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Background: Radiation-associated angiosarcoma of the breast (RAAS) is a rare complication of adjuvant radiotherapy associated with poor survival. The British Sarcoma Group guidelines recommend that all angiosarcomas are referred to a sarcoma multidisciplinary team, although there is no recommendation that patients are managed within a sarcoma service. The aims of this study were to compare survival, complete excision rates and local recurrence rates of patients managed within a sarcoma service and those managed within local hospitals., Methods: All patients with RAAS referred to a regional sarcoma service between 1998 and 2015 were identified from prospective databases. Patient records, and radiology, pathology and operation notes were reviewed retrospectively., Results: Thirty-six patients were operated on with curative intent; 26 were managed by the sarcoma service (of whom 21 underwent radical excision of the irradiated field followed by chest wall reconstruction) and ten were managed locally. Median age was 69·5 (range 43-85) years. Disease-specific survival was significantly longer in patients managed by the sarcoma service than in those managed locally: median 91·1 (range 69·2-113·0) versus 48·8 (18·6-79·1) months respectively (P = 0·012). Overall survival rates were similar (P = 0·112). There was no difference in complete excision rate (18 of 26 in sarcoma service versus 5 of 10 in local services; P = 0·456), although the local recurrence rate was significantly lower among patients managed by the sarcoma service (9 of 26 versus 8 of 10; P = 0·015)., Conclusion: Specialist management of RAAS leads to fewer local recurrences and improved disease-specific survival. Early referral and management within specialist units is recommended., (© 2018 BJS Society Ltd Published by John Wiley & Sons Ltd.)

Chronic exposure to high LET radiation has been shown to cause liver cancer in humans based on studies of patients who received Thorotrast, a colloidal suspension of thorium dioxide formerly used as a radiological contrast agent, and on studies of Russian nuclear weapons workers exposed to internally ingested plutonium. Risk estimates for these exposures and specific subtypes of liver cancer have not been previously reported. Combining published data with tumor registry data pertinent to the Thorotrast cohorts in Germany, Denmark, Portugal, and Japan and to Russian workers, we generally found significantly elevated risks of three major histologic types of liver tumors: hepatocellular carcinoma (HCC), cholangiocarcinoma (CC), and hemangiosarcoma (HS) for Thorotrast exposures. In contrast, HS was the only liver tumor significantly associated with the lower alpha-particle doses experienced by the Russian workers. Excess cases per 1,000 persons exposed to Thorotrast were similar for the three liver cancer subtypes but lower for plutonium exposure. Odds ratios (OR) of HS and CC for Thorotrast were from 26 to 789 and from 1 to 31 times higher than those for HCC, respectively. ORs of liver cirrhosis for Thorotrast exposure ranged from 2.7 (95% confidence interval (CI): 2.2-3.4) to 6.7 (5.1-8.7).

Cutaneous angiosarcoma is a rare aggressive tumour of capillary and lymphatic endothelial cell origin. It presents as multiple purple and red papules and nodules on the head and neck or the extremities. We report an 86-year-old woman with angiosarcoma arising on her chronically lymphoedematous right leg. The lymphoedema, secondary to chronic immobility, had developed gradually over 40 years. No other family members had lymphoedema. The patient presented with a plaque of friable tumour tissue on the lower right leg and dorsum of the foot, and satellite lesions on the knee and groin which initially appeared to be petechial haemorrhages. The satellite lesions in the groin grew into tumour nodules. There was no evidence of a preceding malignancy, nor any operative intervention to the affected limb or abdomen. Histological examination of all tumour specimens revealed moderately to poorly differentiated angiosarcoma. She died within 5 months of the first appearance of the skin nodules.

A case of cutaneous angiosarcoma occurring in a 51-year-old male renal transplant patient is reported. Multiple violaceous nodules surrounded by poorly demarcated red to purple discoloration were found on his scalp. Immunosuppressants consisting of azathioprine and prednisolone had been administered during the 12-year period since the renal transplantation. We diagnosed the lesion clinically as a cutaneous angiosarcoma and performed a wide surgical excision. The histopathological findings confirmed the diagnosis and showed tumour cells in the peripheral margin. Postoperatively, the patient started immunotherapy with systemic administration of recombinant interleukin-2 (rIL-2), but he refused to continue it because of the acute rejection of the transplanted kidney induced by the rIL-2. Instead he received radiation therapy (total 7000 rad) of the scalp. Although no recurrence was noticed for 15 months after the completion of radiation, he died due to lung metastasis from angiosarcoma. We review the seven cases, including ours, of angiosarcoma after renal transplantation that are reported in detail in the literature.

We describe a patient with angiosarcoma of the scalp arising in an area of radiodermatitis caused by X-ray epilation therapy for scalp ringworm as a child. Radiotherapy has been well documented as a causative factor in the formation of cutaneous malignancies, most notably basal cell and squamous cell carcinoma, but only rarely angiosarcoma. This is now the first reported case of angiosarcoma arising after X-ray epilation therapy for scalp ringworm, and we postulate a causative link between the two events.

A cutaneous metastatic angiosarcoma was diagnosed in a 79-year-old woman 19 years after radiotherapy for a carcinoma of the uterine cervix. The diagnosis was confirmed by immunohistochemical staining (factor VIII-related antigen and BMA 120) and electron microscopic examination. Surgical treatment of the large tumour, which was situated in the gluteal region, was not feasible, but electron beam therapy resulted in complete remission. However, a further metastasis occurred in the inguinal region, and management by total excision, radiotherapy, and interferon-alpha treatment was unsuccessful. The patient died 28 months after the initial diagnosis of the neoplasm.

