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Objective: Primary intracranial angiosarcomas (PIAs) are exceedingly uncommon, with the literature predominantly comprising case reports. The clinical characteristics and prognosis of this condition remain elusive. Our objective is to describe the clinical characteristics and surgical prognosis of this rare disease while offering insights into the most effective contemporary treatment strategy., Methods: The authors of this article incorporated a cohort of 28 cases of PIAs, consisting of 3 from our institution and 25 from previously documented literature sources. Subsequently, we conducted both Cox univariate and multivariate analyses to assess the potential risk factors influencing overall survival (OS)., Results: The cohort include 19 males and 9 females with a mean age of 39.6 ± 23.5 years (range: 0.03-73 years). Radiologically, 24 cases were located at supratentorial area, while only 4 cases were located at infratentorial area. 17 cases underwent gross total resection (GTR), and 11 cases underwent Non-GTR. Postoperative radiotherapy was administered to 17 cases, and postoperative chemotherapy was administered to 6 cases. After a mean follow-up time of 21.5 ± 26.4 months, 19 (67.9%) patients died. The 1-year, 2-year, 5-year OS is 55.3%, 50.7% and 24.6%, respectively. Univariate and multivariate Cox regression analysis showed that Non-GTR was the sole factor predicting a shorter OS (p = 0.004)., Conclusion: In this study, we found that PIAs have a higher incidence in males than in females, and most cases show evidence of old hemorrhage on preoperative MRI. Through our statistical analysis, GTR plays a crucial role in for treating this rare disease. Further clinical data are needed to validate our conclusions., (© 2023. The Author(s), under exclusive licence to Springer Science+Business Media, LLC, part of Springer Nature.)

Background: Angiosarcoma of the breast is rare and aggressive. It can occur as a de novo tumor or secondary to breast cancer treatment. The purpose of this study is to analyze differences between patients with primary and secondary angiosarcoma of the breast and investigate potential risk factors for its development., Patients and Methods: The Surveillance, Epidemiology, and End Results program of the National Cancer Institute database was queried to identify patients with angiosarcoma of the breast, trunk, shoulder, and upper arm. The population-based incidence was analyzed. Primary and secondary angiosarcoma cases were identified and compared. Breast cancer characteristics of secondary angiosarcoma patients were compared with all breast cancer patients in the database who did not develop angiosarcoma., Results: Overall, 904 patients were included, and 65.4% were secondary angiosarcomas. These patients had worse survival, were older, more likely to be White, more likely to have regionally advanced disease, and had angiosarcoma tumors of higher pathologic grade. Independent factors associated with development of secondary angiosarcoma among breast cancer patients included White race, older age, invasive tumor, lymph node removal, lumpectomy, radiation treatment, and left-sided tumor. Although the mean time to develop angiosarcoma after breast cancer diagnosis was 8.2 years, the risk continues to increase up to 30 years after breast cancer treatment., Conclusion: Angiosarcoma is rare but increasing in incidence. Secondary angiosarcomas are more common and exhibit more aggressive behavior. Several factors for angiosarcoma after breast cancer treatment could be identified, which may help us counsel and identify patients at risk., (© 2021. Society of Surgical Oncology.)

Background: The incidence of lymph node metastasis (LNM) of angiosarcomas is reported to be less than 15%, and elective neck management has not been indicated. This study evaluated the incidence and pattern of regional LNM in patients with scalp angiosarcomas using the clinical data of its full course to understand time-event sequences of scalp angiosarcomas., Methods: This retrospective study included all consecutive cases of pathology-confirmed angiosarcomas and analyzed 40 cases of scalp angiosarcomas. The survival plots were estimated using the Kaplan-Meier method, and the results are presented mainly in a descriptive manner., Results: The overall survival rate for the patients was 35.8% at 2 years. In contrast to previous reports, regional LNM was observed in more than half of the patients (52.5%) with scalp angiosarcoma. Meanwhile, a direct spread to distant organs occurred in only 27.5% of the patients. Regional LNM could predict clinical manifestation of systemic disease within 3 to 6 months. No differences in survival rates between patients with and without LNM were observed in this series. Occurrence of LNM seemed to be correlated with a high mitotic rate of primary tumors, but not with tumor grade or tumor dimension. The first-echelon lymph nodes from scalp angiosarcoma were peri-parotid, post-auricular, and level 2 lymph nodes., Conclusions: For a localized scalp angiosarcoma, it seems reasonable for initial curative surgery to include prophylactic evaluation of regional lymph nodes for pathologic nodal staging, prognosis estimation, and the decision for systemic treatments.

