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Objective: Breast angiosarcoma is a tumor that can arise as a primary breast tumor or in association with prior radiation therapy. Angiosarcomas are uniquely sensitive to paclitaxel. This study evaluated the impact neoadjuvant paclitaxel (NAC) therapy has on surgical outcomes, tumor recurrence, and survival in breast angiosarcomas., Methods: Patients with angiosarcoma of the breast, either primary or radiation-associated, were identified from a prospective institutional database. Patients receiving NAC were compared to those treated with upfront surgery. Clinical and pathological variables were compared using Student's t-test or Fisher's exact test, differences in survival were calculated using Kaplan-Meier methods., Results: Twenty-four patients with angiosarcoma of the breast were identified, 10 with primary angiosarcoma and 14 with radiation-associated angiosarcoma. Twelve patients received NAC, 6 of each with primary angiosarcoma or radiation-associated angiosarcoma. Of these 12 patients, 11 had a margin negative resection (91%) of which, nine had a complete pathological response on surgical pathology. Of the 12 surgery-first patients, four (n = 4/12, 33%) had positive surgical margins, two of the four underwent reoperation. With a median follow-up of 16 months, four NAC patients had recurrence (33%) compared to six patients in the surgery-first group (58%) (p = 0.41). While not statistically significant, NAC patients had a 33% less risk of recurring compared to surgery-first patients ([hazard ratio =0.67 (95% confidence interval 0.16-2.72; p = 0.6])., Conclusion: NAC for breast angiosarcoma may be associated with high rates of complete pathological response and margin-negative resection. However, this did not impact overall survival. Future prospective control studies and longer follow-up periods are warranted to understand the impact on recurrence and survival., (© 2024 The Author(s). Journal of Surgical Oncology published by Wiley Periodicals LLC.)

Background and Objectives: Neoadjuvant radiation (NRT) is frequently utilized in soft tissue sarcomas to increase local control. Its utility in cutaneous and soft tissue angiosarcoma remains poorly defined., Methods: This retrospective cohort study was performed using the National Cancer Database (2004-2016) evaluating patients with clinically localized, surgically resected angiosarcomas. Factors associated with receipt of NRT in the overall cohort and margin positivity in treatment naïve patients were identified by univariate and multivariable logistic regression analyses. Survival was assessed using Kaplan-Meier analysis., Results: Of 597 patients, 27 (4.5%) received NRT. Increasing age (odds ratio [OR] 0.95, p = 0.025), tumor size more than or equal to 5 cm (OR 3.16, p = 0.02), and extremity tumor location (OR 3.99, p = 0.04) were associated with receipt of NRT. All patients who received NRT achieved an R0 resection (p = 0.03) compared with 17.9% of patients without NRT. Factors associated with risk of margin positivity included tumor size more than or equal to 5 cm (OR 1.85, p = 0.01), and head/neck location (OR 2.24, p = 0.006). NRT was not significantly associated with improved survival (p = 0.21)., Conclusions: NRT improves rates of R0 resection but is infrequently utilized in cutaneous and soft tissue angiosarcoma. Increased usage of NRT, particularly for patients with lesions more than or equal to 5 cm, or head and neck location, may help achieve complete resections., (© 2021 Wiley Periodicals LLC.)

Background and Objectives: Primary liver sarcomas (PLS) are rare. Published series are limited by small numbers of patients., Methods: We reviewed the National Cancer Database (2004-2014) for patients who underwent surgical resection of PLS., Results: Of 237 patients identified, the majority were female (60.8%), with median age of 52 years. Histologies were: epithelioid hemangioendothelioma (n = 67), angiosarcoma (n = 64), leiomyosarcoma (n = 33), embryonal rhabdomyosarcoma (n = 31), carcinosarcoma (n = 16), giant cell sarcoma (n = 14), spindle cell sarcoma (n = 12). Ninety-seven (40.9%) patients underwent lobectomies or extended lobectomies, 41 patients (17.3%) underwent transplantation. Surgical margins were negative in 82.9%. Tumors were well differentiated in 11.3%. Histology type correlated with outcome with the best prognosis for epithelioid hemangioendothelioma (OS: not reached, similar for resection and transplantation) and the worst for angiosarcoma (OS:16.6 mo with resection; 6 mo with transplantation; P = 0.04). Resections with microscopically negative margins were associated with improved survival (58.7 vs 11.3 mo for positive margins; P < 0.001). Chemotherapy and radiation therapy were used in a minority of patients (32.9% and 4.3% respectively) with no improvement in outcomes., Conclusions: Both hepatic resection and liver transplantation can be associated with long term survival for selected primary liver sarcomas such as epitheliod hemangioendotheliomas. Histology type and the ability to resect the tumor with negative margins correlate with outcomes and the decision to operate should be carefully weighed for subtypes with particularly dismal prognosis such as angiosarcomas., (© 2018 Wiley Periodicals, Inc.)

