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.     

A possible hypoxia-induced endothelial proliferation in the pathogenesis of epithelioid hemangioma

Epithelioid hemangioma is an uncommon benign vascular lesion with distinct clinicopathological characteristics. Histologically, this entity mainly composed of well-formed but often immature vessels lined by epithelioid endothelial cells with prominent chronic inflammatory component. There is considerable controversy whether epithelioid hemangioma is a reactive lesion or a true neoplasm. We postulated that the local hypoxia may play a role in the pathogenesis of this vascular tumor. This local hypoxic condition may caused by many incidences such as congenital vascular malformation or trauma. The hypoxia will lead to the proliferation of endothelial cells and the formation of this vascular tumor, which may lead by putative stimulators VEGF and HIF-1. Hypoxia may also promote endothelial cell proliferation through the renin-angiotensin-aldosterone system. Additionally, the inflammatory cells including eosinophils and mast cells may contribute to the endothelial cell proliferation in EH. Further study investigating the associated factors of hypoxia may lead to new, potentially important insights into epithelioid hemangioma, and might also contribute to novel strategies for the management of this entity.     

Epithelioid hemangioma of the foot

Epithelioid hemangioma (angiolymphoid hyperplasia with eosinophilia, EH/ALHE) is a rare benign angioproliferative lesion which typically occurs in the region of the head and neck. In the literature, occurence on the extremity is only rarely described. A case of multiple occurence of EH/ALHE in the skin of the toes and metatarsal bone with osteolysis is reported. Occurence on the extremity, superficial and deep affection and some "atypical" microscopic features may cause diagnostic dilemma. The key diagnostic features of EH/ALHE are vascular channels lined with epithelioid endothelial cells, surrounding layer of myopericytes, absence of atypia and mitotic activity and characteristic inflammation. Immunohistochemistry may be helpful in settling the diagnosis.     

Intravascular (”intimal”) epithelioid angiosarcoma: clinicopathological and immunohistochemical analysis of three cases

Angiosarcomas are rare malignant mesenchymal tumours, characterized morphologically by anastomosing vascular channels lined by atypical and proliferative active endothelial cells. An epithelioid cytomorphology of tumour cells is often seen focally in angiosarcoma, whereas purely epithelioid angiosarcomas are rare. Although angiosarcomas show a vascular differentiation they are almost never confined to pre-existing blood vessels. We describe three cases of intravascular epithelioid angiosarcoma arising in the carotid artery of a 60-year-old man, in the infrarenal part of the abdominal aorta and both renal arteries of a 69-year-old woman, and in the abdominal aorta of a 68-year-old man. In all cases malignant tumour tissue was found incidentally after disobliteration of thrombosed vessels. Histologically, purely epithelioid angiosarcoma composed of solid sheets of epithelioid tumour cells was seen; immunohistochemistry confirmed the endothelial differentiation of neoplastic cells. The reported cases show that angiosarcoma can occasionally arise within a pre-existing vessel. Received: 18 May 1999 / Accepted: 8 June 1999     

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.     

Primary epithelioid angiosarcoma of the adrenal gland

Primary angiosarcomas of the adrenal gland are exceptionally rare vascular tumors. We report a case of a 63-year-old man with an epithelioid angiosarcoma of the left adrenal gland. Visualized on computed tomography as a nonhomogeneous round mass, the tumor measured 3 cm in diameter. Histology showed a vascular tumor composed of epithelioid cells with vesicular nuclei and prominent nucleoli that lined irregular vascular spaces and also formed solid areas and showed pleomorphism and rare mitotic activity. Immunohistochemical stain confirmed the diagnosis of epithelioid angiosarcoma. We report our findings and review previously described literature cases of this rare entity.     

A clinicopathological study of Kimura's disease and epithelioid hemangioma

The clinicopathological features of 9 cases of Kimura's disease and 5 cases of epithelioid hemangioma(EH) were reviewed. Patients with Kimura's disease presented with multiple or solitary large tumors occurring in the thigh, arms as well as parotid gland. Histologically, the lesions were characterized by numerous lymphoid follicles with proliferation of post-capillary venules lined by plump endothelium and a marked eosinophilic infiltration. Patients with EH were older than those with Kimura's disease and presented papular nodular lesions, less than 1 cm in size. Microscopically, there was proliferation of blood vessels lined by epithelioid endothelium with infiltration by lymphocytes and eosinophils. Although there may occasionally be clinicopathological overlap between Kimura's disease and EH, we consider that these two disorders can be recognized as separate entities.     

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.     