Background: Radiation-associated angiosarcoma of the breast (RAASB) is an aggressive malignancy that is increasing in incidence. Only a few previous population-based studies have reported the results of RAASB treatment., Methods: A search for RAASB patients was carried out in the Finnish Cancer Registry, and treatment data were collected to identify prognostic factors for survival., Results: Overall, 50 RAASB patients were identified. The median follow-up time was 5.4 years (range 0.4-15.6), and the 5-year overall survival rate was 69%. Forty-seven (94%) patients were operated on with curative intent. Among these patients, the 5-year local recurrence-free survival, distant recurrence-free survival, and overall survival rates were 62%, 75%, and 74%, respectively. A larger planned surgical margin was associated with improved survival., Conclusions: We found that the majority of RAASB patients were eligible for radical surgical management in this population-based analysis. With radical surgery, the prognosis is relatively good.

Background: Hepatic angiosarcoma is a rare primary liver tumor. The aim of this current study was to evaluate the presentation and treatment outcomes in a modern cohort., Methods: This was a retrospective, multi-institutional, observational study of patients with histopathologic diagnoses of primary hepatic angiosarcoma from four institutions. Clinicopathologic characteristics, treatments, and patient outcomes were examined., Results: Forty-four patients with hepatic angiosarcoma were identified. Patients were predominantly Caucasian and presented at a median age of 63.7 years; 81.4% of patients had bilobar disease and 37.2% had metastatic disease at the time of presentation. Only 10 patients underwent surgical resection. Median overall survival for the entire cohort was 5.8 months (interquartile range 1.9-16.4), and 1-, 3-, and 5-year actual survival was 30.0%, 8.1%, and 5.6%, respectively. There were only two 5-year survivors, both of whom presented with localized disease and underwent curative resection., Conclusion: The prognosis for hepatic angiosarcoma remains quite poor. Surgical resection for localized disease results in the best outcomes. Unfortunately, current imaging modalities are often non- diagnostic, and most patients are unresectable at the time of presentation.

Transthoracic echocardiography (TTE), computed tomography (CT) and magnetic resonance (MR) cannot always clearly identify angiosarcoma. A 22 year old woman presented with dyspnea. Preoperative TTE showed no tumor, and CT and MR both gave the impression that an extra-cardiac, mediastinal tumor was present. Transesophageal echocardiography (TEE), performed during surgery showed an intra-cardiac tumor, which was a well-delineated mass at the intra-ventricular groove of the right heart and surrounding the superior vena cava. The tumor was excised and found to be a malignant angiosarcoma. TEE provides diagnostic advantages over other cardiac imaging techniques and can serve as a useful adjunct during and before intrathoracic surgery.

We reported one case of penile angiosarcoma in a 49-year-old patient who had been misdiagnosed as syphilis. Based on our experience, we suggest that if sexually transmitted diseases and Peyronie's disease can be ruled out, diagnosis of the penile angiosarcoma be warranted if unexplained rash, ulceration on the penis, and induration in corpus cavernosum are observed. It should be noted that if the anti-inflammatory or anti-viral treatment is prescribed for some time but the lesion does not disappear or even gets worse, the penile angiosarcoma should be suspected and penile biopsy is necessary.

