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who had recently given up smoking &#40;smoking history of 132 pack per year&#41;&#44; presented in August&#39;99 with complaints of cough with purulent sputum&#44; hemoptysis&#44; dyspnoea and fever that did not improve with a course of antibiotherapy&#46;</p><p id="p0020" class="elsevierStylePara elsevierViewall">His general condition was good&#44; but he had reduced lung sounds in the lower third of the right hemithorax&#46; The PaO<span class="elsevierStyleInf">2</span> in room air was 73<span class="elsevierStyleHsp" style=""></span>mmHg&#46; Chest X-ray showed an opacity in the lower right hemithorax&#44; and CT scan revealed an endobronchial mass causing obstruction of the lumen of the inferior lobar bronchus &#40;<a class="elsevierStyleCrossRef" href="#f0005">Fig&#46; 1</a>&#41;&#44; with an irregular&#44; approximately 3<span class="elsevierStyleHsp" style=""></span>cm&#44; extension into the lung parenchyma&#46; Sputum cytology showed malignant cells and the histology of the endoscopic bronchial biopsy revealed a malignant tumor with sarcomatous features&#46; There was no evidence of tumor elsewhere on abdominal and brain CT scans and bone scintigraphy&#46; The endobronchial tumor mass was subsequently removed with laser&#44; and histological examination showed a spindle cell tumor with marked nuclear pleomorphism&#44; multinucleated tumor giant cells and numerous mitotic figures &#40;<a class="elsevierStyleCrossRef" href="#f0010">Fig&#46; 2</a>&#41;&#46; There was no evidence of epithelial differentiation&#46; The tumor was immunoreactive for vimentin and focally for CD68&#44; and negative for multiple high and low mol&#46; wt&#46; cytokeratins&#44; epithelial specific antigen&#44; epithelial membrane antigen&#44; actin&#44; smooth muscle actin&#44; desmin&#44; S-100 protein and CD34&#46; CD117 immunostaining showed no mast cells within the tumor&#46; The cell proliferation index with Ki 67 was 60 &#37;&#46; A diagnosis of pleomorphic undifferentiated sarcoma was made&#46;</p><elsevierMultimedia ident="f0005"></elsevierMultimedia><elsevierMultimedia ident="f0010"></elsevierMultimedia><p id="p0025" class="elsevierStylePara elsevierViewall">Exploratory thoracotomy showed mediastinal invasion and radiotherapy was given to the tumor and mediastinum in a dose of 56 grays in 28 fractions&#44; resulting in disappearance of the mass&#46;</p><p id="p0030" class="elsevierStylePara elsevierViewall">The patient has been followed-up regularly for the last 11 years&#44; and has suffered recurrent respiratory infections recovering easily with antibiotherapy&#46; Serial chest CT scans &#40;<a class="elsevierStyleCrossRef" href="#f0015">Fig&#46; 3</a>&#41;&#44; showed an unaltered diffuse density in the subcarinal and right hilar regions extending to the right pulmonary artery&#44; thought to be scarring&#46; The patient has refused to undergo further bronchial endoscopy&#46;</p><elsevierMultimedia ident="f0015"></elsevierMultimedia></span><span id="s0015" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="st0035">Discussion</span><p id="p0035" class="elsevierStylePara elsevierViewall">Primary pulmonary sarcomas are rare tumors&#44; representing about 40 &#37; of &#34;rare&#34; pulmonary neoplasms and 9 &#37; of all sarcomas&#46;<a class="elsevierStyleCrossRef" href="#bb0010"><span class="elsevierStyleSup">2</span></a> To date&#44; approximately 300 cases have been reported in the literature&#46;<a class="elsevierStyleCrossRef" href="#bb0005"><span class="elsevierStyleSup">1</span></a> Sarcomas in the lungs can arise from pulmonary parenchyma&#44; the bronchi or the pulmonary artery and its branches&#46;<a class="elsevierStyleCrossRefs" href="#bb0010"><span class="elsevierStyleSup">2&#44;3</span></a> They typically occur in middle-aged individuals with slight predominance in men&#46; Prior history of radiotherapy or accidental exposure to radiation&#44; confer greater