Pleural Epithelioid Hemangioendothelioma

Epithelioid hemangioendothelioma (EHE) is a rare tumor of vascular origin. While it can be found in any tissue, it is most often found in lung and liver and usually has an intermediate behavior. EHEs originating from pleural tissue have been less frequently described than those from other sites. Furthermore, to date, all of the cited pleural EHEs were described as highly aggressive. In the present report, we describe a rare case of pleural EHE extending to lung and bone in a 31-year-old woman. The histological diagnosis was confirmed by both conventional examination and immunohistochemistry. Her disease stabilized during the 4th course of adriamycin (45 mg/m², day 1-3), dacarbazine (300 mg/m², day 1-3) and ifosfamide (2,500 mg/m², day 1-3) with mesna, and she survived for 10 months after the diagnosis.     

Epithelioid Hemangioendothelioma of the Lung

Light and electron microscopic features of an epithelioid hemangioendothelioma of the lung in a 43-year-old woman are reported. The tumor cells stained for factor VIII-related antigen. A mass excised from the thigh 10 years earlier showed identical appearances.     

Epithelioid Hemangioendothelioma of the Liver in Primary Biliary Cirrhosis

We report a case of primary epithelioid hemangioendothelioma of the liver occurring in a patient with primary biliary cirrhosis, stage III. The hepatic tumor was found incidentally by imaging techniques and was surgically resected under a tentative diagnosis of metastatic carcinoma. The resected tumor (1.8±1.6 cm) showed Wtcal histologic features of epithelioid hemangioendd jelioma. The tumor cells were positive for factor VIII related antigen and were stained with Ulex europaeus lectin I. Ultrastruc-turally, the tumor cells showed cytoplasmic vacuoles, tight junctions, basal lamina, pinocytotic vesicles, bundles of thin filaments (approximately 10 nm in diameter) and Weibel Palade bodies. The non tumorous part of the liver showed features of primary biliary cirrhosis, stage III. This is the first reported case of epithelioid hemangioendothelioma occurring in a liver with primary biliary cirrhosis. Acta Pathol Jpn 39: 607 611, 1989.     

Periosteal Epithelioid Hemangioendothelioma with Leptomeric Fibrils

A case of recurrent periosteal epithelioid hemangioendothelioma of the right femur in a 66-year-old woman is reported. Microscopic examination showed epithelioid tumor cells with frequent intracytoplasmic vacuoles arranged in small nests or cords in hyalinized stroma. Immuno- and lectinhistochemical studies for factor VIII-related antigen and ulex europaeus I lectin indicated the endothelial nature of the tumor cells. Ultrastructurally, a few tumor cells contained leptomeric fibrils and crystalline filamentous aggregates in addition to showing certain features of endothelial cells. There seems to be no previous report of a vascular tumor containing leptomeric fibrils, which are often noted in normal striated muscle cells and their tumors. The bland appearance of the epithelioid tumor cells, poor vasoformative nature and prolonged clinical course differentiated this tumor from conventional angiosarcoma and so-called hemangioendothelioma of bone.     

Periosteal Epithelioid Hemangioendothelioma with Leptomeric Fibrils

A case of recurrent periosteal epithelioid hemangioendothelioma of the right femur in a 66-year-old woman is reported. Microscopic examination showed epithelioid tumor cells with frequent intracytoplasmic vacuoles arranged in small nests or cords in hyalinized stroma. Immuno- and lectinhistochemical studies for factor VIII-related antigen and ulex europaeus I lectin indicated the endothelial nature of the tumor cells. Ultrastructurally, a few tumor cells contained leptomeric fibrils and crystalline filamentous aggregates in addition to showing certain features of endothelial cells. There seems to be no previous report of a vascular tumor containing leptomeric fibrils, which are often noted in normal striated muscle cells and their tumors. The bland appearance of the epithelioid tumor cells, poor vasoformative nature and prolonged clinical course differentiated this tumor from conventional angiosarcoma and so-called hemangioendothelioma of bone.     

