Surgical treatment of pulmonary artery sarcoma

OBJECTIVE: Pulmonary artery sarcomas are rare and usually fatal tumors. The diagnosis is difficult and delayed in most cases. Newer imaging techniques could allow early diagnosis in patients with symptoms of pulmonary vascular obstruction. Surgical resection improves clinical symptoms and offers the only chance of cure. We report the case histories of 7 patients with primary pulmonary artery sarcomas treated by surgical resection with or without adjuvant therapy. METHODS: Seven patients (3 women and 4 men; mean age, 52.3 years; preoperative New York Heart Association functional class III/IV, n = 5/2) underwent operations. Malignancy was preoperatively suspected in 5 patients, and 2 patients had a presumptive diagnosis of chronic pulmonary embolism. Tumor resection with partial or total prosthetic replacement (n = 2), reconstruction (n = 5), or both, of central parts of the pulmonary arteries was performed in 6 patients. Thromboendarterectomy was necessary in 4 patients, and pneumonectomy was necessary in 2 patients. Six patients received adjuvant therapy. RESULTS: There was no perioperative mortality. All patients had a substantial improvement in exercise tolerance and hemodynamics 3 months after their operations. Four patients died 7, 9, 18, and 19 months after their operations because of recurrent tumor or pulmonary metastases. Two patients are alive 21 and 35 months after primary surgical repair, with pulmonary metastases detected by computed tomographic scans. One patient is alive 62 months after resection without clinical or radiologic signs of tumor recurrence or metastasis. CONCLUSIONS: Early diagnosis of primary pulmonary artery sarcomas can be improved by computed tomography and magnetic resonance scanning. Radical surgical resection probably presents the only chance for cure. The role of neoadjuvant or adjuvant treatment modalities has to be defined. Pulmonary artery sarcoma need not necessarily be a fatal diagnosis.     

Surgical resection after radiochemotherapy in patients with unresectable adenocarcinoma of the pancreas

BACKGROUND: The use of chemoradiotherapy for pancreatic cancer has been advocated for its potential ability to downstage locally advanced tumors. This article reports our experience with chemoradiotherapy for patients with unresectable, locally advanced pancreatic cancer (superior mesenteric artery or celiac axis encasement). STUDY DESIGN: Since 1998, 61 patients with radiographically unresectable, pathologically confirmed pancreatic adenocarcinoma have received standard fractionation radiation therapy (total dose, 45 Gy at 1.8 Gy, 5 d/wk) with chemotherapy, which included a continuous infusion of fluorouracil (5-FU: 650 mg/m(2)/D1-D5 and D21-D25) and cisplatin (80 mg/m(2)/bolus D2 and D22). Patients with tumor response at restaging CT scan underwent surgical exploration to determine whether the tumor was resectable. RESULTS: Thirty-eight of 61 (62%) restaged patients demonstrated a disease progression. Twenty-three patients (38%) had an objective response, with, in all cases, persistence of arterial encasement. Twenty-three patients underwent exploratory operations after chemoradiotherapy, and 13 underwent standard Whipple resection. So 13 of 23 (56%) patients who had exploratory operation, or 23 of 61 (21%) patients, underwent surgical resection. With a median followup of 27 months, median survival for the resected patients was 28 months. Median survival was 11 months in the nonresponder group (n = 38) and 20 months in the group who received a palliative procedure (n = 10). CONCLUSIONS: Locally advanced, unresectable pancreatic adenocarcinoma may be downstaged by chemoradiotherapy to allow for surgical resection. Patients whose cancer becomes resectable have a median survival at least comparable with survival after resection for initially resectable pancreatic adenocarcinoma.     

Right ventricular outflow tract reconstruction for primary pulmonary artery sarcoma

Primary pulmonary artery sarcoma is extremely rare and a lethal tumor. Complete resection of the tumor and following adequate reconstruction is the only hope for cure and prolonged survival. We report a case of successful surgical excision of primary pulmonary artery sarcoma involving pulmonary trunk, pulmonary valve and right ventricular outflow tract wall, which were replaced with prosthetic valved conduit Postoperative histopathological diagnosis was leiomyosarcoma. The patient is well without any evidence of recurrence at 10 months after surgery.     

Surgical treatment for pulmonary artery sarcoma.