susceptibility to the development of sarcomas&#46; Symptoms and radiographic features are similar to those seen in other epithelial lung tumors and depend more on tumor localization than on their histological characteristics&#46;<a class="elsevierStyleCrossRef" href="#bb0010"><span class="elsevierStyleSup">2</span></a></p><p id="p0040" class="elsevierStylePara elsevierViewall">Primary sarcomas of the lung present a considerable diagnostic challenge to both the histopathologist and the clinician&#46; Litzsky <a class="elsevierStyleCrossRef" href="#bb0035"><span class="elsevierStyleSup">7</span></a> stresses two points&#59; most malignant spindle cell tumors in the lungs will turn out to be carcinomas with sarcomatoid differentiation and sarcomas metastatic to the lung are far more frequent than primary sarcomas&#46;</p><p id="p0045" class="elsevierStylePara elsevierViewall">The lung presents all the common histological types of primary sarcomas seen elsewhere in the soft tissues&#46; Changing terminology and histological criteria make it difficult to compare the incidence of different types of sarcoma&#46; <a class="elsevierStyleCrossRefs" href="#bb0005"><span class="elsevierStyleSup">1&#8211;7</span></a> Fibrosarcoma&#44; a common sarcoma of the lung in earlier studies&#44; <a class="elsevierStyleCrossRef" href="#bb0030"><span class="elsevierStyleSup">6</span></a> is no longer mentioned and fibrohistiocytic tumors such as malignant fibrous histiocytoma also thought to be common&#44;<a class="elsevierStyleCrossRef" href="#bb0020"><span class="elsevierStyleSup">4</span></a> are rarely encountered&#46;<a class="elsevierStyleCrossRef" href="#bb0010"><span class="elsevierStyleSup">2</span></a> The most common sarcomas now reported&#44; are leiomyosarcoma&#44; synovial sarcoma&#44; malignant peripheral nerve sheath and vascular tumors&#46;<a class="elsevierStyleCrossRef" href="#bb0035"><span class="elsevierStyleSup">7</span></a></p><p id="p0050" class="elsevierStylePara elsevierViewall">It is now accepted that along with morphological features&#44; immunohistochemistry plays an important role in the classification&#44; and the wide range of antibodies currently available means that entities previously diagnosed as fibrosarcoma&#44; malignant fibrous histiocytoma and hemangiopericytoma have now been reclassified&#46; Undifferentiated pleomorphic sarcoma&#44; previously called pleomorphic malignant fibrous histiocytoma&#44; is diagnosed only when no definable line of differentiation can be established&#44;<a class="elsevierStyleCrossRef" href="#bb0040"><span class="elsevierStyleSup">8</span></a> as in our case&#46; Immunohistochemistry is also essential for ruling out the much more frequent pulmonary sarcomatoid carcinomas&#46; In our case&#44; antibodies to high and low molecular cytokeratins&#44; epithelial specific antigen and epithelial membrane antigen ruled out sarcomatoid carcinoma&#44; synovial sarcoma and diffuse malignant mesothelioma&#44; a diagnosis that should be considered when the tumor involves the pleura or mediastinum&#46;<a class="elsevierStyleCrossRef" href="#bb0035"><span class="elsevierStyleSup">7</span></a> Synovial sarcoma is characterized by mast cells within the tumor&#44;<a class="elsevierStyleCrossRef" href="#bb0045"><span class="elsevierStyleSup">9</span></a> and none were detected in this tumor on staining with CD117&#46; Smooth muscle actin&#44; actin and desmin are seen in leiomyosarcoma&#44; and the latter two in rhabdomyosarcoma&#44; S-100 protein in malignant peripheral nerve sheath and liposarcoma&#44; while malignant solitary fibrous tumor and malignant vascular tumors are immunoreactive for CD34&#44; all of which were negative in our case&#46; CD68&#44; focally positive in our case and previously thought to be a marker for pleomorphic undifferentiated sarcoma &#40;malignant fibrous histiocytoma&#41;&#44; is no longer believed to be useful&#46;<a class="elsevierStyleCrossRef" href="#bb0040"><span