Devil in the Dedifferentiated Details: A Rare Case of Primary Pulmonary Dedifferentiated Liposarcoma.

Primary liposarcoma of the lung is an exceedingly rare phenomenon, with fewer than ten cases reported in prior literature as of 2024. Rarer still, dedifferentiated liposarcoma of pulmonary origin is even less frequently reported, with only two cases having been described. Here, we detail the case of a 66-year-old female who presented with a large left-sided obstructing lung mass and underwent bronchoscopy with tumor cryoprobe debulking. Histological examination revealed a spindle cell sarcoma corresponding to a dedifferentiated liposarcoma with leiomyosarcomatous features as murine double minute 2 translocation was identified by fluorescence in situ hybridization and desmin by immunohistochemistry. The patient completed multiple radiotherapy sessions followed by pneumonectomy and a relatively uncomplicated postoperative course. As pulmonary liposarcoma tends to be poorly responsive to typical chemotherapy, standard management of pulmonary liposarcoma in the absence of metastases is surgical intervention to improve patient survival. Thus, we maintain that pulmonologists, oncologists, and radiologists should have a heightened awareness of this unique pathology in patients presenting with solitary lung mass.

A rare phenomenon, primary pulmonary smooth muscle tumor and its management.

Smooth muscle tumors of uncertain malignant potential, the borderline tumors arising from the smooth muscle cells, usually grow slowly and do not fulfill the diagnostic criteria of leiomyosarcoma and its variants, but may behave in a malignant manner. A 15-year-old female patient with an endobronchial mass in the left main bronchus on thoracic computed tomography underwent thoracotomy and tracheobronchoplasty with a wide and safe margin. Histopathological evaluation revealed a smooth muscle tumor of uncertain malignant potential. There were no complications related to the operation during the hospital stay. At six months of surgery, there were no symptoms or signs suggesting any recurrence in her follow-up. In conclusion, In conclusion, pulmonary leiomyomas may rarely present as an endobronchial mass and may mimic asthma by causing respiratory symptoms developing as attacks due to displacement of the mass within the lumen.

A Rare Case of Pulmonary Leiomyosarcoma.

Leiomyosarcoma commonly occurs in the abdomen, retroperitoneum, large blood vessels, and uterus[1]. Cardiac leiomyosarcoma is a rare and highly aggressive sarcoma. We reported a case of a 63-year-old male with pulmonary artery leiomyosarcoma. Transthoracic echocardiography showed a large 4.4×2.3 cm hypoechoic mass in the right ventricular outflow tract and pulmonary artery. Computed tomography pulmonary angiography showed a filling defect in a similar location. The initial impression was PE, but a tumor was not ruled out. An emergency surgery was performed due to progressively worse chest distress and dyspnea. A yellow mass that had adhered to the ventricular septum and pulmonary artery wall was detected to be compressing the pulmonary valve. Immunohistochemistry confirmed tumor cells positive staining for Desmin and smooth muscle actin and negative staining for S-100, CD34, myogenin, or myoglobin, and KI67(+)80%, indicating leiomyosarcoma. Pulmonary leiomyosarcoma showed a side-inserted heart chamber filling defect in CTA and should be excised when the patient suddenly deteriorated.

Diagnosis of pulmonary leiomyosarcoma extending into the main bronchus using repeated transbronchial cryobiopsy.

The diagnosis of pulmonary leiomyosarcoma using bronchoscopy is difficult, and surgical resection is often performed for definitive diagnosis and curative therapy. We report a case of pulmonary leiomyosarcoma, successfully diagnosed using repeated transbronchial cryobiopsy (TBCB). A 69-year-old-woman was found to have an oval mass in the left hilar region extending into the left main bronchus on computed tomography (CT). All transbronchial biopsy specimens were necrotic, but repeated TBCB removed the necrotic tissue from the tumour and finally led to the diagnosis of pulmonary leiomyosarcoma. Proton therapy was administered, which caused shrinkage of the tumour. Thus, TBCB is useful for definitive diagnosis of leiomyosarcoma without surgical biopsy. Repeated TBCB can reduce tumour volume, eliminate atelectasis, and reduce the extent of radiotherapy exposure.

Pulmonary leiomyosarcoma arising in pulmonary hamartoma: an exceptional occurrence in a rare tumor.

A solitary peripheral lung nodule was found in the left lung of a 52-year-old man. It was located in the lower lobe and measured 18.5 cm of major axis on chest computed tomography. A tru-cut core biopsy was obtained and a proliferation of bland, monomorphic, spindle cells in interlacing fascicles was observed. Accordingly, a surgical resection of the neoplasm was subsequently carried out. Macroscopically, the tumor appeared as a well-circumscribed nodule with a firm and whitish cut surface. Histologically, the neoplasm was predominantly composed of bland and monomorphic spindle cells, with a predominantly fascicular growth pattern, in which many tubular and cleft-like spaces of entrapped normal respiratory epithelium were involved. Myxoid change, stromal hyalinization and scattered bizarre mononucleated and multinucleated cells were also observed. Based on clinico-morphological, immunophenotypical and molecular features, we made a diagnosis of malignant transformation of pulmonary adenoleiomyomatous hamartoma into pulmonary leiomyosarcoma. As far as we know, this is the first described case of this exceptionally rare occurrence in an already rare neoplasm.

Primary pulmonary leiomyosarcoma with coexistent pulmonary aspergillosis: a case report.