OBJECTIVE: Pulmonary artery sarcomas are exceedingly rare and the prognosis for patients with pulmonary artery sarcoma is very poor. We retrospectively reviewed the early and late outcomes after treatment for pulmonary artery sarcoma, and the purpose of this study is to report our surgical experience with this fatal disease. METHODS: Between 1999 and 2007, a total of nine patients (mean age, 47.4 years; M:F = 4:5) underwent operations for pulmonary artery sarcoma at our institution. The tumor was radically resected and every effort was made to remove the tumor as completely as possible. Seven patients underwent surgical resection with the aid of hypothermic cardiopulmonary bypass. The completeness of resection was determined intraoperatively by frozen section biopsy of the resection margin. RESULTS: There was no in-hospital mortality. No patients suffered from significant complications related to the operation. Follow-up was completed for all the patients with a mean duration of 19.2 months. During follow-up, six patients died with a median survival time of 17.6 months. The cause of death was related to the recurrence of pulmonary artery sarcoma in all cases. The pattern of recurrence was local recurrence and distant metastasis in three and four patients, respectively. CONCLUSIONS: The early outcomes after surgical treatment for pulmonary artery sarcoma were excellent, and the late outcomes in this series were no worse than those in the previous reports. We suggest that the use of cardiopulmonary bypass is important to obtain a complete resection and the completeness of the resection should be confirmed intraoperatively by frozen section biopsy of the resection margin.     

Effect of neoadjuvant therapy on local recurrence after resection of pancreatic adenocarcinoma

BACKGROUND: It is unknown whether neoadjuvant chemoradiotherapy, compared with adjuvant chemoradiotherapy, decreases the rate of local recurrence after resection of pancreatic adenocarcinoma. STUDY DESIGN: This is a retrospective case review of 102 patients with pancreatic adenocarcinoma who underwent pancreatic resection between 1993 and 2005. RESULTS: Of 102 patients with pancreatic adenocarcinoma who underwent surgical resection, 19 (19%) had no additional treatment, 41 (40%) underwent adjuvant chemoradiotherapy, and 42 (41%) were treated preoperatively with neoadjuvant chemoradiotherapy. Patients selected to receive neoadjuvant therapy were more likely to have locally advanced tumors. Based on initial CT scan, the percentage of patients with unresectable or borderline resectable tumors in the neoadjuvant group was 67%, compared with 22% in the adjuvant group. Nevertheless, patients receiving neoadjuvant chemoradiotherapy were less likely to have a local recurrence develop than patients receiving adjuvant chemoradiotherapy (5% versus 34%, p = 0.02). For those patients with tumors determined to be resectable on initial CT scan, local recurrences were observed in 31% (10 of 32) of patients in the adjuvant therapy group, compared with only 7% (1 of 14) of the neoadjuvant group. Intraoperative radiation therapy, administered to 51% of patients, was not associated with a lower rate of local recurrence. CONCLUSIONS: Neoadjuvant chemoradiotherapy is associated with improved local tumor control in patients undergoing resection for pancreatic carcinoma.     

Leiomyosarcomas of the rectum. Amputation of the rectum or local resection?]

Two cases of high grade leiomyosarcoma of the rectum treated by local excision are reported. The first patient presented a local recurrence associated with liver and pulmonary metastases and eventually died, respectively 11 and 22 months after resection. The second patient's tumor recurred 3 months after excision and was treated with pelvic exenteration and CYVADIC regimen as chemotherapy and died 3 years later from local recurrence. A review of the literature indicates that local surgical excision in comparison to a more radical surgical approach, such as abdominoperineal resection, is as effective in improving survival. Lesions less than 2.5 cm in diameter, confined to the bowel can be treated by local surgical excision. To date no adjuvant therapy is effective in terms of survival or local control.     

Primary Pulmonary Artery Sarcoma: Surgical Management and Differential Diagnosis with Pulmonary Embolism and Pulmonary Valve Stenosis

Abstract   Background and aim of the study: Primary pulmonary artery sarcomas are rare and lethal tumors that are usually diagnosed during surgery or autopsy. We present six cases of primary pulmonary artery sarcomas and discuss clinical features, differential diagnosis, surgical treatment, and outcome of the tumors. Methods: Between January 1994 and July 2008, six patients were identified with the disease during operation. Three patients were initially diagnosed with pulmonary valve stenosis, and two patients had a presumptive diagnosis of chronic pulmonary embolism. Two patients had simple or partial tumor resection. Four patients had radical tumor resection and homograft reconstruction of the pulmonary arteries. Results: Histological examinations showed five malignant mesenchymomas and one fibrosarcoma. One patient died of refractory pulmonary hypertension during operation. Two patients died 4 months postoperatively because of brain metastases. Two patients were alive for 3 and 9 months, respectively after the operation with recurrent tumor. One patient is alive even 2 years after resection with no signs of recurrence or metastasis. Conclusions: Because of similar clinical features, pulmonary artery sarcomas are often confused with other pulmonary vascular obstructive diseases. Computed tomography scanning and gadolinium-enhanced magnetic resonance imaging could be useful methods for differential diagnosis. The prognosis is very poor. The survival time after resection varies from several months to several years depending on the presence of recurrence or metastasis. Early diagnosis and radical surgical resection presents the only opportunity for a potential cure.     