Background: Angiosarcoma is an aggressive tumor rising in incidence from use of therapeutic radiation. Because of its relative rarity, prognostic factors have not been clearly delineated., Methods: Patients who underwent resection of localized angiosarcoma from 2002 to 2012 were identified using the National Cancer Data Base. Factors associated with poor overall survival (OS) were identified using Cox proportional hazards modeling and internally validated with bootstrap resampling. These were used to construct a risk model to identify low-, intermediate-, or high-risk groups., Results: Median OS among 821 patients undergoing resection was 3.4 years. On multivariable analysis of factors known preoperatively, those associated with worse OS included: age >70 years (HR 2.02, P < 0.0001), black race (HR 1.92, P < 0.0001), head and neck primary (HR 1.44, P = 0.003), grade 3 tumor (HR 1.53, P = 0.013), size 3-7 cm (HR 1.64, P < 0.0001), size >7 cm (HR 2.37, P < 0.0001). After including postoperative variables, positive resection margins were associated with worse OS (microscopic, HR 1.59, P = 0.002; macroscopic, HR 3.38, P = 0.008). Stratification by risk group was superior to AJCC stage in discriminating OS., Conclusions: In the largest study to date of patients with angiosarcoma, risk factors for poor OS were identified to create a clinically useful risk model that can prognosticate patients with localized disease following surgical resection. J. Surg. Oncol. 2016;114:557-563. © 2016 Wiley Periodicals, Inc., (© 2016 Wiley Periodicals, Inc.)

Background and Objectives: Angiosarcoma of the breast can be divided into primary and secondary. The objective was to determine clinicopathologic factors associated with breast angiosarcoma and to compare primary versus secondary angiosarcoma., Methods: Breast angiosarcoma cases at Mayo Clinic from 1960 to 2008 were identified. Clinicopathologic factors were reviewed. Characteristics of primary and secondary angiosarcoma were compared., Results: Twenty-seven cases of primary angiosarcoma and 14 cases of secondary angiosarcoma were identified. The median age of primary angiosarcoma patients was lower than that of secondary angiosarcoma--43 years versus 73 years (P < 0.0001). Primary angiosarcoma more frequently presented with a mass, whereas secondary angiosarcoma presented with a rash (P < 0.0001). Median time from radiation to secondary angiosarcoma diagnosis was 6.8 years. Median tumor size was 7.0 cm for primary angiosarcoma and 5.0 cm for secondary angiosarcoma (P = 0.7). Tumors were high grade in 33% of primary angiosarcoma and 82% of secondary angiosarcoma (P = 0.02). Five-year survival for primary and secondary angiosarcoma was 46% and 69%, respectively (P = 0.8)., Conclusion: Primary angiosarcoma occurs in younger patients than secondary and more frequently presents with a mass. Mastectomy is the mainstay of treatment for breast angiosarcoma. Breast angiosarcoma is a rare malignancy with poor long-term prognosis., ((c) 2010 Wiley-Liss, Inc.)

Background: Primary angiosarcomas of breast are rare tumors, with a fatal outcome., Material and Methods: We studied histological prognostic factors and c-kit expression by immunohistochemistry (IHC) in 12 angiosarcomas accessioned at a cancer referral center in India., Results: All patients had primary angiosarcoma; no case of secondary angiosarcoma was accessioned during the study period. Median age of patients was 24.5 years. Nine patients had intermediate grade tumors, one a well differentiated tumor and three patients had high-grade tumors. Interesting cases encountered included an epithelioid angiosarcoma and an angiosarcoma arising on the background of a biphasic tumor. Eight patients had lumpectomy, four mastectomy and two patients were given radiotherapy. Of the nine patients (seven type I/II and two high grade) with follow up, eight patients developed disseminated metastases within a year of presentation. The patient with well-differentiated angiosarcoma also died of metastasis albeit after a longer time. On IHC c-kit staining was weakly seen in two cases., Conclusion: Primary angiosarcoma was fatal in young Indian women even in lower grade tumors. The low expression of c-kit on IHC suggests that targeting this protein for therapy may not be successful in treating these tumors., (Copyright (c) 2006 Wiley-Liss, Inc.)

Background and Objectives: Primary splenic angiosarcoma is a very rare and aggressive neoplasm with a high metastatic rate and dismal prognosis. Since only a few cases have been adequately reported in the medical literature, we report here a further six cases., Methods: The records of all cases of primary splenic angiosarcoma treated at Chang Gung Memorial Hospital from April 1991 to July 2004 were retrospectively reviewed., Results: Of the six cases identified (three men and three women; range, 7-69 years; median, 44 years), four presented with palpable abdominal masses or left upper quadrant abdominal pain. Other systemic symptoms, such as bleeding gums, fatigue, fever, body weight loss, and gastrointestinal bleeding were noted. An abnormal hematogram was found in five patients, with all of these five showing anemia, and three thrombocytopenia. Splenomegaly was present in all patients. Hemoperitoneum due to splenic rupture was noted in one patient. Three patients had distant metastasis to the liver (n = 2), bone (n = 1), bone marrow (n = 1), and small bowel (n = 1) at diagnosis. Liver (n = 3), bone (n = 1), and bone marrow (n = 1) metastases were found in four patients after initial therapy. Five of the six cases underwent a splenectomy, one underwent partial hepatectomy, and one received chemotherapy. The respective 1-, 3- and 5-year survival rates were 60%, 40%, and 40%, and the median survival time was 36 months. One patient was disease-free 162 months after splenectomy., Conclusions: The clinical presentations of splenic angiosarcoma were similar to those of previous reports apart from the higher rate of splenomegaly observed in this study. In contrast to reported pediatric cases, our patient achieved long-term disease-free survival after splenectomy alone., (2005 Wiley-Liss, Inc.)

