Search PubMed⌕ Search

SEARCH · Search PubMed

Results for “HEMANGIOENDOTHELIOMA”

Search indexed PubMed citations on genomics, clinical trials, systematic reviews and public health. Explore titles, authors and supplied subject terms, then open the PubMed record.

Quote a phrase for an exact phrase match. Source license links do not imply unrestricted reuse.

At least 19 recordsLinked to original sources
PubMed2003

Vascular tumors of bone: A study of 17 cases other than ordinary hemangioma, with an evaluation of the relationship of hemangioendothelioma of bone to epithelioid hemangioma, epithelioid hemangioendothelioma, and high-grade angiosarcoma.

Cases filed as vascular tumor of bone other than ordinary hemangioma were reviewed. They were included in the study if there was adequate histologic material and clinical information, clear evidence of bone origin, and at least 5 years follow-up. The study group comprised 17 cases, of which 13 were categorized as hemangioendothelioma of bone, 1 as epithelioid hemangioendothelioma, and 3 as high-grade angiosarcoma. Hemangioendothelioma of bone had growth patterns varying from vasoformative to solid, but well-formed vessels were present in at least some area in all cases. The cells generally had a rounded, epithelioid character, regular nuclei, and relatively few mitotic figures; occasional features included spindle cells and scattered enlarged, hyperchromatic or pleomorphic nuclei. Lymphoplasmacytic and eosinophilic inflammatory infiltrate ranged from prominent to slight or absent, and myxoid or hyaline stroma was never more than focal. Epithelioid hemangioma could not be separated from hemangioendothelioma of bone. The single epithelioid hemangioendothelioma for the most part had cords of relatively uniform epithelioid cells in a prominent myxoid stroma but focally demonstrated an angiosarcoma-like appearance, with irregular vascular spaces and marked nuclear pleomorphism. The high-grade angiosarcomas exhibited predominantly irregular vasoformation combined with solid areas, diffuse nuclear hyperchromatism and pleomorphism, and, in 2 cases, numerous mitotic figures (the third case had only a small biopsy and a postradiation amputation specimen). Of the hemangioendotheliomas of bone, 7 were unicentric and 6 were regionally multicentric either concurrently or sequentially. Three patients had intraosseous local recurrence, 2 had discontinuous regional skin or soft tissue involvement (including the popliteal artery in 1), and 1 had a solitary lung metastasis, but none died of tumor. The patient with epithelioid hemangioendothelioma had multicentric tumors in widely separated bones and died with liver and lung metastases. Two of the high-grade angiosarcomas were unicentric, and the third was regionally multicentric, with a popliteal artery-soft tissue component as well. All 3 of these patients died with metastases in various sites.

Adolescent↗
PubMed2002

Congenital composite hemangioendothelioma: case report and reappraisal of the hemangioendothelioma spectrum.

BACKGROUND: Composite hemangioendothelioma is the most recently described entity of the hemangioendothelioma (HE) spectrum. To the best of our knowledge, only eight cases of this entity have been hitherto described. All of the previous cases affected adults; local recurrences were observed in three cases, and one case showed lymph node metastasis. We herein describe a new and previously unreported congenital case of this rare vascular tumor, arising on the acral extremity of the left forearm, which was diagnosed when the patient was 23 years old. RESULTS: The histological examination disclosed a heterogeneous vascular neoplasm composed of retiform HE-like (80%), spindle cell hemangioma-like (15%), cavernous hemangioma-like (approximately 3%), epithelioid HE-like (approximately 2%) areas, and rare foci with an angiosarcoma-like pattern (< 1%). A distinctive and unique finding of the present case was the presence of large granular eosinophilic macrophages filling some vessels of the retiform HE-like areas. A below-elbow amputation was performed. The patient is alive and well, without evidence of residual or metastatic disease 7 years after the treatment. CONCLUSIONS: The authors expand the concept of composite hemangioendothelioma by adding a congenital case and provide a reappraisal of the hemangioendothelioma spectrum.

Adult↗
PubMed1994

Spindle cell hemangioendothelioma in association with epithelioid hemangioendothelioma.

A forty-two-year-old man presented with a twelve-year history of a multilobular, firm, but nonulcerated tumor (6 by 4.5 cm) of the dorsum of the right foot. He had capillary telangiectasia of both ankles but no lymphedema. Results of tests for human immunodeficiency viruses I and II were nonreactive. The tumor showed a complex combination of features of spindle cell hemangioendothelioma, epithelioid hemangioendothelioma, and areas suggestive of a possible well-differentiated (lymph) angiosarcoma. The tumor was completely excised with minimal healthy margin. There was no evidence of local or distant lesion at one-year follow-up examination.