Background: Secondary angiosarcoma of the breast is a rare but severe long-term complication of breast cancer treated with breast-conserving surgery and radiotherapy. We characterized a population-based cohort of patients with secondary angiosarcomas from two tertiary hospitals to investigate this complication with respect to surgical treatment and outcome., Methods: We identified 35 patients with a history of radiation for breast cancer that developed angiosarcoma in the irradiated field from 1990 to 2009. Of these, 31 underwent surgery and were included for analysis., Results: Angiosarcoma developed after median 7 years (range 3-25 years). R0 resection was obtained in 23 of 31 patients after primary treatment. Local recurrence developed in 19 patients after median 6 months (range 1-89 months). Regional and distant metastases occurred in 13 patients after median 17 months (range 2-50 months); nine which also had local recurrence. Patients whose local recurrence could be operated on had a better survival after treatment than those who were not considered for surgical treatment, median 34 months (range 6-84 months) compared with 6 months (range 5-24 months). The median disease-free survival and disease-specific survival was 16 and 37 months, respectively., Conclusions: Despite R0 resection, two-thirds of the patients developed a local recurrence. Survival among those with local recurrence was better if the patient could be treated with surgery. Overall, the prognosis was dismal and median DSS was just over 3 years.

Pulmonary epithelioid angiosarcoma is rare, and there are no descriptions of the immunocytochemistry of such a case. We report a case with focus on immunocytochemistry of imprint cytological specimens here. A 43-year-old Japanese man presented with cough. Brushing cytology of the tumor using a bronchoscope showed a malignant neoplasm. Subsequently, left lower lobectomy was undertaken. Imprint cytological material of the neoplastic cells noted some clusters of round, or oval, to polygonal epithelioid cells. Histological examination showed the proliferation of epithelioid neoplastic cells containing erythrocytes in the cytoplasm. The immunocytochemistry in imprint cytological specimens showed a focal positive reaction for von Willebrand factor, and the immunohistochemistry of the surgically resected lung tumor showed a focal positive reaction for Fli-1, von Willebrand factor, CD31, and CD34. Finally, we suggest that the immunocytochemical study of imprint cytological materials may supply available information in diagnosing angiosarcoma with epithelioid features lacking characteristic structures.

Background: Angiosarcomas are an uncommon type of malignancy that are generally thought to behave usually in a locally aggressive fashion; they often metastasize to distant sites., Methods: Patients with a diagnosis of angiosarcoma treated at our institution between 1980 and 2006 were analyzed for patient demographics, tumor characteristics, multimodality treatment, and outcomes., Results: A total of 82 patients were divided into those with primary and advanced disease. Overall, the median age was 65 (range, 22-91) years, and 44% of patients were women. Median size of tumors was 3.8 cm, and 76% of tumors were intermediate or high grade. Tumors were located throughout the body: 32 cutaneous, 22 deep soft tissues or organs, 10 radiation or lymphedema field, 8 bone, and 7 nonirradiated breast. Of 46 patients with primary disease, all patients underwent surgical resection, 67% received radiotherapy, and 27% received chemotherapy. Five-year disease-specific survival was 60%, and negative prognostic factors included intermediate or high grade, and tumors arising in a radiated or lymphedema field. Of 36 patients with advanced disease, 36% underwent a palliative operation, 78% received radiation, and 58% received chemotherapy. Median survival was just 7.3 months, and cutaneous tumors predicted a better prognosis compared with other sites., Conclusions: Primary angiosarcomas treated with aggressive surgical resection and the addition of radiation for close margins or worrisome pathologic features can result in long-term survival in most patients. The role of adjuvant chemotherapy is unclear. Patients with advanced disease have a poor prognosis, but there can be dramatic responses to chemotherapy in a minority of patients.