class="elsevierStyleSup">8</span></a> All the tumors that were considered in the histological differential diagnosis are predominantly composed of spindle cells and express vimentin&#46; The problem of overlapping immunohistochemical profiles seen in sarcomas is being addressed increasingly by turning to molecular genetic studies in these tumors&#46;<a class="elsevierStyleCrossRef" href="#bb0045"><span class="elsevierStyleSup">9</span></a></p><p id="p0055" class="elsevierStylePara elsevierViewall">Sarcomas of the lung are histologically indistinguishable from metastatic sarcomas&#46; Careful clinical and radiological examination is required to exclude a primary tumor elsewhere&#46;<a class="elsevierStyleCrossRef" href="#bb0025"><span class="elsevierStyleSup">5</span></a> In the long follow-up of our patient&#44; no tumors have appeared elsewhere in the body&#46;</p><p id="p0060" class="elsevierStylePara elsevierViewall">Primary pulmonary sarcomas may have an aggressive progression&#46;<a class="elsevierStyleCrossRef" href="#bb0015"><span class="elsevierStyleSup">3</span></a> Surgical removal is the treatment of choice for all histological types&#44; followed by radiotherapy of 50&#8211;60 grays in fractionated doses&#44; if the removal is incomplete&#46;<a class="elsevierStyleCrossRef" href="#bb0005"><span class="elsevierStyleSup">1</span></a> Chemotherapy with doxorubicine and ifosfamid is advised only in metastatic or advanced sarcomas&#44; but the response is less than 20 &#37;&#46;<a class="elsevierStyleCrossRef" href="#bb0010"><span class="elsevierStyleSup">2</span></a></p><p id="p0065" class="elsevierStylePara elsevierViewall">Local recurrence is frequent and is the main cause of death&#44; while distant metastases are rare&#46; The most relevant prognostic factors are tumor size at diagnosis&#44; histological grade and complete surgical removal&#46;<a class="elsevierStyleCrossRefs" href="#bb0005"><span class="elsevierStyleSup">1&#44;6</span></a> Reported median survival for these tumors is 24&#8211;36 months&#46; <a class="elsevierStyleCrossRefs" href="#bb0005"><span class="elsevierStyleSup">1&#44;5&#44;6</span></a> However&#44; with complete surgical removal of the tumor&#44; this extends to 47 months&#46;<a class="elsevierStyleCrossRef" href="#bb0005"><span class="elsevierStyleSup">1</span></a> In a series of pulmonary leiomyosarcomas&#44; high-grade tumors had a median survival of 5 months&#44; <a class="elsevierStyleCrossRef" href="#bb0050"><span class="elsevierStyleSup">10</span></a> although one patient with a high-grade tumor was well 12 years after surgery&#46; Another patient subjected a sleeve-lobectomy for a high-grade shwannoma was alive without recurrence after 25 years&#46;<a class="elsevierStyleCrossRef" href="#bb0030"><span class="elsevierStyleSup">6</span></a> Although our patient had a relatively small tumor&#44; the histological type&#44; and inability to carry out complete surgical removal should have suggested a poor prognosis&#46; The response to radiotherapy is inconsistent in literature&#44; with a few patients&#44; usually those in whom complete tumor removal was achieved&#44; responding well&#46;<a class="elsevierStyleCrossRefs" href="#bb0005"><span class="elsevierStyleSup">1&#44;6</span></a></p><p id="p0070" class="elsevierStylePara elsevierViewall">We describe a patient with a primary undifferentiated pleomorphic sarcoma of the lung&#44; whose tumor could not be resected&#44; still alive after 11 years&#46; This clinical case testifies to the importance of reporting primary sarcomas of the lung to improve the understanding of the biological behavior of this group of rare tumors&#46;</p></span></span>"
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CASE REPORT
Primary pleomorphic sarcoma of lung – 11 year survival
Sarcoma pleomórfico primário do pulmão – 11 anos de sobrevivência
M.J. Gonçalvesa,
Corresponding author
mjsagoncalves@hotmail.com

Corresponding author.