Adult↗
PubMed2005

D2-40 immunohistochemical analysis of pediatric vascular tumors reveals positivity in kaposiform hemangioendothelioma.

Kaposiform hemangioendothelioma is a distinctive vascular neoplasm affecting predominantly children and neonates. In neonates it needs to be differentiated from common infantile hemangioma and other vascular lesions of infancy. Kaposiform hemangioendothelioma immunoreacts with vascular endothelial growth factor receptor 3, and partial lymphothelial differentiation of this lesion has been suggested. D2-40 has been recently proposed as a selective marker of lymphatic endothelium. We performed immunohistochemical analysis with the D2-40 antibody on 24 kaposiform hemangioendotheliomas and 48 other pediatric vascular lesions including common infantile hemangioma (n=10), rapidly involuting congenital hemangioma (n=10), non-involuting congenital hemangioma (n=9), verrucous hemangioma (n=9), and pyogenic granuloma (n=10) to define whether this marker can be applied in the diagnosis of vascular lesions of infancy. In all, 23 of 24 (96%) kaposiform hemangioendotheliomas exhibited a distinct staining, while none of the other lesions immunoreacted with D2-40. D2-40 stained the neoplastic spindled cells and lymphatic channels adjacent to vascular lobules of kaposiform hemangioendothelioma. These findings support D2-40 as a new determinate marker for kaposiform hemangioendothelioma, useful in differentiating it from other vascular lesions of infancy and suggest lymphothelial differentiation of the neoplastic component of kaposiform hemangioendothelioma. Further studies are necessary to define the identity of the D2-40 antigen and to elucidate the biologic significance of its selective lymphothelial reactivity..

Actins↗
PubMed1980

Benign hemangioendothelioma involving the central nervous system: "strawberry nevus" of the neuraxis.

Benign hemangioendothelioma is rarely included in discussions of vascular neoplasms involving the central nervous system, whereas it is a well-defined entity outside the neuraxis. We present four cases of benign hemangioendothelioma, including an infantile hemangioendothelioma with venous drainage into the transverse dural sinus, an intracranial hemangioendothelioma adherent to the falx cerebri, a spinal epidural hemangioendothelioma causing spinal cord compression, and a parasellar hemangioendothelioma. Although hemangioendothelioma resembles hemangioblastoma in some ways, it is a distinct entity differing from the other vascular lesions involving the central nervous system.

Adult↗
PubMedDate not supplied

[Cutaneous epithelioid hemangioendothelioma].

INTRODUCTION: Epithelioid hemangioendothelioma is a tumor of soft tissues arising from the vascular endothelium. It is considered as a low-grade malignant tumor. Cutaneous involvement is rare and often associated with multi-systemic localizations. CASE REPORT: We report the case of a 34 year-old woman with a 6-month history of a 1.5 cm erythematous-violaceous, soft, painful cutaneous nodule involving the right forearm. An abdominal sonography had been performed 1 month before the onset of the nodule, because of epigastric pain and was normal. Histological and immunohistological examinations led to diagnosis of an epithelioid hemangioendothelioma. Assessment of the extension with radiology, sonography, tomodensitometry and magnetic resonance imaging, revealed nodules of the liver. Treatment consisted in the wide and complete excision of the tumor. There was no evidence of local recurrence after one year follow-up, and the hepatic lesions were stable. DISCUSSION: Epithelioid hemangioendotheliomas belong to the epithelioid vascular tumor spectrum. They have in common the morphologic epithelioid aspect of endothelial tumor cells. Cutaneous localization is rare, and to the best of our knowledge, only 20 cases of epithelioid hemangioendothelioma with skin involvement have been reported in the literature. Epithelioid hemangioendothelioma can be isolated or associated with internal visceral involvement. The detection of skin lesions should lead to a complete assessment of the extension of the disease to detect any internal localization. Because of low-grade malignancy of the tumor, and the integrity of the liver observed one month before the onset of the disease in our patient, epithelioid hemangioendothelioma appears to be a multicentric disease rather than metastatic hepatic localizations of a primitive skin cancer.

Adult↗
PubMed1994

Cellular hemangioma and angioblastoma of the spine, originally classified as hemangioendothelioma. A confusing diagnosis.