Background: The aim of this study was to analyze the presentation of, surgery for, and prognosis of malignant vascular tumors (MVTs)., Methods: This was an observational single-center study. Patients who underwent operation for MVTs between 1988 and 2004 were included. Data were gathered prospectively in a computerized registry., Results: Of 568 adult patients with soft tissue malignancies, 43 (7.6%) were treated for MVTs. Twenty-four men and 19 women (median age, 55.3 years) were referred for 30 primary tumors and 13 recurrences. Symptoms were observed in 90.7% of the cases (swelling [37.2%], pain [34.9%], extrusion [11.6%], hemorrhage [7%], weight loss [4.7%], loss of energy [4.7%], impaired function [4.7%], and others [30.2%]). Tumors were located in the extremities (n = 16), trunk (n = 3), abdomen (n = 15), retroperitoneum (n = 7), and thyroid gland (n = 2). Twenty-two (51.2%) angiosarcomas, nine (20.9%) malignant hemangiopericytomas, eight (18.6%) malignant epithelioid hemangioendotheliomas, and four (9.3%) lymphangiosarcomas were seen. The median overall survival after surgery was 21.4 months, with 2-, 5-, and 10-year overall survival rates of 41.5%, 38.3%, and 18.8%, respectively. MVTs of the extremities and trunk and localized disease indicated a better prognosis than abdominal or retroperitoneal MVTs (univariate and multivariate analyses: P = .0122 and P = .0287) and metastasized stages (univariate and multivariate analyses: P = .0187 and P = .0287)., Conclusions: A considerable number of patients with soft tissue malignancies undergo surgery for MVT. Various symptoms and a multilocular occurrence are typical. The course of MVTs is aggressive. Tumor site and stage are important prognostic factors. Surgery is potentially curative, especially for localized disease of the extremities and trunk.

A patient with epithelioid angiosarcoma of the gallbladder is described. This is only the second case of an extremely rare but highly aggressive tumor reported in the international literature. Pathophysiological, clinical, and therapeutic aspects are discussed in relation to the available data on angiosarcomas of the gallbladder.

Angiosarcoma of the spleen is a very rare but highly malignant vascular neoplasm. So far only 140 cases have been reported. A 42-year-old patient is presented in which the radiologic imaging misled to the diagnosis of infiltrating echinococcosis. After splenectomy histological and immunohistochemical staining gave proof of metastatic angiosarcoma. The patient died three months later as a consequence of multiple organ dysfunction syndrome. The literature is reviewed in regard to clinical features, diagnosis and therapy.

Fifty-one cases of posterior fossa haemangioblastoma have been seen in our department over a period of 23 years. Seven cases were associated with spinal cord lesions and one with a supratentorial lesion. There were 22 cases of Lindau's disease (43%). The onset was earlier and recurrences commoner than in isolated cases. In 10 cases the development of new tumours in different parts of the central nervous system (CNS) required re-operation, demonstrating the multicentric nature of this disease. The majority of the tumours occupying the fourth ventricle or medulla oblongata (8 cases) were macroscopically solid and had higher post-operative morbidity than the cystic tumours in the cerebellum. The recent development of magnetic resonance imaging (MRI) provides accurate anatomical information of the lesions (especially those of the brain stem and spinal cord) even while asymptomatic. The brilliant enhancement of these tumours on MRI with Gadolinium-diethyline-triamine-pentaacetic acid (Gd-DTPA) has proved to be particularly useful.

Eighteen cases of isolated spinal haemangioblastoma are presented. 13 patients were male and 5 female; average age was 34 years. The site of the tumour was bulbo-cervical in 1 case, cervical in 6, cervico-thoracic in 2, thoracic in 7, thoraco-lumbar in 2. Surgical excision of the tumor was total in all cases and peri-operative mortality was 5%. At average follow-up of 11 years, 8 patients had died (4 from neurological causes and 4 from non-neurological disease), and 9 survived; symptoms and signs improved in 11 cases, and were not improved in 6. The biological and pathological characteristics of this tumour are reported in the light of the present 18 cases and those reported in the literature.

A man presenting with sleep apnoea was found to have a haemangioblastoma at the cervico-medullary junction. The associated hydrocephalus and syringomyelia resolved after excision of the tumour. Postoperatively the patient developed transient bilateral glossopharyngeal neuralgia, presumably due to surgical damages to the tractus solitarius. To the best of our knowledge this is the first reported case with transient bilateral glossopharyngeal neuralgia following resection of a haemangioblastoma of the cervico-medullary junction.

MRI and Cusa have significantly modified the diagnosis and the treatment of intramedullary tumours. Our experience, based on 66 cases, is in favour of surgery performed when the patient's neurological status is still good. Radical surgery, whenever possible, is the best treatment and can be achieved for many histological diagnoses, even in gliomas where we succeeded in 36% of the cases. Surgery is the only way to be sure of the exact histological diagnosis of the lesion which can be suggested in 70% of the cases but not confirmed by MRI.

