Cases reported "Hemangioendothelioma"

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11/50. Spindle cell hemangioendothelioma exhibits the ultrastructural features of reactive vascular proliferation rather than of angiosarcoma.

    A patient with spindle cell hemangioendotheliomas was followed from 1964 to the present time, allowing the authors the opportunity to examine the lesions in the early, mature, and old phases. Organizing thrombi of different stages associated with slit-like vascular proliferation were always observed, whereas cavernous vascular spaces predominated as the lesions became older. Each spindle cell hemangioendothelioma initially developed relatively rapidly and was sometimes painful but then persisted as a silent nodule for decades. Transmission and scanning electron microscopic studies revealed that endothelial cells tended to digitate into the slit-like proliferating channels, became attached to other cells by means of tight junctions, and thus obstructed the channels at sites where thrombi developed repeatedly. The vascular spaces, ranging in nature from slit-like to cavernous, were outlined further by a relatively sparse mantle of ramified or dendritic interstitial cells that corresponded to spindle cells. Most of the cells appeared simply to be fibroblasts, but they developed the features of pericytes when they were close to the endothelial lining of well-developed vascular lumens. Large vascular spaces and phleboliths were surrounded by smooth muscle cells. Approximately 20% of the interstitial cells were dendritic macrophages characterized by phagocytic activity, presence of many lysosomes, and factor xiiia expression. The long and characteristic clinical course, the histologic evidence that thrombosis and its organization was continually occurring within the lesions, and the ultrastructural finding that spindle cell hemangioendotheliomas were composed of different microvascular segments from capillaries to veins, suggest that spindle cell hemangioendotheliomas may develop from a cycle of recanalization after thrombosis that occurs repeatedly because of the unique endothelial growth that was noted. This is in contrast with the previous conception that they were low-grade angiosarcomas.
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keywords = spindle cell, spindle
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12/50. hemangioendothelioma of the frontal bone in a child.

    hemangioendothelioma is a vascular tumor of endothelial cell origin. It may involve bone or soft tissues and can behave like a benign or malignant tumor. In the literature, there are several case reports on the involvement of the head and neck region, but involvement of the frontal bone is extremely rare. This report presents the case of a 4-year-old boy with the diagnosis of spindle cell hemangioendothelioma of the frontal bone, who was treated by wide surgical resection.
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keywords = spindle cell, spindle
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13/50. Spindle cell haemangioendothelioma in an arteriovenous fistula of the ring finger after blunt trauma.

    We present a case of traumatic arteriovenous fistula of the palm and ring finger, which posed management dilemmas and eventually necessitated ray amputation. Subsequent histology revealed a spindle cell haemangioendothelioma that had developed within the fistula. We report the clinical features and management of this patient.
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keywords = spindle cell, spindle
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14/50. Epithelioid and spindle-cell hemangioendothelioma of the spleen. Report of a distinctive splenic vascular neoplasm of childhood.

    A case of a distinctive vascular neoplasm of the spleen in a 3-year-old boy is described. The tumor was characterized histologically by a biphasic growth pattern, with discrete nodular areas composed of atypical round, epithelioid cells with large nuclei and prominent nucleoli, and areas showing an intricate proliferation of vascular channels lined by elongated spindle cells. Immunohistochemical studies showed cytoplasmic staining of the tumor cells with factor viii-related antigen, ulex europaeus lectin, and vimentin antibodies. Stains for keratin, actin, desmin, lysozyme, and S-100 protein were negative in the tumor cells. Electron microscopy revealed a fairly cohesive population of cells that contained mature and immature cell junctions, basal lamina material, and surface pinocytotic activity consistent with vascular endothelial cells. Five-year follow-up has shown the patient to be alive and free of disease. This case appears to represent a previously unreported primary vascular neoplasm of the spleen showing combined features of epithelioid and spindle-cell hemangioendothelioma. The lesion should be distinguished from other benign and malignant vascular proliferations of the spleen such as Kaposi's sarcoma, angiosarcoma, and the recently described littoral-cell angioma.
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ranking = 0.43011990455154
keywords = spindle cell, spindle
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15/50. Intragluteal spindle cell hemangioendothelioma. An unusual presentation of a recently described vascular neoplasm.

    A 40-year-old woman noted a large tumor mass in the left buttock that, on microscopic examination, proved to be a recently described, relatively uncommon spindle cell hemangioendothelioma. This particular neoplasm, which has some features of Kaposi's sarcoma, seems not to have been reported previously in a deep, intramuscular location.
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16/50. Vanek's tumor (inflammatory fibroid polyp). Report of 18 cases and comparison with three cases of original Vanek's series.