STUDY DESIGN: The authors report two cases of vascular tumors of the spine, classified originally as benign and malignant hemangioendothelioma, and after revision, as cellular hemangioma and angioblastomatosis, respectively. OBJECTIVES: Problems in interpretation of the confusing term hemangioendothelioma and treatment modalities for vascular tumors of the spine are discussed. SUMMARY OF BACKGROUND DATA: Hemangioendothelioma is a confusing term and is often used to cover bewilderment at the biological behavior of a vascular tumor. Its spectrum ranges, depending the references used, from benign to malignant and can mistakenly include benign lesions like cellular hemangioma and angioblastoma (solitary and multicentric). METHODS: Of two patients with a cellular tumor of the spine, the clinicopathologic data and modes of treatment are reviewed. The relevant literature is discussed. RESULTS: In the first case, the diagnosis of benign cellular hemangioendothelioma was changed to cellular hemangioma. In the second case, the original diagnosis of malignant hemangioendothelioma with metastasis to liver and lungs was changed to angioblastomatosis, most probably benign. In both cases, a correct interpretation of the initial diagnosis or proper diagnosis would have influenced the mode of treatment. CONCLUSION: Avoid the confusing term hemangioendothelioma. If a vascular lesion is benign, it should be classified as a variant of hemangioma. If malignant as angiosarcoma, use a separate category, in which lesions like angioblastoma and angioblastomatosis can be put until their nature has been clarified.

Adult↗
PubMed2001

[Epithelioid hemangioendothelioma of the spine].

BACKGROUND: Epithelioid hemangioendotheliomas are rare vascular tumors, mostly originating from soft tissue, lungs or liver. CASE REPORT: A 58-year-old woman was admitted to hospital because of upper congestion, Horner's syndrome and segmental sensory disturbance. MRI of the spine showed a spinal mass, reaching from the seventh cervical vertebra to the fourth chest vertebra. Because of worsening of upper congestion and progressive neurological impairment, the patient had to be operated twice. Unfortunately a surgical excision of the infiltrating tumor in sano was not possible and the patient died 4 weeks after the second operation. Histologic examination revealed the diagnosis of infiltrating epithelioid hemangioendothelioma, originating from the first chest vertebra. CONCLUSION: Prognosis of epithelioid hemangioendothelioma is mainly determinated by its location. In advanced stages of disease or lack of surgical cure epithelioid hemangioendotheliomas may be fatal. Therefore epithelioid hemangioendotheliomas should be considered early in the differential diagnosis of patients with uncertain bone lesions.

Cervical Vertebrae↗
PubMed2000

Vascular endothelial growth factor receptor-3 (VEGFR-3): a marker of vascular tumors with presumed lymphatic differentiation, including Kaposi's sarcoma, kaposiform and Dabska-type hemangioendotheliomas, and a subset of angiosarcomas.

Recently, a novel monoclonal antibody to vascular endothelial growth factor receptor 3 (VEGFR-3), a tyrosine kinase receptor expressed almost exclusively by lymphatic endothelium in the adult, has been shown to react with a small number of cases of Kaposi's sarcoma (KS) and cutaneous lymphangiomas. We sought to extend these studies to a large number of well-characterized vascular neoplasms to evaluate diagnostic uses of this antibody and to determine whether it defines them in a thematic fashion. Formalin-fixed, paraffin-embedded sections from 70 vascular tumors were immunostained with antibodies to VEGFR-3 von Willebrand factor (vWF), and CD31. Anti-VEGFR-3 was positive in 23 of 24 KS, 8 of 16 angiosarcomas (AS), 6 of 6 kaposiform hemangioendotheliomas, 4 of 4 Dabska tumors, and 2 of 13 hemangiomas. Positively staining angiosarcomas were characterized either by a prominent lymphocytic component, a hobnail endothelial cell similar to that encountered in the Dabska tumor, or spindled areas resembling KS. No VEGFR-3 expression was noted in any cases of epithelioid hemangioendothelioma, pyogenic granuloma, littoral angioma, or stasis dermatitis. vWF expression was seen in 10 of 13 KS; 13 of 14 AS; 4 of 5 kaposiform hemangioendotheliomas; and all Dabska tumors, hemangiomas, lymphangiomas, epithelioid hemangioendotheliomas, vascular malformations, stasis dermatitis, and splenic littoral angiomas. CD31 expression was present in 12 of 13 KS, 13 of 14 AS, and in all other cases. Expression of VEGFR-3 is a very sensitive marker of KS, kaposiform, and Dabska-type hemangioendotheliomas, suggesting that all show at least partial lymphatic endothelial differentiation. Expression of VEGFR-3 does not reliably discriminate KS from AS. However, the expression of VEGFR-3 by certain AS having Kaposi-like areas, a prominent lymphocytic infiltrate, or hobnail endothelium may define subset(s) having phenotypic, if not pathogenetic and biologic, differences.

