Multimodality treatment including surgery for primary pulmonary sarcoma: Size does matter.

BACKGROUND AND OBJECTIVES: Primary pulmonary sarcoma (PPS) accounts for less than 1.1% of all pulmonary tumors. Few outcome data are reported. We evaluated outcome and prognostic factors in our series. METHODS: We retrospectively reviewed all patients who underwent resection for PPS in our center from 2002 to 2018. Survival was calculated from the date of surgery until last follow-up. Impact on survival of gender, type of lung resection, completeness of resection, grade, size, and TNM staging for lung cancer and soft tissue sarcoma (STS) was assessed. RESULTS: Thirteen patients were included. Eight (61.5%) patients received neoadjuvant treatment. Median tumor size at diagnosis was 11.5 cm (1-30 cm). Type of lung resection was wedge (n = 2, 15%), lobectomy (n = 4, 31%), intrapericardial (n = 3, 23%), and extrapleural pneumonectomies (n = 4, 31%). In-hospital mortality was 8%. Overall 5-year survival was 60%. Median disease-free survival was 17 months. Tumor size was a predictor for survival (P = .02) and recurrence (P = .05). Gender (P = .04) and type of lung resection (P = .04) were predictors of survival. T stage for STS of trunk and extremity, and TNM stage for lung cancer were predictors for recurrence (P = .03 and P = .04, respectively). CONCLUSION: Surgical resection within a multimodality therapy concept in highly selected patients can offer good long-term outcome.

An extremely rare primary sarcoma of the lung with peritoneal and small bowel metastases: a case report.

BACKGROUND: Primary sarcoma of the lung is a very rare malignant tumor accounting for less than 0.5% of all lung tumors and presenting diagnostic and treatment challenge. We describe a case of a patient diagnosed with primary lung undifferentiated pleomorphic sarcoma developing subsequent peritoneal and small bowel metastases, which stand for highly unusual disease presentation. CASE PRESENTATION: A 57-year-old male presented with extensive partially necrotic tumor in the left upper lobe (LUL) of the lung that involved LUL bronchus and extended to the visceral pleura. There was no evidence of nodal or visceral dissemination. After initial presentation, the patient was admitted to the hospital's pulmonology department for further workup. The most likely diagnosis based on biopsy specimen was poorly differentiated sarcoma. Left pneumonectomy with mediastinal lymph node dissection was performed. The final pathohistological diagnosis (PHD) was undifferentiated pleomorphic sarcoma (UPS). Three months after lung surgery, a follow-up CT scan was done which showed a 60-mm obstructive metastatic intraabdominal lesion with small bowel infiltration and further separate peritoneal deposits. Unfortunately, an urgent surgery had to be performed as the patient developed signs of acute abdomen due to bowel perforation. Only 2 months later, the patient passed away at home. CONCLUSIONS: Treatment options of UPS are based on algorithms used in treatment of extremity lesions with well-established role of surgery. However, the role of perioperative chemotherapy remains equivocal with no strong evidence-based data due to the rarity of the disease. Small bowel is an unexpected metastatic site, but of significant clinical relevance.

Primary pulmonary angiosarcoma: A case report.

Primary sarcoma is uncommon in the lung, and primary angiosarcoma is exceedingly rare. We report a case of primary pulmonary angiosarcoma of the left lung with emphasis on its growth pattern in the lung. A 48-year-old Japanese man was admitted to our hospital because of dyspnea on exertion. He was subsequently found to have left pleural effusion. Computed tomography shows a nodular lesion measuring 7 × 4 cm in his left lung. Obstruction of the left inferior lobar bronchus was observed, and endobronchial biopsy suggested angiosarcoma. Left pneumonectomy was performed. On macroscopic examination of the cut surface, multiple nodular lesions were observed particularly in portions around branches of pulmonary artery along bronchioles. Histological examination revealed vascular channel-like structure with vague lumen formations by atypical polygonal or spindle-shaped neoplastic cells. Immunohistochemically, the neoplastic cells are positive for FLI-1, ERG, CD31 and von Willebrand factor/factor VIII-related antigen, but not CD34. Angiosarcoma is a particularly rare form of primary pulmonary tumors, and this case report describes its unique macroscopic growth pattern in the lung.

Pulmonary Sarcomatoid Carcinoma: What Makes This Rare Lung Cancer So Challenging?

