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.     

Recurring bone epithelioid hemangioma

Bone vascular tumors are very rare. Epithelioid types are described according to their architecture, their degree of vascular differentiation, and their cytonuclear atypia. The include epithelioid hemangioma, epithelioid hemangioendothelioma, and angiosarcoma. We report a case of L4 corpus vertebral bone epithelioid hemangioma. The patient was a 25-year-old man with a tumor that recurred twice. The lesion was characterized by a vascular lumen lined by cells with regular nuclei and inflammatory infiltrates. Capillaries were lined by prominent epithelioid endothelial cells, associated with CD31+ and cytokeratin-.     

A case of epithelioid hemangioma of the spine

Epithelioid hemangioma is a rare and benign vascular tumor, most often occurring in the skin. Numerous other localizations, including bones, have been reported. The overall favorable clinical outcome of cutaneous epithelioid hemangioma is now well documented, but it still remains debated in osseous localization, as local recurrences and metastases have been described. We report a case of epithelioid hemangioma of the spine occurring in a 25-year-old male, and discuss main differential diagnoses. Recent studies tend to demonstrate that osseous epithelioid hemangioma, as cutaneous epithelioid hemangioma, should be considered as a benign tumor. Histologically, epithelioid hemangioma consists of a vascular proliferation with diffuse or lobular pattern and features vascular spaces lined by epitheliod endothelial cells with numerous lymphocytes and eosinophils. The main differential diagnosis is represented by epithelioid hemangioendothelioma, the surgical treatment of which must be more aggressive. Precise histological diagnosis is essential for accurate clinical management and to avoid overtreatment.     

Fine-needle aspiration cytology of angiolymphoid hyperplasia with eosinophilia: a case report with electron microscopy and immunohistochemistry

Angiolymphoid hyperplasia with eosinophilia (ALHE) is an uncommon vascular inflammatory lesion usually involving the dermis or subcutaneous tissue of the head-neck region of middle-aged women. Histologically, this lesion shows a florid proliferation of vessels lined by particular endothelial cells and an inflammatory infiltrate composed of lymphocytes and eosinophils. The fine-needle aspiration (FNA) cytology of a nodular lesion in the retroauricolar region of a 18-yr-old woman showed spindle-shaped and polygonal cells with vesicular nuclei and deeply eosinophilic cytoplasm containing well-defined vacuoles in a background of eosinophils and lymphocytes. These features were consistent with a proliferation of epithelioid endothelial cells, and a diagnosis of ALHE was suggested. The histology confirmed the preoperatory diagnosis, and ultrastructural and immunohistochemical studies further demonstrated the endothelial nature of epithelial-appearing cells. Because the clinical appearance of the lesion may mimic a large number of benign and malignant diseases, a preoperatory diagnosis of ALHE is rarely made. The FNA cytology may represent a simple and reliable method with which to study and diagnose proliferations of epithelioid endothelial cells.     

Infection-associated vascular lesions in acquired immunodeficiency syndrome patients

Several reports have recently appeared in the literature describing "unique" non-neoplastic vascular lesions in patients with the acquired immunodeficiency syndrome (AIDS). These lesions may be mistaken clinically and histologically for Kaposi's sarcoma. The terms epithelioid angiomatosis, epithelioid or histiocytoid hemangioma, and pyogenic granuloma have all been used to describe a similar entity in which cat scratch disease bacillus (CSDB) was subsequently identified. Lesions closely resembling this entity occur in patients with bartonellosis. We report a case of a cutaneous vascular lesion on the hand of an AIDS patient in which cytomegalovirus (CMV) and organisms consistent with CSDB were both found. Simultaneous infections with CMV and CSDB have not been previously described. The presence of these organisms in and around endothelial cells may provide the common stimulus for the formation of these reactive vascular proliferations.     

Unusual hemangioendothelioma of the liver with epithelioid morphology associated with marked eosinophilia: autopsy case

Vascular neoplasms characterized by epithelioid endothelial cells consist of several different entities from benignity to high-grade malignancy. Because of histological overlap between them, there is substantial difficulty in classifying them correctly. The present patient, a 33-year-old man, presented with hepatomegaly, striking eosinophilia and elevated serum interleukin-5 level. Biopsy and autopsy revealed an unusual epithelioid vascular tumor in the liver, which is histologically distinct from epithelioid hemangioma, epithelioid hemangioendothelioma, or epithelioid angiosarcoma. The tumor cells had vasoformative and partly solid growth with no severe nuclear atypia and very low mitotic activity, and the histological features were similar to those of the entity recognized as hemangioendothelioma of bone. Organs other than the liver, for example the testes and bone, were also involved. This tumor should be considered in the differential diagnosis of severe eosinophilia.     

