Primary cardiac angiosarcoma masquerading as intracardiac thrombus.

A 49-year-old man with a recent history of atrial tachycardia and intracardiac thrombus presented to the emergency department with melena and cardiac tamponade. Physical examination was notable for a vascular mass at the right lower gingival sulcus and a right chest wall nodule. Enteroscopy revealed a target lesion with friable ulcer in the gastric body. Cardiac MRI revealed a large right atrial mass, previously thought to represent thrombus. The patient was ultimately diagnosed with primary cardiac angiosarcoma (PCAS) by histopathology of gingival, gastric and subcutaneous lesions. This case illustrates the significant morbidity and mortality resulting from aggressive local invasion and growth of PCAS, as well as the challenge of differentiating between primary thrombosis and vascular malignancy. Misdiagnosis of this elusive clinical entity may be costly, potentially resulting in delay of intervention and adverse effects of alternate therapies such as anticoagulation.

[Diagnostic difficulties and legal analysis of potential claims to occupational medicine specialists based on a case of unrecognizable cardiac angiosarcoma]. / Trudnosci diagnostyczne i analiza prawna potencjalnych roszczen wobec lekarza medycyny pracy na przykladzie nierozpoznanego naczyniakomiesaka serca.

The article presents a case of sudden death of a 56-year-old woman at the workplace, caused by a very rare primary cardiac tumor. The patient's family reported a crime to the prosecutor's office suggesting participation of third parties in causing the death or malpractice in physical examinations before the death. A review of clinical data concerning cardiac angiosarcoma, available in electronic databases (e.g., Web of Science, PubMed), was presented, which could be useful in the practice of occupational medicine specialists. A legal analysis of potential claims to occupational medicine specialist in the case of failure to recognize primary cardiac tumors was also included in the article. Med Pr. 2020;71(6):757-64.

Intracardiac metastasis from head and neck squamous cell carcinoma.

Cardiac metastasis from head and neck cancer is an extremely rare and devastating condition with an abysmal prognosis. Most of our knowledge about this condition is from case reports and series. We present a case of squamous cell carcinoma of the tonsils that was complicated by embolic stroke and critical limb ischemia that were found to be secondary to intracardiac metastasis. We believe that this condition is under-reported; hence, we conducted a thorough review of the literature to highlight the characteristics and previous therapeutic experiences with various presentations of cardiac metastasis from head and neck carcinoma. Clinicians are encouraged to report their experience with evaluating and managing this type of metastasis.

A case of surgically resected cardiac rhabdomyoma with progressive left ventricular outflow tract obstruction.

Cardiac rhabdomyoma is the most prevalent cardiac tumors in the pediatric population, in close association with tuberous sclerosis complex. It is usually detected antenatally or postnatally by echocardiography. Clinical presentations depend greatly on the size and position of the tumor mass. Interestingly, rhabdomyoma has a propensity to regress spontaneously and is not usually operated upon, unless the patient becomes hemodynamically compromised. Herein, we report an unusual case of surgically treated cardiac rhabdomyoma in a baby boy presented at birth with a progressive enlarging intraventricular mass, complicated with left ventricular outflow tract obstruction 7 weeks later. Histopathological examination of the intracardiac mass revealed sheets of tumor cells with spider-like morphology (known as "spider cells"), confirmed the diagnosis of rhabdomyoma. Close disease monitoring of patient's hemodynamic status in a newly diagnosed cardiac rhabdomyoma is inevitable as the tumor, although rare, may progress.

Cardiac Malignant Peripheral Nerve Sheath Tumor Accessed By 18F-FDG PET/CT.

This is a case report of a patient presenting an extremely rare cardiac malignancy: malignant peripheral nerve sheath tumour. The 18F-fluorodeoxyglucosis positron emission tomography associated to computed tomography (18F-FDG PET/CT) accesses the tumour anatomy and metabolic activity, thereby making it possible to characterize a malignant neoplasm noninvasively. The diagnostic approach with 18F-FDG PET/CT spared the heart from a likely futile invasive procedure when detecting a distant metastasis and changed the biopsy site and therapeutic planning.

Clinical Treatment of Pediatric Primary Cardiac Tumors: A Single-Institute 12-Year Experience.