Pulmonary sarcomatoid carcinoma (PSC) is a rare and aggressive subtype of non-small cell lung carcinoma (NSCLC). This case report describes a 55-year-old male with a significant smoking history who initially presented with left hemiplegia. Imaging studies revealed brain metastases and a spiculated parenchymal lung nodule in the left apical region. Histopathological examination confirmed PSC through a CT-guided biopsy. The patient's condition rapidly deteriorated, leading to death before the initiation of planned palliative chemotherapy. This report highlights the diagnostic challenges and poor prognosis associated with PSC, emphasizing the need for further research into effective treatment strategies.

SEER-Based Survival Nomogram (1998-2015) Based on 'Stage, Lymph Node Dissection, Tumor Size and Degree of Differentiation, and Therapies' for Prognosis of Primary Pulmonary Sarcoma.

Objective: Primary pulmonary sarcoma (PPS) is very rare in terms of incidence, henceforth, the clinical evidence pertinent to the prognosis of PPS is limited. The aim of this study was to construct a nomogram for evaluating the overall survival (OS) of patients diagnosed with PPS based on the stage, lymph node dissection, tumor size and degree of differentiation, and therapies. Methods: A total of 515 patients diagnosed with PPS during the period of 1998 to 2015 were obtained from the surveillance, epidemiology, and end results database and randomly segregated into 'training group' and 'validation group' with a ratio of 7:3. Regression analysis was executed for the training group to obtain the independent factors influencing prognosis of PPS patients. A nomogram was constructed as per the results obtained through multivariate Cox regression analysis subsequently validated using C index, receiver operating characteristic (ROC) curve, and calibration curves. Results: Age, tumor size, histology type, lymph node surgery, summary stage and differentiation grade were independent factors affecting the prognosis. C index was 0.775 and 0.737 for both training group, and validation group, respectively. Areas under the ROC curve of 1-year, 3-year, and 5-year OS were 87.6 (95% CI: 83.8-91.3), 90.1 (95% CI: 86.2-94.0) and 90.6 (95% CI: 85.8-95.4), respectively, in training group. Area under the curve values of 1-year, 3-year, and 5-year OS in the validation group were 83.1 (95% CI: 75.8-90.5), 82.9 (95% CI: 73.2-92.7) and 87.0 (95% CI: 75.9-98.1), respectively. Based on the nomogram, patients were segregated into low-risk group and high-risk group (degree of risk: cutoff score 193). OS of low-risk group was significantly higher when compared to high-risk group (P < .001) in the training group and validation group. Radiotherapy was a risk factor for the low-risk group and adjuvant chemotherapy has not exhibited influence on OS pertinent to low-risk group. However, adjuvant radiotherapy or chemotherapy both significantly improved the prognosis of PPS patients (P < .001) in the high-risk group. Conclusion: Constructed nomogram could have a strong predictive ability with higher accuracy for the prognosis of patients with PPS. Patients at low risk could not benefit from adjuvant radiotherapy or chemotherapy, while the prognosis clearly improved in the high-risk populations treated with either radiotherapy or chemotherapy.

Complete response to sotorasib as neoadjuvant treatment in patient with locally advanced primary pulmonary sarcoma harboring KRAS mutation: a case report.

Background: Primary pulmonary sarcoma (PPS) is very rare relative to other subtypes of lung cancer. Therefore, evidence-based treatment options for PPS patients have remained unclear. Identification of actionable cancer driver mutations in patients with non-small cell lung cancer (NSCLC) has provided the chance to use targeted treatments and improve patient clinical outcomes. In addition to epidermal growth factor receptor (EGFR), the wide use of high-throughput genomic profiling with next-generation sequencing (NGS) has also identified other cancer driver genes such as Kirsten rat sarcoma (KRAS), human epidermal growth factor receptor 2 (HER2), and mesenchymal epithelial transition (MET). Case Description: In our study, we reported a locally advanced PPS patient harboring KRAS G12C mutation. The clinical stage before neoadjuvant treatment was stage IIIB (c.T3N2M0). The direct KRAS G12C inhibitor sotorasib (AMG-510) was used as neoadjuvant treatment and the patient achieved complete response (CR). Then, the patient underwent video-assisted thoracoscopic surgery (VATS) with reserved spontaneous breathing for surgical resection. The pathological evaluation was indicative of pathological CR (pCR). Further follow-ups are required to evaluate the long-term clinical benefit of neoadjuvant treatment with sotorasib and surgical resection with VATS. Conclusions: To our knowledge, it was the first study to use sotorasib for a PPS patient harboring KRAS G12C mutation in a neoadjuvant setting. Further follow-ups are required to evaluate the long-term clinical benefit of neoadjuvant treatment with sotorasib and surgical resection with VATS.

