Two cases of epithelioid angiosarcoma involving the thyroid and a brief review of non-Alpine epithelioid angiosarcoma of the thyroid

Epithelioid angiosarcoma involving the thyroid is a rare entity, more often described in the Alpine region. Two cases of epithelioid angiosarcoma that affected the thyroid in patients from a non-Alpine location were diagnosed during a 10-year period in our department. The first case occurred in an 89-year-old Chinese man with a history of longstanding goiter, whereas the second case involved a 74-year-old Chinese man with a history of angiosarcoma of the scalp. On histologic examination, both thyroid tumors were composed of plump epithelioid cells with vesicular chromatin and prominent nucleoli, forming vascular structures and solid sheets. Positive staining for CD31 and factor VIII-related antigen confirmed endothelial differentiation in both cases. Both patients died within 5 months following the diagnosis of thyroid disease. The relationship of the scalp angiosarcoma and thyroid disease of the second patient is unclear. A brief review of non-Alpine primary thyroid epithelioid angiosarcoma is presented.     

A non healing ulcer on scalp

Angiosarcoma is a soft tissue tumour with a dismal prognosis. We present a 74 year old male presenting with a non healing ulcer on the scalp. On histopathology a diagnosis of angiosarcoma was made. An early diagnosis and tumour size play a pivotal role in the survival of the patient.     

A case of retiform-hemangioendothelioma with unusual presentation and aggressive clinical features

Retiform hemangioendothelioma (RH) is an extremely rare low-grade angiosarcoma mainly involving the skin and subcutaneous tissue. Clinically patients often present with an asymptomatic slow-growing solitary nodular or plaque-like lesion. RH is characterized by frequent local recurrences but a very low metastatic rate. Here we reported a case of RH in a 61-year-old Chinese woman who presented with a rapid growing cutaneous plaque-like lesion on her right scalp, followed by another lesion behind the right ear. The lesions were associated with paroxysmal sharp needle-stabbing like headache. She underwent wide excision and skin engraftment. Three months post surgery, she experienced tumor recurrence, and died 9 months after the initial diagnosis.     

Fatal poorly differentiated angiosarcoma of the scalp

Cutaneous angiosarcoma is a very rare but aggressive tumor. Angiosarcoma of the scalp is very rare, and a review of the world literature revealed less than 60 cases. Here, the author reports a case of poorly differentiated angiosarcoma of the scalp. The pathological diagnosis was very difficult. A 70-year-old Japanase man was admitted to our hospital complaining of red mass of the scalp. An excisional biopsy was done. The biopsy showed proliferation of malignant spindle cells in the dermis. Apparent differentiation was not recognized. Invasion into the lateral dermis and subcutis was recognized. There were many mitotic figures and a few foci of necrosis. The size was 2 × 2 × 3 cm. Intracytoplasmic vacuoles were recognized in the malignant tumor cells in some places. A few vague vasoformative features were recognized in one very small area. Immunohistochemically, the malignant spindle cells were positive for factor VIII-related antigen (F-VIII-RA), Ulex lectin, CD31, CD34, vimentin, p53 protein. The Ki-67 labeling was 76%. In contrast, the tumor cells were negative for cytokeratins, epithelial membrane antigen, desmin, S100 protein, α-smooth muscle antigen, bcl-2, melanosome, and myoglobin. The intracytoplasmic vacuoles were strongly positive for F-VIII-RA, Ulex lectin, CD31, and CD34, The abortive vasoformative channels were moderately positive for these endothelial markers. A pathologic diagnosis of angiosarcoma of the scalp was made. Chemoradiation and immunotherapy were performed. However, the tumor recurred several times, and ultimately metastasized to the systemic bones and lungs. The patient died of systemic carcinomatosis 33 months after the first manifestation.     

