Sarcoma de la vena pulmonar / Sarcoma of the pulmonary vein

Resumen Los sarcomas son neoplasias primarias, cuya ubicación es rara en grandes vasos y excepcional en las venas pulmonares. Los dos tipos más frecuentes en las venas pulmonares son el leiomiosarcoma y el fibrosarcoma, con una distribución alrededor de la cuarta a la quinta décadas de vida, un pronóstico de 23 meses y 60% de metástasis al momento del diagnóstico. La presentación clínica es inespecífica ya que simula situaciones como embolia pulmonar, falla cardiaca descompensada y masa en la aurícula izquierda. Para su diagnóstico se cuenta con diferentes herramientas, como la ecocardiografía, la tomografía computarizada, la angiografía coronaria, la resonancia magnética y la tomografía por emisión de positrones (PET TC). El tratamiento incluye resección quirúrgica radical, manejo adyuvante con quimioterapia y radioterapia, e incluso, en casos seleccionados, trasplante de corazón. Se presenta el caso de una paciente con diagnóstico inicial de embolia pulmonar, con un episodio de edema pulmonar secundario a masa en la aurícula izquierda y extensión de un sarcoma de vena pulmonar derecha, con desenlace fatal. Se aporta a la literatura con el caso y la revisión de tema.

Abstract Sarcomas are primary neoplasms, whose location is rare in large vessels and in the pulmonary veins is exceptional. The two most frequent types in the pulmonary vein are leiomyosarcoma and fibrosarcoma, distribution around 4 and 5 decades of life, with a prognosis of 23 months and 60% metastasis at the time of diagnosis. The clinical presentation is nonspecific simulating situations such as pulmonary embolism, decompensated heart failure and mass in the left atrium. Different tools are available for its diagnosis, like echocardiography, computed tomography, coronary angiography, magnetic resonance imaging and PET CT. Treatment includes radical surgical resection, adjuvant therapy with chemotherapy, and radiation therapy, even heart transplantation in selected cases. It is presented the case of a patient with an initial diagnosis of pulmonary embolism, with an episode of pulmonary edema secondary to a mass in the left atrium, extension of a sarcoma of the right pulmonary vein, with a fatal outcome. We contributed to the literature with the case and review of theme.

A Case of Primary Right Atrial Angiosarcoma / 日本心臓血管外科学会雑誌

Primary cardiac angiosarcoma is extremely rare and its prognosis remains poor. We present a 61-year-old woman with angiosarcoma who was admitted with exertional dyspnea and considerable hemodynamic compromise. Computed tomography showed a large tumor in the right atrium and multiple liver metastases. The cardiac tumor invaded from the superior vena cava to the atrial septum. After the cardiac tumor was removed, the right atrium, the atrial septum and the superior vena cava were reconstructed with a bovine pericardial patch. The pathological diagnosis was angiosarcoma, and the cut-end was positive for sarcoma. The patient survived 14 months after surgery with the aid of adjuvant chemotherapy.

Right Atrial Paraganglioma: An Extremely Rare Primary Cardiac Neoplasm Mimicking Myxoma

In this report, we present a case of 35-year-old lady who had presented with atypical chest pain and exertional breathlessness for past six months. Transthoracic and transesophageal echocardiograms showed a well-circumscribed, echo-dense mass in the right atrium, attached to the interatrial septum at the level of atrioventricular junction and in the vicinity of coronary sinus ostium. She underwent successful resection of the cardiac mass. Histopathology revealed paraganglioma, which was reconfirmed by immunohistochemistry study. This represents an extremely rare presentation as primary cardiac tumors are 20-times less common than metastatic tumors and paraganglioma is one of the rarest primary cardiac tumors, accounting for < 1% of all cases.

A Case of Primary Cardiac Angiosarcoma Associated with Cardiac Tamponade / 日本心臓血管外科学会雑誌

We report a rare case of primary cardiac angiosarcoma in the right atrium. A 47-year-old man was admitted to our hospital with cardiac tamponade. Echocardiography and computed tomography revealed a tumor in the right atrial cavity. We performed tumor resection to confirm the histological diagnosis, to prevent tumor embolism, and to increase the possibility of improving the prognosis. The tumor was resected with the right atrial wall and right pericardium. The right atrium was then reconstructed with a bovine pericardial patch. The pathological diagnosis was angiosarcoma. The patient survived only about 6 months after surgical resection, but there was no local recurrence. This report presents a very rare case of cardiac angiosarcoma associated with cardiac tamponade.

A Papillary Fibroelastoma in the Left Ventricle / 日本心臓血管外科学会雑誌

A 75-year old woman in whom a left ventricular tumor had been detected by echocardiography 2 years before referral to our hospital, presented with blurry vison for one month. Acute cerebral infarction was diagnosed. We suspected that the infarction was occurred by an embolus from the intraventricular tumor, and resected it through left atrial incision. The resected tumor was 10 mm in size and it resembled a sea anemone. The tumor was pathologically diagnosed as papillary fibroelastoma. The postoperative course was good, with no recurrence for the last 18 months.

