Pediatric Primary Ovarian Angiosarcoma: From Rarity to a Realization

Background

Angiosarcoma is an uncommon high-grade sarcoma in children. Visceral angiosarcoma involving the ovary is extremely rare. Because of the lack of recurrent cytogenetic alterations, histopathological identification of this uncommon tumor in unusual sites like the ovary demands pathologic expertise. Complete surgical resection and radiotherapy are the chief treatment modalities determining survival, with chemotherapy contributing a minor role.

Angiosarcoma of skull base in a 1-year-old child—A case report

Angiosarcoma is a rare tumour of endothelial cell origin whilst malignant skull base tumours are highly unusual in paediatric patients. This case reports an angiosarcoma involving the clivus and basi-sphenoid region of the skull base, in a 1-year-old boy. This tumour is extremely rare in childhood, particularly in this site. The histological features were consistent with a high-grade haemangioendothelioma, categorised as an angiosarcoma [1]. The characteristics of this rare malignancy and the challenges in its management are discussed. This is the youngest reported patient, to our knowledge, with an angiosarcoma of the skull base.     

Primary angiosarcoma of the abdominal aorta: a case report and literature review (aortic angiosarcoma)

Fewer than 140 cases of aortic sarcoma have been reported with only 34 classified as angiosarcoma. These rare malignancies most often mimic aortoiliac occlusive or aneurysmal arteriosclerotic disease both clinically and on imaging studies, and the large majority are unexpected and diagnosed pathologically on a surgical specimen or at autopsy [1–5]. A 42-year-old woman who presented with low back pain and claudication was shown by CT and angiography to have infrarenal aortic occlusion. Angiosarcoma was an unexpected pathologic diagnosis based on tissue removed during aortobifemoral bypass surgery. Pre and postoperative CT and angiography are presented along with a review of the literature.     

The role of radiation therapy in the management of skin cancers

The use of radiation therapy in the management of skin cancer is variable and often anecdotal. Applied as both primary and adjuvant therapy in patients with both nonmelanoma skin cancer and rarer tumors of the skin, a consensus regarding optimal dosing regimens has not yet been reached. Herein, the authors outline the basic concepts of radiation therapy for tumors of the skin and review its use for high-risk nonmelanoma skin cancer, as well as less common malignancies, including angiosarcoma, Merkel cell carcinoma, and sebaceous carcinoma.     

Angiosarcoma of the uterus: report of 2 new cases with deviant clinicopathologic features and review of the literature

A few cases of uterine angiosarcoma have been detailed in the literature: 2 new cases are herein described featuring some unusual clinical or phenotypic differences compared with previously published cases, such as occurrence in premenopausal age or a poorly differentiated histology. The patients were 35 and 81 years old, and both presented with extrauterine spread and evidence of distant metastases. Grossly, they were fleshy, hemorrhagic, and necrotic. Microscopically, they were made up of poorly differentiated, epithelioid, or spindle cells. In one case, the neoplastic growth deceitfully recalled a poorly differentiated leiomyosarcoma showing focal rudimentary endothelial differentiation. The other index case was characterized by a more pronounced vascular pattern, the neoplasm was composed of spindle cells arranged in loose channels. A diffuse immunopositivity for CD31, CD34, and factor VIII–related antigen was detected. Tumor cells were negative for other tested antigens including keratins, desmin, actins, and H-caldesmon. One patient died shortly during the follow-up, whereas the other is alive with evidence of disseminated disease. Occurrence in childbearing age or a deviant histologic pattern, as documented in this report, may be added to the clinicopathologic spectrum of uterine angiosarcoma.     

Primary angiosarcoma in non-irradiated parotid gland – Very unusual malignant tumour

Medical literature and the number of patients diagnosed with head and neck angiosarcomas are very limited. Our case report presents clinical history, imaging procedures, treatment (surgery) and histological findings of angiosarcoma in non-irradiated parotid gland.     

Primary vascular tumours of the kidney

Primary vascular tumours of the kidney are rare and may pose diagnostic difficulties because of their similar clinical, morphological, and immunohistochemical features. This article summarizes the clinical and pathological features of primary renal angiosarcoma and anastomosing haemangioma of the kidney including epidemiology, genetics, and prognosis. Renal anastomosing haemangiomas are benign neoplasms characterized by anastomosing capillary-sized vascular channels. These tumours are rare, with about 75 cases reported in the literature. Most anastomosing haemangiomas are found incidentally on ultrasound, computed tomography, or magnetic resonance imaging. Common symptoms include abdominal pain, haematuria, and abdominal mass. Renal anastomosing haemangiomas are characterized by recurrent mutations in GNAQ and GNA14 genes. The prognosis of anastomosing haemangioma is excellent. Primary renal angiosarcomas are malignant tumours showing endothelial differentiation. To date, 76 cases have been described in the literature. Primary renal angiosarcomas are frequently symptomatic. The clinical features of renal angiosarcomas are similar to those of renal anastomosing haemangiomas, including abdominal pain, haematuria, and abdominal mass. Angiogenesis-related genes and vascular-specific receptor tyrosine kinases such as KDR, TIE1, SNRK, TEK, and FLT1 are upregulated in angiosarcomas. Primary renal angiosarcomas are highly aggressive neoplasms with a poor prognosis despite surgical treatment, chemotherapy, radiotherapy, or targeted therapy.     

血管靶向药物治疗血管肉瘤的1个病例报告和文献复习

血管肉瘤是一种罕见的有侵袭性的起源于血管内皮的肿瘤,预后极差。无法手术的患者治疗方法非常有限,而且没有治疗标准。贝伐珠单抗是一个抗血管内皮生长因子的血管生成抑制剂,是重组人源化的单克隆抗体,可以作为血管肉瘤治疗的一种有前景的选择。这是一个采用贝伐珠单抗联合化疗药物治疗血管肉瘤的病例报道和文献回顾,患者通过治疗得到了短期的缓解获益并提高了生活质量。     

Metastatic squamous cell carcinoma with pseudoangiosarcomatous features and aberrant expression of vascular markers

Squamous cell carcinoma with pseudoangiosarcomatous features is a rare but well-recognized variant of squamous cell carcinoma. These tumors exhibit complex anastomosing channels lined by neoplastic cells, histologically mimicking a vasoformative mesenchymal tumor. Immunohistochemically, the published cases expressed epithelial markers and were consistently negative for vascular markers. Squamous cell carcinoma with pseudoangiosarcomatous features and aberrant expression of vascular markers has never been reported. Herein, we report two cases of metastatic poorly-differentiated squamous cell carcinoma with pseudoangiosarcomatous morphologic features which showed immunoreactivity for vascular markers (CD31, Fli-1, and ERG). One case (left thigh skin squamous cell carcinoma with abdominal wall metastasis) showed strong and diffuse positivity for vascular markers, and the final diagnosis was confirmed with electron microscopy. The second case (squamous cell carcinoma of unknown primary site with bone metastasis) showed patchy positivity for both squamous and vascular markers. This is the first report of squamous cell carcinoma with pseudoangiosarcomatous features and aberrant expression of vascular markers, which resembles angiosarcoma both morphologically and immunohistochemically, and may represent a potential diagnostic pitfall. It is of crucial importance for pathologists to be aware of metastatic squamous cell carcinoma with such unique features, so that misdiagnosis and inappropriate treatment will be avoided.