Mast cells in hemangioma: a double-edged sword

Hemangioma is a common benign vascular tumor characterized by presentation within the few weeks of life, rapid growth during the first year and spontaneous involution over a period of several years. Mast cells play an important role in immunity and angiogenesis by synthesizing and releasing a group of bioactive mediators, cytokines and chemokines. Previous study confirmed that the number of mast cells is highest during the involuting phase, lower in the involuted phase, and lowest in the proliferating phase. There is evidence that several mast cell mediators are angiogenic by regulating endothelial cell proliferation and degrading the extracellular matrix. Mast cells may also secrete antiangiogenic modulators in the involuting stage of hemangioma. It could be postulated that mast cells may play both angiogenic and antiangiogenic roles in different stage of hemangioma. The mast cell secretion may trigger the "angiogenic switch", while the angiogenic roles may function through the other potential angiogenic factors. Better understanding of the exact role of mast cells in hemangioma will contribute to build new therapeutic interventions targeting mast cells in hemangioma.     

Cutaneous angiolymphoid hyperplasia with high endothelial venules is characterized by endothelial expression of cutaneous lymphocyte antigen

Two cases in which the presence of cutaneous lesions with a characteristic admixture of lymphoid hyperplasia and vascular proliferation lead to the diagnosis of so-called acral pseudolymphomatous angiokeratoma (APA) of children are reported. Owing to the prominence of its blood vessels, so striking as to be reminiscent of high endothelial venules (HEVs), APA was initially interpreted as a vascular lesion rather than a pseudolymphoma. This resemblance is further compounded by our finding that cutaneous lymphocyte antigen (CLA), an HEV marker, is also expressed in APA epithelioid blood vessels. Consequently, we believe that "cutaneous angiolymphoid hyperplasia with high endothelial venules" (ALH-HEV), which alludes to the dual nature of proliferating elements and the HEV-like phenotype of the vascular component, would be a better denomination for this entity. Additionally, we speculate that ALH-HEV lymphoid hyperplasia self-perpetuates through the transformation of dermal capillaries into HEVs, which would bind non-skin homing lymphocytes expressing L-selectin and promote their local recruitment and recirculation.     

BILATERAL OVARIAN HEMANGIOMAS ASSOCIATED WITH DIFFUSE HEMANGIOENDOTHELIOMATOSIS: A CASE REPORT

A patient who had disseminated vascular tumors involving the bilateral ovaries, bilateral lungs and pleura, pericardium, and mediastinum is reported. The tumors were histologically of the capillary, and partly the cavernous, type of hemangioma. However, endothelial cell growth was prominent in some areas, especially in the lung, and the histology of the lung tumor resembled epithelioid hemangioendothelioma or intravascular bronchiolo-alveolar tumor (IVBAT). In the endocardium of the right atrium, an endothelial tumorous projection was observed, and there were tiny foci of tumor cell nests in the abdominal venous wall. Small lymphangiomas were also found in the subcapsular region of the spleen. These findings suggest that there had been an abnormal proliferation of systemic endothelial cells and that tumors of endothelial cell origin with diverse histological patterns developed with this condition as a background. The autopsy finding of fibrin thrombi in multiple organs as well as laboratory data including thrombocytopenia suggest that this case belongs to the "Kasabach-Merritt syndrome".     

Endovascular papillary angioendothelioma (Dabska tumor)

The histological diagnosis of endovascular papillary angioendothelioma (EPA) (Dabska tumor) is controversial although the tumor is included in the World Health Organization classification. Papillary endothelial proliferation with a central hyaline core is one of the most characteristic features of EPA; however, this type of proliferation has been observed in other vascular tumors, such as angiosarcoma, retiform hemangioendothelloma and glomeruloid hemangioma. Several vascular tumors have EPA-like foci and EPA is not well defined generally. Endovascular papillary angioendothelioma may not be a distinct entity and may well include a heterogeneous group of lesions.     