Intriguing Case: Atypical Tumor of the Mediastinum: Epithelioid Hemangioendothelioma Containing Metaplastic Bone and Osteoclastlike Giant Cells

A 66-year-old woman presented with a superior-anterior mediastinal mass that contained considerable calcification by computed tomography. Initial biopsy interpretation was inconclusive but suggested a giant cell sarcoma of soft parts because the tumor contained atypical epithelioid cells and osteoclastlike giant cells. After radiation and chemotherapy, the tumor was removed. It contained large epithelioid cells, pale chondroid areas, metaplastic bone, and osteoclastlike giant cells. Immunohistochemical and electron microscopic studies revealed the epithelioid cells to be factor VIII positive and to contain abundant intermediate filaments, micropinocytotic vesicles, Weibel-Palade bodies, basal lamina, and primitive cell-cell junctions. These epithelioid cells also stained for muscle-specific actin (HHF-35), yet were negative for desmin and cytokeratins (CAM 5.2 and AE1/3). The findings were most consistent with those reported to occur in epithelioid hemangioendothelioma.     

Kaposiform Hemangioendothelioma Complicated by Kasabach-Merritt Phenomenon: Ultrastructural Observation and Immunohistochemistry Staining Reveal the Trapping of Blood Components

Abstract

Kaposiform hemangioendothelioma (KHE), a borderline tumor of endothelial origin, is associated with Kasabach-Merritt phenomenon, characterized by profound thrombocytopenia and consumptive coagulopathy resulting from the localized intravascular coagulation (LIC) in the tumor. Previous studies have suggested that the trapping of blood components, including platelets, may underlie the LIC in KHE. However, more evidence is needed to support this hypothesis. In this study, one case of a Chinese infant with a KHE in the left arm was complicated by Kasabach-Merritt phenomenon. The tumor was partially resected and the sample was used for ultrastructural observation and immunohistochemistry staining of Glut-1. Ultrastructural observation found the trapping of erythrocytes, platelets, macrophages, and lymphocytes in the slit-like channels of the tumor nodules, and phagocytic vesicles in the cytoplasm of neoplastic cells. Immunohistochemistry staining further showed numerous Glut-1(+) erythrocytes in the channels. In conclusion, our results provided compelling morphological evidence of the trapping of blood components in KHE, which may interpret the LIC in the tumor and subsequent consumptive coagulopathy.     

Intriguing Case: Atypical Tumor of the Mediastinum: Epithelioid Hemangioendothelioma Containing Metaplastic Bone and Osteoclastlike Giant Cells

A 66-year-old woman presented with a superior-anterior mediastinal mass that contained considerable calcification by computed tomography. Initial biopsy interpretation was inconclusive but suggested a giant cell sarcoma of soft parts because the tumor contained atypical epithelioid cells and osteoclastlike giant cells. After radiation and chemotherapy, the tumor was removed. It contained large epithelioid cells, pale chondroid areas, metaplastic bone, and osteoclastlike giant cells. Immunohistochemical and electron microscopic studies revealed the epithelioid cells to be factor VIII positive and to contain abundant intermediate filaments, micropinocytotic vesicles, Weibel-Palade bodies, basal lamina, and primitive cell-cell junctions. These epithelioid cells also stained for muscle-specific actin (HHF-35), yet were negative for desmin and cytokeratins (CAM 5.2 and AE1/3). The findings were most consistent with those reported to occur in epithelioid hemangioendothelioma.     

Primary Intraoral Epithelioid Hemangioendothelioma Presenting in Childhood: Review of the Literature and Case Report

Epithelioid hemangioendothelioma (EH) is a recently described vascular neoplasm of borderline or intermediate malignant potential. This tumor arises from medium- to large-sized vessels, primarily involves the soft tissues of the extremities as well as the liver and lung, and rarely occurs in the head and neck region. Only eight well-documented cases of intraoral EH have been reported. We present an additional pediatric case of EH confined to the oral cavity and review the literature regarding EH presenting as an intraoral mass. EH is characterized histopathologically as an epithelioid lesion arranged in nests, strands, and trabecular patterns with infrequent vascular spaces. Occasional erythrocytes within intracytoplasmic lumina may be seen in tumor cells. Ultrastructural examination typically shows intracytoplasmic lumina with pseudopodial cellular membrane extensions. The cytoplasm usually contains intermediate filaments infrequently associated with Weibel-Palade bodies. Neoplastic cells are immuno-reactive for factor VIIIR:Ag and Ulex europaeus. Histopathologic features, which may be associated with aggressive clinical behavior, include significant cellular atypia, one or more mitoses per 10 high-power fields, an increased proportion of spindled cells, focal necrosis, and metaplastic bone formation. Because of the intermediate malignant potential of epithelioid hemangioendothelioma, complete tumor resection is recommended for intraoral lesions.     

