Epithelioid angiosarcoma arising in schwannoma: report of three Chinese cases with review of the literature

Angiosarcoma arising in a schwannoma is extremely rare with only eleven cases having been reported in the English literature. We describe here three further cases occurring in adult males with a pre-existing longstanding schwannoma. The tumor arose each from the vagus, ischiadic and adrenergic nerve respectively. None of the patients had von Recklinghausen's disease. Microscopically, the tumor was composed of a mixture of a benign schwannoma and an epithelioid angiosarcoma. The two components changed abruptly within the tumor. The endothelial cell differentiation was confirmed by immunohistochemistry. A review of published reports, including the present cases, suggests a poor prognosis with a high rate of local recurrence, distant metastasis and mortality.     

Epithelioid angiosarcoma of the ilium: a case report

Bone epithelioid angiosarcoma (EA) is rare and characterized by large, mildly to moderately pleomorphic epithelioid cells, with abundant eosinophilic cytoplasm, vesicular nuclei, and prominent nucleoli. The tumors may arise in various locations in bone and the patients may present with unifocal or multifocal osseous disease. We present a unifocal lesion case of EA of the ilium in a 62-year-old woman. A needle biopsy of the ilium was performed and first diagnosed poorly differentiated adenocarcinoma based on CKpan and CK18 immunopositivity. The tumor was treated initially with curettage followed by chemotherapy. The final diagnosis on the surgical specimen was epithelioid angiosarcoma.     

Epithelioid angiosarcoma of the intestinal tract with endothelin-1-like immunoreactivity

Two rare cases of intestinal epithelioid angiosarcoma arising in the sigmoid colon and small intestine are reported. The small intestinal tumours were located in the duodenum and upper jejunum. All tumours showed reddish-black protuberant masses with comparatively clear margins. Histology reveals solid and epithelioid growth of large polygonal cells, mimicking undifferentiated carcinoma. Vascular differentiation such as lumina containing red cells and intracytoplasmic vacuolization is noticed. Intense immunoreactivity toUlex europaeus agglutinin I and JC70 (CD31), and sporadic positive reaction to factor VIII related antigen were detected in one case but not in the other. However, both tumours have cells which show intense endothelin-1 immunoreactivity. Positive immunostaining occurred with low molecular weight keratins suggesting epithelial differentiation and suggested epithelioid angiosarcoma as a diagnosis, a specific entity within angiosarcoma. Because of their histological features, epithelioid angiosarcomas may be confused with undifferentiated carcinomas. Endothelin-1-like immunoreactivity seems to be a good marker for this type of angiosarcoma, even when the tumour cells lack usual marker substances for endothelial cells.     

Epithelioid angiosarcoma: a clinicopathological study of 16 Chinese cases

Aims: To review retrospectively 16 cases of epithelioid angiosarcomas (EAs) with emphasis on their clinical and pathological characteristics, treatment and possible prognostic factors. Methods and results: All eligible cases were searched and acquired from archives of the pathology departments of two hospitals in Shanghai, The Fifth People’s Hospital of Shanghai, Fudan University, and the Shanghai Cancer Center, Fudan University, China. The patients ranged in age from 19 to 77 years, and 5 patients were below 50 years of age. Microscopically, the tumors were mostly composed of large, round or polygonal epithelioid cells that were predominantly arranged in solid sheets or nests. The tumor cells had basophilic or eosinophilic cytoplasm, vesicular nuclei, and prominent nucleoli. Mitotic figures including abnormal mitoses were frequently encountered. In all 16 cases in our series, immunohistochemical studies showed positivity for CD31, and partial positivity for Fli-1, CD34 and factor VIII-related antigen. Of the 14 patients available for follow-up, 3 patients were alive with disease, 9 patients died as a result of the tumor, 1 died of local hemorrhage, and one died of unknown etiology. The median survival was 17.1 months. Conclusions: EA is highly aggressive and carries a very poor prognosis. Therefore, the clinical recognition and correct diagnosis of EA are essential.     

