Primary leiomyosarcoma of the atrium with heterologous differentiation

Zaidi, Shaesta Naseem; Husain, Sufia; Barakah, Dona

doi:10.5144/0256-4947.2017.403

Cited by 2 publications

(5 citation statements)

References 9 publications

(6 reference statements)

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“…The most common location of cardiac malignant cancer is the left atrium and the most incident malignant tumour is angiosarcoma, with aggressive behaviour and a worse prognosis [ 7 ].…”

Section: Discussionmentioning

confidence: 99%

“…As for LMS, the main sites affected are the uterus, abdomen and retroperitoneum [ 11 ], which leads them to be divided into uterine and non-uterine LMS [ 2 ]. As cardiac tumours, they are extremely rare [ 7 , 9 ]. Primary cardiac LMS are extremely rare [ 6 , 8 ] and have a worse prognosis [ 6 , 7 ].…”

Section: Discussionmentioning

confidence: 99%

“…As cardiac tumours, they are extremely rare [ 7 , 9 ]. Primary cardiac LMS are extremely rare [ 6 , 8 ] and have a worse prognosis [ 6 , 7 ].…”

Section: Discussionmentioning

confidence: 99%

“…Among the malignant tumours, secondary neoplasms correspond to the highest number of cases, which come from metastases and constitute more than half of the occurrences [2,3]. On the other hand, primary cardiac cancers are rare, highly aggressive and responsible for an unfavourable prognosis [1,3,6,7]. Among the latter, the various types of sarcomas (angiosarcoma, rhabdomyosarcoma and LMS) stand out, corresponding to 75% of all cardiac cancers [1,[3][4][5].…”

Section: Discussionmentioning

confidence: 99%

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Management of primary cardiac leiomyosarcoma

Formigosa¹,

Santos²,

Martins³

et al. 2023

ecancer

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Background Primary cardiac cancer is a rare event with various clinical presentations and often causes unexpected symptoms or sudden death. Case reports with this diagnosis are scarce. Case presentation We present an unusual manifestation of leiomyosarcoma of the left atrium in a female patient, 33 years old. Presenting difficulty to walk, dyspnoea at rest, skin pallor, cough with hemoptoics and syncope. A transthoracic echocardiogram showed cavitary enlargement of the left atrium, moderate to significant mitral stenosis with an adherent mass in the anterior leaflet, left ventricular systolic function preserved at rest, and mild aortic and tricuspid insufficiency. The procedure was complete resection of the tumour or negative microscopic margins (R0 resection), 25 sessions of radiotherapy, 5 cycles of adjuvant chemotherapy using gemcitabine (900 mg/m 2 on days 1 and 8) and docetaxel (75 mg/m 2 on day 8), with a resolution of the clinical picture. After 5 years of follow-up, the patient had no metastases or recurrence of the initial tumour. Conclusion The nonspecific symptoms presented in the reported case demonstrate that the cardiac tumour can mimic other cardiac disorders, such as coronary artery disease or pericarditis, rarely representing the first manifestation of a previously unknown malignancy.

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“…The most common location of cardiac malignant cancer is the left atrium and the most incident malignant tumour is angiosarcoma, with aggressive behaviour and a worse prognosis [ 7 ].…”

Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

“…As cardiac tumours, they are extremely rare [ 7 , 9 ]. Primary cardiac LMS are extremely rare [ 6 , 8 ] and have a worse prognosis [ 6 , 7 ].…”

Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

See 2 more Smart Citations

Management of primary cardiac leiomyosarcoma

Formigosa¹,

Santos²,

Martins³

et al. 2023

ecancer

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show abstract

“…They usually arise from the uterus, gastrointestinal tract, and retroperitoneum, and very rarely do they originate from the heart [5]. Primary cardiac leiomyosarcomas are exceedingly rare entities with an incidence of only 0.025% of all cardiac sarcomas, with only a bleak number of reported cases [6].…”

Section: Discussionmentioning

confidence: 99%

Primary Leiomyosarcoma of the Left Atrium

Saldanha

Shetty²,

Nirmala³

2019

Journal of Case Reports: Open Access

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Objectives: To present a case of a leiomyosarcoma of the left atrium, presenting as exertional dyspnea in a middle-aged Filipino. This study likewise presents a review of current literature.Results: A 44 years old female presented with a three-day history of exertional dyspnea. Transesophageal echocardiography revealed a mass with pericardial effusion and a large mass occupying almost the entire left atrial cavity. She underwent complete excision with mitral valve repair. Histopathology revealed bundles of fascicles of spindle-shaped cells with eosinophilic infiltrates, positive for SMA, Caldesmon, and Vimentin, and a high Ki67 of >50% --consistent with primary leiomyosarcoma. No adjuvant chemotherapy was done. Within three months of post-operation and lost to follow-up, the patient noted the recurrence of dyspnea. Echocardiography revealed the recurrence of the mass, again obstructing the left atrium.Metastatic lesions were also on both lungs. The patient was planned a 2nd resection of the mass, followed by systemic chemotherapy, but died due to obstructive heart failure.Summary/conclusion: Primary cardiac leiomyosarcomas (PCLMS) are extremely rare tumors. Studies reveal female preponderance with mean age of 48 years. The tumors seem to originate from the cardiac chambers, without the involvement of the pericardium and great vessels. Increased tumor burden, metastatic disease, high proliferative index, and high-grade tumors usually translate to a worse prognosis. Complete surgical resection of the tumor is still the mainstay of treatment followed by doxorubicin, ifosfamide, or dacarbazine-based regimens.

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Primary leiomyosarcoma of the atrium with heterologous differentiation

Cited by 2 publications

References 9 publications

Management of primary cardiac leiomyosarcoma

Management of primary cardiac leiomyosarcoma

Primary Leiomyosarcoma of the Left Atrium

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