Transmanubrial osteomuscular sparing approach for lung cancer invading the anterior part of the thoracic inlet

Standard treatment for lung cancer presenting as a superior sulcus tumor is induction chemoradiotherapy followed by surgery, which yields rates of about 70% complete resection and 50% 5-year survival rate. However, the surgical technique to achieve complete resection for superior sulcus tumor invading major anatomical sites including the subclavian artery is challenging. The anterior transcervical thoracic approach applied by Dartevelle and colleagues provides excellent exposure of the subclavian vessels. Grunenwald and associates have improved on this approach to preserve the clavicle and sternoclavicular joint. We applied the transmanubrial osteomuscular-sparing approach in two patients. In both cases, exposure of the subclavian vessels was excellent. In one case, the subclavian artery was resected and reconstructed with a polytetrafl uoroethylene graft. This patient has continued to show recurrence-free survival for more than 5 years. We outline our experience and review the literature on the surgical approach for superior sulcus tumor invading the anterior part of the thoracic inlet.     

Combined resection and adjuvant therapy improves prognosis of sarcomas of the pulmonary trunk.

BACKGROUND: Diagnosis of pulmonary artery sarcoma is difficult and frequently delayed owing to the insidious, non-specific nature of the symptoms. We present our experience with surgery for this rare disease. METHODS: Between 1995 and 1998, four patients underwent surgery for pulmonary trunk sarcomas. They presented with similar symptoms and were initially treated for thromboembolic disease of the lung. Preoperative diagnosis was established using computer tomography and echocardiography. Postoperatively, two patients received radiotherapy and one combined radio-/chemotherapy. RESULTS: One patient underwent thrombendarteriectomy of the trunk with adjuvant radiotherapy and remains tumor-free after three years and one showed complete regression of metastases after chemotherapy and remains well after more than one year. The other two patients died within two months of surgery, one due to a tumor mass in the inferior caval vein and the other because of cerebral metastases. CONCLUSIONS: According to the literature, mean survival rate is 1.5 months without resection and 10 months with resection, with survival up to four years following combined resection and adjuvant therapy. Our findings are in agreement with this.     

Surgical treatment for esophageal cancer. Current issues

Esophageal cancer is one of the most difficult malignancies to cure. The prognosis remains unsatisfactory despite significant advances in surgical techniques and perioperative management. The optimal treatment strategy for localized esophageal cancer has not yet been established. Surgical resection remains the mainstay of treatment for esophageal cancer, and curative resection is the most important surgery. Extended esophagectomy with three-field lymphadenectomy provides the highest quality of tumor clearance and prolongation of patient survival. There has been intense effort in developing novel strategies to treat patients with resectable esophageal cancer. Various combined-modality approaches have been attempted to improve treatment outcomes. Definitive chemoradiotherapy has an impact on long-term survival in patients with resectable esophageal cancer. Accordingly, there are three main combined-modality approaches: esophagectomy with adjuvant chemotherapy or chemoradiotherapy; primary definitive chemoradiotherapy with or without salvage esophagectomy, and preoperative chemoradiotherapy followed by planned esophagectomy. Recently, owing to the remarkable advances in optical technology, minimally invasive esophagectomy using endoscopic instruments has been introduced into esophageal cancer surgery. This article reviews recent changes in the treatment of esophageal cancer surgery, and considers the role of esophagectomy.     

Pulmonary resection for metastatic breast cancer.

Thirty-three patients treated primarily with surgical excision of pulmonary metastases from breast cancer were compared with 30 patients treated primarily with systemic chemohormonal therapy. Treatment for patients in the surgical group included pulmonary resection alone in 20, resection plus adjuvant systemic therapy in nine, and resection plus adjuvant radiation therapy in four. Treatment for patients in the medical group included systemic therapy alone in 22 and systemic therapy plus local radiation therapy in eight. Mean survival in the surgical group was significantly longer than that in the medical group, even when only those patients who manifested single pulmonary nodules were compared (58 months vs 34 months). The overall 5-year survival rate after treatment of lung metastasis was significantly greater for the surgical group than for the medical group (36% vs 11%). The results of this study indicate that surgical resection should be considered in patients with breast cancer who develop operable pulmonary metastases without evidence for concomitant extrapulmonary disease. In selected patients, such therapy may result in a survival benefit.     