Fine-needle aspiration diagnosis of angiosarcoma of the spleen: a case report and review of the literature

Primary angiosarcoma of the spleen is a very rare neoplasm with a poor prognosis. The definitive diagnosis is usually based on the histologic evaluation of the splenectomy specimen. We describe a case of angiosarcoma diagnosed by fine-needle aspiration cytology prior to splenectomy. A 69-year-old white woman presented with heterogeneous lesions in the spleen during a follow-up computed tomographic scan for a history of liposarcoma of the right buttock. A malignant endothelial neoplasm was diagnosed by fine-needle aspiration cytology using immunocytochemistry, and a splenectomy confirmed the presence of angiosarcoma. To our knowledge, this is the first well-documented and confirmed case of primary angiosarcoma of the spleen diagnosed by fine-needle aspiration cytology. This report emphasizes the value of fine-needle aspiration cytology as an important diagnostic tool in splenic neoplasms.     

头皮低分化皮肤血管肉瘤的临床病理分析

目的 探讨头皮低分化皮肤血管肉瘤的临床免疫病理特征.方法 分析8例头皮低分化皮肤血管肉瘤患者的临床特点,观察其组织病理形态和CD31、CD34、第八因子相关抗原、波形蛋白、细胞角蛋白(AEl/AE3和CAM5.2)、上皮细胞膜抗原(EMA)、癌胚抗原(CEA)和S-100蛋白免疫组织化学染色.结果 患者平均发病年龄为69.0岁,男:女为5:3,临床早期常在头面部出现暗红斑,后期发生浸润性暗红斑块,均伴结节,偶发溃疡.组织病理检查示真皮广泛梭形细胞局部或广泛实性浸润,常见细胞异形,未见明确血管腔样结构.梭形细胞强阳性表达CD31、第八因子相关抗原和波形蛋白,弱阳性表达CD34、AEl/AE3和CAM5.2,而EMA、CEA和S-100蛋白阴性.结论 头面部出现淤斑样浸润性斑块和结节的老年患者,应及时做组织病理检查,CD31、CD34、第八因子相关抗原和波形蛋白免疫组织化学标记有助于明确诊断.     

Primary diagnosis of angiosarcoma by fine needle aspiration: Lessons learned from 3 cases

Angiosarcomas are rare malignancies arising from cells of endothelial origin and are aggressive sarcomas that can occur in any anatomic site. They are reported to have predilection for the scalp, extremities and breasts. The incidence of these tumors is increasing, which has been suggested to be attributable to the growing use of radiotherapy to treat breast and other malignancies. There is currently limited literature describing the primary cytologic diagnosis of angiosarcoma on fine needle aspirate material. We describe the findings of three cases of angiosarcoma diagnosed by fine needle aspiration. Our three cases offer distinct radiologic, clinical and cytopathologic points‐of‐view: a thyroid angiosarcoma, a mediastinal angiosarcoma and a skin angiosarcoma. The cytomorphology of angiosarcoma is characterized by large highly atypical spindle to epithelioid cells with abundant cytoplasm in dispersed single cells or loose aggregates. The nuclei are large and pleomorphic with vesicular chromatin and prominent nucleoli. Mitoses are readily identified. The background can be bloody and/or necrotic. Occasional intracytoplasmic lumens are a helpful morphologic feature suggesting vascular differentiation. HHV‐8 immunostaining may aid in the differential diagnosis with Kaposi sarcoma while epithelioid hemangioendothelioma can be distinguished based on morphologic features. Given the metastatic potential and high mortality rate associated with these tumors, this entity is an important consideration in the contexts herein described.     

Angiosarcoma of scalp: a case report

Cutaneous angiosarcomas are rare and constitute less than 1% of all malignant mesenchymal tumours. Most angiosarcomas of skin arise in the following clinical settings: 1. face and scalp of elderly 2. following lymphoedema and 3. post radiation. We present a case of an elderly man presenting with scalp lesion of 18 months duration. Histological examination of the biopsy revealed features of an angiosarcoma, which showed imunohistochemical positivity for factor VIII related antigen.     