Bilateral ovarian hemangiomas associated with diffuse hemangioendotheliomatosis: a case report

A patient who had disseminated vascular tumors involving the bilateral ovaries, bilateral lungs and pleura, pericardium, and mediastinum is reported. The tumors were histologically of the capillary, and partly the cavernous, type of hemangioma. However, endothelial cell growth was prominent in some areas, especially in the lung, and the histology of the lung tumor resembled epithelioid hemangioendothelioma or intravascular bronchiolo-alveolar tumor (IVBAT). In the endocardium of the right atrium, an endothelial tumorous projection was observed, and there were tiny foci of tumor cell nests in the abdominal venous wall. Small lymphangiomas were also found in the subcapsular region of the spleen. These findings suggest that there had been an abnormal proliferation of systemic endothelial cells and that tumors of endothelial cell origin with diverse histological patterns developed with this condition as a background. The autopsy finding of fibrin thrombi in multiple organs as well as laboratory data including thrombocytopenia suggest that this case belongs to the "Kasabach-Merritt syndrome."     

Sclerosing angiomatoid nodular transformation of the spleen (SANT): a case report

We report a case of sclerosing angiomatoid nodular transformation of the spleen incidently discovered in a 41-year-old man. The macroscopic examination showed the presence of a reddish brown, well delineated but not encapsulated, multinodular lesion being histologically characterized by nodules made up of complex vascular structures lined by monomorphous but non atypical endothelial cells, surrounded by fibrin and a collagen stroma rich in spumous macrophages and hemosiderophages. The immunohistochemical markers carried out showed the presence of capillaries, veins and sinusoids normally found within the splenic parenchyma, but adopting an unusual configuration. This distinct entity, recently described and completely benign, must be included in the differential diagnosis of the vascular lesions of the spleen, which includes, among others, the hemangioma, the littoral cell angioma, the hemangioendothelioma and the inflammatory myofibroblastic tumor.     

Uterine glomeruloid hemangioma in a patient without POEMS syndrome

Cutaneous glomeruloid hemangioma is a hallmark of POEMS syndrome. These patients have elevated serum levels of vascular endothelial growth factor. The authors report an extracutaneous uterine glomeruloid hemangioma in an 82-year-old woman with a history of breast and endometrial carcinomas. Within the lumen of myometrial vessels, a lobular, glomeruloid proliferation of capillary-like CD31 and vascular endothelial growth factor receptor-1-positive endothelial cells was found. The capillary loops were lined by endothelial cells, most of them containing PAS-positive and immunoglobulin-positive eosinophilic hyaline globules (thanatosomes). This vascular proliferation was consistent with a glomeruloid hemangioma. Although an extracutaneous glomeruloid vascular proliferation has been found in the retroperitoneal adipose tissue in a patient with POEMS syndrome, this study reports what seems to be the first case of visceral glomeruloid hemangioma in a patient without POEMS syndrome. The authors hypothesize that the glomeruloid endothelial cell proliferation with vascular endothelial growth factor receptor-1 expression may be a paraneoplastic phenomenon.     

Epithelioid haemangioma (angiolymphoid hyperplasia with eosinophilia) and Kimura''s disease in Chinese

Although Kimura's disease has often been considered to be identical to angiolymphoid hyperplasia with eosinophilia (epithelioid haemangioma), recent studies suggest that they are different clinicopathological entities. In this study, we have made a detailed morphological comparison of 10 cases of epithelioid haemangioma and 40 cases of Kimura's disease occurring in the Chinese population. The epithelioid haemangiomas occurred in the subcutaneous tissue, skin and maxillary antrum, whereas Kimura's disease affected the subcutaneous tissue, major salivary glands and lymph nodes. Distinctive features of epithelioid haemangiomas were exuberant proliferation of vessels lined by cuboidal to hobnail endothelial cells with irregular nuclei and cytoplasmic vacuoles, fibromyxoid matrix, involvement of muscular coat of blood vessels and zonation of inflammatory infiltrate towards the peripheral portion of the lesion. Distinctive features of Kimura's disease were florid lymphoid infiltrate with prominent lymphoid follicles, vascularization of germinal centres, germinal centre necrosis, marked eosinophilia with or without eosinophil abscess formation, proliferation of high endothelial venules, and sclerosis. The histological features suggest that epithelioid haemangioma is a proliferation of atypical endothelial cells, possibly neoplastic, that is associated with a variable inflammatory infiltrate, whereas Kimura's disease is primarily an inflammatory condition in which high endothelial venules are usually found.     