Ultrastructural and Immunohistochemical Study of Epithelioid Hemangioendothelioma of Bone: Coexpression of Epithelial and Endothelial Markers

Four cases of epithelioid hemangioendothelioma of bone—a borderline malignant tumor of vascular origin —were studied ultrastructurally and immuno histochemically. The epithelioid tumor cells were positive for vimentin, polyclonal and monoclonal cytokeratins, and the endothelial markers factor Vlll-related antigen (FVIII:RAg) and Ulex europaeus agglutinin I. The coexpression of polyclonal cyto keratin and FVIII:RAg was demonstrated by means of step sections in the same tumor cells. The endothelial origin of epithelioid tumor cells was supported ultrastructurally by identification of Weibel-Palade bodies.     

Hepatic epithelioid hemangioendothelioma metastasized to the peritoneum,omentum and mesentery: a case report

Epithelioid hemangioendothelioma (EHAE) is a malignant vascular tumor derived from endothelial cell often misdiagnosed as Hepatic carcinoma on the basis of radiological features. Till now etiology of this rare curiosity is unknown but it is related with use of oral contraceptives pills (OCP), liver trauma, exposure to vinyl chloride and hepatitis. We herein report on a case which failed to be diagnosed by cytopathology, computed tomography (CT) and magnetic resonance imaging (MRI). Patient was a 46 yr old man presented with abdominal distension for a month. Initial liver function test (LFT) was increased whereas renal function test (RFT) and alpha-fetoprotein (AFP) were normal. His abdominal ultrasound revealed multiple hypoechoic nodules and multiple liver calcifications. Subsequently laparoscopic omental biopsy and Ultrasound guided liver biopsy was done showing the neoplastic cells scattered in fibrous stroma. The immunohistochemistry for endothelial tumor cells stained positive for Vimentin (+++), CD10 (+++), CD34 (++), CD31 (+), Factor VIII antigen (focal) (+) and low proliferative activity for ki-67. Our case is very interesting in which patient admitted with nonspecific symptoms of abdominal pain and diagnosed to be a Malignant Hepatic EHAE metastasized to the peritoneum, omentum and mesentery. The patient was on thalidomide 50 mg/day and increased to 100 mg/day. 5-Flurouracil (FU) intraperitoneal chemotherapy and other symptomatic and supportive treatment was given to the patient. Our case highlights on the importance of immunohistopathological diagnosis, compare the radiological findings of this disease and discuss the treatment strategy with review of available literature.     

Primary kaposiform hemangioendothelioma of a long bone: two cases in unusual locations with long-term follow up

Kaposiform hemangioendothelioma (KHE) is a rare vascular neoplasm of low malignant potential that mainly affects infants and adolescents. The tumor almost exclusively occurs in somatic soft tissue or the retroperitoneum. We report herein two cases of primary KHE occurring in a long bone without cutaneous changes with long-term follow up in young patients. The patients were a 9-year-old girl and 5-year-old boy presenting with lytic lesions of the femur and humerus, respectively, without cutaneous lesions. Histologically, the neoplasms were comprised of nodules of spindle- to oval-shaped cells growing in an infiltrative fashion. The neoplastic cells formed poorly canalized or slit-like blood vessels alternating with solid spindle areas. Immunohistochemical studies showed that the tumor cells expressed CD31, CD34 and Fli1, but not HHV8, LNA-1 or GLUT1. D2-40 stained the neoplastic spindle cells and lymphatic channels adjacent to vascular lobules. The girl remains well with 15 years and 6 months follow up after a second complete excision. The boy has no signs of recurrence or metastasis nearly 5 years after local complete excision. To our best knowledge, this is the first report in the English literature of primary long bone occurrences of KHE without cutaneous changes with long-term follow up.     

Composite hemangioendothelioma arising from the kidney: case report with review of the literature