    Eighteen cases of Vanek's tumors are presented. The patients included nine men and nine women between the ages of 45 and 93 years (mean, 66.2 years). Nine cases were clinically diagnosed as polyps of the gastric antrum, five cases as polyps of the stomach (not otherwise specified), one polyp was located in the ileum and the three remaining polyps in the small intestine (not otherwise specified). The thirteen polyps with available size information measured from 0.4 to 5 cm in the greatest diameter (mean, 2.2 cm). Immunohistochemically, the affections were positive for vimentin (18/18) and CD34 (15/18). All the cases negative for CD34 also lacked concentric onion skin-like formations of the spindle cells around glands and vessels. The different immunophenotype and absence of concentric formations could be explained by the existence of two different lesions commonly designated as Vanek's tumor (inflammatory fibroid polyp) or by the hypothesis of various evolutional stages. In the differential diagnosis, it is important to distinguish namely eosinophilic gastroenteritis, gastrointestinal stromal tumor, inflammatory pseudotumor, hemangioendothelioma, and hemangiopericytoma. In contrast to gastrointestinal stromal tumors, genetically no substitution, deletion, or insertion occurred in c-kit exon 11 in all analyzed samples. Likewise, no deletion or insertion in part of c-kit exon 9 was observed.
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ranking = 0.2
keywords = spindle cell, spindle
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17/50. Epithelioid and retiform hemangioendothelioma of the skull bone--report of four cases.

    hemangioendothelioma (HE) is a borderline or intermediate type of vascular neoplasm. We report clinical and histopathological characteristics of four cases of HE arising from the skull bones because of its extreme rarity in this location. The age of the patients ranged from 6-45 years. Three patients presented with a painless swelling over the head and one case had sphenoid wing mass with dimness of vision and proptosis. Radiographic images showed a well-demarcated, osteolytic lesion in the skull bone in all the four, one case in addition had sclerotic edges and another had specks of calcification. Grossly, the tumour was very vascular with hemorrhagic areas. Histologically, three cases showed features of an epithelioid variant of HE, with short strands and solid nests of rounded to slightly spindled, eosinophilic endothelial cells, some of them having small intracellular vacuoles. The stroma was myxoid--hyalinised with focal mixed inflammatory infiltrate. One case had features of a 'retiform' histological variant composed of numerous elongated vessels lined by a single layer of hobnail endothelial cells, focal lymphocytic infiltrate and papillae with hyaline collagenous cores. The tumour cells in all the four were immuno-labelled by antibody to factor viii-associated protein. The tumour cells lacked cytological atypia and mitosis was sparse. These features were important in prognostication as low-grade tumours can be cured by complete wide-resection.
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ranking = 0.046023980910308
keywords = spindle
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18/50. Kaposiform hemangioendothelioma: case report and literature review.

    We report the identification of a kaposiform hemangioendothelioma (KH) in the oropharynx of a 3-year-old boy. This is a rare endothelial-derived spindle cell neoplasm affecting children and early adolescents with features common to capillary hemangioma and Kaposi sarcoma. Nine cases of head and neck KH have been reported, this being the first in the otolaryngology literature. Our patient underwent wide local excision and has remained tumor free for over 1 year. KH should be considered in the differential diagnosis of a vascular lesion demonstrating unexpected behavior from that of a hemangioma.
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ranking = 0.2
keywords = spindle cell, spindle
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19/50. Multifocal kaposiform haemangioendothelioma.

    Kaposiform haemangioendothelioma (KHE) is a rare, locally aggressive vascular spindle cell proliferation, with resemblance to Kaposi's sarcoma. This tumour usually occurs in skin and retroperitoneum of infants and young children and is often complicated by the Kasabach-Merritt phenomenon (KMP). A 3-year-old boy presented with a right submandibular swelling due to lymphadenopathies, a violaceous skin lesion at the left commissure of the lips and an ill-defined lesion in the right thyroid lobe. There were some signs of KMP. Histological examination revealed a typical infiltrative multilobular spindle cell proliferation with slit-like vascular spaces in these three localisations. Immunohistochemical stains showed positivity for CD34 and CD31 and many alpha-smooth muscle actin-positive spindle cells around the vascular spaces. There was no Herpes virus type 8 expression. The presented case is unique in two ways. First, thyroid involvement of KHE has never been described in the literature until now. Secondly, and most remarkably, the multifocal presentation in three anatomically distinct and separated localisations is extremely unusual.
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ranking = 0.6
keywords = spindle cell, spindle
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20/50. Haemangioendothelioma on the conjunctiva of the upper eyelid.

    A 62-year-old man visited the authors' clinic complaining of a mass on the palpebral conjunctiva of the right upper eyelid. The 2.0 cm x 1.2 cm sized, red and painless mass underwent incision and biopsy for histopathological examination. The mass was confirmed to be Kaposiform haemangioendothelioma characterized by densely packed spindle cells. These cells were positive to vimentin, CD31 and factor viii-related antigen by immunohistochemical stain. The mass was completely resolved by oral steroid therapy and has not recurred through the presentation. Kaposiform haemangioendothelioma generally occurs in infant and adolescent periods and is characterized by rapid progression and invasion to adjacent tissue. Herein, an unusual case of Kaposiform haemangioendothelioma affecting the conjunctiva of the upper eyelid on a middle-aged man is reported.
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ranking = 0.2
keywords = spindle cell, spindle
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