Primary Pulmonary Leiomyosarcoma Research Articles

Primary Pulmonary Leiomyosarcoma : an Extremely Rare, Difficult-to-Manage Case of Lung Cancer

Background: Primary Pulmonary Leiomyosarcoma (PPL) known as <0.5% of total lung cancer. Histopathological examinations are the pillars of PPL diagnosis as clinical manifestations and radiological features are usually not specific. Treatments of PPL include surgical resection, radiotherapy, and chemotherapy.
 Case Illustration: A 51-year-old male, smoker, with a productive cough for 2 weeks, accompanied by chest pain, weight loss, and loss of appetite. Imaging studies showed a solid mass in the right lung, which was confirmed through bronchoscopy, cytology, histology, and immunohistochemistry evaluations. Bronchoscopy showed an intraluminal mass in the right main bronchus suggesting malignancy. Immunohistochemistry of Desmin and Smooth Muscle Actin, which were positive, confirmed the diagnosis of PPL of the right lung stage T4N1M1a IVA.
 Discussion: Surgical resection is the gold standard treatment for PPL whose clinical conditions and tumor spread still allowed for safe operation. Surgical resection did not undergo because already in stage IVA. Radiotherapy and chemotherapy can be added for patients who are unable to have surgery. There are no guidelines regarding chemotherapeutic regimens that are recommended for PPL. Cisplatin/carboplatin and etoposide are two chemotherapeutic agents that are commonly used in other variants of lung cancer. Cisplatin is successful in 5-23% of patients and etoposide is successful in 8%. Doxorubicin and ifosfamide are usually used for soft tissue sarcoma too. After administration of 4 cycles of carboplatin/etoposide, a chest CT scan with contrast recist shows the progression of PPL.
 Conclusion: Carboplatin and etoposide have been long used as therapy for lung cancer. Currently, available literature shows that their effectiveness in PPL is still considered low. Nevertheless, more studies are needed to further explore the possibilities of using carboplatin and etoposide in PPL patients.
 
 Keywords: chemotherapy, lung cancer, primary pulmonary leiomyosarcoma

Rare lung cancers—Primary pulmonary leiomyosarcoma: A case report

SummaryPrimary pulmonary sarcomas (PPS) are rare mesenchymal lung cancers, which do not present clinically or radiological different to lung carcinomas. Definite PPS diagnosis can only be made by histological analysis and detailed staging examinations in order to exclude a secondary pulmonary malignancy such as metastatic soft tissue sarcoma or another solid tumour. Here we present the case of a 66-year-old woman with a pulmonary mass infiltrating the diaphragm and the mediastinal adipose tissue, which was identified as leiomyosarcoma. The patient received curative surgery with complete tumour R0 resection. The prognosis of PPS is defined by tumour size, lymph node status and histological grading. Surgery is the mainstay of therapy and there is no definitive indication for adjuvant therapy for R0-resected and lymph-node-negative patients like in our case. However, multimodal therapy approaches such as (neo)adjuvant chemo- and radiotherapy can contribute to improving locoregional tumour control, which is the most important prognostic factor. With our case report we want to raise awareness for pulmonary sarcomas as a relevant proportion of rare lung cancers which have to be kept in mind during the differential diagnosis. Moreover, we aim to discuss the complex and individual interdisciplinary management.

A case of primary pulmonary leiomyosarcoma completely resected after neoadjuvant chemotherapy

BackgroundPrimary pulmonary leiomyosarcoma is a rare malignant tumor. We herein report a case of primary pulmonary leiomyosarcoma that was completely resected by surgery after neoadjuvant chemotherapy.Case presentationA 60-year-old man presented with cough. Chest computed tomography showed an 11-cm mass in the right upper lobe of the lung that had invaded the superior vena cava. Endobronchial ultrasound-guided transbronchial needle aspiration revealed leiomyosarcoma of the lung. We considered complete resection of the tumor to be very difficult because of the tumor invasion into the right atrium inflow of the superior vena cava, so we performed chemotherapy using doxorubicin for five cycles. After chemotherapy, the tumor size decreased to 5.6 cm, and we performed right upper lobectomy with combined resection of the superior vena cava. The tumor was completely resected by surgery. The patient is alive without recurrence 17 months postoperatively.ConclusionsWe encountered a case of primary pulmonary leiomyosarcoma that was successfully treated by surgery after neoadjuvant chemotherapy. Doxorubicin monotherapy was effective in this case. Surgery combined with neoadjuvant chemotherapy should be considered for such cases, as a long-term survival can be achieved by complete resection of primary pulmonary leiomyosarcoma.

