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.     

以皮肤水疱为主要表现的血管肉瘤1例

报告1例以皮肤水疱为主要表现的血管肉瘤。患者男,78岁,面部、额部、头皮紫红色肿物、水疱5月余,经组织病理学检查确诊为血管肉瘤,免疫组化检查显示CD31( )、CD34(血管 )。     

Primary nonphylloides breast sarcomas

BACKGROUND: The prevalence of primary breast sarcoma is low, occurring in fewer than 1% of women with breast malignancies. The purpose of this study was to examine the presentation, treatment, and prognosis of patients presenting with these neoplasms. METHODS: This was a retrospective review of patients with a primary breast sarcoma treated at Mayo Clinic, Rochester, Minnesota, between 1975 and 2001. Follow-up information was obtained. RESULTS: Of the 55 patients, 17 had breast-conserving therapy and 38 women had mastectomy. The mean patient age at presentation was 52 years (range 22 to 82). The types of sarcoma included angiosarcoma (18), malignant fibrous histiocytoma (11), stromal sarcoma (8), liposarcoma (4), leiomyosarcoma (4), dermatofibrosarcoma protuberans (4), osteosarcoma (3), fibrosarcoma (2), and rhabdomyosarcoma (1). Follow-up information was available for 53 patients, with a mean follow-up of 81 months. Twenty-nine of 53 patients (55%) developed recurrent sarcoma, and 23 patients (43%) died of their disease. Twenty-seven patients had no evidence of recurrence, and 3 patients were alive with disease at last follow-up. Overall median survival of patients with breast sarcoma was 58 months. Patients with angiosarcoma had a poorer outcome than other sarcoma patients. Twelve of 18 patients (67%) died of angiosarcoma, compared with 11 of 32 patients (34%) of all other sarcoma patients combined. Of 34 patients who did not receive adjuvant chemotherapy or radiation, 13 died of their disease (38%), as compared with 10 of 16 patients (63%) who did receive adjuvant therapy.CONCLUSIONS: While primary nonphylloides breast sarcomas are rare tumors, their treatment and prognosis are poor. Adjuvant chemotherapy and radiation did not improve survival in this report. Surgical extirpation remains the only effective treatment.     

Malignant vascular tumors of liver in neonates

Malignant vascular tumors of the liver are rare in children, especially in neonates. We report two cases. One was initially diagnosed as infantile hemangioendothelioma. Rapid growth and appearance of pulmonary metastasis while on aggressive medical treatment suggested malignant transformation. The other neonate presented with intractable ascites and liver biopsy showed histological features of angiosarcoma. Ascites has rarely been reported as a presenting feature of angiosarcoma in literature. Both patients died. To date, surgery, chemotherapy, and radiotherapy have not improved the outcome of malignant vascular tumors of liver.     

Hemangioendothelioma of bone. A case report with massive tissular necrosis

Hemangioendothelioma or histiocytoid hemangioma of bone is a low grade tumor, which may be a quite indolent and controlable lesion, even in the rare case it develops distant metastasis. Our case presents features of such lesion by light microscopy and with a peroxidase-antiperoxidase technique for Factor VIII related antigen, but it exhibits an unusual extensive tissular necrosis. Necrosis does not seems to make worse the usual course, after six years and ten months of conservative surgical treatment and radiation therapy.     

子宫血管肉瘤2例报告并文献复习

目的 分析2例起初被误诊为其他类型子宫恶性肿瘤的子宫血管肉瘤病例并复习相关文献,以提高临床医生对其临床病理特征、治疗和预后的认识。 方法 收集并分析山东大学齐鲁医院妇科收治的2 例子宫血管肉瘤患者的临床资料,结合相关文献对该疾病进行总结。 结果 病例1接受了根治性手术,术后给予异环磷酰胺+表柔比星方案化疗6个周期,术后无进展生存期为62个月,总生存期为66个月;病例2接受了根治性手术,术后给予异环磷酰胺+表柔比星方案化疗4个周期,尚无复发迹象。 结论 子宫血管肉瘤临床上极为罕见,易被误诊,诊断时应借助病理及免疫组化与其他子宫恶性肿瘤相鉴别,手术联合术后辅助化疗可能是子宫血管肉瘤一种有效的治疗方法。     

