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Primary cardiac intimal sarcoma masquerading as mitral stenosis.

ABSTRACT: Intimal sarcomas are a very rare medical entity and usually arise from the pulmonary artery or the thoracic aorta. Sarcomas should be considered in the differential diagnosis in patients with suspected mitral valve disease. Echocardiography should be performed as early as possible to establish a prompt diagnosis and management.

SUBMITTER: Spartalis M

PROVIDER: S-EPMC5538049 | biostudies-other | 2017 Aug

REPOSITORIES: biostudies-other

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An Unusual Cause of Functional Mitral Stenosis: A Left Atrial Intimal Sarcoma.

Project description:Primary cardiac tumors are rare, with an incidence of <0.1% in postmortem series; sarcomas comprise 75% of these. Cardiac sarcomas may be life-threatening at the time of presentation. We describe a left atrial intimal sarcoma presenting with constitutional symptoms, obstructive shock, and systemic emboli, and treated with proton beam therapy. (Level of Difficulty: Intermediate.).

| S-EPMC8311188 | biostudies-literature

The Insidious Cardiac Tumor: A Primary Left Atrium Intimal Cardiac Sarcoma in a Young Patient.

Project description:This case presentation discusses a rare cardiac malignancy initially thought to be a benign tumor. A 36-year-old presented with syncope, dyspnea, Computed Tomography Pulmonary angiography study obtained was negative for pulmonary emboli but revealed a left atrial mass. A transesophageal echocardiogram (TEE) confirmed a mass with multiple lobes and a broad base attached to the septum, encroaching into the right atrium, aortic root wall, base of the anterior mitral leaflet flowing to the mitral orifice in diastole also obstructing the right pulmonary vein. Despite a quick diagnosis and plan to begin treatment, the patient rapidly declined owing to the extent and aggressive nature of this cardiac malignancy. This case reports the insidious nature of these tumors as well as how challenging and life threatening they are at the time of their clinical manifestation.

| S-EPMC6476112 | biostudies-literature

Unusual cause of heart failure: Mitral stenosis and pulmonary venous obstructions caused by the direct invasion of primary cardiac sarcoma.

Project description:Cardiac tumor can produce a variety of symptoms and clinical findings depending on the location, size, and histologic type. It may cause heart failure usually by interfering with intracardiac blood flows associated with the mass effects. Here, we report an extremely rare case of heart failure caused by primary cardiac sarcoma with a review of the literature. The cause of heart failure was moderate to severe mitral steno-insufficiency due to the direct tumoral invasion of mitral valve apparatus combined with obstruction of the pulmonary veins.

| S-EPMC6269419 | biostudies-literature

Primary Cardiac Sarcoma Involving the Mitral Valve, an Insidious Form of Heart Failure.

Project description: Not available

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A case report of primary cardiac intimal sarcoma presenting with atrial fibrillation and a left atrial mass.

Project description:BackgroundIntimal sarcoma is an exceedingly rare type of primary cardiac tumour. It is characterized by poorly differentiated spindle-shaped cells that can mimic smooth muscle and is strongly associated with MDM2 genetic amplification. Owing to its rarity and non-distinctive histological features, diagnosis remains a significant challenge.Case summaryIn this case report, we describe a case of primary cardiac intimal sarcoma in a 37-year-old woman who presented with atrial fibrillation (AF) and a left atrial mass. Despite having a histological sample from an excised left atrial mass, the diagnosis was not made until she presented with back pain secondary to metastatic disease to the spine.DiscussionPrimary cardiac intimal sarcoma is an extremely rare diagnosis. The mainstay management of intimal cardiac sarcoma is aggressive surgical resection. Unfortunately, the prognosis of cardiac sarcomas remains very poor, with a mean survival between 3 months and 1 year. This case of cardiac intimal sarcoma highlights the difficulty in establishing a diagnosis, particularly given the unusual presentation of AF.

| S-EPMC8633751 | biostudies-literature

Stepwise Use of Multimodality Imaging in a Rare Cardiac Intimal Sarcoma.