, M.M. Mendesb, F. Joãoc, J.M. Lopesd, M. Honavare
a Serviço de Infecciologia, Departamento de Medicina, Hospital Pedro Hispano – Unidade Local de Saúde de Matosinhos, EPE, Matosinhos, Portugal
b Serviço de Medicina Interna, Departamento de Medicina, Hospital Pedro Hispano - Unidade Local de Saúde de Matosinhos, EPE, Matosinhos, Portugal
c Serviço de Pneumologia, Departamento de Medicina, Hospital Pedro Hispano – Unidade Local de Matosinhos, EPE, Matosinhos, Portugal
d Departamento de Patologia, Faculdade de Medicina da Universidade do Porto, H.S. João & IPATIMUP, Porto, Portugal
e Serviço de Anatomia Patológica, Hospital Pedro Hispano – Unidade Local de Saúde de Matosinhos, Matosinhos, Portugal
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        "titulo" => "Sarcoma pleom&#243;rfico prim&#225;rio do pulm&#227;o &#8211; 11 anos de sobreviv&#234;ncia"
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    "textoCompleto" => "<span class="elsevierStyleSections"><span id="s0005" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="st0025">Introduction</span><p id="p0005" class="elsevierStylePara elsevierViewall">Primary sarcomas of the lung are rare&#44; accounting for less than 0&#46;5 &#37; of lung malignancies&#44; and their classification is not consensual in the literature&#44; largely due to changes in terminology and in diagnostic criteria that have taken place since the advent of immunohistochemistry&#46;<a class="elsevierStyleCrossRefs" href="#bb0005"><span class="elsevierStyleSup">1&#8211;5</span></a></p><p id="p0010" class="elsevierStylePara elsevierViewall">Because of their rarity&#44; the treatment of these tumors is not standardized&#59; although complete surgical removal confers the best length of survival&#46; <a class="elsevierStyleCrossRef" href="#bb0005"><span class="elsevierStyleSup">1</span></a> Tumor size and grade are additional factors that may influence the outcome&#46;<a class="elsevierStyleCrossRef" href="#bb0030"><span class="elsevierStyleSup">6</span></a></p></span><span id="s0010" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="st0030">Case report</span><p id="p0015" class="elsevierStylePara elsevierViewall">A 66 year-old retired painter and bricklayer&#44; who had recently given up smoking &#40;smoking history of 132 pack per year&#41;&#44; presented in August&#39;99 with complaints of cough with purulent sputum&#44; hemoptysis&#44; dyspnoea and fever that did not improve with a course of antibiotherapy&#46;</p><p id="p0020" class="elsevierStylePara elsevierViewall">His general condition was good&#44; but he had reduced lung sounds in the lower third of the right hemithorax&#46; The PaO<span class="elsevierStyleInf">2</span> in room air was 73<span class="elsevierStyleHsp" style=""></span>mmHg&#46; Chest X-ray showed an opacity in the lower right hemithorax&#44; and CT scan revealed an endobronchial mass causing obstruction of the lumen of the inferior lobar bronchus &#40;<a class="elsevierStyleCrossRef" href="#f0005">Fig&#46; 1</a>&#41;&#44; with an irregular&#44; approximately 3<span class="elsevierStyleHsp" style=""></span>cm&#44; extension into the lung parenchyma&#46; Sputum cytology showed malignant cells and the histology of the endoscopic bronchial biopsy revealed a malignant tumor with sarcomatous features&#46; There was no evidence of tumor elsewhere on abdominal and brain CT scans and bone scintigraphy&#46; The endobronchial tumor mass was subsequently removed with laser&#44; and histological examination showed a spindle cell tumor with marked nuclear pleomorphism&#44; multinucleated tumor giant cells and numerous mitotic figures &#40;<a class="elsevierStyleCrossRef" href="#f0010">Fig&#46; 2</a>&#41;&#46; There was no evidence of epithelial differentiation&#46; The tumor was immunoreactive for vimentin and focally for CD68&#44; and negative