Histopathology of Spindle Cell Vascular Tumors

Vascular tumors with a spindled morphology represent a diagnostic challenge in soft tissue pathology. It may be difficult to distinguish certain benign entities in this category from spindled vascular tumors of intermediate malignancy or even spindled variants of angiosarcoma. This article focuses on vascular tumors characterized by a predominantly spindled morphology, including spindle cell hemangioma, acquired tufted angioma (angioblastoma of Nakagawa), kaposiform hemangioendothelioma, Kaposi sarcoma, and spindle cell variants of angiosarcoma.     

Breakages at YWHAE,FAM22A,and FAM22B loci in uterine angiosarcoma: A case report with immunohistochemical and genetic analysis

Described herein is the first reported case of a uterine angiosarcoma with breakages at three loci, YWHAE (17p13), FAM22A (10q23) and FAM22B (10q22). A 62-year-old postmenopausal woman was found to have endometrial thickening of her uterus. An endometrial biopsy indicated a malignant, spindle cell neoplasm. A total hysterectomy with bilateral salpingooophorectomy was performed. Histologic examination of the uterine specimen showed a malignant tumor consisting of irregular rudimentary vascular channels and solid small nests diffusely infiltrating to the middle of the myometrial wall. The tumor cells were epithelioid, and displayed eosinophilic cytoplasm and vesicular nuclei in some areas of the tumor. Immunohistochemically, the tumor cells showed vascular differentiation; they were diffusely positive for CD31 and D2-40 but were negative for factor VIII and CD34. In the course of the procedure of differential diagnoses, we included fluorescence in situ hybridization analysis for detection of a FAM22B-YWHAE fusion gene resulting from t(10;17)(q22;p13), recently reported in a series of endometrial stromal sarcoma, and unexpectedly identified breakages at three loci, i.e. YWHAE (17p13), FAM22A (10q23) and FAM22B (10q22). Collectively, these findings suggest that abnormality in the loci of YWHAE, FAM22A and FAM22B, which are known to be associated with oncogenesis of endometrial stromal sarcoma, may contribute to the development of uterine angiosarcoma.     

Angiosarcoma of the spleen presenting as spontaneous splenic rupture: A rare case report and review of the literature

INTRODUCTIONAngiosarcoma of the spleen is a rare malignancy of vascular origin with a high rate of metastasis and poor prognosis. We report one such rare case of spontaneous splenic rupture, along with a review of current literature.PRESENTATION OF CASEA 30 year old man presented to our emergency services with severe abdominal pain, distension, hypotension and splenomegaly. Investigations revealed a marked anaemia, coagulopathy, severe lactic acidosis, and acute kidney injury. Imaging demonstrated splenomegaly with acute haemorrhage and lymphadenopathy. Laparotomy and splenectomy revealed piecemeal spleen and nodular omentum. The patient suffered an intra-operative cardiorespiratory arrest, and despite successful resuscitation, fatally arrested postoperatively in ICU. Histology revealed a primary splenic angiosarcoma with omental metastases.DISCUSSIONPrimary splenic angiosarcoma was first reported in 1879, with only 200 cases reported to date, largely as isolated case reports, with an annual incidence of 0.14–0.25 per million. With variable symptomatology and a potential to present with life-threatening complications, early diagnosis is paramount. CT scanning shows distinctive changes and is invaluable in disease assessment. Tissue diagnosis is often possible only after splenectomy. Spontaneous rupture carries the worst prognosis.CONCLUSIONPrimary splenic angiosarcoma is a rare and aggressive malignancy that often presents with metastatic disease, and largely carries a dismal prognosis. Definitive diagnosis is challenging, but imaging with CT scanning can show characteristic changes and establish any metastatic disease. With no established adjuvant therapy long term outlook remains poor even if treated successfully by surgery.