BACKGROUND: Primary cardiac tumors in children are extremely rare. This study aimed to analyze clinical treatment and follow-up of pediatric primary cardiac tumors. PATIENTS AND METHODS: We performed a retrospective analysis by searching the medical records of 75 patients diagnosed with pediatric primary cardiac tumors from June 2005 to August 2017 in our institution. We followed operative patients every half year in the first postoperative year and then at least every year. If the patients had no serious symptoms or hemodynamic changes, they received nonoperative management and were followed regularly every year. RESULTS: Nineteen patients underwent surgery at our department for serious symptoms and critical hemodynamic changes. Four patients had postoperative complications. Two died of low cardiac output syndrome and arrhythmia after surgery. One patient with myxomas had tumor recurrence and one had been found of another rhabdomyoma after surgery. The other 14 patients recovered well. Fifty-six patients had nonoperative management. Four were lost in follow-up. Two patients with malignant tumors died of unknown causes after discharge. The remaining patients had no severe symptoms or tumor growth during follow-up. CONCLUSIONS: Clinical treatment of pediatric primary cardiac tumors should be performed individually. Most pediatric primary cardiac tumors are benign, and spontaneous regression is possible, especially for rhabdomyomas. The principle purpose of surgical treatment is to restore normal hemodynamics and protect important structures and cardiac tissue.

Isolated metastasis of hepatocellular carcinoma in the right ventricle.

BACKGROUND: Hepatocellular carcinoma (HCC) with right ventricle metastasis without inferior vena cava and right atrium involvement is very rare and the prognosis of HCC with RV metastasis is generally poor. The mass in the cardiac chamber may lead to lethal instability of hemodynamics, however, the initial symptom is probably non-specific, which means that diagnosis timely becomes even harder. CASE PRESENTATION: We present a 63-year-old male with isolated metastasis of HCC in the right ventricle which caused inflow obstruction. Moreover, we reviewed a series of studies of isolated metastasis of hepatocellular carcinoma between 1980 and 2018, and summarized the relative outcomes. CONCLUSIONS: Isolated metastasis of hepatocellular carcinoma in the right ventricle is extraordinarily rare. It may damage cardiac structure and broke hemodynamic balance. Multimodality imaging plays an important in accurate pre-operation assessment. Nowadays, palliative treatments could relieve fatal symptoms to some degree, however, standard treatment has not been well established.

Somatic PRKAR1A mutation in sporadic atrial myxoma with cerebral parenchymal metastases: a case report.

BACKGROUND: Atrial myxomas are generally considered benign neoplasms. The majority of tumors are sporadic and less than 10% are associated with an autosomal dominant condition known as the Carney complex, which is most often caused by germline mutation in the gene PRKAR1A. Whether this gene plays a role in the development of sporadic myxomas has been an area of debate, although recent studies have suggested that some fraction of sporadic tumors also carry mutations in PRKARIA. Extra-cardiac complications of atrial myxoma include dissemination of tumor to the brain; however, the dissemination of viable invasive tumor cells is exceedingly rare. CASE PRESENTATION: We present here a 48-year-old white woman who developed multiple intracranial hemorrhagic lesions secondary to tumor embolism that progressed to 'false' aneurysm formation and invasion through the vascular wall into brain parenchyma 7 months after resection of an atrial myxoma. Whole exome sequencing of her tumor revealed multiple mutations in PRKAR1A not found in her germline deoxyribonucleic acid (DNA), suggesting that the myxoma in this patient was sporadic. CONCLUSIONS: Our patient illustrates that mutations in PRKAR1A may be found in sporadic lesions. Whether the presence of this mutation affects the clinical behavior of sporadic tumors and increases risk for metastasis is not clear. Regardless, the protein kinase A pathway which is regulated by PRKAR1A represents a possible target for treatment in patients with metastatic cardiac myxomas harboring mutations in the PRKARIA gene.

IgG4-related Disease Involving the Cardiovascular System: An Intracardiac Mass and a Mass Lesion Surrounding a Coronary Artery.

A 61-year-old Japanese man with IgG4-related autoimmune pancreatitis developed a mass in the right atrium (RA) and a mass lesion surrounding the left anterior descending coronary artery. We performed an intracardiac echo catheter-guided percutaneous biopsy of the RA mass, and histologically diagnosed it as IgG4-related disease. Oral corticosteroid therapy gradually downsized the mass lesions. We encountered a very rare case with mass lesions in the cardiovascular system of the IgG4-related disease that were able to be diagnosed using an intracardiac echo-guided biopsy.

Image of the month: Ventricular myxoma mimicking hypertrophic cardiomyopathy.

A 5-year-old boy with an incidentally detected cardiac murmur was referred for evaluation. Tall R waves were noted in the electrocardiogram in leads V3 and V4. Transthoracic echocardiography suggested asymmetric septal hypertrophy with diffuse thickening of the inter-ventricular septum with normal thickness of the posterior left ventricular wall. Upon closer interrogation, a masquerading sessile cardiac mass was identified adherent to the left ventricular side of the inter-ventricular septum which appeared to contract with each cardiac cycle, mimicking hypertrophic cardiomyopathy.