A Case of Primary Myxofibrosarcoma of the Heart Recurring 7 Months after the First Cardiac Operation / 日本心臓血管外科学会雑誌

Primary cardiac myxofibrosarcoma is an extremely rare malignant tumor of the heart, which can be difficult to diagnose. We report such a case, which recurred 7 months after the initial heart surgery. A 63-year-old woman with progressive dyspnea was seen by a physician. A transthoracic echocardiography revealed a large mobile mass in the left atrium, causing severe mitral stenosis. The patient was referred to our hospital for an operation. The tumor, which arose from the anterior mitral annulus, was resected, and mitral and tricuspid annuloplasty were performed. The histopathological diagnosis was myxoma. The postoperative course was uneventful. Seven months later, the patient reported suffering from dyspnea and leg edema. The echocardiography showed multiple recurrences of tumor in the left atrium. A second operation was performed to reduce the mass volume as a palliative treatment because some large fragile tumors occupied the left atrium. The patient died 5 months after the second operation due to metastasis. The final diagnosis by histopathology was myxofibrosarcoma.

A Case of Cardiac Angiosarcoma Successfully Treated with Postoperative Conformal Dynamic Arc Radiotherapy / 日本心臓血管外科学会雑誌

Cardiac angiosarcoma is a rare heart malignancy. The prognosis is reported to be very poor. Here, we report a case of cardiac angiosarcoma which was treated by postoperative conformal dynamic arc radiotherapy. The patient has been in good health with no recurrence for 18 months after surgery. The patient was a 71-year-old woman, who presented edema and general malaise. Echocardiography and computed tomography revealed a right atrial mass and massive pericardial effusion, which was thought to be the cause of tamponade. Intraoperatively, we found a large tumor arising from right atrial wall spreading and invading to the inferior vena cava, diaphragm, and right pericardium. We abandoned complete resection of the tumor. We only resected the part of the tumor under cardiopulmonary bypass. The subsequent defect of the right atrial wall was reconstructed with bovine pericardial patch. The pathological diagnosis was consistent with angiosarcoma, and the margin was positive for the tumor. In addition to reduction surgery, we adopted radiotherapy (conformal dynamic arc radiotherapy, 10MV-X-ray, 54 Gy/18 Fr/4.5 week, fractionated radiotherapy). The CT, one month after the radiation, showed a significant reduction in tumor size. Moreover, no tumor could be pointed out by echocardiography 18 months after surgery. Advances in technology and methodology has made tumor control possible without significant side effects. We conclude that reduction surgery accompanied with postoperative radiotherapy is promising in maintaining quality of life and in improving life expectancy.

A Case of Emergency Surgery for a Huge Primary Right Atrial Angiosarcoma with Right Ventricular Failure and Shock / 日本心臓血管外科学会雑誌

We encountered a rare case of a 75-year-old woman who fell into right ventricular failure and shock with a comparatively rapid course due to a huge primary right atrial angiosarcoma occupying the right atrium. An emergency surgical excision of the tumor was performed and the right atrium was reconstructed with an EPTFE patch under cardiopulmonary bypass. On account of the positive margin, postoperative radiotherapy was added. There was no local recurrence, but adjuvant chemotherapy was performed for multiple lung and liver metastases 14 months after surgery. Primary cardiac angiosarcomas are extremely rare and have dismal prognoses. Although a complete surgical resection is the cornerstone of treatment, multidisciplinary therapy may improve patient outcomes.

A Case of Malignant Mesenchymoma of the Heart

Malignant mesenchymomas by definition are composed of two or more cellular types that would ordinarily derive from primitive mesenchyme. They grow rapidly, recur frequently, metastasize, and can be found in a wide variety of locations. Malignant mesenchymomas as primary cardiac tumor are extremely rare with poor prognosis. Only 15 cases of cardiac malignant mesenchymoma were reported in worldwide literature in 1961-1992. We report a case of primary cardiac malignant mesenchymoma in 58 year-old female patient admitted due to hemoptysis and mild exertional dyspnea.

Cardiac Fibroma of the Ventricular Septum: A case report

This is an autopsy case of a 6 month old girl who suddenly died of respiratory distress during sleep. She had suffered from mild but frequent episodes of common cold and was treated for eczema for several days. At autopsy, the heart was enlarged and weighed 100 gm. A firm and gray-white tumor, measuring 4.5 x 3.8 x 2.8 cm, was located in the interventricular septum and encroached upon the wall of left ventricle. The mass was well demarcated but was not encapsulated. Neither necrosis nor calcification was present. Microscopically the tumor was composed of haphazardly arranged bundles of collagen fibers and fibroblasts. Myocardial cells are intermingled with the fibroblasts at the margin of the tumor. Massive edema of the lung and congestion of the liver and spleen were pronounced.