Cases reported "Vascular Neoplasms"

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1/8. Epithelioid hemangioma of the temporal artery clinically mimicking temporal arteritis.

    A rare case of epithelioid hemangioma (angiolymphoid hyperplasia with eosinophilia) arising in the right temporal artery of a 68-year-old Japanese woman was investigated. The patient had been treated with corticosteroids (Predonine 5 mg/day p.o.) for 4 years for idiopathic thrombocytopenic purpura. Headaches in the right temporal region with repeated high fevers first appeared 1 year prior to the biopsy of a nodule at the artery. The clinical diagnosis was temporal arteritis (giant cell arteritis). The specimen consisted of a short segment of the superior arterial branch having a sheet-like proliferation of the epithelioid endothelial cells in the lumen, which was almost occluded, focal rupture of the media, and marked proliferation of the capillaries (neovascularization) radiating out from the media to the adventitia. There were scattered foci of inflammatory cell infiltration composed mainly of small lymphocytes in the adventitia, but there were no histological changes suggestive of giant cell arteritis. The lesion was thus characterized by the proliferation of epithelioid endothelial cells in the lumen of the artery, and neovascularization between the media and adventitia. The histological features were very close to epithelioid hemangioma except for the absence of an eosinophilic reaction. The long-term administration of corticosteroids may have suppressed it. The authors believe this is the first case of epithelioid hemangioma arising from the temporal artery without a history of trauma. It showed some unique features both clinically and histopathologically, which contributed to the study of the yet not clearly classified vascular proliferative lesion, epithelioid hemangioma.
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2/8. Giant cavernous sinus teratoma: a clinical example of a rare entity: case report.

    OBJECTIVE AND IMPORTANCE: Teratomas represent 0.5% of all intracranial tumors. These benign tumors have tissue representative of the three germinal layers: ectoderm, mesoderm, and endoderm. Most teratomas are midline tumors located predominantly in the sellar and pineal regions. cavernous sinus location is very rare; only two purely intracavernous teratomas have been reported. CLINICAL PRESENTATION: A 14-year-old boy presented with a history of progressive right eye proptosis and visual acuity impairment, headaches, and a neuralgia-like facial pain in the right V1 distribution. A head computed tomographic scan and magnetic resonance imaging disclosed a large tumor filling the right cavernous sinus and extending into the ipsilateral middle fossa. These scans also demonstrated mixed signals derived from different tissues conforming to the tumor (fat, cartilage, muscle strands, bone, and a primordial tooth). Heterogeneous enhancement was seen after infusion of contrast medium. Significant bone erosion and remodeling was evident in the middle fossa floor and right orbit, with secondary proptosis. A presumptive diagnosis of mature teratoma was made. INTERVENTION: With the use of a right frontotemporal interfascial approach, a combined extra- and intradural dissection of the tumor was performed. The lesion entirely occupied the cavernous sinus, laterally displacing the Gasserian ganglion and trigeminal branches (predominantly V1 and V2). The internal carotid artery and Cranial Nerve VI were medially displaced by the tumor mass. The lesion was composed of different tissues, including hair, fat, cartilage, muscle, nerve-like tissue, bone, and a primordial tooth. The tumor was removed completely, and the pathological report confirmed the diagnosis of a mature teratoma. There was no evidence of recurrence at the 8-month follow-up examination. CONCLUSION: Because of the lesion's location in the lateral wall of the cavernous sinus, a total removal was achieved with the use of standard microsurgical techniques. knowledge of the microanatomy is essential in treating intracavernous pathology. We present the third reported case of a giant mature teratoma of the cavernous sinus.
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3/8. Dumbbell-shaped leiomyosarcoma of the inferior vena cava with foci of rhabdoid changes and osteoclast-type giant cells.

    An inferior vena cava (IVC) tumor was incidentally found in a 67-year-old Japanese man. The resected tumor was lobulated and multinodular, measuring 14.0 x 6.5 x 7.0 cm, showing a dumbbell-shaped appearance with a central constriction. The tumor showed both intra- and extra-luminal growth. The tumor was primarily composed of well-differentiated leiomyosarcoma. Spindle tumor cells in the well-differentiated area were positive for vimentin, muscle actin, alpha-smooth muscle actin, and desmin. Foci of rhabdoid cells and osteoclast-type multinucleated giant cells were also found. Rhabdoid cells ultrastructurally had paranuclear aggregates or whorls of intermediate filaments that were positive for vimentin, low molecular weight cytokeratin, and desmin. Osteoclast-type multinucleated giant cells were positive for only CD68 antigen, suggesting a reactive histiocytic lineage. To the best of our knowledge, this is the first case of IVC leiomyosarcoma accompanied by both rhabdoid tumor cells and osteoclast-type reactive multinucleated giant cells. These unusual features should be kept in mind in the diagnosis of dumbbell-shaped retroperitoneal tumors that involve the IVC.
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4/8. Late recurrence of cardiac sarcoma presenting as giant pulmonary artery aneurysm.

    We report the case of a 46-year-old woman who had undergone cardiac transplantation for a malignant, right ventricular sarcoma. Five years later, she experienced pulmonary hypertension and a pulmonary artery aneurysm. Medical management of the pulmonary hypertension being unsuccessful, she underwent surgical exploration of the pulmonary artery aneurysm and bilateral pulmonary endarterectomy. Intra-operative findings revealed pulmonary artery sarcoma and an unresectable pulmonary artery aneurysm.
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5/8. Left trisegmentectomy and combined resection of the inferior vena cava, without reconstruction, for giant cystadenocarcinoma of the liver.

