Zara Shah  ( Section of Neurosurgery, Department of Surgery, Aga Khan University Hospital, Karachi )
Mishkat Shehzad  ( Section of Neurosurgery, Department of Surgery, Aga Khan University, Karachi, Pakistan. )
Faizan Saeed  ( Section of Neurosurgery, Department of Surgery, Aga Khan University, Karachi, Pakistan. )
Saqib Kamran Bakhshi  ( Section of Neurosurgery, Department of Surgery, Aga Khan University Hospital, Karachi, Pakistan. )
Muhammad Shahzad Shamim  ( Section of Neurosurgery, Department of Surgery, Aga Khan University Hospital, Karachi. )

Primary cardiac neoplasms are very rarely encountered, and the most common type of malignant tumours occurring primarily in the heart are sarcomas. These carry a lethal prognosis on account of their late presentation and aggressive spread. They have a high propensity for cerebral metastases. Such cases are exceptionally uncommon, and till date only a few examples are available. Currently, there is no standard protocol for the management of primary cardiac sarcoma with brain metastases.

 

Keywords: Brain metastasis, cardiac sarcoma, craniotomy

 

DOI: https://doi.org/10.47391/JPMA.23-29

 

Introduction

 

Primary neoplasms of the heart are a rare occurrence, while many countries lack data, incidence in the United States of America ranges from 1.38 to 30 per 100,000 people per year, out of which 80% are benign and 20% are malignant.¹ Cardiac sarcomas are the most common type of primary malignant tumours of the heart and carry a poor prognosis due to delayed presentation, lack of awareness and an advanced tumour stage at the time of presentation.² Of all primary malignant cardiac tumours, patients with cardiac sarcomas are the most at risk for developing distant metastases and tend to metastasize to the lungs, liver and mediastinum.³ Cerebral metastasis, however, is rare and has shown to have the worst prognosis.⁴ Although many modes of management have been recommended, a standardized treatment protocol has not been established for patients presenting with cardiac sarcomas metastasized to the brain.

 

Review of Literature

 

Due to rarity of brain metastasis from primary cardiac sarcomas, the management remains a dilemma.

 

 

 

(Figure-1,2) We conducted a thorough search on PubMed and Google Scholar for evidence on primary cardiac sarcomas metastasizing to brain. Bishop et al. reported the largest cohort of patients diagnosed with cardiac angiosarcoma (CA) and metastasis to brain. Although being the largest reported cohort, the authors reported only 26 cases which shows the rarity of the diagnosis. The median age of the patients at diagnosis was 37 years and the median time from CA diagnosis to the detection of brain metastasis was 11 months. In 13 (50%) patients, CA metastasized to the brain when the systematic disease was progressing whereas in 9 patients (35%), brain metastasis developed when the systematic disease was stable. The median number of brain metastasis at diagnosis was 3 (IQR1-7). Eighteen (69%) patients were symptomatic at brain metastasis diagnosis with symptoms including headaches in 12(46%) , nausea or vomiting (9,35%), neurological (7,27%) and altered mental status (3,12%). Twenty-three (88%) patients had intracranial haemorrhage (IH) during the course of the disease including 21 (81%) at the time of diagnosis.  Eighteen (69%) patients had treatable brain metastasis at the time of the diagnosis and were managed with whole brain radiotherapy (RT) (6,33%), surgery (4,22%), stereotactic radiosurgery (4,22%), surgery and RT (2,11%) and systematic therapy (2,11%). Nearly all of the patients (23, 88) died from the disease and most patients died of a terminal haemorrhage (TH) (13, 57%) which was defined as an IH that caused death or resulted in a transfer to hospice care. However, 9 (39%) patients died due to systematic progression whereas 1 (4%) died to due intracranial progression. The authors have recommended surveillance brain imaging and prompt local therapy once brain metastasis is diagnosed to reduce the risk of TH.⁵

