Angiosarcoma of the testis

 A primary angiosarcoma of the testis in a 74-year-old patient was a highly anaplastic epthelioid angiosarcoma, which was positive for endothelial markers immunohistochemically. The tumour was unrelated to testicular germ cell neoplasm; the patient had received no previous radiation or chemotherapy. Received: 18 August 1998 / Accepted: 16 November 1998     

Cutaneous angiosarcoma–A report of 20 Taiwanese patients

Angiosarcoma is a rare soft tissue sarcoma of poor prognosis. A retrospective single-center study was conducted to characterize cutaneous angiosarcoma. Twenty patients were enrolled. The mean age was 76 years and male to female ratio 3:1. Ninety percent had primary angiosarcoma. Scalp was the most commonly involved site. Metastatic disease was noted in 20% when angiosarcoma was diagnosed. Median overall survival time was 20 months. In this single center series, cutaneous angiosarcoma was a disease of early metastasis and poor prognosis. Physician should be aware of vascular lesions on the scalp and face regions especially in male elderly patients.     

Angiosarcoma: state of the art and perspectives

We propose a literature review of available data on angiosarcoma (AS). AS account for 1% of adult soft tissue sarcoma. Two risk factors are well-establish chronic lymhoedema, previous radiotherapy. Clinical presentations of AS are heterogeneous. Large resection followed, if possible, by adjuvant radiotherapy is the cornerstone of curative intent treatment of localized forms. There are no convincing data supporting the administration of adjuvant chemotherapy. For metastatic or locally advanced AS, doxorubicin and weekly paclitaxel seem to provide the longer progression-free survival. Three phase II or parts of phase II trials have been published in the last 2 years, investigating weekly paclitaxel, sorafenib and imatinib, demonstrating that clinical trials are feasible for such rare diseases. Biological evidences for the key role of angiogentic factors have been accumulated during the last years and support the further investigation of anti-angiogenetic agents alone and almost combination with chemotherapy in such disease.     

Angiosarcoma-like metastatic carcinoma of the liver

Two cases of metastatic carcinoma strikingly simulating angiosarcoma in the liver are described. The first case was a 53-year-old female with cystic liver tumors which were found 22 months after surgery for ureteral cancer. The second case was an 81-year-old female with multiple tumors in the liver and the pancreas, and a post-mortem examination was carried out. She had undergone surgery for skin cancer three years before. Both cases had an angiosarcoma-like appearance macroscopically and microscopically. Immunohistochemically, the tumor cells of both cases were negative for CD31, CD34, and Factor VIII-related antigen and positive for several types of cytokeratin, suggesting that they were not angiosarcomas but carcinomas. Angiosarcoma is the most common sarcoma arising in the liver. Thus, metastatic carcinoma, which resembles angiosarcoma, might be mistaken for angiosarcoma.     

Sorafenib for patients with advanced angiosarcoma: a phase II Trial from the French Sarcoma Group (GSF/GETO)

Background.

Angiosarcomas account for <2% of all soft tissue sarcomas. This subtype is one of the most aggressive forms of soft tissue sarcoma. The prognosis for angiosarcoma patients in the advanced phase remains poor with current cytotoxic agents (progression-free survival [PFS] time of ∼4 months and overall survival [OS] time of ∼8 months). We investigated the antitumor activity of sorafenib in patients with metastatic or advanced angiosarcomas in a phase II trial.

Methods.

We conducted a stratified phase II trial. The primary endpoint was the progression-free rate (PFR) at 9 months according to the Response Evaluation Criteria in Solid Tumors. A two-stage design (optimal Simon design) was used. Patients received sorafenib (400 mg twice daily) for 9 months until unacceptable toxicity or tumor progression. Central pathological and radiological reviews were performed. Data on stratum A (superficial angiosarcoma) and stratum B (visceral angiosarcoma) are currently available. This trial is registered with ClinicalTrials.gov (identifier, {"type":"clinical-trial","attrs":{"text":"NCT00874874","term_id":"NCT00874874"}}NCT00874874).

Findings.

Strata A and B recruited 26 and 15 patients, respectively. The median age was 63 years (range, 31–82 years), with 17 male and 24 female patients. Fourteen cases arose in irradiated fields. Thirty patients (73.0%) had been pretreated with conventional chemotherapy. No unexpected toxicity occurred. The PFR at 9 months was 3.8% in stratum A and 0.0% in stratum B. The median PFS times were 1.8 months and 3.8 months, respectively, whereas the median OS times were 12.0 months and 9.0 months, respectively. No responses were observed in chemotherapy-naïve patients, whereas a 40% tumor control rate and 23% response rate were observed in the pretreated population. In this cohort, no activating mutation of the KDR gene (exons 15, 16, 24) was detected.

Interpretation.

Sorafenib showed limited antitumor activity in pretreated patients only, for both visceral and superficial angiosarcoma, but tumor control was of short duration.     

