Case Of Primary Pulmonary Sarcoma Research Articles

Surgical Management of Primary Pulmonary Artery Sarcoma

Primary pulmonary artery sarcoma is a rare cardiac tumor with a dismal prognosis without surgical therapy. It is often confused with the more common chronic pulmonary emboli which may delay the appropriate diagnosis or lead to suboptimal surgery. The objective of this study was to evaluate the short and long-term survival and local recurrence rate of pulmonary artery sarcoma cases operated on at our institution using an anatomic resection approach for the pulmonary trunk and main pulmonary arteries rather than endarterectomy. We searched our prospectively collected cardiac tumor database for cases of primary pulmonary sarcoma operated at our institution between June 2000 and September 2018 and followed until January 3, 2021. We used an anatomic resection and replacement technique for involved pulmonary root and main pulmonary arteries with endarterectomy used only for disease distal to the first arterial branch when lung preservation was possible. The primary endpoints for our study were survival from the time of initial diagnosis and survival from the time of our surgery. Secondary endpoints were operative 30-day mortality and incidence of local recurrence or metastatic disease. We identified 20 consecutive cases of surgical resection of primary pulmonary sarcoma. The median age at surgery was 52.5 years (IQR 43.5-60.5). Complete pulmonary root resection and reconstruction using a pulmonary homograft were needed in 16/20 (80%) of cases. All resections employed cardiopulmonary bypass with cardioplegic arrest. A pneumonectomy was needed in 7/20 (35%) of patients. A negative margin (R0) resection was achieved in 9 patients (45%) and margins were microscopically positive (R1) on final pathology in 9 patients (45%). Two patients (10%) had gross tumor (R2) at the resection margin. Operative mortality was 2/20 (10%). Median survival was 2.8 years from diagnosis (95% CI 1.3-8.8) and 2.7 years from surgery by our team (95% CI 0.8-5.9). Survival from first initial diagnosis at 1, 3, 5, and 10 years was 85.0%, 49.1%, 49.1%, and 16.4%. Survival from our surgery by our team at 1, 3, 5, and 10 years was 70%, 48.8%, 41.8%, and 8.4%. Surgical resection of primary pulmonary artery sarcoma with an approach utilizing an anatomic resection of the pulmonary root and main pulmonary arteries when involved and pneumonectomy or endarterectomy when there is disease distal to the first branch artery can be done with a reasonable operative risk and long-term survival when compared to the natural history of the disease.

Analysis of 19 cases of primary pulmonary sarcoma

背景与目的原发性肺肉瘤（primary pulmonary sarcoma, PPS）是一种极少见的肺部原发性恶性肿瘤，起源于肺间叶组织，极易误诊，本研究旨在探讨原发性肺肉瘤的临床特点，以期为临床早期诊断及治疗提供更全面的参考依据。方法分析总结四川大学华西医院1996年-2011年经病理证实的19例PPS患者的临床资料并进行文献复习。结果19例患者中男性12例，41岁-68岁患者占57.9%，平均年龄41岁。主要症状为咳嗽、咳痰以及咯血；影像学表现中8例见“毛刺征”，5例见“分叶征”，主要病理学类型为滑膜肉瘤、平滑肌肉瘤以及恶性纤维组织细胞瘤。手术治疗17例，化疗1例。随访17例，随访率89.5%，中位生存时间为18个月。结论原发性肺肉瘤临床及影像学均无特征性表现，易被误诊，死亡率高。

Primary pulmonary sarcoma-a study of 8 cases

To summarize the diagnosis, pathology, treatment and prognosis of 8 cases of primary pulmonary sarcoma. The clinical data of 8 patients with primary pulmonary sarcoma in the PUMC Hospital from 1986 to 2009 were retrospectively analyzed. The clinical manifestations included cough (n=5), chest pain (n=2), hemoptysis (n=2), short of breath (n=2), and hoarseness due to the oppression of the recurrent laryngeal nerve (n=1). The pathological diagnoses included leiomyosarcoma (n=1), synovial sarcoma (n=1), malignant fibrous histiocytoma (n=1), and unknown tumors due to poor differentiation (n=5). The 1- and 5-year survival rate was 37.5 % and 12.5%, respectively. The primary pulmonary sarcoma is a rare disease with non-specific clinical manifestations. The early diagnosis is difficult and the prognosis is poor. Currently no satisfactory therapy is available for this disease.

A Case of Primary Pulmonary Sarcoma with Morphologic Features of Malignant Fibrous Histiocytoma

A malignant fibrous histiocytoma is a malignant soft tissue neoplasm that occurs frequently in the metaphyseal ends of the long bones of adults. The lung is a common site for metastasis but it is a rare site for a primary malignant fibrous histiocytoma. Here we report a case of a primary malignant fibrous histiocytoma of the lung. The patient was a 53-year-old man who presented with a moderate amount of a left pleural effusion and an illdefined mass in the left lower lobe on a chest radiograph and a local invasion to the left 10th and 11th rib on chest CT. Under the strong suspicion of lung cancer with a pleural invasion, a serial diagnostic thoracentesis was performed. The cytologic examination of the pleural effusion revealed no malignant cells. Consequently, a thoracoscopic pleural biopsy was performed. The histological examination revealed slender spindle cells and scattered epitheloid cells arranged in a vague storiform or a whirling pattern. Immunohistochemicaily, the tumor cells tested positive for vimentin and negative for cytokeratin, desmin, CD 34 and PAS. These features were consistent with a malignant fibrous histiocytoma. This case is an unusual addition to the small number of published reports on a primary malignant fibrous histiocytoma of the lung.