Biomarkers, Tumor↗
PubMed1998

[Case report of epithelioid hemangioendothelioma of the frontal region metastatic to the parotid gland].

BACKGROUND: The epithelioid hemangioendothelioma is a soft tissue tumor of vascular origin. Typical localisations are subcutis, cutis, liver, lungs and bones. It has been described in 1982 by Weiss and Enzinger as a separate tumor entity. Due to the unpredictable biological behaviour of the tumor, a borderline malignancy is assumed. CASE REPORT: We report on the case of a 44-year old woman with a metastasising epithelioid hemangioendothelioma in the head and neck area. The primary tumor was located in the subcutis of the left forehead. Due to local recurrences surgical treatment was performed three times after the initial removal in 1993. At the time of the last local recurrence in 1996, a tumor in the left parotid gland was diagnosed and was the reason for a lateral parotidectomy. Pathohistologically, a metastasis of the epithelioid hemangioendothelioma was found. Postoperative radiotherapy was performed and no recurrence occurred until now (22 months follow-up). CONCLUSION: Metastatic epithelioid hemangioendotheliomas are rarely found in the head and neck area. Literature has not yet reported on metastasis formation in the parotid gland. The case illustrates the potentially malignant behaviour of epithelioid hemangioendotheliomas. Hence, therapy should consist of a combination of radical tumor removal and post-operative radiotherapy.

Adult↗
PubMed2006

Primary peritoneal epithelioid hemangioendothelioma.

Epithelioid hemangioendothelioma is an uncommon malignant vascular tumor of intermediate grade that occurs in a variety of organs and soft tissues. Diagnosis of epithelioid hemangioendothelioma is often complicated by the rarity of the tumor, and because the tumor shares many morphologic features with other peritoneal neoplasms. This report presents 3 cases of epithelioid hemangioendotheliomas arising as primary tumors of the peritoneum and reviews 7 previously reported cases to establish the demographic, clinical, morphologic, immunohistochemical, and ultrastructural features of this neoplasm. These cases demonstrate that the light microscopic features are very similar to epithelioid hemangioendothelioma arising at more conventional sites, such as the liver, that immunohistochemical analysis provides a reliable approach for confirming or establishing the diagnosis, and that at least one endothelial marker (either CD31, CD34, or factor VIII) should be positive for a definitive diagnosis. This series identifies the characteristics of peritoneal epithelioid hemangioendothelioma that can be reliably used for diagnosis.

Adult↗
PubMed2002

Infantile hepatic hemangioendothelioma presenting as early heart failure: report of two cases.

Hepatic hemangioendothelioma is rare. We report on hepatic hemangioendotheliomas in 2 young infants, with initial manifestations of respiratory distress and congestive heart failure. Serum alpha-fetoprotein (alphaFP) level was as high as 26,343 microg/l at 14 days old in 1 case, but was only 18 microg/l in the other case. The 2 patients were treated with prednisolone and hepatic artery ligation, respectively, with no residual sequelae after 12 months of follow-up. In this article, the clinical courses of these 2 young infants are reviewed, and the management of infantile hepatic hemangioendothelioma complicated with heart failure is discussed. In our experience, early heart failure caused by infantile hepatic hemangioendothelioma can be well controlled, especially with prednisolone therapy or by hepatic artery ligation. Spontaneous regression has been reported. However, without early recognition and therapeutic intervention, progression to decompensated heart failure may lead to death. Furthermore, it is necessary to differentiate infantile hepatic hemangioendothelioma (IHH) from hepatic malignancies.

Female↗
PubMed1997

[Spindle-cell hemangioendothelioma with monomelic and multifocal form in a child].