for multiple high and low mol&#46; wt&#46; cytokeratins&#44; epithelial specific antigen&#44; epithelial membrane antigen&#44; actin&#44; smooth muscle actin&#44; desmin&#44; S-100 protein and CD34&#46; CD117 immunostaining showed no mast cells within the tumor&#46; The cell proliferation index with Ki 67 was 60 &#37;&#46; A diagnosis of pleomorphic undifferentiated sarcoma was made&#46;</p><elsevierMultimedia ident="f0005"></elsevierMultimedia><elsevierMultimedia ident="f0010"></elsevierMultimedia><p id="p0025" class="elsevierStylePara elsevierViewall">Exploratory thoracotomy showed mediastinal invasion and radiotherapy was given to the tumor and mediastinum in a dose of 56 grays in 28 fractions&#44; resulting in disappearance of the mass&#46;</p><p id="p0030" class="elsevierStylePara elsevierViewall">The patient has been followed-up regularly for the last 11 years&#44; and has suffered recurrent respiratory infections recovering easily with antibiotherapy&#46; Serial chest CT scans &#40;<a class="elsevierStyleCrossRef" href="#f0015">Fig&#46; 3</a>&#41;&#44; showed an unaltered diffuse density in the subcarinal and right hilar regions extending to the right pulmonary artery&#44; thought to be scarring&#46; The patient has refused to undergo further bronchial endoscopy&#46;</p><elsevierMultimedia ident="f0015"></elsevierMultimedia></span><span id="s0015" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="st0035">Discussion</span><p id="p0035" class="elsevierStylePara elsevierViewall">Primary pulmonary sarcomas are rare tumors&#44; representing about 40 &#37; of &#34;rare&#34; pulmonary neoplasms and 9 &#37; of all sarcomas&#46;<a class="elsevierStyleCrossRef" href="#bb0010"><span class="elsevierStyleSup">2</span></a> To date&#44; approximately 300 cases have been reported in the literature&#46;<a class="elsevierStyleCrossRef" href="#bb0005"><span class="elsevierStyleSup">1</span></a> Sarcomas in the lungs can arise from pulmonary parenchyma&#44; the bronchi or the pulmonary artery and its branches&#46;<a class="elsevierStyleCrossRefs" href="#bb0010"><span class="elsevierStyleSup">2&#44;3</span></a> They typically occur in middle-aged individuals with slight predominance in men&#46; Prior history of radiotherapy or accidental exposure to radiation&#44; confer greater susceptibility to the development of sarcomas&#46; Symptoms and radiographic features are similar to those seen in other epithelial lung tumors and depend more on tumor localization than on their histological characteristics&#46;<a class="elsevierStyleCrossRef" href="#bb0010"><span class="elsevierStyleSup">2</span></a></p><p id="p0040" class="elsevierStylePara elsevierViewall">Primary sarcomas of the lung present a considerable diagnostic challenge to both the histopathologist and the clinician&#46; Litzsky <a class="elsevierStyleCrossRef" href="#bb0035"><span class="elsevierStyleSup">7</span></a> stresses two points&#59; most malignant spindle cell tumors in the lungs will turn out to be carcinomas with sarcomatoid differentiation and sarcomas metastatic to the lung are far more frequent than primary sarcomas&#46;</p><p id="p0045" class="elsevierStylePara elsevierViewall">The lung presents all the common histological types of primary sarcomas seen elsewhere in the soft tissues&#46; Changing terminology and histological criteria make it difficult to compare the incidence of different types of sarcoma&#46; <a class="elsevierStyleCrossRefs" href="#bb0005"><span class="elsevierStyleSup">1&#8211;7</span></a> Fibrosarcoma&#44; a common sarcoma of the lung in earlier studies&#44; <a class="elsevierStyleCrossRef" href="#bb0030"><span class="elsevierStyleSup">6</span></a> is no longer mentioned and fibrohistiocytic tumors such as malignant fibrous histiocytoma also thought to be common&#44;<a class="elsevierStyleCrossRef" href="#bb0020"><span