In another case series of 39 patients, Siontis et al. highlighted patterns of metastatic disease, histologic subtypes, treatment modalities, and overall survival of these patients. Twelve (31%) of the 39 patients developed brain metastases and the median time from diagnosis of the cardiac sarcoma to the discovery of brain metastases was 8.5 months. High grade undifferentiated pleomorphic sarcoma in 6 (50%) was the most common histological variant whereas other histological types included angiosarcoma in 3(25%), leiomyosarcoma in 2(17%), and fibrosarcoma in 1(8%). Left-sided cardiac tumors were more commonly associated with brain metastases in (9/12, p=0.01) compared to right-sided and pericardial tumours. All patients presented with neurologic symptoms. Median overall survival (OS) for the patients was 13.6 months with a post-brain metastases median survival of 5.6 months. Four (33%) of the 12 patients who had received RT with a median post-brain metastases survival of 8 months, 4 (33%) patients underwent surgical resection followed by radiation with a post-brain metastases survival of 5.5 months and only 1 (8%) patient underwent metastasectomy alone with a post-brain metastases survival of 12 months. The authors concluded that due to the increased high risk of brain metastases of cardiac sarcomas, patients should be routinely screened through brain imaging at diagnosis and follow-up.⁶

Lin et al. reported a single case of primary CA with  brain metastasis. A 63-year-old male presented with right arm weakness and brain imaging revealing a 2 cm haemorrhagic mass in the left frontal lobe. Further computed tomography imaging demonstrated multiple pulmonary nodules, a right atrial appendage mass, and a right T10 pathologic transverse process fracture. The patient underwent a left frontal craniotomy for tumour resection after which the pathological evaluation was significant for metastatic angiosarcoma. The right atrial mass was assumed to be the originating source of the angiosarcoma. The patient then underwent seven cycles of chemotherapy for the systematic disease. At 10 months, repeat magnetic resonance imaging of the brain revealed new sub-centimeter brain metastasis in the right frontal lobe. However, the patient decided to continue to monitor the new lesion while continuing chemotherapy and was last reported to be alive at 12 months after surgery.⁷

In another case report, Sun et al. reported a case of primary cardiac pleomorphic undifferentiated sarcoma with right hemisphere cerebral infarction and right temporo-parietal metastatic sarcoma. A 33-year-old male underwent a surgical excision of a cardiac left atrial mass which was diagnosed as undifferentiated pleomorphic sarcoma. Post-operatively the patient developed altered mental status and left sided weakness. Brain imaging demonstrated right cerebral hemisphere infarction for which he underwent decompressive craniectomy. Four months post decompressive craniectomy, the patient presented with a right temporo-parietal occupying lesion which was resected and diagnosed as malignant high differentiation soft tissue sarcoma. The patient however, refused any palliative radiotherapy or chemotherapy throughout the course of the disease. The patient was last reported as still alive 12 months after the diagnosis; however, he was suffering from global aphasia and left-side limb paralysis.³

 

Conclusion

 

Metastases of primary cardiac sarcomas to the brain is rare. Current literature mostly reports cases with a lack of studies reporting large cohorts. However, existing data suggests the risk of developing brain metastases in patients with primary cardiac sarcomas is increasing, which necessitates routine brain imaging at diagnosis and follow up to ensure early diagnosis and improved disease outcomes.

 

References

 

1.      Poterucha TJ, Kochav J, O'Connor DS, Rosner GF. Cardiac Tumors: Clinical Presentation, Diagnosis, and Management. Curr Treat Options Oncol. 2019;20:66.

2.      Shanmugam G. Primary cardiac sarcoma. Eur J Cardiothorac Surg. 2006;29:925-32.

3.      Sun YP, Wang X, Gao YS, Zhao S, Bai Y. Primary cardiac sarcoma complicated with cerebral infarction and brain metastasis: A case report and literature review. Cancer Biomark. 2017;21:247-50.

4.      Guan T, Wei Q, Tang Y, Zhao H, Lu Z, Feng W, et al. Metastatic patterns and prognosis of patients with primary malignant cardiac tumor. Front Cardiovasc Med. 2022;9:1009765.

5.      Bishop AJ, Zheng J, Subramaniam A, Ghia AJ, Wang C, McGovern SL, et al. Cardiac Angiosarcomas: Risk of Brain Metastasis and Hemorrhage Warrants Frequent Surveillance Imaging and Early Intervention. Am J Clin Oncol. 2022;45:258-63.

6.      Siontis BL, Zhao L, Leja M, McHugh JB, Shango MM, Baker LH, et al. Primary Cardiac Sarcoma: A Rare, Aggressive Malignancy with a High Propensity for Brain Metastases. Sarcoma. 2019;2019:1960593.

7.      Lin CT, Ducis K, Tucker S, Tranmer B. Metastatic Cardiac Angiosarcoma to the Lung, Spine, and Brain: A Case Report and Review of the Literature. World Neurosurg. 2017;107:1049 e9- e12.