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

Autor: Haeyon Cho, In‐Hye Song, Uiree Jo, Ji‐Seon Jeong, Hyun Jung Koo, Dong Hyun Yang, Sung‐Ho Jung, Joon Seon Song, Kyung‐Ja Cho
Jazyk: angličtina
Rok vydání: 2023
Předmět:
Zdroj: Cancer Medicine, Vol 12, Iss 16, Pp 16815-16828 (2023)
Druh dokumentu: article
ISSN: 2045-7634
DOI: 10.1002/cam4.6303
Popis: Abstract Background Primary 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). Methods We 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. Results Forty‐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
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