Primary ovarian angiosarcoma: A case report and literature review

Primary ovarian angiosarcoma is extremely rare. Only 16 cases have histologicaliy been reported to date In the Ilterature. A case of angiosarcoma arising In the right ovary of a 46-year-old female is presented. Grossly, the resected right ovary was completely replaced by a solid tumor mass, which revealed multiple necrotic and/or hemorrhagic foci. This case revealed the typical histological features of angiosarcoma with sinusoldal and solid patterns of anaplastic tumor cells. Immunohlstochemically, tumor cells were strongly and diffusely positive for CD31 and CD34, in particular, along the cytoplasmic membrane of the tumor cells. Ultrastructurally, tumor cells possessed the intermediate junctions between tumor cells, discontinuous basal laminae attached to the irregularly shaped blood vessels and occasional cytoplasmic pinocytotlc vesicles. These findings confirmed the case as being one of angiosarcoma of the ovary. The patient died 9 months after surgery as a result of developed multlfocal brain metastases. A total of 17 cases reported as primary ovarian anglosarcoma, including this presented case, are clinicopathologically reviewed.     

甲状腺上皮样血管肉瘤2例报道及文献复习

目的：探讨甲状腺肿瘤上皮样血管肉瘤的临床病理学特征。方法：对2例原发于甲状腺的上皮样血管肉瘤进行光镜观察和免疫组化标记。结果：2例均为老年患者,以颈部包块就诊,其中1例伴有地方性甲状腺肿。镜下显示,肿瘤主要由实性片状或巢状排列的上皮样细胞组成,瘤细胞核大,空泡状,含有明显的核仁,部分瘤细胞的胞质内可见含有红细胞的空泡或小管腔形成。除实性区域外,可见内衬上皮样瘤细胞的不规则性血管腔,网状纤维染色能清晰显示这些管腔结构,特别是在HE染色达CK8和Tg。结论：原发于甲状腺的上皮样血管肉瘤具有一定的临床病理学特点,光镜形态、免疫组化标记及电镜观察均支持瘤细胞具内皮细胞分化,应被视为甲状腺肿瘤中一种独立的病理学类型,而不应笼统地归入到未分化癌中。     

心包内及后纵隔恶性神经鞘瘤1例报告并文献复习

目的:提高对心包内恶性神经鞘瘤的临床认识.方法:对我院治疗的1例心包内及后纵隔恶性神经鞘瘤合并心包填塞患的临床病理资料进行报告,并分析国内外文献报道的6例心包内恶性神经鞘瘤的临床资料.结果:心包内恶性神经鞘瘤以心包填塞为主要症状,心脏彩超及胸部CT检查是临床上诊断心包内肿瘤的主要手段,确诊依靠组织病理学及免疫组化检查,手术切除肿瘤能缓解心包填塞症状.结论:心包内恶性神经鞘瘤临床罕见,预后较差,手术切除肿瘤是其有效的治疗方法之一.     

Postirradiation epithelioid angiosarcoma of the breast: a case report with immunohistochemical and electron microscopic study

A case of postirradiation epithelioid angiosarcoma of the breast in a 72-year-old woman is reported. She had had right breast conserving surgery, axillary lymph node dissection, and 50 Gy external beam radiation therapy for infiltrating ductal carcinoma. A skin lesion on the irradiated breast appeared 5 years after completion of radiation. Angiosarcoma was diagnosed in a contralateral axillary mass 8 months later. Light microscopically, the tumor was characterized by a sheet-like growth of epithelioid cells with focal vasoformative areas. Tumor cells were reactive for factor VIII-related antigen, cytokeratin and CD34. Electron microscopically, the tumor cells were round with smooth cell borders. They were closely apposed, occasionally forming a small lumen containing single red blood cells or aggregates of platelets. Groups of tumor cells were enclosed by an external lamina. The tumor cells had abundant cytoplasm with sparse organelles. Rare suggestive Weibel-Palade bodies were present. The immunohistochemical and ultrastructural findings in this postirradiation tumor were in agreement with previously reported findings in non-irradiation-induced epithelioid angiosarcomas.     

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.     

Epithelioid angiomyolipoma of the liver: case report and review of the literature

Hepatic angiomyolipoma is a rare tumor. Here we describe a case and review the literature.     