Kimura病和上皮样血管瘤的临床病理学观察

目的探讨Kimura病和上皮样血管瘤的临床病理学特征、免疫学表型及两者的鉴别诊断。方法分析和观察9例Kimura病和3例上皮样血管瘤的临床资料和组织学形态,应用免疫组织化学(SP法)研究其免疫表型,所用抗体包括CD43、CD3、CD45RO(UCHL-1)、CD20、CD79α、Ki-67、CD31、CD34、第Ⅷ因子相关抗原(FⅧRAg)、CD68(KP-1)和细胞角蛋白(AE1／AE3)。结果临床上,Kimura病多见于男性患者,而上皮样血管瘤多见于女性。两者均好发于头颈部,但Kimura病多表现为皮下多发性结节或局部肿胀,部分病例伴有区域淋巴结肿大,而上皮样血管瘤则多表现为皮肤单发的小结节或丘疹样病变。组织学上,两者均可位于真皮内或皮下,但Kimura病均以增生的淋巴组织为主,其中7例可见淋巴滤泡形成,内含活跃的生发中心,滤泡问可见大量增生的毛细血管后微静脉型血管,其内皮呈肿胀状或扁平状,血管周围和滤泡旁可见大量的嗜酸性粒细胞浸润,3例内可见嗜酸性微脓疡形成。3例上皮样血管瘤均以成簇增生的血管为主,其中2例为毛细血管型,1例为小至中等大的血管型。血管内衬胞质丰富、深嗜伊红染的上皮样内皮细胞。除内衬管腔外,内皮细胞还可在血管腔内生长或在血管周围呈实性片状增生或排列成条索状,部分细胞的胞质内含有空泡,拟形成原始血管腔。2例的问质内可见多少不等的慢性炎症细胞反应,但不见淋巴滤泡形成,嗜酸性粒细胞浸润也远不如Kimura病明显。免疫组织化学显示,Kimura病中的淋巴滤泡表达B细胞抗原,滤泡间的淋巴细胞多表达T细胞标记,而上皮样血管瘤中的内皮细胞强阳性表达CD31和FⅧRAg等内皮标记。结论Kimura病和上皮样血管瘤是两种完全不同的病种,前者是一种淋巴组织的增生,而后者则是一种良性的血管瘤,掌握两者的临床特点和组织学形态对避免将两者相互混淆具有十分重要的意义。     

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.     

Status of intraoperative cytology in the diagnosis of epithelioid hemangioma

Whereas evaluation of the frozen section of a subcutaneous retro-auricular mass was equivocal, the correct diagnosis of epithelioid hemangioma could be suggested on examination of intraoperative cytological smears. It is proposed that in the absence of cytological cues of malignancy and in the presence of the proper clinical setting, the constellation of vascular structures, eosinophils, lymphocytes, and clusters of cuboidal cells with vacuoles in their abundant acidophilic cytoplasm is indicative of epithelioid hemangioma. Diagn. Cytopathol. 1998;18:227–229. © 1998 Wiley-Liss, Inc.     

Splenic hamartoma and capillary hemangioma are distinct entities: Immunohistochemical analysis of CD8 expression by endothelial cells

The histologic and immunohistologic features of two morphologically similar splenic tumors, a capillary hemangioma and a splenic hamartoma, are reported. The hemangioma was composed predominantly of small vascular channels lined by endothelium expressing factor VIII-related antigen and lacking T-subset antigen (CD8). In contrast, the splenic hamartoma was predominantly a spindle cell lesion with numerous vascular channels coursing through the tumor; these contained splenic-type endothelium expressing both CD8 and factor VIII-related antigen. Our results justify the concept that the splenic hamartoma is a tumor of splenic origin or a true hamartoma and is distinct from the splenic capillary hemangioma.     

Concurrent hepatic adenomatoid tumor and hepatic hemangioma: a case report

A 45-year-old male with alleged asymptomatic hepatic hemangioma of 4 years duration had right upper-quadrant pain and was referred to a tertiary hospital. Computed tomography and magnetic resonance imaging scans revealed a hypervascular mass of about 7 cm containing intratumoral multilobulated cysts. A preoperative liver biopsy was performed, but this failed to provide a definitive diagnosis. The patient underwent a partial hepatectomy of segments IV and VIII. The histologic findings revealed multifocal proliferation of flattened or cuboidal epithelioid cells and a highly vascular edematous stroma. Immunohistochemistry findings demonstrated that the epithelioid tumor cells were positive for cytokeratin (AE1/AE3), vimentin, calretinin, and cytokeratin 5/6, and were focally positive for CD10, and negative for WT1 and CD34, all of which support their mesothelial origin. Immunohistochemistry for a mesothelial marker should be performed for determining the presence of an adenomatoid tumor when benign epithelioid cells are seen.     

