma by histologic and immunohistochemical findings. Postoperative nasal flow, feeding, and sight were unimpaired. At the 9-month follow-up, the infant remained free of disease.
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2/109. Combined malignant hemangiopericytoma and deep venous thrombosis. A case report.

    Malignancies, antiproliferative drug treatment, cancer-related conditions like immobilization, perioperative status and radiotherapy are risk factors for hypercoagulability. Setting aside mass or invasion-related venous thrombosis, the differential diagnosis regarding the etiopathogenesis (paraneoplastic syndrome or antiproliferative treatment) is usually problematic. The authors report a case of combined malignant hemangiopericytoma and recurrent deep venous thrombosis in the right inferior limb. Through a literature review, the following issues are discussed: 1) the criteria for cyto-histopathologic assessment; 2) the involvement of pericytes both in coagulation and platelet aggregation; 3) the importance of discriminating true paraneoplastic syndromes from other tumor-related clinical manifestations; 4) the response to external radiotherapy of malignant hemangiopericytoma as limited disease; 5) the poor results of doxorubicin-ifosfamide polychemotherapy and dacarbazine monochemotherapy in metastatic disease. Although doxorubicin-ifosfamide treatment was in progress in the reported case, the authors conclude that the recurrent deep venous thrombosis is likely to be paraneoplastic, even if such a diagnosis has not been previously reported in the literature.
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3/109. Nasal haemangiopericytoma: report of two cases with literature review.

    Haemangiopericytoma (HPC) is a rare vascular tumour produced by proliferation of pericytes. One hundred and thirty-three cases of HPC have been reported in the nose and the paranasal sinuses. We present two more cases. Wide surgical excision via lateral rhinotomy, midfacial degloving, or endonasal removal is the treatment of choice. radiotherapy has been used post-operatively in cases of incomplete removal. life-long follow-up is required to evaluate local recurrence and late metastases.
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4/109. Hypoglycaemia associated with the production of insulin-like growth factor ii and insulin-like growth factor binding protein 6 by a haemangiopericytoma.

    Non-islet-cell tumour-induced hypoglycaemia (NICTH) is, in most cases, attributable to tumour production of insulin-like growth factor ii (IGF-II). Tumour-derived IGF-II has a higher than normal molecular weight (big 'IGF-II') and an impaired ability to form the normal ternary 150 kD complex with IGF binding protein-3 (IGFBP-3) and the acid-labile subunit (ALS). Consequently, tumoral IGF-II circulates mainly in smaller binary complexes which have a higher bioavailability than the ternary complex. We had the opportunity to analyze IGFs and IGF-related factors in both pre- and post-operative blood, tumour tissue and tumour cyst fluid from a patient with a disseminated haemangiopericytoma and severe hypoglycaemia. In addition, the effect of serum and tumour cyst fluid on autophosphorylation of the insulin receptor was examined. Patient serum contained low levels of IGF-I, IGFBP-3 and ALS, while the concentrations of IGFBP-2 and IGFBP-6 were markedly elevated. The total level of circulating IGF-II was within the normal range, but Biogel P-60 gel filtration of patient serum revealed that 77% of the IGF-II was present in high molecular weight forms (normal: 10-15%), which decreased to 53% after partial removal of the tumour. Most of the IGF-II immunoreactivity in pre- and post-operative patient serum was associated with 50-60 kD complexes with only a minimal contribution (<10%) from the 150 kD complex. Tumour cyst fluid contained excessive amounts of both big IGF-II and IGFBP-6. Northern blot analysis of total mRNA isolated from the tumour demonstrated high expression of the IGF-II gene and abundant 1.1 kb IGFBP-6 transcript, while the genes encoding IGFBP-3, -4 and -5 were only weakly expressed and mRNA of IGFBP-1, -2 and IGF-I could not be detected. mRNAs for the IGF type II receptor could be easily demonstrated, whereas those for the insulin- and IGF type I receptor were hardly detectable. In contrast to patient serum tumour cyst fluid strongly stimulated the insulin receptor in vitro. The present study suggests an important role of the simultaneous production of IGF-II and IGFBP-6 in the pathophysiology of tumour-induced hypoglycaemia.
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5/109. Massive intrathoracic extrapleural haemangiopericytoma: deployment of radiotherapy to reduce vascularity.

    Haemangiopericytoma is a rare, highly vascular, slow-growing tumour which has both malignant and benign varieties. We report a case of a 63-year-old man in whom it was treated by initial radiotherapy followed by complete surgical excision. The preoperative radiotherapy markedly reduced the vascularity of the tumour. A brief review of the pathology and nature of tumour is provided.
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6/109. Management of sinonasal hemangiopericytomas.

    The purpose of the present study is to report four cases of sinonasal hemangiopericytoma (HP) diagnosed and treated in our department between 1987 and 1998. The pretreatment findings and the treatment are described and discussed in the light of the literature. HP are unusual vascular tumors, featuring pericytes distributed around normal vascular channels. Two of these four cases were located in the nasal cavity and the other two were located in the maxillary sinus. Inside the nasal cavity, HP presented as a protruding reddish-gray mass with marked bleeding on contact. Electron microscopy and immunohistochemical techniques are essential for diagnosis and to distinguish HP from other sarcomatous tumors. Preoperative assessment included routine CT, MRI, arteriography and selective embolization. These tumors must be treated surgically with complete excision. An endonasal approach was performed in two cases of intranasal HP, while a combined external-endonasal approach was required for the other two cases of HP.
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7/109. Haemangiopericytoma of kidney: a report of 2 cases.

    Haemangiopericytoma is a rare neoplasm of the kidney. There are no unique radiological or clinical identifiers that can aid in preoperative diagnosis. Surgery is the only reliable therapy, as both chemotherapy and radiotherapy have proven ineffective in several series. The outcome is difficult to predict, the only reliable predictor is presence or absence of metastasis. The rarity of this lesion prompts the report of these two cases.
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8/109. Preoperative embolization in surgical treatment of mediastinal hemangiopericytoma.

    The case of a 47-year-old man with a tumor of the posterosuperior mediastinum is reported. Surgical biopsy sample revealed a hemangiopericytoma, but radical excision was impossible because of massive bleeding. Percutaneous embolization of mediastinal tumor was performed to reduce peroperative blood loss. It allowed uneventful complete removal of the lesion. We recommend preoperative embolization in cases of hypervascular mediastinal tumors.
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9/109. Intracranial infantile hemangiopericytoma.

    hemangiopericytoma (HP) is a rare vascular tumor that usually occurs in adults, but 10% occur in children. Tumors occurring in the first year of life are even more rare and are referred to as infantile hemangiopericytoma. In this article, we report the imaging, operative, and pathological findings in a patient with an infantile HP.
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10/109. Cerebral haemangiopericytoma treated with conservative surgery and radiotherapy.

    Cerebral haemangiopericytomas are rare tumours that resemble meningiomas but behave more aggressively, with a tendency to metastasize. We report two patients with haemangiopericytoma who had limited surgical resections owing to perioperative blood loss but who had massive tumour shrinkage after a course of radical radiotherapy. We suggest a more conservative surgical approach to the management of these tumours.
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