A Mimic of Sarcomatoid Adrenal Cortical Carcinoma: Epithelioid Angiosarcoma Occurring in Adrenal Cortical Adenoma

The adrenal gland is a site of various neoplasms; however, it is rarely involved by sarcomas. We present herein an unusual adrenal neoplasm consisting of epithelioid angiosarcoma and adrenal cortical adenoma. In this report, the authors highlight the diagnostic challenges associated with an epithelioid angiosarcoma occurring in an adrenal cortical neoplasm by providing a comprehensive discussion on the spectrum of vascular proliferations seen in the adrenal gland along with a roadmap for practicing pathologists. The presence of angiosarcoma within an adrenal cortical adenoma may represent a collision tumor; however, one can speculate that the rich vasculature of endocrine lesions can also create a favorable milieu for the occurrence of this phenomenon. While the latter needs to be further clarified, the presented case should be added to the unusual clinical presentations of vascular lesions of the adrenal gland mimicking a sarcomatoid adrenal cortical carcinoma.     

Epithelioid angiosarcoma of the adrenal gland associated with chronic arsenical intoxication?

Epithelioid angiosarcoma is a rare tumor quite recently described. There is no accurate epidemiological study of this tumor. Among the internal organs, the liver is the one most frequently affected with angiosarcoma while there is no reference to the adrenal gland as a primary site. It is well known that the direct exposure to arsenicals (especially of vineyard cultivators) may be an important causative factor in the pathogenesis of the disease. A 59-year-old male vineyard cultivator with an epithelioid angiosarcoma of the right adrenal gland is described. The histologic characteristics as well as the immunohistochemical profile of the tumor are presented and the literature is briefly reviewed.     

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-.     

Primary adrenal angiosarcoma

We report the case of a 69-year-old man who presented with an adrenal angiosarcoma which had been discovered during prostatic adenocarcinoma staging. Twenty-two cases have been reported in the literature. All of them were of epithelioid type. Because of the epithelioid appearance and frequent expression of epithelial immunohistochemical markers, they can be confounded with adrenal carcinomatous metastases. Careful screening for vascular differentiation and immunohistochemistry with endothelial markers are essential for the diagnosis.     

Primary epithelioid angiosarcoma of the pleura: a case report and review of literature

Primary angiosarcoma of the pleura is an extremely rare malignancy. Herein, we report the case of an elderly Chinese patient with primary left pleural epithelioid angiosarcoma. The 76-year-old man presented with a 4-month history of a cough with sputum expectoration and weight loss of 4 kg within one month. A chest scan showed a massive oval-shaped mass in the left pleural cavity. We then performed a left thoracotomy for tumor resection and surgical exploration. Histological examination of the resected specimen showed few viable tumor cells with significant atypia; tumor cells had large nuclei and prominent nucleoli and were arranged in a crack-like, sheeted pattern. Moreover, there was a significant amount of fibrinous exudates, hemorrhage, degeneration, and necrosis. With immunohistochemical analysis, tumor cells had strong expression of CD31, CD34, FLI-1, vimentin. Morphological and immunohistochemical findings supported the diagnosis of epithelioid angiosarcoma.     

Primary adrenal gland epithelioid sarcoma: A case report and literature review

Epithelioid sarcoma is a malignant mesenchymal neoplasm with morphologic and immunophenotypic epithelioid differentiation, which rarely arises in solid organs. We report a case of primary epithelioid sarcoma in the adrenal gland of a 31‐year‐old female. The patient initially presented with nausea and rectal bleeding, and subsequent imaging studies revealed a 4.4 cm left adrenal gland mass and left retroperitoneal lymphadenopathy. Clinical and radiological studies did not reveal tumor elsewhere in the patient. Histologic features were those of epithelioid sarcoma, proximal type with cohesive clusters of epithelioid tumor cells harboring frequent mitoses, and areas of necrosis. Immunohistochemical stains showed strong, diffuse expression of epithelial markers (pancytokeratin), and CD34 and Fli‐1. Partial and focal positive staining of CK7 was also noted. Nuclear expression of SMARCB1 (INI‐1) protein was lost. ERG was negative in this case. We believe that this is the second‐case report of a primary adrenal gland epithelioid sarcoma. Fli‐1 positivity was seen in our case, and ERG was negative as shown in some recent publications regarding epithelioid sarcomas.     