Surgical treatment of pulmonary artery leiomyosarcoma: a good survival without adjuvant therapy

Primary pulmonary artery leiomyosarcoma is a rare tumor that can be misdiagnosed as acute or chronic pulmonary thromboembolic disease. In this report, we present a case of a 58-year-old woman initially diagnosed with chronic thromboembolic pulmonary disease, but who was later found to have pulmonary artery leiomyosarcoma. A complete mass resection was performed surgically. The definitive pathologic examination was consistent with pulmonary artery leiomyosarcoma. Although the patient did not receive adjuvant therapy after the surgery, she had a good outcome and was free of disease 8 months after surgery.     

Primary sarcoma of the right ventricle: surgical and adjuvant therapy

We describe surgical and adjuvant therapeutic management of a right ventricular (RV) sarcoma and pulmonary artery occlusion. Echocardiographic evaluation of a 39-year-old man with exertional dyspnea revealed a tumor mass in the right ventricle, pulmonary trunk, and bilateral pulmonary arteries. The tumor was resected with concomitant pulmonary valvotomy, but the resection was incomplete. The RV outflow was reconstructed with an allograft patch, and a stentless valve was implanted for pulmonary valvular function. The pulmonary trunk and arteries were enlarged with allograft patches. The tumor was undifferentiated sarcoma and caused postoperative pulmonary artery restenosis. Radiotherapy improved pulmonary perfusion (reduction of RV pressure), but the patient died of brain metastasis. Undifferentiated cardiac sarcomas associated with pulmonary hypoperfusion should be resected even if incompletely, and radiation therapy could alleviate reduced pulmonary perfusion.     

Does Surgical Resection of Pulmonary Metastases of Head and Neck Cancer Improve Survival?

Background  The prognosis of patients with metastasized head and neck cancer is poor. Limited experience exists with the benefit of resection of lung metastases and systematic mediastinal and hilar lymph node dissection on survival of patients with head and neck carcinoma. Methods  Eighty patients undergoing metastasectomy for pulmonary metastases of primary head and neck cancer entered the study. Multivariate analysis was performed by Cox regression analysis. Survival differences between patients operated and those not operated on were analyzed by matched pair analysis. Results  From 1984 until 2006, pulmonary metastases were diagnosed in 332 patients treated for head and neck cancer; 80 of these were admitted to our department for resection. Metastases of the primary head and neck tumor were confirmed histologically in 67 patients. The median overall survival after resection of lung metastases was 19.4 months and was statistically significantly better compared with patients who were not operated on (P < .001). The multivariate analysis after metastasectomy revealed that incomplete resection of pulmonary lesions, complications associated with surgery, and adjuvant therapy of the primary tumor are independent negative prognostic factors for survival. We observed a trend to improved survival in patients without hilar or mediastinal lymph node metastases. Conclusion  The survival rate of patients operated on was statistically significantly higher than that of patients with conservative treatment. Even patients with multiple or bilateral pulmonary lesions after curative treatment of a primary tumor should be operated on if there is no contraindication against an extended surgical procedure and a complete resection of the metastases seems achievable. The first two authors contributed equally to this work.     

直肠癌局部切除术的外科疗效与预后分析

目的 分析局部切除术治疗直肠癌的疗效及影响复发和预后的因素.方法 回顾性分析采用局部切除术治疗的46例中低位直肠癌患者临床资料,并对其预后影响因素进行单因素及多因素分析.结果 46例患者中经肛门局部切除38例,经骶尾部切除8例;术后并发症发生率为10.9%,围手术期病死率为0;局部复发率为17.4%,Tis、T1及T2期病变的局部复发率分别为0,12.0%,33.3%.肿瘤大体类型、肿瘤直径和分化程度与术后局部复发相关.总体5年生存率为83.3%,Tis、T1、T2期5年生存率分别为100%、90.9%、66.7%.单因素分析显示,肿瘤大小、分化程度、术后放化疗、脉管癌栓和局部复发是影响预后的因素.多因素分析显示,局部复发是影响预后的主要因素.结论 早期（Tis、T1期）高中分化癌,病灶直径≤3 cm,无脉管浸润的隆起型直肠癌,局部切除术是有效的治疗方法.T2期及以上的肿瘤应首选根治性手术,不能耐受根治术者或拒行腹壁人工肛门者,可考虑姑息性局部切除术后加行辅助放、化疗.     