Reported herein is a medical curiosities vascular tumor primary arising from the kidney and exhibiting unique histopathological features. A 32-year-old woman underwent a total nephrectomy of right kidney because of a mass localized in the inferior pole. Distinct from other vascular lesions, on histology the tumor had a peculiar composite pattern, consisting of benign and malignant vascular components, which were haphazardly intermixed without any definite margins. The malignant component was composed of epithelioid hemangioendothelioma (45%) and angiosarcoma (50%) with moderate differentiation. Immunohistochemically, the oval to cuboidal to spindle tumor cells expressed only endothelial markers (CD31, CD34 and factor VIII-related antigen). And the angiosarcomatous component was characterized by the presence of a greater proliferation index Ki-67. Unlike other epithelial tumors, smooth muscle actin (SMA), cytokeratin, EMA and S-100 were all negative in the epithelioid tumor cells. These findings led to the diagnosis of a low-grade vascular neoplasm with morphological features consistent with so-called composite hemangioendothelioma (CHE). At 11 month follow up the patient was alive, without evidence of tumor recurrence. CHE is an extremely rare vascular neoplasm, with borderline malignant potential, which mostly occurs in distal extremity of the limbs at the cutaneous level and, only 30 cases have been previously described until now. To our knowledge, this is the first report of CHE arising from the kidney and widens the spectrum of primary vascular tumors arising in the kidney.     

网状血管内皮瘤八例临床病理学分析

目的探讨8例网状血管内皮瘤（ RH）的临床病理学特征,并扩展其瘤谱。方法回顾性复习8例RH的临床资料、病理学形态和免疫学表型,分析随访结果,并复习文献。结果患者平均年龄40岁（范围13～69岁）,本组均为女性。肿瘤分别发生于头颈部和下肢皮肤（5例）、长骨（2例）和脾脏（1例）。皮肤病变表现为局部缓慢性生长的斑块或皮下结节,骨病变表现为患肢疼痛,脾脏病变表现为左季肋部隐痛不适。大体上呈灰黄或红褐色,平均直径3.0 cm,范围0.8～5.0 cm。镜下观察,肿瘤主要由内衬鞋钉样或火柴头样内皮细胞的细长分支状血管组成,形成特征性的网状结构,类似于睾丸网,部分区域内可见条索状或实性巢状排列的上皮样瘤细胞。除细长的网状血管外,3例于局部可见扩张的血管腔,腔内有乳头簇形成,表面被覆鞋钉样内皮细胞,轴心玻璃样变,形态上类似Dabska瘤。血管周围的间质多伴有淋巴细胞浸润,并可有胶原化。本组1例发生区域淋巴结转移。免疫组织化学标记结果显示,网状血管内皮细胞表达 CD31（8／8）、人凝血因子Ⅷ（5／8）、CD34（5／7）和D2-40（1／2）。随访6例（18～67个月）,2例局部复发,无病例发生远处转移。结论RH是一种好发于成年人的中间型血管内皮瘤,以细长分支状血管和鞋钉样内皮细胞为特征,除皮肤外,部分病例可原发于骨和脾脏。部分病例兼有Dabska瘤样形态特点,提示与Dabska瘤有延续性,归属鞋钉样血管内皮瘤同一瘤谱。熟悉网状血管内皮瘤的形态学特点有助于与其他具有鞋钉样内皮细胞的血管肿瘤相鉴别。     

Hemangioendothelioma of the spleen.

Hemangioendotheliomas of the spleen are rare and are considered to be of intermediate/borderline malignancy. We report such a case in a patient who presented with chronic anemia but who otherwise was asymptomatic. The tumor involved half the organ and was solitary and nonencapsulated. Microscopically, it was composed of vascular and stromal elements. Both types of elements showed moderate atypia and rare mitoses. The lining cells stained positively with antibodies to factor VIII-related antigen and Ulex europaeus lectin. The stromal component showed evidence of myofibroblastic differentiation. One year after splenectomy, all hematologic parameters slowly improved and returned to normal. The clinicopathologic differences between hemangioma, angiosarcoma, and hemangioendothelioma are discussed, and cases that have recently been reported in the literature are reviewed.     

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.     

Epithelioid Hemangioendothelioma of the Spermatic Cord

This report describes an epithelioid hemangioendothelioma arising on the spermatic cord of a 50-year-old man who presented with a nodule in the right hemiscrotum. Histologic examination showed a cellular tumor composed of epithelioid cells, many of which had intracytoplasmic lumina containing red blood cells. Immunohistochemistry showed focal positivity of tumor cells with the endothelial markers CD34, factor VIII-related antigen, and Ulex and with the anticytokeratin antibodies CAM5.2 and AE1/AE3. Ultrastructural examination revealed polygonal tumor cells forming vascular spaces and intracytoplasmic lumina containing red blood cells. This is the second documented occurrence of epithelioid hemangioendothelioma at this site. The case illustrates the value of immunohistochemistry and electron microscopy in establishing a diagnosis. Int J Surg Pathol 8(1):75-78, 2000