class="elsevierStyleSup">4</span></a> are rarely encountered&#46;<a class="elsevierStyleCrossRef" href="#bb0010"><span class="elsevierStyleSup">2</span></a> The most common sarcomas now reported&#44; are leiomyosarcoma&#44; synovial sarcoma&#44; malignant peripheral nerve sheath and vascular tumors&#46;<a class="elsevierStyleCrossRef" href="#bb0035"><span class="elsevierStyleSup">7</span></a></p><p id="p0050" class="elsevierStylePara elsevierViewall">It is now accepted that along with morphological features&#44; immunohistochemistry plays an important role in the classification&#44; and the wide range of antibodies currently available means that entities previously diagnosed as fibrosarcoma&#44; malignant fibrous histiocytoma and hemangiopericytoma have now been reclassified&#46; Undifferentiated pleomorphic sarcoma&#44; previously called pleomorphic malignant fibrous histiocytoma&#44; is diagnosed only when no definable line of differentiation can be established&#44;<a class="elsevierStyleCrossRef" href="#bb0040"><span class="elsevierStyleSup">8</span></a> as in our case&#46; Immunohistochemistry is also essential for ruling out the much more frequent pulmonary sarcomatoid carcinomas&#46; In our case&#44; antibodies to high and low molecular cytokeratins&#44; epithelial specific antigen and epithelial membrane antigen ruled out sarcomatoid carcinoma&#44; synovial sarcoma and diffuse malignant mesothelioma&#44; a diagnosis that should be considered when the tumor involves the pleura or mediastinum&#46;<a class="elsevierStyleCrossRef" href="#bb0035"><span class="elsevierStyleSup">7</span></a> Synovial sarcoma is characterized by mast cells within the tumor&#44;<a class="elsevierStyleCrossRef" href="#bb0045"><span class="elsevierStyleSup">9</span></a> and none were detected in this tumor on staining with CD117&#46; Smooth muscle actin&#44; actin and desmin are seen in leiomyosarcoma&#44; and the latter two in rhabdomyosarcoma&#44; S-100 protein in malignant peripheral nerve sheath and liposarcoma&#44; while malignant solitary fibrous tumor and malignant vascular tumors are immunoreactive for CD34&#44; all of which were negative in our case&#46; CD68&#44; focally positive in our case and previously thought to be a marker for pleomorphic undifferentiated sarcoma &#40;malignant fibrous histiocytoma&#41;&#44; is no longer believed to be useful&#46;<a class="elsevierStyleCrossRef" href="#bb0040"><span class="elsevierStyleSup">8</span></a> All the tumors that were considered in the histological differential diagnosis are predominantly composed of spindle cells and express vimentin&#46; The problem of overlapping immunohistochemical profiles seen in sarcomas is being addressed increasingly by turning to molecular genetic studies in these tumors&#46;<a class="elsevierStyleCrossRef" href="#bb0045"><span class="elsevierStyleSup">9</span></a></p><p id="p0055" class="elsevierStylePara elsevierViewall">Sarcomas of the lung are histologically indistinguishable from metastatic sarcomas&#46; Careful clinical and radiological examination is required to exclude a primary tumor elsewhere&#46;<a class="elsevierStyleCrossRef" href="#bb0025"><span class="elsevierStyleSup">5</span></a> In the long follow-up of our patient&#44; no tumors have appeared elsewhere in the body&#46;</p><p id="p0060" class="elsevierStylePara elsevierViewall">Primary pulmonary sarcomas may have an aggressive progression&#46;<a class="elsevierStyleCrossRef" href="#bb0015"><span class="elsevierStyleSup">3</span></a> Surgical removal is the treatment of choice for all histological types&#44; followed by radiotherapy of 50&#8211;60 grays in fractionated doses&#44; if the removal is incomplete&#46;<a class="elsevierStyleCrossRef" href="#bb0005"><span class="elsevierStyleSup">1</span></a> Chemotherapy with doxorubicine and ifosfamid is advised only in