Background and Objectives: Primary sarcoma occurring in breast is rare and comprises 0.5-1% of all breast neoplasm. Majority of the series include both stromal and cystosarcoma phyllodes, only a few hundred cases of sarcomas other then cystosarcoma are reported., Patients and Methods: We carried out a retrospective analysis of 19 patients with primary sarcoma of the breast treated between 1982 and 2002., Results: Mean age of the patients was 38.6 years (12-70 years). Gradually progressive swelling was the commonest presenting feature. There were eight cases of angiosarcoma, four cases of spindle cell sarcoma, two each of pleomorphic sarcoma and stromal sarcoma, and one each of malignant fibrous histiocytoma, embryonal rhabdomyosarcoma, and sarcoma (NOS). Eight of these were high-grade (42%). Eight patients underwent either radical or modified mastectomy, three underwent wide excisions, and one underwent quadrantectomy. Ten (52.6%) patients received postoperative adjuvant radiation. Two patients received chemotherapy. After a mean follow-up time of 34.5 months (median 25 months), eight patients failed. Failure was local in five, opposite breast in one, and both local and distant in two. The disease free survival at 3-year was 39%. In univariate analysis only the margin of first surgery was found to be a significant predictor of survival (P = 0.05)., Conclusions: Primary sarcomas of the breast are aggressive tumors. Surgical treatment should consist of at least simple mastectomy. All attempts should be made to achieve a negative margin as this appears to be the only factor influencing survival in these patients., (Copyright 2004 Wiley-Liss, Inc.)

Stewart-Treves syndrome (STS) is the rare occurrence of angiosarcoma in a setting of postmastectomy upper extremity lymphedema. A collective comparison of outcomes following various initial treatment options in STS has not previously been reported. We reviewed 160 cases of STS reported in the literature since 1966. We analyzed the relationship between initial treatment and survival in all 92 of these patients for whom detailed treatment and outcome data had been reported. There was no significant difference in survival comparing those initially treated with wide excision (n = 16) and those treated with amputation (n = 45) (P = 0.40). Even in the setting of initial surgical treatment, overall long-term survival was poor (<40%). There have been even fewer long-term survivors among those treated initially with regional chemotherapy (n = 7) or radiation therapy (n = 24). An update on STS and a discussion of recent advances in the understanding of its molecular pathogenesis that may result in future treatment improvements are presented.

Data for prognostic factors in angiosarcoma (AS) are limited, prompting a large-scale study of AS with multivariate analysis. To analyze prognostic factors in angiosarcoma (AS), clinical and histologic findings in 55 patients collected from hospitals in Japan were reviewed. Prognostic factors were evaluated by univariate and multivariate Cox's proportional hazards models. The study involved 32 males and 23 females, ages 18-93 (median, 69) years. The primary sites of tumors included head and neck (32 cases), trunk (10), extremities (3), spleen (3), breast (3), and other (4). The overall 2-year survival rate was 21%. Univariate analysis of clinical factors including age, sex, size and depth of tumor, tumor-related symptoms, interval between onset of symptoms and admission, surgical procedures, adjuvant chemotherapy, and adjuvant radiotherapy showed that age, tumor size, and mode of treatment were significant for survival. Histologic factors analyzed were mitotic counts, cellularity, cellular pleomorphism, extent of necrosis, vascular differentiation, and nonspecific diagnosis. Only mitotic counts were significant for prognosis. Multivariate analysis on these four factors revealed that tumor size, mode of treatment, and mitotic counts were independent prognostic factors.

Splenic hamangiosarcomas are rare tumors, usually discovered at autopsy. In a few instances the diagnosis was made premortem, at the time of splenectomy for spontaneous rupture. The tumors usually present with abdominal pain, left upper guadrant mass and tenderness, and occasionally with a microangiopathic type of anemia. The histogenesis of the tumor is in dispute. Some authors feel that they are degenerations of hemangiomas. Others feel that they arise de novo in the spleen. There is not proven association of thorotrast administration or vinyl chloride exposure to the development of hemangiosarcomas in the spleen. The prognosis of the tumor is uniformly poor and most of the patients surviving laparotomy have followed a uniformly fatal clinical course. In a few cases treated with chemotherapy there has been no evidence of clinical benefit. The case report in this article presented with essentially all the features enumerated above.