Angiosarcoma of the adrenal gland

We report the first case of primary angiosarcoma of the adrenal gland, to our knowledge. A 54-year-old man presented with chronic left upper quadrant abdominal pain, and a left adrenal mass was subsequently found on computed tomography of the abdomen. The tumor was surgically removed and a diagnosis of adrenal angiosarcoma, supported by findings of immunoperoxidase and ultrastructural studies, was made. Seven months later, recurrent tumor resulted in an en bloc resection of lateral gastric wall, tail of pancreas, left kidney, and spleen. One year after the initial surgery, the patient was free of tumor. Angiosarcoma should be added to the list of possible primary adrenal gland malignancies.     

Malignant PEComa of the adrenal gland

Perivascular epithelioid cell neoplasms, also known as PEComas, are unique mesenchymal tumors exhibiting perivascular epithelioid cell differentiation, characterized by a mixed myogenic and melanocytic phenotype. PEComas arising in visceral organs outside of the kidney, liver, and lung are rare, and often pose problems in diagnosis. Examples of this neoplasm originating in the adrenal gland are limited. The present report details the clinical and pathologic features of an unusual case of a pure epithelioid PEComa (epithelioid angiomyolipoma) of the adrenal gland exhibiting clinically malignant behavior in the form of pulmonary metastases, a feature not previously described in tumors of this site. The diagnosis was supported by immunohistochemical studies demonstrating expression of myoid and melanocytic antigens. The present case serves to emphasize the potential of PEComa for clinically aggressive behavior and the importance of distinguishing this tumor from other epithelioid neoplasms that are more commonly encountered in the adrenal gland.     

Immunohistochemical characterization of thyroid gland angiomatoid tumors

The histogenesis of thyroid gland angiomatoid tumors, probably as a primary angiosarcoma, has been a controversy for many years. These tumors may be variants of undifferentiated (anaplastic) carcinomas. We report a thyroid gland angiomatoid carcinoma in a 61-year-old African American male. The tumor had a heterogeneous pattern with both sarcomatous and epithelioid areas. Tumor cells lined some vascular-like spaces and others had intracytoplasmic lumens containing red blood cells. The tumor cells were found to express multiple endothelial (factor VIII-related antigen, CD31, CD34, and Ulex europaeus I lectin) and epithelial (cytokeratin and epithelial membrane antigen) markers as well as thyroglobulin by immunohistochemistry. This rare presentation demonstrates the heterogeneous nature of thyroid gland angiomatoid carcinoma with both epithelial and endothelial differentiation.     

Epithelioid angiosarcoma of the pleura: a case report.

Angiosarcomas of the pleura are very rare tumors and it is difficult to differentiate them from other common pleural tumors such as mesothelioma and metastasic carcinoma clinically and pathologically. We report a case of a young Korean woman with angiosarcoma arising in the pleura. A 34-yr-old woman presented with dyspnea and chest tightness and pain for several months. A computed tomographic scan of the chest showed diffuse thickening of the left pleura and effusion with passive atelectasis. At thoracotomy the left pleura was thick and indurated. Histologically, the decorticated pleura revealed infiltration of sheets or cords of polygonal and epithelioid tumor cells showing rudimentary vascular differentiation. Immunohistochemically, the tumor cells were strongly positive for CD31, CD34, and vimentin, whereas weakly positive for factor VIII, and negative for cytokeratin, which are characteristic and specific findings of angiosarcoma.     