A giant pelvic malignant schwannoma: a case report and literature review

Schwannomas are usually benign tumors that arise from well-differentiated Schwann cells. They rarely occur in the retroperitoneum. Here, we present a case of a 60-year-old man with a giant retroperitoneal pelvic mass. Imageological diagnosis suggested a large heterogeneous mass of 16 cm in diameter located in the abdominopelvic retroperitoneum. Complete intralesional enucleation was achieved without any adjacent organs injury except a severe bleeding which was ceased as we applied the bilateral inferior vesical artery embolization. Final histopathological result showed the tumor was a low malignant Schwannoma. The patient’s symptoms were greatly improved after operation. Unfortunately, a local recurrence was detected at the six-month follow-up appointment with consequent losing to follow up.     

Primary splenic angiosarcoma with fever and anemia: a case report and literature review

Primary splenic angiosarcoma is an extremely rare and aggressive neoplasm. The prognosis of this disease is dismal, and the mean survival is less than 6 months after the diagnosis. This neoplasm typically presents with abdominal pain, splenomegaly, weight loss, and spontaneous splenic rupture. Fever is a very rare presentation of splenic angiosarcoma. Here we report the case of a 64-year-old man who presented with fever and anemia. A laparoscopic splenectomy was performed and revealed splenic angiosarcoma. The postoperative course was uneventful and the patient received 5 cycles of adjuvant chemotherapy with ifosfamide plus epirubicin. He remained disease free at 9 months after surgery. This is the first case of splenic angiosarcoma with fever as the initial presentation that was treated with laparoscopic splenectomy to be reported in the English literature.     

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。     

Primary renal angiosarcoma: A case report and review of the literature

Primary renal angiosarcoma is very rare. To our knowledge, only 15 cases have been reported to date. A 77-year-old Japanese man with a unilateral kidney presented with massive hematuria followed by renal failure. A renal tumor was suspected and a left nephrectomy was performed. The histopathological diagnosis was angiosarcoma of the kidney. A hemorrhagic tumor measuring 10 × 5 cm and clotted blood was found in the modularly area. The atypical tumor cells had a sinusoidal and solid appearance, and showed Immunohistochemically positive reactions for some of the endothelial markers. The patient died about 21 months after the nephrectomy and the autopsy revealed massive metastases to the liver and retroperitoneum. One of the differential diagnoses of the case was anglomyolipoma, because the tumor cells were relatively bland in their histological appearance with entrapped fat cells in the pelvic area. Fifteen case reports with titles that included the term 'hemangiosarcoma/anglosarcoma', 'hemangioendothelloma/endothelloma' or 'vascular sarcoma' of the kidney were reviewed and compared to the present case.     

原发性卵巢血管肉瘤伴浆液性囊腺瘤1例及文献复习

目的探讨原发性卵巢血管肉瘤伴浆液性囊腺瘤的临床病理特征及鉴别诊断。方法对病例进行病理组织学和免疫组织化学观察,复习文献。结果该例卵巢血管肉瘤伴浆液性囊腺瘤临床表现为下腹隐痛、腹部肿块。眼观见肿块呈囊性,囊壁增厚,切面见红褐色出血性肿块。镜下以充满红细胞的大小不等的囊腔、相互沟通的不规则管道、大部分由梭形细胞构成的实性区域为特征,伴有浆液性囊腺瘤形成。免疫表型：CD31、CD34、FⅧRAg、PCNA呈广泛性强阳性,SMA在中等大小的血管及部分肿瘤细胞呈强阳性,Ki-67为散在阳性,CK（AE1／AE3）为阴性。结论原发性卵巢血管肉瘤十分罕见,必需注意其鉴别诊断。     

Primary angiosarcoma of the kidney: case analysis and literature review

Objective: To study the clinical presentation, diagnosis, treatment and prognosis of primary angiosarcoma of the kidney. Methods: We treated a patient with primary angiosarcoma, then searched the published papers with the terms of ‘primary angiosarcoma of the kidney’ and ‘primary renal angiosarcoma’ in PubMed database, found 27 patients with detailed data, and analyzed their characters in the clinical presentation, diagnosis, treatment and prognosis. Results: The primary angiosarcoma occurred mainly from 50 years old to 69 years old, predominated in male patients. The clinical presentation was flank pain and hematuria, and the nephrectomy was the mainstay of the treatment; the maximum diameter and the metastasis status at the time of diagnosis had important prognostic value. Conclusions: The primary angiosarcoma is a rare carcinoma and lacks of specific presentation. Accurate diagnosis depends on pathological examination. Surgery is the mainstay of the treatment, but the prognosis is poor.     

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.