Bilateral Primary Pulmonary Leiomyosarcoma: A Case Report

Introduction: Primary pulmonary leiomyosarcomas are a group of rare malignant tumors. They are subdivided into those originating from pulmonary parenchyma, bronchial tree, or pulmonary arteries. They tend to expand locally, and presentation depends on the site of the tumor. Diagnosis can be challenging. Case Presentation: A 40-year-old woman presented with dyspnea and severe sepsis due to a lung mass and postobstructive pneumonia. She was diagnosed as having bilateral endobronchial primary pulmonary leiomyosarcoma and treated with bilobectomy; however, she died shortly after surgery due to complications. Conclusion: Considering the rarity of her condition, this case offers a unique opportunity to investigate its presentation, diagnosis, and treatment strategies through a study of the literature.

Primary Pulmonary Leiomyosarcoma: A Case report

Primary Pulmonary Leiomyosarcoma: A Case report

Primary pulmonary leiomyosarcoma-case report of a rare aggressive neoplasm.

Primary pulmonary leiomyosarcoma-case report of a rare aggressive neoplasm.

Pulmonary artery leiomyosarcoma: A clinical dilemma

Leiomyosarcomas are rare neoplasms of the smooth muscles. Primary pulmonary leiomyosarcomas, which constitute approximately 0.2%–0.5% of all primary lung malignancies, are extremely rare and highly lethal. They may originate from the smooth muscle cells of the bronchial wall, the blood vessels, or the pulmonary interstitium, and their rare occurrence, localization, and nonspecific clinical symptoms mean that correct diagnosis and proper management are often delayed. Here, we report a rapidly growing primary pulmonary leiomyosarcoma, which invaded the right atrium, vena cava superior, mediastinum, right hilar area, and left pulmonary artery within 4 months. On histopathology, a transthoracic needle biopsy of the mass confirmed leiomyosarcoma, and delayed presentation meant that there was a local spread to the neighboring structures at the time of diagnosis.

Primary pulmonary leiomyosarcoma: A population-based study

ObjectivesPrimary pulmonary leiomyosarcoma (PPL) is a rare diagnosis with poorly understood clinicopathological characteristics and disease progression. What we know is derived from limited case reports, so identification of features associated with patient survival is warranted. Materials and methodsA population cohort study was conducted using prospectively extracted data from the Surveillance, Epidemiology and End Results (SEER) database for patients with histological diagnoses of PPL from 1973 to 2013 including demographic, treatment and outcome data. ResultsA total of 231 patients with PPL were included (mean 65.8 ± 14.9 years-of-age). 43 were categorized with a regional stage, and 60 had localized stage, whereas 70 had distant stage. Most (60.4%) patients received cancer-directed surgery, and radiotherapy was performed for 14.1% of patients. Median overall survival (OS) was 14.0 months, and 1-, 3- and 5-year OS for PPL patients was 52.7%, 29.0% and 22.2%, respectively. Cancer-directed surgery significantly improved median OS for patients with PPL by almost 29.0 months (33.0 vs 4.0 months; p < .01). Multivariate Cox analysis showed that distant stage was independent prognostic factors for PPL (HR = 2.22, 95% CI 1.30–3.79). Conversely, cancer-directed surgery was an independent protective factor and this decreased risk of death by 57% (HR = 0.43, 95% CI 0.27–0.67) for patients with PPL. ConclusionPPL is a rare pathological lung cancer, surgical resection and distant stage were associated with prognosis. In order to understand PPL more thoroughly, more cases with adequate information are required.

PRIMARY PULMONARY LEIOMYOSARCOMA WITH BRAIN METASTASIS IN A 38‐YEAR OLD FILIPINO FEMALE

RespirologyVolume 22, Issue S3 p. 188-188 APSR Poster Abstracts PRIMARY PULMONARY LEIOMYOSARCOMA WITH BRAIN METASTASIS IN A 38-YEAR OLD FILIPINO FEMALE First published: 23 November 2017 https://doi.org/10.1111/resp.13207_262Citations: 1Read the full textAboutPDF ToolsRequest permissionExport citationAdd to favoritesTrack citation ShareShare Give accessShare full text accessShare full-text accessPlease review our Terms and Conditions of Use and check box below to share full-text version of article.I have read and accept the Wiley Online Library Terms and Conditions of UseShareable LinkUse the link below to share a full-text version of this article with your friends and colleagues. Learn more.Copy URL Share a linkShare onFacebookTwitterLinkedInRedditWechat No abstract is available for this article.Citing Literature Volume22, IssueS3Supplement: 22nd Congress of the Asian Pacific Society of Respirology, International Convention Centre, Sydney, Australia, 23–26 November 2017November 2017Pages 188-188 RelatedInformation

Primary pulmonary leiomyosarcoma: a case report

Primary pulmonary leiomyosarcoma (PPL) is a very rare tumour. Presentation depends on the site of origin of tumour. Early detection and complete resection result in long-term survival. Complete surgical resection offers the only chance of cure. The role of radiotherapy or chemotherapy is not established.

10: A lung nodule unveiling a rare primary pulmonary leiomyosarcoma

10: A lung nodule unveiling a rare primary pulmonary leiomyosarcoma

Primary pulmonary leiomyosarcoma: A case report.

