Radiation-induced osteosarcoma 17 years after mediastinal irradiation following surgical removal of thymoma

Radiation-induced osteosarcoma is a rare complication after irradiation of primary malignancies. In the chest wall, it is usually secondary to radiotherapy for breast cancer or lymphoma. We report a rare case of radiation-induced osteosarcoma of the sternum after mediastinal irradiation of a thymoma. A 49-year-old woman presented with a sternal tumor 17 years after surgery plus mediastinal irradiation (50 Gy) for a stage III thymoma. On biopsy, this second tumor was diagnosed as a radiation-induced osteosarcoma. Systemic survey revealed additional metastatic spread to vertebrae and pelvis. Despite intensive combination chemotherapy that initially stabilized her disease, the patient died 2 years after the diagnosis was made. Because thymoma patients receiving mediastinal irradiation are thus at additional risk of radiation-induced secondary malignancy, long-term follow-up is advisable.     

Malignancies involving the pericardium

Malignancies rarely arise from the pericardium. Mesothelioma, the most common of these, is usually unresectable and almost always incurable. Malignancies may secondarily involve the pericardium by direct extension. Carcinoma of the lung and malignant thymoma with limited direct invasion of the pericardium both can undergo complete and potentially curative resections, but adjuvant therapy is usually indicated. More frequently, malignancies involve the pericardium by a process of retrograde lymphangitic spread or hematogenous dissemination. These patients present with a symptomatic pericardial effusion and occasionally pericardial tamponade. Subxiphoid pericardiostomy and drainage is a safe procedure that provides effective and durable symptomatic relief in these terminally ill patients. More aggressive open procedures should be reserved for loculated or recurrent pericardial effusions.     

腹膜后局限性Castleman病20例临床诊治及预后分析

目的 探讨腹膜后局限性Castleman病的临床特征、诊治策略及影响预后的因素,提高伴副肿瘤性天疱疮(PNP)的Castleman病的诊治水平.方法 回顾性分析1993年1月至2009年5月在北京大学第一医院普通外科接受手术治疗的20例腹膜后Castleman病患者的临床资料,并将13例腹膜后Castleman病伴PNP患者的临床资料与同期7例单纯腹膜后Castleman病患者进行比较,分析两者在发病特点、部位、实验室检查、手术策略、病理特征及外科治疗效果的不同.结果 (1)本组腹膜后Castleman病多见于中青年(中位年龄36岁),发病部位多位于肾旁(14/20,70%)及髂窝(4/20,20%),左侧腹膜后多见,病理分型以透明血管型为主,腹膜后Castleman病合并PNP组的性别、年龄、肿瘤发病部位、大小及病理分型与单纯腹膜后Castleman病组无明显区别(P>0.05);(2)本组腹膜后Castleman病合并PNP患者较易合并闭塞性细支气管炎(8/13),有血清肿瘤标志物癌胚抗原或CA242升高现象(3/8);(3)本组腹膜后Castleman病常有包膜,与邻近脏器边界清晰,手术较为容易,但合并PNP的腹膜后Castleman病的生物学行为有恶性倾向及伴发子灶特征,有侵及邻近血管及术后局部复发可能;(4)Kaplan-Meier及Log-Rank生存分析显示,腹膜后Castleman病合并PNP患者5年生存率为42.8%,平均生存时间30个月,明显低于单纯腹膜后Castleman病组(P<0.05),是否合并闭塞性细支气管炎以及肿瘤能否根治切除是影响腹膜后Castleman病患者预后的重要因素.结论 腹膜后Castleman病伴PNP具有独特的临床特征,早期诊断和切除肿瘤、及时终止致病抗体的产生,是成功治愈的关键.     

Paraneoplastic Extralimbic Encephalitis Associated with Thymoma: A Case Report

Here we a report a rare case of extralimbic encephalitis associated with thymoma. A 66-year-old woman was admitted to our hospital with cramping in her right leg and inability to walk. Magnetic resonance imaging of the brain showed multifocal high intensity signals on T2 flare images in the cerebral cortex, and chest computed tomography showed a 5-cm anterior mediastinal mass, which was considered to be a thymoma. We speculated that she had paraneoplastic encephalitis associated with thymoma. She underwent a thymectomy and was diagnosed with type B1 thymoma. On postoperative day 6, her neurological symptoms began to improve. On postoperative day 31, she was discharged without complications. Limbic encephalitis is a paraneoplastic neurological syndromeassociated with thymoma, but extralimbic encephalitis has been described in the literature very rarely. We report the case of extralimbic encephalitis associated with thymoma along with a literature review.     

