Leiomyosarcoma of the pulmonary veins with extension to the left atrium

Gyhra, Alberto; Santander, Claudio K.; Alarcón, Emilio; Mucientes, Francisco; Carrillo, Hernán

doi:10.1016/0003-4975(96)80208-6

Cited by 29 publications

(13 citation statements)

References 4 publications

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“…The first was in 1996, consisting of a 43-year-old Chilean 6 woman who underwent open heart surgery, and was later was diagnosed as a primary cardiac leiomyosarcoma with vimentin and MSA positive tumor markers, as in this report. The second case was a Mexican report published in 2002 in which a 31-year-old Mexican 7 woman presented during surgery a large infiltrative tumor in the left atrium.…”

Section: Discussionmentioning

confidence: 72%

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Immunohistochemical Diagnosis of Primary Cardiac Leiomyosarcoma in a Latin American Patient

et al. 2016

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Primary cardiac malignancies are rare entities. Although sarcomas enclosed the main group of malignant heart neoplasms, primary cardiac leiomyosarcomas are extremely rare and constitutes less than 8% of cardiac tumors. Leiomyosarcoma usually originates from the pulmonary veins and have a worm-like shaped structure. In this article, we present a case of a 40-year-old Hispanic man diagnosed with a cardiac tumor who underwent surgical resection, during pathological examination the tissue samples were consistent with a malignancy of mesenchymal origin that contained irregular bundles of spindle cells. Subsequent immunohistochemical study categorized the mass as a primary heart leiomyosarcoma with positive smooth muscle actin and muscle specific actin. As usual in this type of malignancy, patient’s clinical status declined overtime, recurrence was diagnosed two months after surgery, and four months after the procedure the patient was discharge for palliative care.

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Section: Discussionmentioning

confidence: 72%

“…In this article, we present the third case, 6,7 to our knowledge, of a Latin American patient with this type of tumor, in addition a literature review of the histopathological and clinical aspects of this entity are provided.…”

Section: Introductionmentioning

confidence: 99%

Immunohistochemical Diagnosis of Primary Cardiac Leiomyosarcoma in a Latin American Patient

et al. 2016

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“…It has been reported that immediate postoperative chemotherapy may markedly prolong survival. 5,6 Polychemotherapy with doxorubicin hydrochloride, ifosfamide, etoposide and vincristine sulfate reduced the size of mass dramatically in reported cases. 6,7 However, the rarity of the tumor has precluded randomized studies.…”

Section: Discussionmentioning

confidence: 91%

“…A biopsy would enable a preoperative diagnosis to be made, but in all the previously reported cases, the preoperative diagnosis seemed to be impossible. Extensive resection including the atrial wall, 2 cardiac valves and lung 4,5 followed by reconstruction with a pericardial patch 4,6 and homograft replacement 7 have been reported, but long-term survivors are rare. Even after extensive resection, the prognosis is very poor due to early local recurrence or metastasis.…”

Section: Discussionmentioning

confidence: 99%

Primary Leiomyosarcoma of the Left Atrium

et al. 1999

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rimary malignant tumors of the heart are extremely rare, and leiomyosarcoma is the rarest among these tumors. The prognosis of leiomyosarcoma is reported to be very poor even if surgical resection, with or without adjuvant chemotherapy and radiotherapy, is performed. Here we present our case of primary leiomyosarcoma of the left atrium and discuss the diagnosis, treatment and prognosis by reviewing the literature. Case ReportA 69-year-old woman had intravenous fluid administered at a clinic due to general fatigue, but as she subsequently developed dyspnea with cyanosis and respiratory arrest, she was transferred to hospital with cardiopulmonary resuscitation and was admitted to the cardiac care unit after intubation. Chest roentgenography demonstrated severe lung congestion and mild cardiomegaly. Transesophageal echocardiography revealed what was suspected to be a giant myxomatous tumor in the left atrium, which extended to the orifice of the mitral valve and obstructed the pulmonary veins (Fig 1); however, no tumor stalk was observed on the atrial septum or left atrial wall. A hemodynamic study demonstrated that the pulmonary artery pressure and mean pulmonary capillary wedge pressure were 42/32 and 30 mmHg, respectively, and the cardiac index was 3.6 L min -1 m -2 .The patient underwent surgery under standard cardiopulmonary bypass via median sternotomy. After administration of antegrade cold crystalloid cardioplegia, the left atrium was incised longitudinally. The tumor occupied most of the left atrial cavity and adhered to the wall, so a right atriotomy with atrial septal incision was also performed to expose and excise the tumor. The tumor, which was solid and partially calcified with thrombus, was carefully dissected and resected. An area of the tumor extended into the pulmonary veins and obstructed the inflow of the left atrium and this was excised and removed as much as possible, but no attempt was made to excise the atrial wall or pulmonary veins, because it was unclear whether the tumor arose from the left atrium or the pulmonary veins. The mitral valve was normal and not invaded by the tumor. After repairing the left and right atria, the patient was weaned from the cardiopulmonary bypass without difficulty.The excised tumor was 5×6 cm in dimension, 95 g in weight, and grayish in color with thrombotic nodules on its surface. Histopathological examination demonstrated a spindle cell tumor, with a high rate of mitosis indicating Jpn Circ J 1999; 63: 414 -415 (Received December 4, 1998; revised manuscript received January 25, 1999; accepted February 3, 1999 A 69-year-old woman with symptoms of congestive heart failure had a left atrial leiomyosarcoma, an extremely rare cardiac tumor, which obstructed the mitral valve and pulmonary veins. Surgical resection was performed, but no other adjuvant therapy was administered because the patient refused it. Recurrence of the tumor occurred soon after surgery and the patient died 81 days postoperatively. (Jpn Circ J 1999; 63: 414 -415)

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“…Chemotherapy and radiation therapy have not been shown to be effective and surgical resection remains the only mode of treatment. When these tumours extend into the heart, cardiopulmonary bypass is required to achieve negative margins and to prevent tumour embolism 3 …”

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confidence: 99%