Project description: Not available

| S-EPMC10635890 | biostudies-literature

Problems and countermeasures for surgical resection of primary pulmonary artery intimal sarcoma.

Project description:BackgroundPulmonary artery intimal sarcoma (PAIS) is a rare but aggressive malignancy. This study clarified the problems and countermeasures of surgical treatment by examining surgical cases of PAIS.MethodsBetween January 2007 and October 2020, 10 patients with PAIS who underwent surgery at our hospital were retrospectively examined.ResultsThe surgical procedure that aimed at complete resection was pulmonary resection only (three cases), along with pulmonary artery vascular replacement (six cases) and pulmonary endarterectomy (PE) (one case). The positive rate of vascular stumps was 7/10. In all cases, chest computed tomography scan showed positive margins of ≤20 mm between the tumor and surgical dissection (6/6). In addition, the distance between the location of the tumor on computed tomography and the dissection line during surgery needed to be at least 20 mm (2/3). However, even at a distance of 25 mm, one case with a positive margin was observed. Postoperative recurrence was 8/9 cases, and the median recurrence period was as short as 10 months (range, 3-19 months). Postoperative treatment was required in 7/9 cases (operation/chemotherapy/radiotherapy/chemoradiotherapy/heavy ion radiotherapy =1/2/2/1/1). The median survival was 15 months (range, 0.5-36 months).ConclusionsExtended surgery should be performed as much as possible, with a distance of at least 20 mm between the location of the tumor on computed tomography scan and the incision line during surgery. The median postoperative recurrence period was as short as 10 months. Therefore, intensive care for intrathoracic recurrence follow-up is required for 1 year after surgery.

| S-EPMC10007893 | biostudies-literature

Primary intimal sarcoma of the left atrium presenting with constitutional symptoms.

Project description:Intimal (spindle-cell) sarcomas are exceptionally rare and are highly aggressive cardiac tumors. The authors describe a case of a 43-year-old female, presenting with a 3-month history of constitutional symptoms with fever, night sweats, anorexia and weight loss, associated with productive cough and pleural effusion that was admitted with clinical suspicion of pulmonary tuberculosis. The patient developed sudden acute heart failure symptoms during hospitalization, leading to mechanical ventilation. Computed tomography scan with contrast showed a cardiac tumor filling the left atrium causing compression of pulmonary veins. Surgical resection was performed and histologic examination revealed an intimal sarcoma. Although commenced on adjuvant chemotherapy, local tumor recurrence occurred with pericardium invasion. The patient died within 4 months of initial diagnosis. This report aims to describe an unusual presentation of this rare disease entity, and to discuss its highly aggressive clinical course.

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Intimal Sarcoma: An Extremely Rare Case of a Left Atrial Tumor with Partial Obstruction of the Mitral Orifice.

Project description: Not available

| S-EPMC8071820 | biostudies-literature

Mitral and tricuspid stenosis caused by light chain cardiac amyloid deposition.

Project description:Cardiac amyloidosis results in an infiltrative restrictive cardiomyopathy, with a number of characteristic features: biventricular hypertrophy, abnormal myocardial global longitudinal strain with relative apical sparing, biatrial dilation, and small pericardial effusion along with conduction abnormalities. Amyloid deposits leading to hemodynamically significant valvular heart disease are very rare. We describe a rare case of concomitant moderately severe tricuspid and mitral valve stenosis because of ongoing amyloid deposition in a patient with progressive multiple myeloma and fat pad biopsy-proven light chain amyloidosis. Worsening infiltrative cardiomyopathy and valvulopathy despite evidence-based chemotherapy and heart failure pharmacotherapy led to end-stage disease and death. Valvular involvement in cardiac amyloidosis requires early recognition of the underlying disease condition to guide directed medical therapy and prevent its progression. In this instance, valvuloplasty or valve replacement is not a viable option.

| S-EPMC7261535 | biostudies-literature

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