Rare Case of Primary Pulmonary Pleomorphic Liposarcoma Treated With Multimodal Therapy.

Background: Pleomorphic liposarcoma (PLS) is a very rare type of primary pulmonary sarcoma. Clinical data about these tumors are limited, and optimal treatment has not yet been defined. Case Report: A 32-year-old male presented with pain and heaviness over the right chest. Contrast-enhanced computed tomography (CECT) of the chest showed a heterogeneous hypodense pleural-based mass and a mediastinal mass. The patient was treated with chemoradiotherapy, followed by excision and adjuvant chemotherapy. Seven months after completion of treatment, he presented with an abdominopelvic mass and soft tissue peritoneal deposits. The mass was resected, and second line chemotherapy resulted in a partial response. The patient was routinely followed. Six months after completion of the second round of chemotherapy, CECT showed multiple soft tissue deposits in the right lumbar region, right hemipelvis, and presacral region with no evidence of pulmonary disease. Chemotherapy elicited a partial response. Three years from the date of diagnosis, the patient was alive with stable disease. Conclusion: This case is unique because of the rare primary site of PLS presentation and the rare presentation of peritoneal metastasis. Citing such cases would help us to define adequate treatment protocols for this aggressive tumor.

Angiosarcoma of the pulmonary artery mimicking pulmonary thromboembolism.

Primary pulmonary angiosarcomas (PPAs) are rare, and often, their diagnosis is delayed because of insidious clinical symptoms and imaging findings mimicking pulmonary thromboembolism (PE). A 33-year-old female patient presented with chest pain and progressive dyspnoea. Her initial diagnosis, based on clinical symptoms and CT pulmonary angiography (PA) findings, was PE. However, after treatment with anticoagulants, the patient failed to improve. A follow-up CTPA and further CT-guided biopsy results were compatible with angiosarcoma.

A Paraneoplastic Leukemoid Reaction in Primary Lung Sarcoma.

Pulmonary malignancies are known to have high prevalence and mortality. They are associated with different paraneoplastic syndromes, especially pulmonary carcinomas, because they are more common than pulmonary sarcomas. We present a case of a 56-year-old African American male who was admitted to our institution with a three-month history of a dry cough, progressive shortness of breath, and two to three days of right arm swelling. He had extreme leukocytosis (WBC count of 106,500 cells/mm3). Computed tomography (CT) scan of the thorax demonstrated an irregular, thick-walled 14-cm lung mass occupying the middle and upper hemithorax. CT-guided biopsy of the mass confirmed the diagnosis of lung sarcoma.

Primary lung sarcoma mimicking left atrial myxoma.

A case of left atrial myxoma, diagnosed on echocardiography in the emergency department, was referred for open heart surgical intervention. On reviewing chest X-ray, right lung, lower lobe whiteout was noted which instigated the need to perform a computed tomography (CT) chest. On CT chest, a heterogenously hypodense mass was seen along the posterior and inferior aspect of the right hemithorax. It had ill-defined planes with left atrium and extension into the left atrium through the right inferior pulmonary vein. On the basis of imaging, the etiology was sarcomatous/malignant vascular mass of pulmonary origin and unnecessary open-heart surgery was averted. The patient was transferred to the oncology department for further management.

Results of treating primary pulmonary sarcomas and pulmonary carcinosarcomas.

OBJECTIVE: Primary pulmonary sarcomas (PPS) and pulmonary carcinosarcomas (PCS) are rare aggressive lung malignancies. We reviewed our 21-year experience with the surgical and nonsurgical treatment of both tumors, comparing their clinical, histopathologic, and treatment results. METHODS: All patients with PPS or PCS who underwent surgical and nonsurgical treatment between 1998 and 2019 at our cancer center were retrospectively reviewed. Multivariable Cox proportional hazards model was constructed. RESULTS: In total, 100 patients were analyzed: 45 with PPS and 55 with PCS. Among patients with PPS, 31 of 45 (69%) underwent surgery with 1 (3%) operative mortality. For patients with PCS, 29 of 55 (53%) underwent surgery with no operative mortality. Patients with PPS were younger than PCS (P < .01). Fewer patients were smokers among PPS (58%) versus PCS (93%) (P < .01). For resected PPS, mean tumor size was 8.2 ± 4.1 cm (range 2.2-18.0) compared with 10.1 ± 5.0 cm (range 3.9-17.0) for unresected PPS. Tumor size for resected PCS was 6.2 ± 2.6 cm (range 2.0-10.5) versus 6.8 ± 3.5 cm (range 1.2-13.5) for unresected PCS. Of resected patients, 5 of 31 (16%) with PPS and 9 of 29 (31%) with PCS were node positive. Overall survival estimates were as follows: for PPS, median survival and 5-year overall survival for resected versus unresected cases were 39.6 months/28.7% versus 4.9 months/7.8%. For PCS, survival estimates were 23.6 months/31.0% versus 14.9 months/28.2%, respectively. In multivariable analyses (N = 100), age, smoking history, histology, and surgery were risk factors of survival. CONCLUSIONS: At initial evaluation, PPS and PCS presented with large-sized tumors and usually were not stage I. Surgery had a positive impact on survival among patients with PPS. Whenever feasible, surgical resection, even in locally advanced disease, may yield long-term survival in these aggressive lung tumors, although the level of evidence is low.