Haemangioblastomas located in the posterior fossa have rarely been described in patients older than 60 years of age. The authors report a case of a 95 year-old man who presented with an obstructive hydrocephalus secondary to a posterior fossa tumour. Computed tomography and angiography suggested intracerebellar metastasis. Despite the advanced age of the patient and his poor general condition, surgery was performed with the patient in a supine position. A haemiangioblastoma was found and completely removed. The postoperative course was without complications. This case demonstrates that the combination of modern anaesthesia, supine operating position, and the use of microsurgical techniques allow successful operations on the posterior fossa even in very old patients.

A case of primary haemangioblastoma with a marked blood supply from multiple meningeal branches of the internal and external carotid arteries is reported, and the clinical implication of detailed angiographic study of unusual feeders in this highly vascular tumour is discussed.

477 patients with soft tissue sarcomas, including 87 children (18.2%) were treated at the Surgical Center of the University of Heidelberg between 1950 and 1980. 22.8% of the patients with solid tumors (excluding brain tumors) in childhood were soft tissue sarcomas. In the pediatric age group there were 30% rhabdomyosarcoma and 20% angioblastic sarcoma. Since introduction of combined tumor therapy (surgery, radiation, and chemotherapy) the survival rate increased impressively from (29% to 58%).

Between 1975 and 1983, 17 patients who underwent surgery for haemangioblastomas of the brain (16 lesions occurring in the posterior fossa) were studied with CT scans (17 cases) and vertebral angiograms (15 cases). The CT scans were superior for demonstrating a cystic tumour, its topography in the posterior fossa, its relationship to the fourth ventricle, and the presence and degree of an obstructive hydrocephalus. The vertebral angiographies were more sensitive and specific than CT for revealing the vascular nature, and were imperative in order to establish the supply and drainage of the tumours. It is clear that CT and angiography each make an important contribution toward defining these tumours. Their association is necessary.

The clinical features, diagnostic and surgical aspects of haemangioblastoma of the posterior cranial fossa are discussed in the light of a series of 61 personal cases and the published data. CT and vertebral angiography are compared as diagnostic tools. With the aid of microsurgery even haemangioblastomas of the brainstem and fourth ventricle can be removed successfully.

A series of 81 patients operated for intramedullary spinal cord tumours over a span of 12 years (1975-1986) is presented. The mean age of the patients was about 37 years and 9 patients were in the paediatric age (0-15 years). In 29 cases (36%) the tumours were located within the cervical region; in 34 cases the tumours were located in the thoracic region (42%) and in 18 cases the tumours were located within the medullary cone (22%). Ependymoma accounted for 52% of the cases (42 cases), astrocytoma for 20% (16 cases), lipoma for 7% (6 cases), hemangioblastoma for 6% (5 cases), metastatic tumours for 5% (4 cases); in the remaining 8 cases (10%) rare or very rare spinal tumours were found. The 9 patients in the paediatric age and 8 additional cases who had undergone previous surgery were then excluded from this review. In 59% of the patients with ependymoma and in 90% of the patients with astrocytoma severe neurological deficit were already present before the operation. Total tumour removal was accomplished in 81% of the cases with ependymoma, total or subtotal removal was achieved in 50% of the cases with astrocytoma. At long term follow-up study fair or good functional results were observed in 19 out of 41 cases of ependymoma (46%) and in 3 cases out of 10 of astrocytoma (30%). The surgical outcome was mostly related to the preoperative neurological conditions and to tumour malignancy grade.

Experiences with 47 haemangioblastomas of the central nervous system seen in our department over a period of 33 years are described. Among these cases there were six tumours of the brain stem and four of the cerebrum. It is pointed out that, after a diagnosis of brain stem tumour has been made, a shunt procedure is indicated if high intracranial pressure is present. Radiation does not seem to help this kind of patient. Polycythaemia was observed in both types of tumours. One case of spinal cord tumour was seen during the same period, and in another case multiple tumours of the brain stem and the spinal cord were observed.