Clinical outcomes of angiosarcoma: a single institution experience

Kim, Jae‐Joon; Seo, Seyoung; Kim, Jeong Eun; Jung, Sung-Ho; Song, Si Yeol; Ahn, Jin‐Hee

doi:10.1186/s40880-019-0389-1

Cited by 5 publications

(5 citation statements)

References 18 publications

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“…Surprisingly, only one of the patient in our current series was living after 65 months. According to previously published angiosarcoma data from our hospital, 16 the mean overall survival for localized angiosarcoma was 21.6 months compared to only 12.56 months for primary cardiac angiosarcoma in our current study cases. Although the 5‐year survival rate for common angiosarcoma in our present series was about 40% regardless of the tumor location, there was no patient in our cohort with a primary cardiac angiosarcoma that survived past 5 years.…”

Section: Discussionsupporting

confidence: 62%

Primary cardiac sarcomas: A clinicopathologic study in a single institution with 25 years of experience with an emphasis on MDM2 expression and adjuvant therapy for prognosis

Cho

Song

et al. 2023

Cancer Medicine

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BackgroundPrimary cardiac sarcomas are rare and their clinicopathologic features are heterogeneous. Among them, particularly intimal sarcoma is a diagnostic challenge due to nonspecific histologic features. Recently, MDM2 amplification reported to be a characteristic genetic event in the intimal sarcoma. In this study, we aimed to identify the types and incidence of primary cardiac sarcomas that occurred over 25 years in tertiary medical institutions, and to find clinicopatholgical significance through reclassification of diagnoses using additional immunohistochemistry (IHC).MethodsWe reviewed the primary cardiac sarcoma cases between January 1993 and June 2018 at Asan Medical Center, South Korea, with their clinicopathologic findings, and reclassified the subtypes, especially using IHC for MDM2 and then, analyzed the significance of prognosis.ResultsForty‐eight (6.8%) cases of a primary cardiac sarcoma were retrieved. The tumors most frequently involved the right atrium (n = 25, 52.1%), and the most frequent tumor subtype was angiosarcoma (n = 23, 47.9%). Seven cases (53.8%) were newly reclassified as an intimal sarcoma by IHC for MDM2. Twenty‐nine (60.4%) patients died of disease (mean, 19.8 months). Four patients underwent a heart transplantation and had a median survival of 26.8 months. This transplantation group tended to show good clinical outcomes in the earlier stages, but this was not statistically significant (p = 0.318). MDM2 positive intimal sarcoma showed the better overall survival (p = 0.003) than undifferentiated pleomorphic sarcoma. Adjuvant treatment is beneficial for patient survival (p < 0.001), particularly in angiosarcoma (p < 0.001), but not in intimal sarcoma (p = 0.154).ConclusionOur study supports the use of adjuvant treatment in primary cardiac sarcoma, as it was associated with a significantly better overall survival rate. Further consideration of tumor histology may be important in determining the optimal use of adjuvant treatment for different types of sarcomas. Therefore, accurate diagnosis by MDM2 test is important condsidering patient's prognosis and treatment.

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Section: Discussionsupporting

confidence: 62%

Primary cardiac sarcomas: A clinicopathologic study in a single institution with 25 years of experience with an emphasis on MDM2 expression and adjuvant therapy for prognosis

Cho

Song

et al. 2023

Cancer Medicine

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“…Other forms of CAS include those that present on the trunk/extremities, radiation induced angiosarcoma of the breast, and Stewart–Treves Syndrome. A retrospective study analyzing clinical outcomes of angiosarcomas in various locations found that median OS (21.7 months) was longest in patients with CAS located on the trunk [ 20 ].…”

Section: Discussionmentioning

confidence: 99%

Cutaneous Angiosarcoma of the Head and Neck—A Retrospective Analysis of 47 Patients

Ramakrishnan

Mokhtari

Charville

et al. 2022

Cancers

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Cutaneous angiosarcoma (CAS) is a rare and aggressive malignant tumor with blood vessel or lymphatic-type endothelial differentiation. It has a poor prognosis with lack of standardized treatment options. This study retrospectively evaluated the clinical characteristics and treatment outcomes of 47 patients with CAS of the head and neck treated at an academic sarcoma center. Patient data were collected from the electronic medical records. 62% of patients were male with the scalp being the most commonly affected area (64%). The majority of patients presented with localized disease (53%). Median overall survival (OS) was 3.4 years with an OS of 36% at 5 years. There was a statistically significant increase in OS for patients who underwent surgery compared to those who did not (5.4 vs. 2.8 years). In contrast, radiotherapy (RT) or chemotherapy did not significantly increase OS. 45% of patients had recurrence of disease during their treatment course with a median time to recurrence of 22.8 months. There was not a significant difference in OS for patients who underwent immunotherapy compared to those who underwent chemotherapy, although only a few patients received immunotherapy. We found that surgery was an effective treatment modality in patients with easily resectable disease, while RT, chemotherapy, and immunotherapy did not significantly improve OS.

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“…Overall prognosis of angiosarcoma is poor and depends on patient's age, tumor location, size, histological grade and extent of tumor progression (7). The reported outcome in children is also poor but our case and two other cases of congenital cerebral angiosarcoma reported by Suzuki et al ( 9) and Kirk et al (10) had good prognosis even without any other treatment except for wide surgical excision.…”

Section: Discussionmentioning

confidence: 53%

“…Angiosarcomas have been reported as aggressive tumors with poor prognosis (7), however the prognosis depends on factors such as tumor size, grade, and patients' age. In this article we report a rare occurrence of a congenital angiosarcoma of scalp with dural and skull bone invasion and a favorable outcome.…”

Section: Introductionmentioning

confidence: 99%

Congenital Angiosarcoma, a Case Report and Review of Literature

et al. 2020

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Introduction: Angiosarcoma, originating from vessels, constitutes about 0.2% to 0.3% of all pediatric soft tissue sarcomas. Prognosis of angiosarcoma is poor and depends on patient’s age, tumor location, size, histological grade and extent of tumor progression. Case Presentation: We report a rare case of a congenital angiosarcoma of scalp with dural and skull bone invasion in a one-month-old boy. His mother noticed the mass 2 days after birth as a very small insignificant nodule, but it grew rapidly afterward. Conclusions: The treatment consisted of only a wide surgical resection. After 15 months there was no sign of local recurrence or metastasis was noticeable and the tumor showed favorable outcome. This case indicates the possibility of a better clinical behavior in congenital angiosarcoma.

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Clinical outcomes of angiosarcoma: a single institution experience

Cited by 5 publications

References 18 publications

Primary cardiac sarcomas: A clinicopathologic study in a single institution with 25 years of experience with an emphasis on MDM2 expression and adjuvant therapy for prognosis

Primary cardiac sarcomas: A clinicopathologic study in a single institution with 25 years of experience with an emphasis on MDM2 expression and adjuvant therapy for prognosis

Cutaneous Angiosarcoma of the Head and Neck—A Retrospective Analysis of 47 Patients

Congenital Angiosarcoma, a Case Report and Review of Literature

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