41例肝脏原发性恶性血管肿瘤的临床病理特点

目的 探讨肝脏原发性恶性血管肿瘤（PHMVT）的临床病理学及预后的特点。方法 回顾性分析1982年1月至2012年12月在我院手术切除并经病理组织学证实的41例PHMVT,对其临床表现、病理学特点及预后进行分析。结果 41例患者中,肝上皮样血管内皮瘤（EHE） 19例（46.3％）,平均瘤体直径为49cm（1.2～6.3cm）;肝血管肉瘤（PHA）14例（34.1％）,平均瘤体直径为8.3cm（3.0～14.0cm）;肝婴儿型血管内皮瘤（IHE）5例（12.2％）,平均瘤体直径为3.8cm（1.2～6.3cm）;肝恶性血管外皮瘤（MHP）3例（7.3％）,平均瘤体直径为7.8cm（2.1～13.0cm）。免疫组化显示41例肿瘤细胞均表达Vimentin、CD34或因子Ⅷ。EHE患者术后中位生存期为87个月,优于PHA的12个月（P＜0.05）;2例有完整随访资料的MHP患者分别于术后43个月死亡和术后84个月复发;IHE患者术后均无复发,预后良好。结论 PHMVT的病理类型与预后有关,PHMVT的恶性程度由高到低依次为PHA＞MHP＞EHE＞IHE。     

Rapid clinical course of cerebral metastatic angiosarcoma from the heart

We report here one case of rapid and aggressive course of cerebral metastatic angiosarcoma from the heart. A 36-year-old man presented with 10-days history of headache. Magnetic resonance imaging demonstrated subacute hemorrhage with a small region of enhancement in right parietal region and the pathological diagnosis was angiosarcoma. Transthoracic echocardiography demonstrated 3.2×3 cm sized mass on right atrial wall. Newly developed lesion was reoperated, three and four weeks later respectively, and whole brain radiotherapy of total 30 Gy was done. With the interval of two months, gamma knife surgery was done for new lesions two times, which were well controlled. Newly developed lesions rapidly happened even in the adjuvant treatment. He died 9 months after the diagnosis because of the aggravation of primary cancer. The cerebral metastatic angiosarcoma from the heart showed the rapid aggressive behavior and the closed follow-up could be needed for the adjuvant treatment.     

Angiosarcoma of the thyroid

Hemangiosarcoma of the thyroid gland is a very rare entity. The therapy of this tumor is difficult because of its locally aggressive and destructive behavior with a high recurrence rate. We report a case of 60-year-old man with history of 1 month rapidly enlarging neck mass in the thyroid region. Ultrasound examination and CT scan showed resistance arising from the left thyroid lobe. FNAC revealed necrotic tumor mass without any cytological specification. Because of the clinical findings, a total thyroidectomy was indicated. The surgery was complicated by invasion of the tumor mass into the surrounding tissues. Therefore, only left side hemithyroidectomy was performed. Histopathology including histochemistry proved positive staining for Factor VIII, Fasciin, and CD31 in tumor cells. In this case, adjuvant oncological treatment was planned, but the patient died because of massive bleeding from the tumor mass. The histological diagnosis of thyroid hemangiosarcoma is mostly difficult. The treatment of the first choice is radical surgery. If the tumor cannot be treated by surgery, then radiotherapy, eventually chemotherapy is indicated. The prognosis is not favorable.     

原发性肋骨血管肉瘤的诊治探讨

目的：研究肋骨血管肉瘤的临床病理特征、治疗及转归。方法：对１例原发性肋骨血管肉瘤的组织病理学、免疫组化结果进行观察分析,结合文献进行讨论。结果：本例血管肉瘤组织学表现为肿瘤细胞呈梭形或上皮样细胞形态。免疫组化染色表达ＣＤ３１、ＣＤ３４。结论：肋骨原发性血管肉瘤极少见,结合组织学形态并联合应用多项血管内皮标记物可作出明确诊断。早期发现、局部手术切除和结合术后放化疗有助于延长患者生存期。     

24例原发性心脏及心包恶性肿瘤的诊断与治疗

目的 探讨原发性心脏及心包恶性肿瘤的诊断和治疗方法.方法 收集24例原发性心脏及心包恶性肿瘤患者的临床资料.24例患者分别接受了不同的辅助检查,包括心电图、超声心动图(UCG)、胸片、CT、冠状动脉CT、三维CT、MRI、心包穿刺和组织病理检查.10例患者行肿瘤根治切除,6例行肿瘤局部切除,8例行开胸探查和局部活体组织检查.结果 24例原发性心脏及心包恶性肿瘤患者均经病理学检查确诊.行肿瘤根治切除术的10例患者中,1例术后第7天死于呼吸循环衰竭,2例尚存活,2例久访,其余5例术后生存时间为1～4年,死于复发或转移.6例行肿瘤局部切除的患者存活4～16个月,死于心功能衰竭和恶液质等.8例仪行开胸探查和局部活体组织检查的患者中,6例在1年内死亡,2例失访.结论 UCG、CT、MRI、三维CT、冠状动脉CT、心血管造影等检查对原发性心脏及心包恶性肿瘤的诊断及手术方法选择有帮助,但确诊仍需依靠病理学检查.早期手术治疗,尽量完全切除肿瘤,有助于延长患者生存期.