A Case of Primary Pulmonary Sarcoma with Morphologic Features of Biphasic Synovial Sarcoma

Synovial sarcoma is a malignant soft tissue neoplasm that occurs frequently in the extremities of young adults, near large joints. The lung is a common site of metastasis but an extremely unusual primary site for synovial sarcoma. We report an unusual case of primary synovial sarcoma that arose in the lung of a 59-year-old woman. The tumor had histologic and immunophenotypic features consistent with biphasic synovial sarcoma These features included of an intimate admixture of cytokeratin and epithelial membrane antigen(EMA)-positive neoplastic epithelial cells and vimentin-positive fibroblast-like spindle cells. The patient had a closed thoracomy drainage and doxycycline pleurodesis for malignant loculated effusion and showed tumor extension in the left whole lung 4 months after pleurodesis. This case is an usual addition to the small number of published reports on primary pulmonary synovial sarcoma The distintive features of this neoplasm allow it to be distinguished from a variety of primary and metastatic malignancies in the lung.

Pulmonary Artery Sarcoma Mimicking Pulmonary Embolism

Two cases of primary pulmonary sarcoma which were initially diagnosed as pulmonary thromboembolism are presented. The clinical and radiologic features of pulmonary sarcoma are reviewed, with special emphasis on the features which distinguish it from thromboembolism.

Intra-arterial infusion therapy for pulmonary tumours.

On a total of 47 patients with pulmonary tumours (45 carcinomas and two pulmonary sarcomas) intra-arterial infusion therapy was performed via the bronchial artery (34 cases), the pulmonary artery (11 cases), the internal thoracic artery (1 case) and the intercostal artery (1 case). Cytostatic drugs were given to 43 patients (mainly in the form of single-drug therapy) and four patients received a preparation of proteolytic enzymes. In the majority of cases (34 of 47 patients) intra-arterial therapy was followed by intravenous cytostatic treatment. We can evaluate the effect of the intra-arterial therapy on the basis of the number and extent of tumour remissions, as well as of acute or subacute side effects. Effects over longer period and the survival times of patients can only be evaluated to a limited extent. We observed one complete tumour remission, five partial remissions (size of tumour reduced by more than 50%) and eight minor remissions (reduction by less than 50%); in 18 cases there was no measurable change in the tumour and in nine cases progression was observed (six cases cannot be evaluated.) Complete or partial remissions were observed only after treatment via the bronchial artery and after cytostatic therapy. These tumours were rather poorly vascularised and remissions occurred irrespective of cell type. There are certain indications that intra-arterial chemotherapy may be superior to equivalent intravenous chemotherapy, but the evidence so far available is not conclusive.

Primary Pulmonary Sarcoma: A Case Report

It is generally agreed that the incidence of primary malignant pulmonary neoplasms has increased markedly in recent years, although the extent of the increase varies in different countries. American observers (1, 2) believe that primary malignant tumors of the lung-form about 1 or 2 per cent of all cancers. According to Hunt (3) and Davidson (4), European statistics show a higher incidence, this type of tumor being found in about 7 per cent of all autopsies on patients dying with malignant disease, and ranking next in frequency to carcinoma of stomach, breast, uterus, and esophagus. Recent reports (5, 6, 7) also indicate that intrathoracic tumors are apparently increasing much more rapidly than the general increase in cancer would warrant, due probably to better diagnostic methods and increased human longevity. It is evident, however, that the greater incidence of these tumors is due to the increased recognition of pulmonary carcinoma and that primary sarcoma of the lungs still remains an obscure and comparatively rare condition. Adler (8) in 1911 collected from the literature 90 cases of pulmonary sarcoma, but the evidence presented in many cases is insufficient to warrant their inclusion in this group. Among 9246. autopsies at the Breslau Pathological Institute only four primary sarcomas of the lung were found (9). Stout (10) states that true mesoblastic malignant tumors of the lung are very rare; and Ewing (11) that mesoblastic tumors only occasionally arise in that organ. Ball (12), in a review of the German, French, and English literature since 1900, was able to collect only 14 authentic cases of primary pulmonary sarcoma. The following case, therefore, because of its rarity and its unusual extensions into neighboring structures, was deemed worthy of reporting in detail.

Primary Pulmonary Sarcoma: A Review, With Report of an Additional Case

The case of primary pulmonary sarcoma to be reported in this paper aroused such interest in the subject that a review of the literature was undertaken. The reports, on the whole, were found to be fragmentary and at times misleading because of incomplete descriptions, scarcity of illustrations, and the ill-defined nomenclature used by writers in various countries, particularly in earlier years. Impressions derived from the present study may, therefore, have to be modified as more accurate reports appear. The present review includes only the English, French, and German literature since 1900, except for one very well illustrated case recently reported by Campatelli (11). The year 1900 was chosen because, in reviewing the entire series of reports, it was a striking observation that in the two decades prior to that year cases were reported with a frequency so much higher than that encountered since that one is forced to conclude that many cases were erroneously diagnosed, though not denying that some cases were authentic. In some instances, even since 1900, it has been difficult, because of incomplete descriptions, to decide whether or not a case should be included in this compilation. Where any major doubt has existed in the mind of the author, these cases have been excluded, though it is recognized that some are probably authentic.