Angiosarcoma of the scalp: Report of two cases with fatal pulmonary complications and a review of Japanese autopsy registry data

Summary Two cases of angiosarcoma of the scalp were reported. The patients were elderly men and died from pulmonary complications, including pneumothorax, pulmonary haemorrhage and pneumonia, associated with metastatic tumours in the lungs. The data recorded from 95 autopsies of patients with angiosarcoma in Japan during 1980–1984 were analyzed. According to the anatomical distribution of the primary tumour, the patients could be subdivided into a scalp group and non-scalp group. In both groups, the most common metastatic site was the lung. The patients of the scalp group had more frequent pulmonary complications such as pneumonia, haemothorax, atelectasis and pneumothorax, when compared with the patients of the non-scalp group. In particular, pneumothorax was observed only in the patients of angiosarcoma of the scalp. The results indicate that angiosarcoma of the scalp tends to metastasize to the lung, especially to the subpleural or surface pleural area, and these metastatic tumours are prone to necrosis, causing characteristic pulmonary complications.Supported in part by a grant-in-aid for Cancer Research from the Ministry of Education, Science and Culture of Japan     

Pitfalls in the cytologic diagnosis of angiosarcoma of the breast by fine-needle aspiration: A case report

This paper describes a case of angiosarcoma of the breast in a 26-yr-old female. The tumor, originally thought to be granulation tissue on fine-needle aspiration biopsy, was correctly identified as a malignant neoplasm of probable mesenchymal origin on a repeat FNA biopsy 4 mo later. A diagnosis of angiosarcoma was made on a subsequent excisional biopsy. Review of the cytologic features revealed findings that should suggest angiosarcoma, especially when correlated with the clinical history. The authors describe the cytological features seen in this case, and discuss an important pitfall in the diagnosis of angiosarcoma of the breast on FNA. Diagn Cytopathol 1994; 11:297–300. © 1994 Wiley-Liss, Inc.     

Aicardi syndrome,metastatic angiosarcoma of the leg,and scalp lipoma

An infant girl with Aicardi syndrome, scalp lipomas, and angiosarcoma of a limb is reported. The cavernous hemangioma of the leg was benign when biopsied at age 5 months but became malignant at 11 months. Angiosarcoma caused multiple distant metastases which were evident at autopsy at age 19 months. This is the first case of Aicardi syndrome associated with lipoma and metastatic angiosarcoma. © 1993 Wiley-Liss, Inc.     

Epithelioid angiosarcoma at chest wall which needs to be carefully distinguished from malignant mesothelioma: report of a rare case

Angiosarcoma is a malignant soft tissue tumor the cells of which variably recapitulate the morphologic and functional features of normal endothelium. Most lesions are located in the deep muscles of the lower extremities followed by the arm, trunk and head and neck. Herein we present a case of epithelioid angiosarcoma which is a variant of angiosarcoma at chest wall in a 73-year-old female. Morphologically, the tumor cells are arranged predominantly in luminal structures which can be seen in both angiosarcoma and malignant mesothelioma. Most of the tumor cells are large rounded “epithelioid” cells with abundant eosinophilic cytoplasm which can be also seen in both tumors. The epithelioid of cytomorphology and the localization at chest wall of this case may remind of a diagnosis of malignant mesothelioma which should be carefully distinguished from epithelioid angiosarcoma from imaging and morphology. CT scanning of the patient shows a mass at her chest wall, the majority of which is around the rib but not inside the lung which indicates a tumor originates more likely from soft tissues of chest wall but not pleura. Immunohistochemical staining shows that the tumor cells are positive for cytokeratin, CD31, Vimentin and WT1, and negative for CEA, TTF-1, Calretinin, Mesothelial Cell (MC), CD56, CK19, and Hepatocyte. Thus this case is diagnosed as epithelioid angiosarcoma but not malignant mesothelioma. From this case we suggest that carefully reading and understanding of the imaging are a very important clue for appropriate diagnosis. A misdiagnosis may occur on the basis of misunderstanding of tumor localization and a consequent inappropriate immunohistochemical staining programme.     