Coexisting primary pulmonary leiomyosarcoma (PPL) with pulmonary Aspergillosis in immunocompetent patients is a rare occurrence. Here, we presented a 54-year-old woman presented with a dry cough for two months. Bronchoscopy revealed pulmonary aspergillosis. The patient was treated with antifungal therapy for one month without improvement. To evaluate further, a chest computed tomography (CT) scan showed a large heterogeneous enhancing mass in the lower lobe of the left lung with left atrium thrombosis. Computed tomography-guided biopsy was performed, and histopathology demonstrated the diagnosis of PPL. The metastasis workup staging showed multiple metastases in vertebrae, scapula, rib, and liver. The patient was treated with chemotherapy followed by tumor bed radiotherapy. Unfortunately, her general condition worsened, and she passed away with overall survival of fourteen months. In conclusion, clinicians should be alert to underlying malignant disease if airway Aspergillus infection is suspicious in patients without strong risk factors for invasive fungal infection.

Lung metastases and subsequent malignant transformation of a fumarate hydratase -deficient uterine leiomyoma.

Uterine leiomyomas, or fibroids, are very common smooth muscle tumors. Their potential to metastasize or transform into leiomyosarcomas is extremely low. Here, we report a patient who underwent hysterectomy due to a large leiomyoma and who was diagnosed with pulmonary tumors seven and nine years later. Histopathological re-evaluation confirmed the cellular leiomyoma diagnosis for the uterine tumor, whereas the pulmonary tumors met the diagnostic criteria of a leiomyosarcoma. Whole-exome sequencing revealed very similar mutational profiles in all three tumors, including a somatic homozygous deletion in a rare, but well-established leiomyoma driver gene FH. Tumor evolution analysis confirmed the clonal origin of all three tumors. In addition to mutations shared by all three tumors, pulmonary tumors harbored additional alterations affecting e.g. the cancer-associated genes NRG1 and MYOCD. The second pulmonary leiomyosarcoma harbored additional changes, including a mutation in FGFR1. In global gene expression profiling, the uterine tumor showed similar expression patterns as other FH-deficient leiomyomas. Taken together, this comprehensive molecular data supports the occasional metastatic capability and malignant transformation of uterine leiomyomas. Further studies are required to confirm whether FH-deficient tumors and/or tumors with cellular histopathology have higher malignant potential than other uterine leiomyomas.

A case of pulmonary leiomyosarcoma achieving long-term survival after multiple metastasectomies.

A 58-year-old man who underwent lower lobectomy of the right lung for primary pulmonary leiomyosarcoma (PPL) 4 years ago presented with epigastric pain and was diagnosed with small bowel intussusception caused by an intestinal mass. Partial resection of the small intestine was performed, and pathological examination revealed metastatic leiomyosarcoma. Masses in the left adrenal gland, subcutaneous tissue of the left upper arm, right pleura, jejunum, right trapezius muscle, and right adrenal gland were subsequently detected in the following 4 years. Resection was performed for each tumor, which was histologically confirmed as metastatic leiomyosarcoma. However, 1 month after the last surgery, multiple systemic metastases were found, thus, he is currently undergoing chemotherapy. The patient has been alive for 8 years and 4 months after the first operation for PPL. PPL is an extremely rare disease with no established treatment strategy for recurrences. Aggressive metastasectomy may be beneficial in selected cases.

Primary Pulmonary Leiomyosarcoma: An Unusual Cause of Pleural Effusion.

Primary pulmonary leiomyosarcomas (PPLs) are extremely rare tumors of the lungs. They can present with non-specific symptoms or can also be asymptomatic with clues towards diagnosis being found on routine examination or radiographs. We present a case of a 54-year-old woman who presented with worsening shortness of breath and spells of dizziness. Her chest radiographs showed right-sided pleural effusion and CT revealed a large enhancing pleural mass with compression atelectasis and mediastinal shift. She underwent a thoracoscopy and right pleural biopsy. Histopathology and immunohistochemistry were most consistent with leiomyosarcoma. An extensive search for a possible primary in other sites was unrevealing, thus diagnosing the patient with PPL. She was managed with surgery and radiotherapy.

Metastatic primary pulmonary leiomyosarcoma to the pancreas diagnosed by endoscopic ultrasound-guided fine-needle aspiration.

A 77-year-old female patient consulted our hospital for an abnormal shadow observed on chest X-ray. Computed tomography revealed the shadow of a mass in the right lower lung lobe and two shadows of masses in the pancreatic head and body. 18F-fluorodeoxyglucose-positron emission tomography showed an intense uptake only in the fields corresponding to these three masses. Each mass was diagnosed as leiomyosarcoma by transcutaneous needle biopsy of the pulmonary mass and endoscopic ultrasound-guided fine-needle aspiration of the pancreatic masses. The primary site was the lung because the pulmonary lesion was solitary, and no tumor was found in other organs. In English language literature, a case of primary pulmonary leiomyosarcoma with metastasis solely to the pancreas has not yet been reported to the best of our knowledge.

A case of primary pulmonary leiomyosarcoma completely resected after neoadjuvant chemotherapy.

BACKGROUND: Primary 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 PRESENTATION: A 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. CONCLUSIONS: We 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.

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.

OBJECTIVES: Primary 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 METHODS: A 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. RESULTS: A total of 231 patients with PPL were included (mean 65.8 ±â€¯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. CONCLUSION: PPL 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: 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.

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.