[Primary pulmonary myxoid sarcoma associated with translocation EWSR1-CREB1 and locorregional micrometastases]. / Sarcoma pulmonar mixoide primario asociado a traslocación EWSR1-CREB1 con micrometástasis locorregional.

Primary myxoid pulmonary sarcoma is a rare entity with an endobronchial growth. Although it should be included in the differential diagnosis of other sarcomas, its prognosis is usually favorable. We present the case of a 51-year-old patient with a mesenchymal tumor in the right lung, diagnosed as a primary pulmonary myxoid sarcoma, positive for EMA, vimentine and with a Ki-67 less than 5%; FISH revealed a EWSR1-CREB1 translocation.

Primary Pulmonary Synovial Sarcoma in a 49-Year-Old Male.

Sarcoma is a malignant tumor arising from the mesenchymal tissues such as striated skeletal and smooth muscles, adipose tissue, bone, cartilage, and synovial tissue. The synovial subset of primary pulmonary sarcoma is very rare and is only described in a handful of cases. Our case describes the diagnostic approach and treatment regimen for a 49-year-old male with no significant past medical history. The diagnosis of a primary pulmonary synovial sarcoma was made through the use of endobronchial ultrasound (EBUS), transbronchial needle aspiration (TBNA), histologic analysis, and immunostaining. The treatment utilized a multimodal approach including resection, chemotherapy, and radiotherapy.

Primary Sarcoma of the Lung - Prognostic Value of Clinicopathological Characteristics of 26 Cases.

BACKGROUND/AIM: Primary sarcomas of the lung (PSL) represent a rare, largely unknown entity. We herein present a retrospective study of 26 patients diagnosed with PSL. PATIENTS AND METHODS: For a period of 10 years, the records of patients from 5 centers were gathered and analyzed. RESULTS: Median age at diagnosis was 61.96 years (range=31-75 years). Eight patients (33.33%) had mediastinal node invasion (MNI). From 17 patients (70.83%) with localized disease, 11 patients (64.70%) underwent surgery. Recurrence rate was 72.72%. Median disease-free interval was 15 months. The median overall survival (OS) of patients with metastatic disease was 4 months and 10 months for the whole population. Only surgery had an impact on survival. CONCLUSION: Prognosis of PSL is somber. The high proportion of patients with MNI at diagnosis may serve as an indication for surgical evaluation of mediastinum and raises the question whether patients with locoregional PSL should be treated with a more aggressive approach.

Successful Multidisciplinary Treatment for Aggressive Primary Pulmonary Undifferentiated Pleomorphic Sarcoma.

Undifferentiated pleomorphic sarcoma (UPS) was previously known as malignant fibrous histiocytoma (MFH). This sarcoma occurs preferentially in the extremities and retroperitoneal space; primary pulmonary UPS/MFH is rare. We report a 52-year-old woman referred to our hospital with dyspnea and severe cough. Chest computed tomography (CT) revealed a pulmonary mass in the left upper lobe and pleural effusion. Cytology of the effusion showed no malignancy; however, the tumor increased rapidly in size, and the patient's respiratory symptoms worsened. The tumor occupied almost all of the left upper lobe and involved the adjacent pericardium. She underwent left upper lobectomy with pericardial resection and reconstruction. Postoperative pathology of the resected specimen showed undifferentiated pulmonary sarcoma, pT4N0M1a stage IV A, and genetic analyses revealed the v-Ki-ras2 Kirsten rat sarcoma viral oncogene homolog (KRAS) mutation. The patient's dyspnea recurred 1 month postoperatively, and CT showed marked pleural effusion. An 18F-fluorodeoxyglucose positron emission tomography demonstrated abnormal diffuse accumulation of 18F-fluorodeoxyglucose in the left pleural cavity. We initiated five cycles of chemotherapy with doxorubicin and ifosfamide, and the patient has been well without recurrence for 24 months after multidisciplinary treatment with surgery followed by systemic combination chemotherapy. We successfully treated our patient with primary pulmonary UPS/MFH using a multidisciplinary approach, even though this sarcoma carries a poor prognosis and is insensitive to both chemotherapy and radiotherapy.

