Isolated cardiac metastasis from sacral chordoma
Y Oda1, R Takada, K Koitabashi
1Department of Internal Medicine, Tokyo Koseinenkin Hospital, Japan.
Insights
A rare cardiac metastasis from a chordoma caused right heart failure in a 64-year-old woman. Despite palliative interventions, treatment remains challenging due to extensive tumor infiltration.
Area of Science:
- Cardiovascular Pathology
- Oncology
- Surgical Oncology
Background:
- Chordomas are rare bone tumors, with cardiac metastasis being exceptionally uncommon.
- Right heart failure can be precipitated by cardiac masses obstructing outflow or infiltrating myocardial tissue.
Observation:
- A 64-year-old female presented with symptoms of right heart failure and pericardial effusion.
- Biopsy confirmed cardiac metastasis from a primary chordoma, identified by matching tissue from four years prior.
- Immunohistochemical staining aided in confirming the diagnosis of cardiac chordoma metastasis.
Findings:
- The cardiac tumor infiltrated the right ventricle free wall, causing significant outflow obstruction.
- Palliative surgical attempts, including tumor resection and a right atrium-to-pulmonary artery shunt, were unsuccessful due to tumor extent and prosthesis failure.
- Current management involves palliative chemotherapy, as definitive treatment options are limited.
Implications:
- This case highlights the extreme rarity of cardiac chordoma metastasis and the diagnostic challenges it presents.
- It underscores the limited therapeutic options and poor prognosis associated with advanced cardiac chordoma.
- Further research into novel treatment strategies for rare cardiac tumors is warranted.
Abstract:
A 64-year-old woman presented with right heart failure caused by a cardiac tumor centered in the free wall of the right ventricle, accompanied by pericardial effusion. A match between the biopsy specimen and tissue removed 4 years earlier resulted in the diagnosis of a cardiac metastasis from a chordoma. Immunohistochemical staining was also useful in establishing the diagnosis. To alleviate the right ventricular outflow obstruction, a palliative operation was planned, resecting the tumor and performing a right ventriculoplasty, which was cancelled due to the extent of infiltration of the tumor, and instead a right atrium to pulmonary artery shunt was attempted using a vascular prosthesis, only to fail due to an inability to maintain blood flow through the prosthesis. Presently there are no definitive treatment options available, and some palliative chemotherapy is being performed. Single cardiac metastases from a chordoma are extremely rare.