In cases of congenital lymphoedema the finding of ulceration, violaceous nodules or papules, or apparent traumatic ecchymoses should act as a diagnostic beacon warning of dangers. A case is reported of a high-grade angiosarcoma developing in a patient with congenital hereditary lymphoedema (Milroy's disease). This is the second paper to report this complication, the third case report and the first case in which the diagnosis is substantiated by immunohistochemistry and lectin histochemistry. A review of cases of angiosarcoma complicating congenital hereditary and non-hereditary lymphoedema is also presented.

A case of angiosarcoma of the colon is reported. The tumor developed in the fibrous capsule of a retained sponge that was lost 25 years earlier during a gynecologic surgical procedure. The postoperative course was dominated by a fatal consumptive thrombohemorrhagic disorder. Angiosarcoma associated with a retained foreign body and development of hemorrhagic diathesis in angiosarcoma are discussed.

We report a case of angiosarcoma of the glans penis in a 77 year old male Caucasian. The tumour developed 18 years after a course of radiotherapy for a penile ulcer which was an intra-epidermal squamous carcinoma. The differential diagnosis and the concept of radiotherapy-induced angiosarcomas are discussed.

Diagnosed from 1970 through 1975, the annual incidence rate for angiosarcoma of the liver among residents of New York State (excluding New York City) was 0.25 per million. A case-control study indicated that direct exposure to arsenic, vinyl chloride (VC), and thorium dioxide was a significantly important factor in the etiology of this disorder (P less than 0.02). Direct exposure to these chemicals could not be demonstrated for 19 (73%) of the 26 study patients. The fact that 5 of these patients lived nearer to VC fabrication or polymerization plants than did their matched controls lent some support to the hypothesis that indirect modes of exposure, not specifically related to occupation, might be important in the etiology of this disorder.

A patient is discussed who had angiosarcoma of her lymphedematous right breast develop four years after segmental mastectomy for infiltrating ductal carcinoma. The lymphedema developed and persisted after an indolent and recurrent postoperative infection. The possibility that the second malignancy is a consequence of the chronic lymphedema, similar to the angiosarcomas of lymphedematous extremities after radical mastectomy, is cautiously entertained. This hypothesis is worthy of consideration as more breast conservation surgery is being done, with or without adjuvant radiation therapy, and accumulating evidence suggests that lymphedema of the breast is a common complication of surgery followed by radiation.

Von Hippel-Lindau disease is an autosomal dominant multisystem disorder, the commonest presenting manifestations of which are haemangioblastomas of the cerebellum and retina. Affected individuals are at risk of developing a number of other lesions, the most serious of which are renal carcinoma, haemangioblastomas elsewhere in the central nervous system and phaeochromocytoma. Patients with this disease can therefore present to a number of disciplines during their lifetime and unless the possibility of von Hippel-Lindau disease is considered, the patient may wrongly be assumed to have an isolated lesion. Twenty patients with cerebellar haemangioblastomas were seen between 1972 and 1985; the diagnosis of von Hippel-Lindau disease was subsequently established in 8. Although the diagnosis had not previously been considered, in retrospect 7/8 cases were known to be at risk of this syndrome. These cases came from 7 families and an additional 4 relatives are also known to have been affected. Mean age of presentation for the various manifestations of von Hippel-Lindau disease, each of which occurred in one or other of our cases, has been calculated from 9 of our patients and 107 others reported in the literature. Clinically significant manifestations almost invariably developed before the age of 50 years. Limited screening of our index cases and their at-risk relatives demonstrated one asymptomatic renal carcinoma. We propose a protocol for screening all individuals at risk of von Hippel-Lindau disease, which involves annual retinal examination from five years, and biennial computerized tomography of the head and abdomen from fifteen and twenty years, respectively.

Female R rats mated with an R male and inoculated in utero with polyoma virus after "fetectomy" developed tumors. These tumors originated in the uterus and were of fetal origin (visceral yolk sac). Histologically, they were hemangiomas or hemangiosarcomas. The latter were transplantable and grew in tissue culture. Infectious polyoma virus could not be isolated from the tumor cells kept as transplantable lines or cultured in vitro. However, the tumor cells were positive for the polyoma-specific surface antigen, polyoma tumor-specific transplantation antigen(s), and polyoma nuclear T antigen.

We describe a patient who developed an angiosarcoma on her face at the age of 24. The tumour arose from an angiomatous naevus that had been treated with radiotherapy when the patient was 7 months old.

A hemangiosarcoma of the abdominal wall developed in a long-standing, nonhealing radiation-induced ulcer ten years after therapeutic irradiation for endometrial carcinoma.

Data on 93 autopsy cases (group A) of thorotrast-associated liver cancers were obtained from the "Annual of Pathological Autopsy Cases in Japan" from 1958 to 1979, and data on 78 autopsy cases (group B) of thorotrast-associated liver cancers were obtained from the Japanese literature from 1953 to 1980. Cholangiocarcinoma (CLC) constituted 58% of group A and 55% of group B. The curve of the cumulative numbers of patients with CLC versus year in group A was almost linear, showing an increasing risk per surviving patients with advancing time. Angiosarcoma (AGS) occurred in 25% of group A and 24% of group B. The number of patients with AGS increased significantly after 1969 in both groups (P less than 0.05). In group B, age and years after thorotrast injection of patients with AGS were statistically higher than those of patients with CLC (age: P less than 0.05; years after thorotrast injection: P less than 0.0001). Hepatocellular carcinoma (HPC) accounted for 17 and 21% of groups A and B, respectively. When yearly distribution, age, and time after thorotrast injection of patients with HPC were correlated with those of patients with other liver cancers, the only statistically significant difference between patients with HPC and patients with CLC (P less than 0.02) was in the years after thorotrast administration. Since 1977 multiple primary liver cancers including AGS developed in thorotrast-administered patients in both groups.