Disnea típica en un paciente con diagnóstico atípico / Typical dyspnea in a patient with an atypical diagnosis

RESUMEN El sarcoma primario de arteria pulmonar es un tumor poco frecuente y con muy mal pronóstico. Su incidencia es difícil de estimar. Presenta una sintomatología inespecífica que dificulta y retrasa su diagnóstico. Presentamos un paciente de 62 años diagnostica do inicialmente de tromboembolismo pulmonar con una clínica discordante. Se decide continuar el estudio, que confirma posteriormente el diagnóstico de sarcoma primario de arteria pulmonar mediante biopsia.

ABSTRACT Primary pulmonary artery sarcoma is a rare tumor with a very poor prognosis. Its inci dence is hard to estimate. It presents with nonspecific symptoms, delaying the diagnosis and making it challenging. We present a 62-year-old patient initially diagnosed with pulmonary thromboembolism, exhibiting discordant symptoms. Further investigation was pursued, ultimately confirming the diagnosis of primary pulmonary artery sarcoma through biopsy.

Primary pulmonary sarcoma - long-term treatment outcomes and prognostic factors.

INTRODUCTION: Primary pulmonary sarcoma (PPS) is a rare type of non-epithelial malignant tumour of the lungs. Lung sarcomas are usually of metastatic character, originating from the primary tumour located in another organ. Pathomorphological diagnosis is difficult and therefore definitive diagnosis is usually determined after surgical treatment. Five-year survival of radical operated patients is 42-69%. AIM: To evaluate prognostic factors in PPS. MATERIAL AND METHODS: Twenty-two patients who underwent surgical treatment between 1994 and 2009 were analysed. RESULTS: Ten types of sarcomas were identified. All 22 analysed patients underwent operations and in 14 (63.6%) cases it was R0 resection (pneumonectomies, lobectomies, non-anatomical resections). In 8 out of 22 patients a locally advanced form was identified. Median overall survival (OS) in the group of all patients was 22 months, 5- and 10-year OS was 27% and 18% respectively, whereas in patients who underwent non-anatomical lung resections it was 80% and 60% respectively with median OS of 213 months. Overall survival was dependent on gender, completeness of treatment and size of tumour. Haemoptysis was a poor prognostic factor. Primitive neuroectodermal tumour was a type of sarcoma with a negative effect on progression-free survival. No impact of age, type of sarcoma, degree of histological malignancy, stage or adjuvant treatment on OS was noted. CONCLUSIONS: Primary radical surgical resection prolongs patient survival. Patients with tumours larger than 5 cm and men have worse prognoses. Future studies conducted on larger groups of patients diagnosed with PPS are necessary.

Incidence of pulmonary non-epithelial tumors: 18 years' experience at a single institute.

Primary and metastatic non-epithelial tumors of the lung and pleura (NETs-LP) are presumed to represent a small proportion of pulmonary neoplasm, and their epidemiologic characterization is lacking. Moreover, classification of pulmonary mesenchymal tumor according to 'WHO Classification of Tumors of Lung and Pleura' (2004) is simple and necessary to give a supplementation like new 'WHO Classification of Tumors of Soft Tissue and Bone' (2013). Non-epithelial tumors of the lung and pleura diagnosed from January 1995 to April 2013 were retrospectively reviewed and classified in accordance with 'WHO Classification of Tumors of Soft Tissue and Bone' (2013). Of 681 cases represented in the study, smooth muscle tumor (13.1%) was most common, and tumor of uncertain differentiation (10.5%) and fibroblastic/myofibroblastic tumor (5.7%) were followed. Smooth muscle tumor (12.6%) was the common primary tumor, whereas chondrogenic or osteogenic tumor (21.6%) was common in metastatic ones. Pulmonary vascular sarcoma (n=15) was the most frequent primary pulmonary sarcoma with following synovial sarcoma (n=9) and malignant type of solitary fibrous tumor (n=9). This study is the first trial of epidemiologic investigation in Korea about NETs-LP, indirectly representing their incidence in Korea and northeast Asia, and we hope that this presentation offers guidance in further study of NETs-LP.