Commentary: Primary cardiac sarcoma—Systemic disease requires systemic therapy

Ravi, Vinod; Reardon, Michael J.

doi:10.1016/j.jtcvs.2019.10.087

Cited by 5 publications

(5 citation statements)

References 5 publications

(3 reference statements)

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“…Despite this, the combination of surgery, chemo- and radiation-therapy has been reported with more than twice the median survival, if compared to surgery alone. 13 , 14 …”

Section: Discussionmentioning

confidence: 99%

Primary cardiac sarcoma: a case report of a therapeutic challenge

Andrei

Scheggi

Stefàno

et al. 2020

European Heart Journal - Case Reports

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Background Primary cardiac sarcomas are very rare and the prognosis is poor both because the diagnosis is typically made at an advanced stage of the disease and because data are insufficient to identify a standard treatment. Surgical resection is the cornerstone of therapy with the need to develop new therapeutic strategies. Case summary We present a case of a young man admitted to the emergency department due to worsening dyspnoea. A left-sided sarcoma was diagnosed and treated with surgery, chemo- and radiation therapy, and subsequently with heart transplant for local recurrence of the disease. Endomyocardial biopsy made during the routine follow-up period was complicated by pericardial tamponade and cardiogenic shock and the patient was managed with veno-arterial extracorporeal membrane oxygenation, until recovery of left ventricular function (left ventricular ejection fraction of 55%). After 1 year a kidney transplant was performed. After 42 months from diagnosis, the patient is in good general condition. Discussion Primary cardiac sarcomas are treated with surgery to reach R0 (free resection margins) and with chemo- and radiation therapy with adjuvant purposes. Auto-transplantation is also performed, while conventional heart transplant must be customized on an individual basis, after excluding metastases. A multidisciplinary assessment should be performed and the single patient treated with a personalized approach, in relation to his performance status, location of the mass, and stage of the disease.

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“…Despite this, the combination of surgery, chemo- and radiation-therapy has been reported with more than twice the median survival, if compared to surgery alone. 13 , 14 …”

Section: Discussionmentioning

confidence: 99%

Primary cardiac sarcoma: a case report of a therapeutic challenge

Andrei

Scheggi

Stefàno

et al. 2020

European Heart Journal - Case Reports

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“…This regimen is continued, as tolerated, for maximal response around the sixth cycle at which point surgical resection is considered after review by a multidisciplinary cardiac tumor review board. 1,11 Adjuvant systemic therapy is often recommended following surgery, even if negative margins are achieved. For patients with metastatic disease, similar chemotherapy regimens are recommended, and local therapies, often consisting of palliative RT, are used as needed.…”

Section: Methodsmentioning

confidence: 99%

“…Therefore, for patients with localized disease, our current strategy includes initiating neoadjuvant systemic therapy using doxorubicin (60 to 75 mg/m 2 ) and ifosfamide (10 mg/m 2 ) chemotherapy with restaging studies every 2 cycles to evaluate response. This regimen is continued, as tolerated, for maximal response around the sixth cycle at which point surgical resection is considered after review by a multidisciplinary cardiac tumor review board 1,11. Adjuvant systemic therapy is often recommended following surgery, even if negative margins are achieved.…”

Section: Methodsmentioning

confidence: 99%

Cardiac Angiosarcomas

Bishop¹,

Zheng

Subramaniam

et al. 2022

American Journal of Clinical Oncology

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Purpose: We evaluated a cohort of patients with cardiac angiosarcomas (CA) who developed brain metastases (BM) to define outcomes and intracranial hemorrhage (IH) risk. Methods: We reviewed 26 consecutive patients with BM treated between 1988 and 2020 identified from a departmental CA (n=103) database. Causes of death were recorded, and a terminal hemorrhage (TH) was defined as an IH that caused death or prompted a transfer to hospice. Results: The prevalence of BM was 25% (n=26/103). A total of 23 patients (88%) had IH, including 21 (81%) at initial BM diagnosis, of which 18 (86%) required hospitalization. The median platelet count at the time of IH was 235k (interquartile range, 108 to 338k). Nearly all patients died of disease (n=23, 88%) and most patients died from TH (n=13, 57%). TH occurred at BM presentation in 6 (46%) patients, whereas 3 (23%) had TH from known but untreated lesions, 2 (15%) had continued uncontrolled IH during radiation therapy, and 2 (15%) from new BM. Platelet count <50k was not associated with TH (P=0.25). Subsequent IH occurred in 9 patients (35%), and importantly, no patients who completed radiation therapy (n=10) for BM died from TH. Conclusion: Patients with CA frequently develop BM, and the risk of IH is high, resulting in an alarming rate of TH despite normal platelet counts. Therefore, early diagnosis and intervention are warranted. We recommend surveillance brain imaging, and importantly, once BM is detected, prompt local therapy is warranted to try and mitigate the risk of TH.

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“…1,5,11,14,23 Finally, surgeons and heart tumor teams should take to heart the complexity of primary cardiac sarcoma and understand that it is a systemic disease requiring a systemic approach. 24,25 17…”

Section: Don't Let Your Ego Cause Harmmentioning

confidence: 99%

The 7 Pillars of Primary Cardiac Sarcoma Treatment

Atkins,

Ravi,

Reardon

2024

Innovations�(Phila)

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Commentary: Primary cardiac sarcoma—Systemic disease requires systemic therapy

Abstract: Primary cardiac sarcoma is a rare and deadly disease. Surgical resection for appropriate tumors is first-line therapy. Adjuvant chemotherapy increases survival in this usually systemic disease.

Cited by 5 publications

References 5 publications

Primary cardiac sarcoma: a case report of a therapeutic challenge

Primary cardiac sarcoma: a case report of a therapeutic challenge

Cardiac Angiosarcomas

The 7 Pillars of Primary Cardiac Sarcoma Treatment

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