metastatic or advanced sarcomas&#44; but the response is less than 20 &#37;&#46;<a class="elsevierStyleCrossRef" href="#bb0010"><span class="elsevierStyleSup">2</span></a></p><p id="p0065" class="elsevierStylePara elsevierViewall">Local recurrence is frequent and is the main cause of death&#44; while distant metastases are rare&#46; The most relevant prognostic factors are tumor size at diagnosis&#44; histological grade and complete surgical removal&#46;<a class="elsevierStyleCrossRefs" href="#bb0005"><span class="elsevierStyleSup">1&#44;6</span></a> Reported median survival for these tumors is 24&#8211;36 months&#46; <a class="elsevierStyleCrossRefs" href="#bb0005"><span class="elsevierStyleSup">1&#44;5&#44;6</span></a> However&#44; with complete surgical removal of the tumor&#44; this extends to 47 months&#46;<a class="elsevierStyleCrossRef" href="#bb0005"><span class="elsevierStyleSup">1</span></a> In a series of pulmonary leiomyosarcomas&#44; high-grade tumors had a median survival of 5 months&#44; <a class="elsevierStyleCrossRef" href="#bb0050"><span class="elsevierStyleSup">10</span></a> although one patient with a high-grade tumor was well 12 years after surgery&#46; Another patient subjected a sleeve-lobectomy for a high-grade shwannoma was alive without recurrence after 25 years&#46;<a class="elsevierStyleCrossRef" href="#bb0030"><span class="elsevierStyleSup">6</span></a> Although our patient had a relatively small tumor&#44; the histological type&#44; and inability to carry out complete surgical removal should have suggested a poor prognosis&#46; The response to radiotherapy is inconsistent in literature&#44; with a few patients&#44; usually those in whom complete tumor removal was achieved&#44; responding well&#46;<a class="elsevierStyleCrossRefs" href="#bb0005"><span class="elsevierStyleSup">1&#44;6</span></a></p><p id="p0070" class="elsevierStylePara elsevierViewall">We describe a patient with a primary undifferentiated pleomorphic sarcoma of the lung&#44; whose tumor could not be resected&#44; still alive after 11 years&#46; This clinical case testifies to the importance of reporting primary sarcomas of the lung to improve the understanding of the biological behavior of this group of rare tumors&#46;</p></span></span>"
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        "resumen" => "<p id="sp0020" class="elsevierStyleSimplePara elsevierViewall">Primary sarcomas of the lung are uncommon&#44; potentially aggressive neoplasms that need to be distinguished from other malignant pulmonary tumors and whose biological behavior is as yet not fully understood&#46;</p><p id="sp0025" class="elsevierStyleSimplePara elsevierViewall">Primary undifferentiated pleomorphic sarcoma was diagnosed in the right lung of a 66 year-old man&#44; who has survived over 11 years after treatment with endobronchial tumor debulking by laser therapy and radiotherapy&#46;</p>"
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        "resumen" => "<p id="sp0030" class="elsevierStyleSimplePara elsevierViewall">Os sarcomas prim&#225;rios do pulm&#227;o s&#227;o neoplasias raras e potencialmente agressivas&#44; que devem ser distinguidos de outros tumores pulmonares malignos e cujo comportamento biol&#243;gico n&#227;o est&#225; completamente esclarecido&#46;</p><p id="sp0035" class="elsevierStyleSimplePara elsevierViewall">Os autores apresentam um caso cl&#237;nico de sarcoma pleom&#243;rfico indiferenciado do pulm&#227;o&#44; que foi diagnosticado a um homem de 66 anos&#46; Na altura do diagn&#243;stico foi realizada desobstru&#231;&#227;o br&#244;nquica por laser e radioterapia&#44; posteriormente foi observada uma sobreviv&#234;ncia superior a 11 anos&#46;</p>"
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ISSN: 21735115
Original language: English
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