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Background. The purpose of the study was to describe the clinicopathologic characteristics and clinical outcomes of patients with primary breast angiosarcoma. METHODS. The institutional database was searched to identify breast angiosarcoma patients seen between 1965 and 2002. Survival outcomes were estimated by the Kaplan‐Meier method. The log‐rank test was used to compare groups. Cox proportional hazards models were used for multivariate analysis. RESULTS. In all, 69 patients were identified. Median follow‐up was 40 months (range, 0‐413 months). Median age was 46. Median tumor size at diagnosis was 5.5 cm. Thirteen (18.8%) patients received prior radiation for invasive breast carcinoma. Most patients underwent total mastectomy with (41%) or without (45%) axillary dissection. Regional metastasis to axillary lymph nodes was rare. There were 38 recurrences and 27 deaths. The 5‐year overall (OS) and recurrence‐free survival (RFS) rates were 61% (95% confidence interval [CI], 49%–76%) and 44% (95% CI, 33%–58%) with estimated medians of 100 and 37 months, respectively. In Cox proportional hazards models, OS and RFS were significantly associated only with T size and not with patient age, prior radiation, or chemotherapy administration. Of 29 patients treated with chemotherapy at recurrence, there were 4 complete and 10 partial responses (48%) with an anthracycline‐ifosfamide or gemcitabine‐taxane combination. CONCLUSIONS. Breast angiosarcoma is frequently advanced at diagnosis and has a tendency for local‐regional recurrence. A significant number of responses to chemotherapy was observed in the metastatic setting. These data suggest that a multidisciplinary therapeutic approach should be employed in high‐risk patients with large primary tumors. Cancer 2007. © 2007 American Cancer Society.

Rosai-Dorfman disease is a rare subtype of non-Langerhans cell histiocytosis. With the last major report published in 1990, there is a paucity of contemporary data on this disease. Our objective was to report the clinicopathological features, treatments and outcomes of patients seen at a tertiary referral center. Sixty-four patients with histopathological diagnosis of Rosai-Dorfman disease were identified from 1994 to 2017 (median age 50 years; range, 2-79). The median duration from symptom onset to diagnosis was seven months (range, 0-128), which was also reflected in the number of biopsies required to establish the diagnosis (median 2; range, 1-6). The most common presentation was subcutaneous masses (40%). Of the 64 patients, 8% had classical (nodal only) and 92% had extra-nodal disease (67% extra-nodal only). The most common organs involved were skin and subcutaneous tissue (52%), followed by lymph nodes (33%). Three patients had an overlap with Erdheim-Chester disease, which had not been described before. Two of these were found to have MAP2K1 mutations. Commonly utilized first line treatments were surgical excision (38%) and systemic corticosteroids (27%). Corticosteroids led to a response in 56% of the cases. Of those treated initially, 15 (30%) patients developed recurrent disease. The most commonly used systemic agent was cladribine (n=6), with 67% overall response rate. Our study demonstrates that Rosai-Dorfman disease has diverse clinical manifestations and outcomes. While this disease has been historically considered a benign entity, a subset of patients endures an aggressive course necessitating the use of systemic therapies.