Primary cardiac sarcoma

Choi

et al. 2009

Circ J

Circ J 2009; 73: 779 -782 rimary cardiac malignant tumors are very rare and account for a quarter of malignant cardiac tumors. [1][2][3] Sarcomas constitute almost all (95%) of the primary cardiac malignancy. 3,4 We report herein a middle-aged man who presented with dyspnea and had a discordantly huge leiomyosarcoma in the right ventricle (RV) that was elongated into the right atrium (RA) and the main pulmonary artery (MPA) through the tricuspid and pulmonary valves, respectively, with impending obstruction of inflow and outflow tracts of the RV. Case ReportA 45-year-old man visited the emergency room because of progressive shortness of breath for 1 month. He had no medical history and had been doing well without any constitutional symptoms. His blood pressure was 110/60 mmHg, pulse rate 94 beats/min, body temperature 36.7°C, and he had a respiration rate of 22 breaths/min. A physical examination revealed a healthy general appearance and systolic murmur (GIII/GVI) along the left upper parasternal border. An electrocardiography showed a heart rate of 96 beats/min with a S1Q3T3 sign, T wave inversion on the right precordial leads, and an incomplete right bundle branch block implying an acute pressure and volume overload of the RV (Figure 1). On laboratory study, the level of serum pro-B type natriuretic peptide was elevated up to 3,783 pg/ml. Other cardiac enzymes were within normal ranges. Mild thrombocytopenia (126×10 9 /L), prolonged prothrombin time (13.5 s), elevated alanine aminotransferase (57 U/L), and small ascites were also found.Transthoracic and transesophageal echocardiography (TTE/TEE) showed an 8×4 cm-sized, huge tumor that fully filled the RV and extended into the MPA through the RV outflow tract and into the RA through the tricuspid valve (TV; Figures 2A,C). The tumor had a broad-based stalk and was attached to the RV apex and interventricular septum (IVS). A Color Doppler study showed the near obstruction of inflow and outflow tracts of the RV caused by the huge tumor (Figures 2B,D). The apical anterior wall of the RV was thickened and showed partial indentation with increased echogenicity and a small amount of pericardial effusion around the RV apex. The left ventricle showed a D-shape becaues of the compression effect of the bulky tumor shifting the IVS to the left side. A cardiac magnetic resonance imaging (CMR) with gadolinium enhancement disclosed the huge RV tumor with a low-signal intensity similar to a myocardium on a T1-weighted image, and a mild highsignal intensity on a T2-weighted image (Figures 3A-C). The tumor showed heterogeneous enhancement at the delayed phase, especially at the proximal portion of the tumor attached to the RV apex ( Figure 3D).The patient underwent urgent surgery on the third hospital day to relieve the obstruction. During the cardiopulmonary bypass, the RV was not collapsed owing to the large intracavitory mass. The smooth-surfaced hard tumor originated from the RV apex and IVS, and protruded into the RA and MPA. The tumor was markedly adhered to the entire...

Section: Discussionmentioning

confidence: 99%

“…[3][4][5][6] Pleomorphic leiomyosarcoma is a morphologic variant of leiomyosarcoma and its incidence is not known because of its rarity.…”

Section: Discussionmentioning

confidence: 99%

mentioning

confidence: 99%

See 1 more Smart Citation

Huge Primary Pleomorphic Leiomyosarcoma in the Right Ventricle With Impending Obstruction of Both Inflow and Outflow Tracts

Choi

et al. 2009

Circ J

The Annals of Thoracic Surgery

“…Against transplantation was the history of previous lymphoma (eventually predisposing to de novo lymphoma induced by malignancy immunosuppression) and the poor results usually obtained with this treatment for this type of patients, although case series are very limited. Mean survival for patients with cardiac sarcoma has been established between 9 and 11 months [1]. Our patient has been monitored for 7 years until now, without evidence of recurrence of any type and without cardiac rejection, which certainly is an unusually good result.…”

mentioning

confidence: 94%

“…Despite these poor results, transplantation remains the only option for selected patients with nonresectable tumors but localized disease [5]. Adjuvant chemotherapy or radiotherapy, or both, do not seem to improve survival and are not useful [1,5] and were not used in our patient.…”

mentioning

confidence: 99%

Long-Term Survival With Heart Transplantation for Fibrosarcoma of the Heart

Coelho

Banazol

Soares

et al. 2010

Primary sarcoma of the heart is a rare disease that has an ominous prognosis with either medical or surgical therapy. We report a case of a 25-year-old woman with sarcoma of the heart who received a transplant and is clinically well after 7 years. We believe that transplantation must be considered in this kind of pathology for selected cases.(Ann Thorac Surg 2010;90:635-6) © 2010 by The Society of Thoracic Surgeons P rimary tumors of the heart are rare clinical entities and represent 0.001% to 0.03% of all cardiac surgical cases [1-3]; of these, 25% are malignant tumors, and among them, sarcomas are the most prevalent. Sarcomas of the heart are known to be highly invasive, and when the diagnosis is made, about 80% are already disseminated. Average survival at 9 months is 10% [2].The best therapeutic options for these patients are still being discussed. Total resection is not possible for most patients, and the local recurrence rate is very high. Transplantation has certainly been an option for selected isolated cases, but long-term results and precise indications are still not clear [1].Our patient was a 25-year-old woman who was diagnosed with Hodgkin lymphoma at age 16 years. She was treated with chemotherapy and mediastinal radiotherapy and experienced total remission. As sequela, she had some degree of lung fibrosis and hypothyroidism.In August 2001, the patient was unexpectedly admitted to the hospital with complaints and signs of right-sided heart failure. An echocardiogram showed a tumor mass filling the right ventricular cavity, and a computed tomography scan and cardiac magnetic resonance imaging confirmed those findings and excluded any other locations. Without other diagnosis and given her severe New York Heart Association functional class IV condition, she underwent an open heart operation.A biopsy specimen was obtained from the tumor mass inside the right ventricle, and the pathology report identified sarcoma. Considering the ominous result, partial debulking of the tumor was done, trying to maintain the cavity-filling function of the ventricle without putting the patient at risk with a too-aggressive resection, namely at the septal area where we could create a ventricular septal defect.The patient's postoperative period was without complications. She was referred for chemotherapy and underwent a perfusion of ifosfamide (1500 mg/m 2 ) for 3 days. One month later, she was readmitted with gross rightsided heart failure, and the echocardiogram showed extensive recurrence of the tumor mass, with protrusion of the tumor through the tricuspid valve into the right atrium (Fig 1). At this point, there seemed to be no other medical or surgical options owing to the type of the recurrent tumor involving the heart. Computed tomography scans and liver and bone perfusion scans were able to exclude any metastasis or invasion at distant locations, and cardiac transplantation came as a last option.The patient underwent cardiac transplantation in December 2001 that was performed with a biatrial technique. The tumor i...

Holland‐Frei Cancer Medicine

Tumors of the Heart and Great Vessels

Chahinian¹,

Gutstein²,

Fuster³

2017

Overview Primary tumors of the heart and great vessels are rare, but secondary tumors are significantly more common. Clinical manifestations of cardiac tumors depend on the location and size of the mass, not on the histopathology of the tumor. Several noninvasive imaging modalities including echocardiography, cardiac MRI, cardiac CT, and PET are currently available to provide complementary information on the location, extent, and tissue characteristics of a cardiac tumor. The clinical management of patients with cardiac tumors varies based on the type of tumor, clinical symptoms, and overall prognosis.