Fine-needle aspiration of scalp lesions

A variety of inflammatory and neoplastic scalp lesions are encountered in surgical pathology. However, the literature on fine-needle aspirations (FNAs) of the scalp is exceedingly rare. We report on a series of 70 FNAs involving cutaneous and subcutaneous lesions on the scalp. A total of 70 fine-needle aspirations of cutaneous and subcutaneous scalp lesions was reviewed to identify patterns of metastasis to the scalp and to demonstrate the effectiveness of FNA in diagnosing these lesions. There were 42 male and 28 female patients, ranging in age from 29-91 yr (mean, approximately 61 yr). Sixty-one patients had a previous history of malignancy. Of these aspirates, 59 were neoplastic, consistent with the patient's known primary. One case was an abscess, and the remaining case was unsatisfactorvy for cytologic evaluation. Follow-up biopsy revealed granulomatous inflammation. The most common primary tumor to metastasize to the scalp was lung carcinoma, which was seen in 18 cases, followed by hematopoietic malignancies in 14 cases. Melanoma was identified in 6 cases, head and neck tumors in 5 cases, renal malignancies in 4 cases, gastrointestinal tumors in 3 cases, sarcoma in 3 cases, breast and prostate malignancy in 2 cases each, and an olfactory neuroblastoma and meningioma in 1 case each. The remaining 9 aspirates were from patients who did not have a previous history of malignancy. These included 6 benign aspirates consisting of 3 epidermal inclusion cysts, 2 lipomas, and 1 neurofibroma. Two aspirates were malignant and included 1 primary squamous-cell carcinoma and 1 metastatic adenocarcinoma of unknown origin. The remaining case was unsatisfactory for cytologic evaluation. Follow-up biopsy of this lesion showed noncaseating granulomas. Of the aspirates from patients with a previous history of malignancy, 97% were neoplastic. Lung carcinoma and hematopoietic malignancies were the most common neoplasms that metastasized to the scalp. Since the scalp is a common site for metastasis, awareness of this fact is useful to both oncologists and dermatologists. It must be understood that FNA can provide a rapid and accurate diagnosis in the evaluation of scalp masses.     

Cutaneous angiosarcoma arising after radiotherapy treatment of a breast carcinoma. Description of a case and review of the literature

Cutaneous angiosarcomas occurring as a complication of radiation therapy for breast cancer are rare. To the best of our knowledge 37 cases have been reported in English literature during the last two decades. We describe a case of angiosarcoma arising in the skin of a breast previously irradiated for breast carcinoma. The patient, a 74-year-old woman, had been treated with conservative surgery and radiotherapy for a pT1N0 infiltrating ductal carcinoma of the left breast in 1993. Nine years later a skin lesion, which was interpreted as a recurrence, arose in the same site. A biopsy obtained from the lesion, and initially considered to be negative for cancer, showed istological features consistent with those of the atypical vascular lesion. Seven months later the same lesion transformed in a 5 x 3 reddish mass which at histological examination was diagnosed as an high grad post-irradiation angiosarcoma of the skin of the breast. Data on previously reported post iradiation angiosarcoma of the breast are reviewed; further consideration on differential diagnosis between those lesions and atypical vascular lesions are presented.     

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。     

Papillary endothelial hyperplasia of adnexal vasculature

Papillary endothelial hyperplasia is an exuberant predominantly intravascular endothelial proliferation which may simulate angiosarcoma both clinically and histopathologically. This report describes what, to the best of our knowledge, is a unique case of papillary endothelial hyperplasia within para-ovarian and paratubal veins in relation to a multicystic mesothelial and haemorrhagic ovarian mass. Papillary endothelial hyperplasia should be included in the differential diagnosis of vascular lesions in this site.     