BACKGROUND: Spindle-cell hemangioendothelioma is a soft tissue skin tumor recently identified histologically. It can occur at all ages but generally is seen in young adults. The lesion usually occurs as a subcutaneous mass involving the limbs. CASE REPORT: A particular case of spindle-cell hemangioendothelioma was observed in an 18-month-old child. The lesions progressed with a monomelic distribution on the upper limb. Histological diagnosis of spindle-cell hemangioendothelioma was achieved at the age of 6 years. DISCUSSION: The age of the patient and the monomelic distribution is particular in this case of spindle-cell hemangioendothelioma, inciting a nosological discussion on this disease and other vascular tumors of childhood and the relationship of these types of lesions with Maffucci's syndrome. Although no anomalies have been detected to date, radiological surveillance is needed as cases of Maffucci's syndrome associated with spindle-cell hemangioendothelioma is described in the literature.

Hand↗
PubMed2006

Kaposiform hemangioendothelioma arising from the internal auditory canal.

Kaposiform hemangioendothelioma is a rare vascular tumor and locally aggressive endothelial-derived spindle cell neoplasm, which occurs almost exclusively in infants and adolescents. Radiologically, hemangioendothelioma, including Kaposiform hemangioendothelioma, is seen as a highly vascularized well-enhancing tumor, but no characteristic findings differentiate Kaposiform hemangioendothelioma from other soft-tissue tumors, particularly when the tumor is too small to have any locally aggressive features or identifiable large vessels. We present a case of Kaposiform hemangioendothelioma in the internal auditory canal that had no differential features on initial MR images and rapidly grew into a huge mass in a few months.

Ear Neoplasms↗
PubMed1996

Uterine polypoid hemangioendothelioma: conservative treatment.

BACKGROUND AND OBJECTIVE: A polypoid uterine hemangioendothelioma was treated by conservative means. STUDY DESIGN/MATERIALS AND METHODS: The diagnosis of hemangioendothelioma was confirmed by histopathology and angiography. The patient desired to retain her fertility; therefore, the hemangioendothelioma was treated by hysteroscopically guided Nd:YAG laser ablation. RESULTS: Five years of follow-up by hysteroscopy and dilation and curettage reveal no evidence of recurrence. The patient continues to have regular menses. CONCLUSION: Conservative management of a uterine hemangioendothelioma by Nd:YAG laser ablation was successful over 5 years of follow-up.

Adult↗
PubMed1980

Histopathologic studies of benign infantile hemangioendothelioma of the parotid gland.

Five hundred and eighty-four cases of primary parotid gland tumors and tumor-like conditions were examined. Primary benign hemangiomas of the parotid gland were relatively rare, comprising 29 cases (4.9%) of 584 tumors. These could be divided into either cavernous hemangioma or so-called benign hemangioendothelioma. In our series, the incidence of the latter was 1.5% (nine cases), or 31.0% of the total number of hemangiomas. The so-called benign hemangioendotheliomas were found in patients under the age of 12 months, during the infantile period, and occurred predominantly in females and on the right side. Histologically, this tumor showed a uniform growth pattern so that each lobular portion exhibited a strikingly identical appearance. The vascular lumina of the tumor were lined by flattened endothelial cells that were occasionally mixed with some plump cells showing mild atypism. Electron microscopy revealed that the tumor consisted of two distinct components, one being definite endothelial cells lining the vascular channels and the other pericytes located outside of the endothelium embracing the vascular channels. The tumor cells can be interchangeable with one another. It seems that this tumor is derived from the intralobular vascular system of the parotid gland. This tumor is benign in nature and found almost exclusively during the infantile period. Hence, from the clinicopathologic aspect, "benign infantile hemangioendothelioma" seems to be a more suitably descriptive term than simply "benign hemangioendothelioma".

Adolescent↗
PubMed1983

Malignant hemangioendothelioma of the thyroid and factor VIII-related antigen.

Thirty-six malignant hemangioendotheliomas of the thyroid were examined immunohistochemically using antibody probes to factor VIII-related antigen in order to reevaluate the histogenesis of this neoplasia. The 36 cases were reclassified according to their light microscopic features without prior knowledge of the immunohistochemical results. Three different tumor types were discerned: Group I: classical hemangioendotheliomas (20 cases); Group II: borderline cases between malignant hemangioendotheliomas and anaplastic carcinomas (14 cases) and Group III: anaplastic carcinomas with hemangio-endotheliomatous features (2 cases). Factor VIII-related antigen could be demonstrated in 12 (60%) tumors of group I, 3 (21%) tumors of group II and in neither tumor of group III. Five control cases with the typical histological picture of anaplastic carcinoma of the thyroid were negative for factor VIII-related antigen. The results of our study suggest that at least part of the tumors termed as malignant hemangioendotheliomas are in fact derived from endothelial cells.

Aged↗