Postradiation angiosarcoma of the small intestine: a case report and review of literature

Postradiation angiosarcoma is typically a high-grade sarcoma that presents mainly in the skin and superficial tissues. Postradiation angiosarcoma arising in the small intestine is rare with only 11 cases documented in the English-language literature. Herein, we report a postradiation angiosarcoma of the small intestine 9 years after radiotherapy for uterine cervical adenocarcinoma. The patient presented with symptoms of intestinal obstruction. At exploratory laparotomy, tumor nodules involved the small bowel. Microscopically, the neoplasm was composed of spindled and epithelioid cells arranged in solid aggregates and focally forming vascular channels. The diagnosis of angiosarcoma was confirmed immunohistochemically by tumor cell expression of CD31, CD34, and factor VIII-related antigen. The patient died 10 months after laparotomy. The diagnosis of PRA should be entertained for any poorly differentiated neoplasm arising in a previously irradiated site. The correct diagnosis of PRA depends upon histomorphologic identification of vascular differentiation, coupled with immunohistochemical expression of endothelial-related markers.     

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     

Oral and salivary gland angiosarcoma: a clinicopathologic study of 29 cases.

Angiosarcomas of the oral and salivary gland area are extremely rare, mostly presented as case reports. We wanted to study the clinicopathologic features of a series of oral and salivary gland angiosarcomas. Cases coded as "angiosarcoma" were retrieved from the Oral and Maxillofacial Pathology Department of the Armed Forces Institute of Pathology. Patient folders and pathology were reviewed and recorded; immunohistochemistry and follow-up were obtained. Inclusion required oral or salivary gland location, vasoformative growth, cytologic atypia, mitoses, and vascular markers. Skin, bone, and subcutaneous angiosarcomas were excluded. Primary and secondary (metastatic) oral angiosarcomas were included. The 22 primary angiosarcomas involved tongue (n = 9), parotid (n = 4), lip (n = 4), submandibular gland (n = 3), and 1 each of soft and hard palate. The 7 secondary angiosarcomas involved the gingiva (n = 4) and parotid gland (n = 3). Overall, patient ages ranged from 6-90 years (mean, 55 years). There were 15 males and 14 females. Symptoms included a mass with recent enlargement and bleeding. Tumor sizes ranged from 0.8-7.0 cm (mean, 2.6 cm). Histologically, all tumors were vasoformative; 86% had solid and 17% had distinctive papillary areas. Eight (28%) were classified as the epithelioid subtype. Immunohistochemical stains showed that the tumor cells were positive for Factor VIIIrag in 19/21, CD31 in 16/19, CD34 in 7/12, and Ulex in 1/1. Primary tumors were classified as low grade (n = 7, in all locations except salivary gland), intermediate (n = 7), and high grade (n = 8); all secondary tumors were high grade. Follow-up was available on 14/22 primary and 7/7 secondary angiosarcomas. Of primary tumors, two tongue angiosarcoma patients died at 1 and 9 years, but 4 were alive without disease over a mean of 7.3 years (range, 1-13 years). Four primary salivary gland angiosarcoma patients were alive without disease over a mean of 5.8 years (range, 1-14 years), and 1 had only a late (15 years) metastasis and death (at 20 years). Three primary lip angiosarcoma patients were without disease over a mean of 14.3 years (range, 13-16 years). Of secondary tumors, three salivary gland angiosarcoma patients died within 1 year, and all four secondary gingival angiosarcoma patients died of disease within 3 years. Assessing follow-up of primary oral and salivary gland angiosarcoma patients by grade, 5 patients with high-grade tumors had no evidence of disease over a mean of 7.6 years (range, 1-16 years), 3 patients with intermediate-grade tumors had no evidence of disease over a mean of 12.7 years (range, 11-14 years), 2 patients with intermediate-grade tumors died of disease at 9 and 20 years, 3 patients with low-grade tumors had no evidence of disease over a mean of 6.3 years (range, 1-14 years), and 1 patient with low-grade tumor died of disease at 1 year. Primary oral and salivary gland angiosarcomas, albeit rare, mostly involve the tongue, parotid gland, and lip of adults, often with relatively good outcome. Although the most common angiosarcoma morphology in this area is spindled vasoformative and solid, almost one third of oral and salivary gland angiosarcomas are the rare epithelioid angiosarcoma variant. Most gingival and few parotid angiosarcomas appear to be metastases from other locations, with many patients succumbing to death within 3 years. Despite predominantly high- or intermediate-grade morphology, patients with primary angiosarcoma of the tongue, salivary gland, and lip have a better prognosis than do patients with primary cutaneous or deep soft tissue angiosarcoma, including those patients with secondary oral and salivary gland involvement.     

