Leiomyosarcoma of Pulmonary Vein Presenting as Left Atrial Mass: An Outline of Management Principles

Abstract

Leiomyosarcoma of the pulmonary vein is rare and has poor prognosis. Its clinical features are nonspecific and mimic benign conditions. Early diagnosis is challenging. Most cases have been diagnosed only at autopsy or on postoperative histology specimens. Treatment is essentially palliative complete surgical excision. We outline the principles of management with the case of a 39-year-old man with leiomyosarcoma of the left pulmonary veins extending into the left atrium. Extensive investigation to achieve early diagnosis and determine extent of disease is essential. Frozen section guided adequate excision of all cardiac tumours and resection of involved lung tissue achieve local disease control. Adjuvant chemoradiotherapy has been shown to enhance survival.

Efficacy of Paclitaxel in a Patient with Inoperable Pulmonary Vein Leiomyosarcoma

“…Including the present case, only 30 cases with leiomyosarcoma of the pulmonary veins have been reported to date [ 2 , 7 , 8 , 9 , 10 , 11 , 12 ]. Owing to the scarcity of this disease, little is known about the optimal treatment modality of pulmonary vein leiomyosarcoma.…”

“…The benefits of chemotherapy are also unclear. In some cases, postoperative chemotherapy might prolong the survival [ 8 , 10 ], but a possibility that the supposed efficacy of chemotherapy was coupled with surgical interventions should be taken into account because surgery alone might contribute to the prolongation of survival [ 5 ]. In addition to unknown efficacy, there have been no data on an optimal chemotherapy regimen.…”

“…In addition to unknown efficacy, there have been no data on an optimal chemotherapy regimen. Doxorubicin, ifosfamide, etoposide, dacarbazine, vincristine, and cyclophosphamide have been used as in other soft tissue sarcomas, and there was no observed exact efficacy of chemotherapy [ 2 , 8 , 10 ].…”

Immunohistochemical Diagnosis of Primary Cardiac Leiomyosarcoma in a Latin American Patient

“…11 Hence, intravascular leiomyosarcoma may resemble a myxoma due to the similarity in the echocardiographic characteristics and location in the left atrium. However, establishing the origin of the tumor in the pulmonary veins might be the initial sign to differentiate a leiomyosarcoma from a myxoma.…”

“…However, nowadays it is well known that favorable prognosis is mainly influenced by complete surgical resection, although sarcomas are often irresecable. 3 Nevertheless, pathological diagnosis based on histopathological and immunohistochemical studies should be performed, as some subtypes presents a lower overall survival rates in comparison to others, leiomyosarcoma have a poor survival rate of 6 to 12 months without surgery and 24 months with surgery 11 when comparing with a benign atrial myxoma which have a relative good prognosis. 19 Currently, the main risk factors associated with lower overall survival rates in heart leiomyosarcoma are age >50 years and inadequate surgical margins.…”