Primary pulmonary leiomyosarcoma (PPL) is an extremely rare malignant tumor. It has been revealed that PPL may originate from the smooth muscle of the pulmonary parenchyma, pulmonary arteries and bronchi. Patients with PPL may be asymptomatic or present with symptoms similar to those observed in other primary lung tumors. The present study reports the case of a 48-year-old man who presented with a lung mass and underwent a right upper-middle lobe bronchoscope tumor resection. The patient was subsequently diagnosed with PPL. Following the bronchoscopic tumor resection, chemotherapy was administered to the patient; however, the patient succumbed to the disease after the second cycle of chemotherapy.

Primary Pulmonary Leiomyosarcoma: Could It Be Cured by Radiation Therapy?

Primary leiomyosarcoma of lung is extremely rare and often diagnosed as a mass on routine chest radiography. Although advances have been made in treatment protocols, leiomyosarcoma remains one of the more difficult soft tissue sarcomas to treat. Surgical resection is usually curative for small and well-differentiated sarcomas. For poorly differentiated and non-resectable tumors, chemotherapy and radiation therapy are used as neoadejuvant or palliative treatment options. Generally, leiomyosarcomas are known to be resistant to radiation therapy alone. The authors experienced a 68-year-old woman who was diagnosed leiomyosarcoma by routine chest radiography. Although disease progression was observed despite of chemotherapy, radiation therapy reduced the size of tumor. This paper describes the curative potential of radiation therapy for primary pulmonary leiomyosarcomas through a case report and literature review. Key words: Leiomyosarcoma; Radiotherapy; Chemotherapy

Primary Pulmonary Leiomyosarcoma Treated with Chemotherapy and Surgery

Primary pulmonary leiomyosarcomas are a rare malignant tumor with generally poor prognosis. They pose important problems of differential diagnosis especially with pulmonary metastatic sarcoma. They are subdivided into two categories those originating from pulmonary parenchyma and those originating from pulmonary arteries. The authors describe a case of 54-year-old woman that had her left upper lobe mass checked. The diagnosis was made based on the microscopic examination of the percutaneous trans-thoracic needle biopsy. The general examination did not show any metastatic lesions in other areas. The treatment was based on chemotherapy followed by a surgical resection. The patient did not experience recurrences or complications.

Primary Pulmonary Leiomyosarcoma With Cardiac Metastases

Primary Pulmonary Leiomyosarcoma With Cardiac Metastases

Primary pulmonary leiomyosarcoma

Primary pulmonary leiomyosarcoma (PPL) is an extremely rare malignant tumor. In the case presented here, a 52-year-old Chinese female with a lung mass underwent a right upper-middle lobectomy with pulmonary artery sleeve resection and reconstruction, and was thereafter diagnosed with PPL. After 28 months, the patient was well and without local recurrence or distant metastasis.

Primary Pulmonary Leiomyosarcoma

Primary Pulmonary Leiomyosarcoma

小児に発生した肺原発平滑筋肉腫の1切除例

症例は10歳，男児．学校検診で心雑音を指摘され，近医を受診し，胸部X線写真で右上肺野に2 cm大のcoin lesionを指摘された．8ヵ月の経過観察の後，2.6 cm大に増大したため，当科紹介受診となった．胸部CTで右S1に26×24 mm大の境界明瞭，辺縁平滑で内部均一な結節影を認め，MRIでT1低信号，T2軽度低信号を呈し，肺腫瘍を疑った．経皮針生検で腫瘍は細胞異型の乏しい紡錘形細胞からなり，desminに強陽性，細胞分裂像は1/10HPFsで平滑筋腫と診断されたが，FDG-PETでSUVmax 8.7と高集積を認めたため，悪性腫瘍を疑い，胸腔鏡下に右上葉切除＋ND2a.1を行った．術後病理診断は低悪性度の平滑筋肉腫であった．肺原発平滑筋肉腫の本邦報告例のうち男性に発症したものとしては最年少と考えられる1例を経験したので報告する．

気管支内腔に進展した肺平滑筋肉腫の1手術例

気管支内腔に進展した肺平滑筋肉腫の1手術例

Primary Pulmonary Leiomyosarcoma—A Case Report

Sarcomas are mesenchymal tumors that originate from the stromal elements of the bronchial wall or from interstices of lung parenchyma. Pulmonary sarcomatous neoplasms are a rare and diagnostically challenging group of tumors. They constitute only 0.2%–0.5% of all primary lung malignancies. Primary pulmonary leiomyosarcomas are subdivided into those originating from pulmonary parenchyma, bronchial tree or pulmonary arteries. Here we present a case of 43-year-old African-American female with chronic cough, fatigue and weight loss. Early detection is the key to the successful management of these patients. The available treatment option is complete resection of tumor. These cases provide an interesting juxtaposition to the management of typical lung cancer.