Bone marrow metastasis of angiosarcoma

We report a rare finding of bone marrow metastasis from an angiosarcoma. The patient was a 36-year-old man who initially presented with a high-grade angiosarcoma of the spleen and was treated with splenectomy and chemotherapy. He developed leukoerythroblastic anemia three years after splenectomy. Bone marrow biopsy revealed extensive infiltrate by angiosarcoma with typical features of spindle tumor cells and anastomosing vascular channels. The immunohistochemistry showed tumor cells positive for the endothelial markers of CD31, CD34, and von Willebrand factor. Angiosarcomas are rare and aggressive tumors. Although metastases occur commonly, bone marrow findings have been rarely documented. We have found in the literature two other cases of bone marrow metastasis of angiosarcoma, and all these patients had a primary tumor of the spleen. It would seem that splenic angiosarcomas have a virtually unique propensity for infiltration in the bone marrow.     

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.     

Primary pulmonary epithelioid angiosarcoma presenting as a solitary pulmonary nodule on image

Primary angiosarcoma of lung is a rare condition. Only about 20 cases have appeared in English published reports so far. Its rarity and consequent low index of suspicion makes clinical diagnosis difficult. Pathological diagnosis of the epithelioid variant of pulmonary angiosarcoma is particularly challenging. We report a case of primary pulmonary epithelioid angiosarcoma as a solitary pulmonary nodule in image study in a 41-year-old man with a brief review, to contribute it to the sparse literature on this disease.     

A case of pulmonary malignant epithelioid hemangioendothelioma misdiagnosed as adenocarcinoma by fine needle aspiration cytology

Malignant epithelioid hemangioendothelioma (MEHE) is a rare vascular tumor with a biological behavior that lies between those of classical epithelioid hemangioendothelioma and angiosarcoma. Furthermore, MEHE is rarely diagnosed by fine needle aspiration cytology. The authors describe the cytological features of MEHE in a 41-year-old man who presented with increasing dyspnea over a period of 1 month before admission. Computed tomography of the chest showed a 3 cm poorly defined mass in the right lower lobe. Fine needle aspiration cytology demonstrated cellular smears of loosely cohesive clusters of epithelioid cells with numerous intracytoplasmic lumens in a necrotic background. Cellular features included fine chromatin and vesicular or slightly hyperchromatic nuclei with inconspicuous nucleoli and intranuclear inclusions. Nuclear membranes were relatively irregular with indentation. Mean N/C ratio was not increased, presumably due to a moderate amount of cytoplasm. The histologic examination displayed epithelioid and spindle cell proliferation with necrosis accompanying a classical epithelioid hemangioendotheliomatous area. The immunohistochemical evaluation was confirmatory and showed immunoreactivity for vascular markers. The authors also reviewed FNAB findings of epithelioid angiosarcoma, primary adenocarcinoma, and bronchioloalveolar carcinoma of the lung to identify cytomorphologic differences by literature bases. MEHE of the lung is difficult to diagnose cytologically because of its rarity and its cytomorphologic similarities with other malignant epithelial and mesenchymal tumors. However, it may be possible to distinguish it from other entities when the possibility of this unusual vascular neoplasm is suspected and ancillary studies are supportive.     

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。