Leiomyosarcoma of the right inferior pulmonary vein: 2 years survival with multimodality therapy
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Primary leiomyosarcoma of the heart is an extremely rare and aggressive tumor. The authors report a case of a 29-year-old man with a leiomyosarcoma of the right inferior pulmonary vein who underwent surgery, chemotherapy, and radiotherapy. The patient experienced two local recurrences and he finally died 2 years after onset of symptoms because of multiple distal metastases.
Keywords:Cardiac tumors Sarcoma Pulmonary vein Chemotherapy
Leiomyosarcomas represent a minority of primary cardiac tumors and are associated with a poor outcome because of local recurrence and distal metastases. No consensus exists regarding the best operative strategy. We report a case of a 29-year-old man with a leiomyosarcoma of the right inferior pulmonary vein treated with a multimodality therapy including surgery, chemotherapy, and radiotherapy with a 2 years survival after onset of symptoms.
Immunohistochemically the tumor cells showed a wide intracellular positive stain for vimentine, desmin, smooth muscle actine, and caldesmon. There was no immunoreactivity for vascular markers such as CD31 and CD34. Ki67 proliferation marker was present in 40 % of cells (Fig. 2). These morphological features were consistent with a diagnosis of a high grade leiomyosarcoma arising from the right inferior pulmonary vein with positive resection margins and the absence of tumoral infiltration of the left atrial wall.
The specimen obtained at the time of the right inferior lobectomy extended to the left atrium was 15 × 12 × 10 cm in dimension and 380 gr in weight. The tumor measured 7 × 5 × 4 cm and presented wide necrotic areas; it originated from the right inferior pulmonary vein and protruded into its lumen, and extended into the left atrium but still respecting the left atrial wall. The pulmonary parenchyma presented a sub-pleural whitish nodule in the basal pyramid measuring 0.5 cm in diameter and located at 5 cm from the primary tumor. Both the primary tumor and the satellite nodule presented the same histological features of the first specimen; of note there were no lymphatic metastasis.
The third specimen was 5 × 4 × 3 cm in dimension and 90 gr in weight and contained a 4 × 4 × 1.5 cm tumor implanted on the left atrial wall. Histologically the tumor consisted of both epithelioid and fusiform cells, with eosinophilic cytoplasm and hypercromatic nuclei; necrotic areas represented 60 % of the tumor volume.
Primary cardiac tumors are extremely rare with an incidence ranging from 0.001 to 0.03 % at autopsy, while cardiac metastases occur 20–40 times more frequently than primary neoplasms . Three-quarters of the primary cardiac tumors are benign and nearly half the benign heart tumors are myxomas . Sarcomas, usually angiosarcomas, represent the most frequent malignant heart tumors accounting for 90–95 % of all malignant heart tumors, followed by lymphomas ; leiomyosarcoma accounts for about 9 % of all primary malignant heart tumors . Vascular leiomyosarcoma represents only 2 % of soft tissue leiomyosarcomas; these rare tumors mainly derive from smooth muscle cells of vessel walls with predominant localization in the inferior vena cava and less frequently in the pulmonary arteries . Pulmonary vein leiomyosarcoma is an extremely rare tumor first described at autopsy by Kidd and coll. in 1961 ; previously only 4 cases of sarcomas arising from of the pulmonary vein and filling the left atrium have been reported at autopsy .
Less than 20 cases of surgically treated cases of pulmonary vein leiomyosarcomas have been reported since 1989 [3, 5, 6, 7, 8, 9, 10, 11, 12, 13]. This entity shows no sex predilection, the mean age of presentation is 48 years (range 23–74) and symptoms are unspecific and consist of dyspnea, hemoptysis, cough, palpitations, and chest pain. Usually leiomyosarcomas of the pulmonary vein enter the left atrium and can involve the mitral valve causing obstructing symptoms. These tumors are very aggressive with a high risk to develop local and distal recurrences; metastases have been described at liver , scalp , and axillary lymph node . Echocardiography and CT are useful to define the sites and the extent of involvement which determine the therapeutic approach. No consensus exists regarding the best operative strategy; however, wide surgical resection remains the cornerstone for treatment of this tumor with variable outcomes in literature ; additionally given the likely inadequacy of surgical margins and the risk of metastases, adjuvant chemotherapy is the preferred post surgical treatment modality . Mean survival for most cardiac sarcomas is about 9–11 months  and patients who underwent a complete resection show a better survival compared with patients who did not . Therefore, total surgical resection with CPB and the addition of chemotherapy should offer these patients significant palliation and an opportuny for increased length of survival.
We present a case of leiomyosarcoma of the pulmonary vein and to our knowledge this is the fourth case report in a patient younger than 30 years [3, 9, 11]; additionally 2 years or more survival after the diagnosis of this aggressive tumor has been documented only in 4 patients who underwent surgery included this case [10, 11]. Despite rare frequency, accumulation of cases and longer follow-up are required to assess the definitive therapy other than surgical excision and prognostic factors of this rare entity.
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