从胰腺癌患者的长期生存看预后因素及治疗模式

Pancreatic cancer has the characteristics of high malignancy, early dissemination within the pancreas,extrapancreatic nerve plexus invasion, lymph node metastasis and vascular invasion. The 5-year survival rate of pancreatic cancer patients was under 5% even for those who had undergone surgical resection. Based on the review of the literatures including 42 pancreatic cancer patients who survived for 5-20 years after the operation, we concluded that curative resection of pancreatic cancer was still a reliable means in achieving long-term survival; factors influencing the results of resection of pancreatic cancer were lymph nodes involvement, poor differentiated tumor, extrapancreatic nerve plexus invasion, tumor size, residual tumor, curative resection and adjuvant chemotherapy; early diagnosis, aggressive surgery for patients with indications of resection, appropriate surgical procedure and postoperative adjuvant chemotherapy are essential factors to ensure a long term survival of patients with pancreatic cancer.     

从胰腺癌患者的长期生存看预后因素及治疗模式

胰腺癌恶性程度高,具有早期胰腺内播散,胰腺外神经丛侵犯,淋巴结转移与血管侵犯的特点.即使外科切除,大部分患者5年生存率仍<5%. 1长期生存的特征     

Adjuvant treatments for resectable pancreatic cancer

Pancreatic cancer remains one of the most challenging malignancies to treat successfully. The majority of patients present with unresectable advanced-stage cancer, and only 20% of patients can undergo resection. Even if surgical resection is performed, the recurrence rate is high and the survival rate after surgery is poor. Therefore, effective adjuvant therapy is needed to improve the prognosis of patients with pancreatic cancer. Until now, no universally accepted standard adjuvant therapy for this disease has been available: chemoradiotherapy followed by chemotherapy is considered the optimal therapy in the United States, while chemotherapy alone is the current standard in Europe. However, recent randomized controlled trials (RTOG [Radiation Therapy Oncology Group] 9704; CONKO [Charité Onkologie]-001; and a Japanese study) have suggested a benefit of adjuvant chemotherapy with gemcitabine for patients with resectable pancreatic cancer. This article will review the clinical trials of adjuvant therapy for this disease, including the results of recent trials.     

Surgical treatment of primary pulmonary artery tumor

We report two cases of rare primary pulmonary artery (PA) tumor. Case 1 was a 65-year-old man with malignant fibrous histiocytoma which caused severe stenosis over the main PA and right PA. Case 2 was a 49-year-old woman with leiomyosarcoma which caused severe stenosis from the right ventricular outflow tract (RVOT) to the main PA. Both cases underwent endarterectomy, tumor resection, and PA reconstruction under cardiopulmonary bypass. The symptoms of right heart failure improved postoperatively. Tumor recurrences were however recognized in both cases in the early postoperative period, and both patients died of the disease 12 (case 1) and 21 (case 2) months after operation respectively. Tumor resection by endarterectomy techniques may be incomplete because of frequent local recurrence. Postoperative adjuvant therapy in addition to radical resection and reconstruction of the PA and tumor might increase the length of survival.     

Long-term outcome after multimodality treatment for stage III thymic tumors

BACKGROUND: Surgery remains the cornerstone of therapy for thymic tumors, but the optimal treatment for advanced, infiltrative lesions is still controversial. The introduction of multimodality protocols has substantially modified survival and recurrence rate. We reviewed our 13-year prospective experience with multimodality treatment of stage III thymoma and thymic carcinoma. METHODS: Since 1989 we have prospectively used a multimodality approach in 45 stage III thymic tumors. Sixteen patients (35%) had myasthenia gravis. Twenty-three patients (51%) had pure or predominantly cortical thymoma (group 1), 11 (24.5%) had well-differentiated thymic carcinoma (group 2), and 11 (24.5%) had thymic carcinoma (group 3). Tumors that were not considered radically resectable at preoperative workup underwent biopsy and induction chemotherapy (15 patients, 33%) followed by surgical resection; all patients were referred for adjuvant chemoradiotherapy. RESULTS: No operative mortality was recorded; 1 treatment-related death during adjuvant chemotherapy was observed in group 1. Complete resection was feasible in 91% of patients in groups 1 and 2 and 82% in group 3. The overall 10-year survival was 78%. Ten-year survival for groups 1 and 2 was 90% and 85%, respectively; 8-year survival for group 3 was 56%. During follow-up, tumor recurrence was noted in 3 patients (13%) from group 1, 3 (27%) from group 2, and 3 (27%) from group 3. CONCLUSIONS: Multimodality treatment with induction chemotherapy (when required) and adjuvant chemoradiotherapy offers encouraging results for stage III thymic tumors; the outcome is more favorable for cortical thymoma and well-differentiated thymic carcinoma.