Potential roles of allograft inflammatory factor-1 in the pathogenesis of hemangiomas

Hemangiomas are benign tumors of the vascular endothelium and are the most common tumors of infancy. These tumors are characterized by an initial phase of rapid proliferation in the first months of life, which is followed, in most cases, by spontaneous slow involution. Despite their high prevalence, their detailed pathogenesis remains unknown. Recent studies suggest that immunity responses, inflammatory cells and their precursors, myeloid cells, play important roles in the growth and involution of hemangiomas. The allograft inflammatory factor-1 is a powerful gene that is involved in several kinds of inflammatory response-related diseases. Studies also show that it is implicated in angiogenesis, proliferation and differentiation of stem cells, and development of tumors. Taken all these evidences into consideration, we hypothesize that allograft inflammatory factor-1 plays potential roles in pathogenesis, proliferation and involution of hemangioma. Investigating the role of allograft inflammatory factor-1 in the proliferation and involution of hemangioma will lead to a better understanding of pathogenesis of this lesion. Furthermore, the subtle regulation of allograft inflammatory factor-1 in the involution of hemangiomas will help design a new anti-angiogenic therapy for some tumors.     

Monocytes: a possible source of hemangioma endothelial cells

Hemangiomas have a distinctive proliferative phase characterized by rapid proliferation of capillaries within the first year of life, followed by an involuting phase characterized by spontaneous and steady regression of the tumor that may last up to 5-10 years. Angiogenesis has been proposed to be the predominant mechanism of vessel formation in hemangiomas. Emerging evidence shows that endothelial progenitor cells play an important role in the physiological and pathological angiogenesis. However, the numbers of endothelial progenitor cells in hemangioma are small. Recent studies show that large numbers of monocytes, the ancestor of endothelial progenitor cells, are present in hemangioma. In this paper, we hypothesize that monocyte is a possible source of hemangioma endothelial cells according to previous research evidence. This hypothesis suggests that monocytes contribute to early expansion of hemangioma. Further investigation is needed to determine which factors influence the homing and the differentiation of monocytes in hemangioma and potential therapeutic implications of these monocytes.     

Excessive Endothelial Cell Proliferation Occurring in an Organizing Thyroid Hematoma: Report of a Case and Review of the Literature

The authors report a case of a thyroid hematoma containing an exuberant endothelial cell proliferation. No underlying vascular neoplasm was apparent and the hematoma occurred in a substernal goiter from a 90-yr-old male. Review of the literature revealed four similar previously reported cases of endothelial cell proliferation found in thyroid hematomas for a total of five cases. A fine needle aspiration or biopsy procedure antedated the definitive surgery in four patients and may be responsible for the formation of the hematoma in these cases. Areas of the endothelial proliferation resembled papillary endothelial hyperplasia (Masson’s hemangioma) in four cases and cavernous hemangioma in three. However, these lesions occurred outside of vascular lumina and instead were intimately associated with fibrinous material of the hematoma. In addition, two cases contained foci of endothelial cells with nuclear atypia or intracytoplasmic lumens requiring distinction from angiosarcoma. These vigorous endothelial proliferations most likely represent alterations in the organization of a thyroid hematoma, a process similar to papillary endothelial hyperplasia, which is usually intravascular. Primary vascular neoplasms of the thyroid are exceedingly rare. Nevertheless, awareness of exuberant endothelial cell proliferation as a potential, albeit rare, manifestation of a thyroid hematoma should prompt careful attention to distinguish this reactive process from vascular neoplasms such as hemangioma or angiosarcoma.     

Anastomosing hemangioma arising from the kidney: a case of slow progression in four years and review of literature

Reported herein is a renal anastomosing hemangioma which developed slowly in the past four years. A 25-year-old woman was found a mass localized in the upper portion four years ago, and only slow progression in the past four years. She underwent a laparoscopic partial nephrectomy of right kidney and diagnosed as anastomosing hemangioma. On histology the vascular components of the tumor had an anastomosing pattern without well-definite margins. Immunohistochemically, only endothelial markers (CD31, CD34) were expressed on the vascular components of tumor cells. Smooth muscle actin (SMA), cytokeratin (CK), EMA and S-100 and so on were all negative in the epithelioid tumor cells. The patient was alive at 16 months after operation, without any evidence recurrence or metastasis. Anastomosing hemangioma is an extremely rare vascular neoplasm; only 23 cases were previously described until now. Our report of anastomosing hemangioma arising from the kidney with slow progression will improve the knowledge of primary vascular tumors arising in the kidney.     

Epithelioid hemangioendothelioma and related lesions

Epithelioid hemangioendothelioma (EH) is the prototype of a group of vascular tumors characterized by an epithelioid or histiocytoid endothelial cell. This family also includes the epithelioid hemangioma (angiolymphoid hyperplasia with eosinophilia) and epithelioid forms of angiosarcoma. This review discusses the principal clinical, pathologic, and biologic differences among these three lesions. In particular the various manifestations of EH of soft tissue, bone, lung (previously called intravascular bronchioloalveolar tumor), and liver are discussed. Long-term follow-up data of EH of soft tissue and lung are provided.