Multicentric contiguous variant of epithelioid angiosarcoma of the bone. A rare variant showing angiotropic spread

Epithelioid angiosarcoma of the bone is a rare tumor and is a diagnostic challenge. Here we present an autopsy case of a 62-year-old man with multifocal osteolytic lesions in the extremities and the pelvis. The initial diagnosis of a tibial biopsy was poorly differentiated adenocarcinoma. On the occasion of autopsy, a fungating thrombotic nodule was found at the anterior wall of the right atrium, and small hemorrhagic infarcts with tumor thrombi were found in the lung. Histologically, the above lesions were identical to the former tibial biopsy and they showed large eosinophilic epithelioid cells with irregular ovoid nuclei and prominent eosinophilic nucleoli. Rare intracytoplasmic lumina were identified. Immunohistochemically, the tumor cells were positive for cytokeratins (CAM5.2 and AE1/AE3), CD31, factor VIII-related antigen, and vimentin. This case showed angiotropic spread of the tumor only to the right atrium and the lung, with no solid mass in other organs. Multicentric epithelioid angiosarcoma of the bone is a pitfall in pathological diagnoses, especially if a strong radiological impression of metastatic carcinoma is provided. Therefore, pathologists should be aware of this rare variant.     

Epithelioid angiosarcoma: A neoplasm with potential diagnostic challenges

Epithelioid angiosarcomas are extremely rare tumors associated with poor prognosis and early metastases. Its epithelioid cytomorphology and limited vasoformation make it difficult to distinguish from more common malignancies, such as, carcinoma. This can be a potential diagnostic pitfall for the cytopathologist. In this report, the patient is a 24‐year‐old man presenting with testicular pain, a pelvic mass, and innumerable liver nodules. Immediate interpretation of the needle core biopsies of the pelvic mass and liver lesions initially favored a poorly differentiated adenocarcinoma. Unusual positive immunohistochemical stains for CD30 and CK7 ultimately led the investigation toward a tumor of mesenchymal origin. Further, immunohistochemical evaluation demonstrated positive CD31 and Factor VIII staining and established the final diagnosis of epithelioid angiosarcoma. The tumor cells were negative for CD34, CK20, alpha‐fetoprotein, placental‐like alkaline phosphatase, hepatocyte paraffin 1, polyclonal carcinoembryonic antigen, CD10, CA‐125, prostate‐specific antigen, and prostatic acid phosphatase. This case is reported to illustrate the importance of considering the diagnosis of epithelioid angiosarcoma when encountering an “epithelioid” neoplasm particularly with unusual immunoreactivity for CK7 and CD30. Diagn. Cytopathol. 2010. © 2009 Wiley‐Liss, Inc.     

Angiosarcomas of the breast developing post radiotherapy

Aims : This paper describes two contrasting cases of post-radiotherapy angiosarcomas of the breast. They illustrate the spectrum of malignant vascular tumours that may occur in the breast. These tumours are extremely rare and knowledge of their natural history is limited. The prognosis suggested by the few published cases of poorly or moderately differentiated angiosarcomas suggest a poor prognosis often with a fatal outcome. Methods and results : This was confirmed by our first case a poorly differentiated angiosarcoma, occurring 10 years after radiotherapy for breast carcinoma with death occurring from metastatic angiosarcoma 6 months later. Our second, more recently diagnosed case, was a well-differentiated angiosarcoma, occurring 5 years post radiotherapy for breast cancer. She remains well 12 months after the diagnosis of sarcoma but her long-term outlook is more problematic to predict. Only five previous cases of well-differentiated post-radiotherapy angiosarcoma of the breast have been reported with limited follow-up (average 2 years). Conclusions : Communication with the authors of four of the papers since their publication has provided valuable additional information and indicates a potentially good outlook for this woman in the longer term. A second cellular component was also found in these malignant vascular tumours. These spindle cells distinct from the malignant endothelial cells have not to our knowledge been described previously and their possible nature is discussed.     

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.