    A 54-year-old woman with giant liver cystadenocarcinoma underwent left trisegmentectomy with combined resection of the inferior vena cava (IVC) and the right hepatic vein. As a result, only the right inferior hepatic vein was preserved as a drainage vein. Because the perivertebral plexus and the azygos vein were both well developed, neither veno-venous bypass nor IVC reconstruction was performed. The developed collateral veins acted as the venous drainage pathway to maintain a stable systemic circulation. On the seventh postoperative day, portal vein flow dramatically decreased and the patient tended to liver failure. Prostaglandin E(1) (PGE(1)) was administrated via the superior mesenteric artery. The portal flow then gradually increased and liver failure was avoided. Six months after the operation, she was re-admitted due to obstructive jaundice and presented with complete stenosis of the common bile duct (CBD). The jaundice persisted and liver dysfunction progressed. The patient died seven months after the operation. The confluence of the right inferior vein and the IVC could have been deformed, causing outflow blockade. The intrinsic shunt was not good enough to act as the drainage pathway, and IVC reconstruction may have been needed.
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6/8. Pleomorphic carcinoma of the lung with mediastinal extension following malignant lymphoma: report of a case.

    In following up a patient with non-Hodgkin's lymphoma, we encountered a case of pulmonary pleomorphic carcinoma with mediastinal direct invasion. A 65-year-old man with hemoptysis was found to have an abnormal shadow in the right upper lung field. A 6.4 x 4.8-cm tumor adjacent to the upper mediastinum occupied the right anterior segment of the upper lobe (S3) and invaded the superior vena cava (SVC). The serum level of neuron-specific enolase was elevated to 11.9 ng/ml. A specimen from a transbronchial lung biopsy of the right B3b bronchus revealed giant tumor cells. A right upper lobectomy with SVC reconstruction was performed. The resected tumor was diagnosed as a pulmonary pleomorphic carcinoma with a large component of giant and spindle cells, and it is considered to be a rare histologic type.
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7/8. Angiomatoid features in fibrohistiocytic sarcomas. Immunohistochemical, ultrastructural, and clinical distinction from vascular neoplasms.

    The typical example of malignant fibrous histiocytoma (MFH) or dermatofibrosarcoma protruberans (DFSP) does not require ancillary studies for diagnosis. However, hemorrhage with cystic change consisting of blood-filled spaces may closely mimic a vascular neoplasm. Eight fibrohistiocytic sarcomas exhibiting these angiomatoid features, initially mistaken for vascular neoplasms, were identified from personal consultation files and review of 157 consecutive sarcomas (1985 through 1993) at the University of california-(Davis) Medical Center. They included five MFH giant-cell-type sarcomas, two MFH angiomatoid-type sarcomas, and one DFSP. Immunohistochemical analysis of paraffin-embedded material showed vimentin diffuse positive, CD68 (KP-1) diffuse positive, and factor viii negative in all eight sarcomas; actin HHF-45 focal positive in six, diffuse positive in one, and negative in one sarcoma; desmin focal positive in two and negative in six sarcomas; and S100 protein, cytokeratin AE1:AE3, cytokeratin 10.11, and EMA negative in all eight sarcomas. Electron microscopy of three tumors exhibited neoplastic cells with fibroblastic, myofibroblastic, and histiocytic features. weibel-palade bodies or neolumens diagnostic of vascular differentiation were absent. The clinical characteristics and behavior of these sarcomas reflect entities in the spectrum of fibrohistiocytic lineage (MFH subtypes and DFSP) rather than vascular neoplasms. patients with deep, large, giant-cell-type MFHs did poorly (two of four patients died from disease at 8 and 25 months). Both patients with angiomatoid MFHs showed local recurrences from large incompletely excised head and neck lesions. One died of disease at 21 months and the other is free of disease 12 months following excision of a local metastasis to the opposite side of the neck. The patient with DFSP had an 18-cm locally recurrent scalp tumor that extended into bone. Immunohistochemical and ultrastructural confirmation of fibroblastic, myofibroblastic, and histiocytic lineage and exclusion of vascular differentiation help to establish the correct diagnosis in these fibrohistiocytic sarcomas with angiomatoid features. The clinicopathologic features of these eight cases reaffirm the practical utility of MFH and DFSP as diagnostic entities in the spectrum of fibrohistiocytic sarcomas.
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8/8. Vascular leiomyosarcoma arising from the inferior vena cava diagnosed by intraluminal biopsy.

    A 61-year-old woman developed pain in the right thigh, paraplagia of the lower extremities and lumbago in November 1996. A lumbar spine roentgenogram showed lytic change in L2, and magnetic resonance imaging showed a patchy destructive lesion and compression of the dural sac from the right by a tumour. Computed tomography (CT) myelography showed a motheaten destructive lesion in L2 and projection of the tumour into the spinal canal. Abdominal ultrasound, CT and cavography showed dilatation of the inferior vena cava (IVC) and an intraluminal tumour about 2x2.8x4 cm in size in the IVC. The tumour arose from the IVC just beneath the renal vein and extended to just short of the right atrium. Both vertebral and intraluminal biopsy materials showed the same morphology, in which atypical spindle cells admixed with multinucleated giant cells proliferated in a fascicular growth pattern. Neoplastic cells were strongly positive for alpha-smooth muscle actin. We diagnosed vascular leiomyosarcoma arising from the IVC with metastasis to the lumbar vertebrae. Cases of vascular leiomyosarcoma diagnosed by intraluminal biopsy are rare.
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