骨原发性上皮样血管肉瘤2例报道并文献复习

目的提高对骨原发性上皮样血管肉瘤(epithelioid angiosarcoma,EA)的认识,避免误诊。方法对2例原发于骨的EA进行临床病理、组织学及免疫组化分析,并进行文献复习。结果 2例均为男性,为骨组织内的多灶性、溶骨性病变,组织学上肿瘤主要由实性片状排列的上皮样细胞组成,瘤细胞核大,空泡状,含有明显核仁,可见细胞内空泡和血管腔样结构形成。免疫组化标记显示瘤细胞表达CD31、FⅧRAg、CK、vimentin。分别进行单纯化疗和手术治疗。1例失访,1例2个月后死亡。结论骨原发性EA是一种具有上皮样特征的高度恶性血管源性肿瘤,必须与转移癌等鉴别,CD31、CK等血管标记物对鉴别诊断具有重要的意义。     

Epithelioid hemangioma of bone: a potentially metastasizing tumor?

The case of a 15-year-old boy with a severe swelling of the 2nd toe in the left foot and a large swelling of the left inguinofemoral region is described. Histologic examination of the foot lesion showed a typical epithelioid hemangioma of bone that extended into the soft tissues, with a secondary location in the groin, suggestive of lymph node involvement. Subsequently, imaging also revealed swelling of iliac and para-aortic lymph nodes, which probably indicates further lymph node spread.. Besides the fact that epithelioid hemangioma of the toe has not been reported yet, our case showed a non-continuous localization, most likely in draining lymph nodes. This finding suggests metastatic disease, an event that is extremely rare for epithelioid hemangioma. As such, epithelioid hemangioma might be comparable to another benign vascular tumor, the retiform hemangioendothelioma, which occasionally metastasizes to the lymph nodes without systemic spread.     

Epithelioid angiomatous nodule of the nasal cavity

Epithelioid angiomatous nodule (EAN) is a recently described cutaneous vascular lesion with epithelioid features. We report two additional cases occurring in an unusual location in the nasal cavity. The patients were two young adults (17 and 26 years) presenting with recurrent unilateral epistaxis. Computerized tomography scan and endoscopy revealed small tumors that had developed in the nasal septum and the turbinate, measuring 1 and 1.5 cm, respectively. Endoscopic resection was performed. No recurrence was observed in the follow-up. Pathological examination revealed a well-limited but unencapsulated nodule composed of large epithelioid cells with round nuclei. Some tumor cells contained intracytoplasmic vacuoles. Mitotic activity was low without necrotic areas. Tumor cells were strongly immunostained with antibodies to CD34 and CD31. The main differential diagnosis is an epithelioid hemangioma sharing many morphologic features with EAN. We report these cases to inform of a possibly unusual location of EAN.     

Ovarian teratoma showing a predominant hemangiomatous element with stromal luteinization: report of a case and review of the literature

A solid well-circumscribed ovarian tumor in a 63-year-old Japanese woman is reported. Histologically, the tumor consisted predominantly of a hemangiomatous component with a small cyst lined by a respiratory ciliated epithelium. The remaining ovarian stroma showed a marked proliferation of luteinized cells that were strongly positive for inhibin immunohistochemically. Neither dermoid nor other teratomatous components were found, even in the serial sections of the tumor. To date, vascular tumors of the ovary are uncommon and some cases of ovarian hemangioma have been reported. However, their origin, whether a monodermal teratoma or a true neoplasm arising in the ovarian stroma, has not yet been elucidated. When a hemangiomatous proliferation is found in an ovarian tumor, a thorough microscopic search for teratomatous components is necessary before diagnosing the tumor as a pure hemangioma.     

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.     

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.     

Angiomatöse Skelettläsionen im Kindesalter

Currently, vascular lesions are being classified either as tumors with inherent proliferative potential or as vascular malformations with early manifestation and growth commensurate with the growth of the child. This new classification is also applicable to skeletal vascular lesions. 30% of all skeletal vascular lesions manifest in the first two decades of life, with a preponderance of malformations. They are attributed to disturbances in the complex cascade of angiogenesis and a minority may be hereditary. Multiple cutaneous vascular malformations are associated with mutations of the TIE2- and Glomulin-Genes. Vascular malformations with involvement of multiple skeletal elements may pursue an aggressive course and manifest as massive osteolysis. Among the epithelioid vascular tumours of bone, also in young patients, benign epithelioid hemangioma can be distinguished on a morphological basis from epithelioid hemangioendothelioma as an entity with low malignant potential.