Comparison of open subxiphoid pericardial drainage with percutaneous catheter drainage for symptomatic pericardial effusion

BACKGROUND: The optimal therapy for symptomatic pericardial effusions remains controversial. This paper compares outcomes after the two most commonly used techniques, percutaneous catheter drainage and operative subxiphoid pericardial drainage. METHODS: We performed a 5-year retrospective, single-institution study to analyze outcomes after either percutaneous catheter drainage or subxiphoid open pericardial drainage for symptomatic pericardial effusions. RESULTS: Symptomatic pericardial effusions in 246 patients were treated by open pericardiotomy and tube drainage (n = 150) or percutaneous catheter drainage (n = 96). Drainage duration, total drainage volume, and duration of follow-up (2.6 years) were similar in both groups. Effusions were classified malignant in 79 (32%) patients and benign in 167 (68%) patients. No direct procedural mortality occurred, but the hospital mortality was 16 patients (10.7%) in the open group and 22 (22.9%) in the percutaneous group (p = 0.01) The 5-year survival rate was 51% in the open group versus 45% in the percutaneous group, despite a greater percentage of the open group having a preoperative malignant diagnosis (35% versus 28%). Symptomatic effusions recurred in 16.5% of the percutaneous group compared with 4.6% in the open group (p = 0.002), and sclerosis did not appear to reduce recurrence rates (10.7% with sclerosis versus 15.6% without; p > 0.05). The diagnosis of malignancy was confirmed in 16 of 27 (59%) percutaneous procedures performed on patients with known malignancy. In the open group, cytologic and pathologic evaluation of the pericardial specimen revealed malignancy in 32 of 52 (62%) patients with known malignancy. CONCLUSIONS: Subxiphoid and percutaneous pericardial drainage of symptomatic pericardial effusions can be performed safely; however, death occurs from underlying disease. Open subxiphoid pericardial drainage with pericardial biopsy appears to decrease recurrence but does not improve diagnostic accuracy of malignancy over cytology alone.     

Mediastinoscope-controlled parasternal fenestration of the pericardium: definitive surgical palliation of malignant pericardial effusion

ABSTRACT: BACKGROUND: The tumorous infiltration or carcinosis of the pericardium could cause pericardial effusion in up to one-third of cases of malignancy, thus potentially interfere with the otherwise desirable oncological treatment. The existing surgical methods for the management of pericardial fluid are well-established but are not without limitations in the symptomatic relief of malignant pericardial effusion (MPE). The recurrence rate ranges between 43 and 69% after pericardiocentesis and 9 to 16% after pericardial drainage. The desire to overcome relative limitations of the existing methods led us to explore an alternative approach. METHODS: The standard armamentarium of the Carlens collar mediastinoscopy procedure was utilized in a Chamberlain parasternal approach of the pericardial sac. The laterality of approach was decided based upon the pleural involvement, as tumor-free pericardiopleural reflection is required. A pericardio-pleural window at least 3 cm in diameter was created. From January 2000 to December 2009, 22 cases were operated on with mediastinoscope-controlled parasternal fenestration (MCPF). Considering the type of the primary tumor, there were 11 lung cancer, 6 breast cancers, 2 haematologic malignancies and in 3 patients the origin of malignancy could not be verified. RESULTS: There were no operative deaths. We lost one patient (4.5%) in the postoperative hospital period. All of the surviving patients had a minimum of 2 months of symptom-free survival. We detected transient recurrence of MPE in one patient (4.5%) 14 days after the MCPF, which disappeared spontaneously after 24 hours. CONCLUSION: The MCPF offers a real alternative in certain cases of pericardial effusion. We recommend this method especially for the definitive surgical palliation of MPE.     

Chemotherapy for thymic tumors: induction, consolidation, palliation

Although thymoma and thymic carcinoma are rare malignancies, they constitute a large proportion of tumors of the anterior mediastinum. Surgery forms the mainstay of therapy; however, thymic malignancies are sensitive to chemotherapy and radiation therapy also. Systemic chemotherapy is primarily used for treatment of metastatic or recurrent disease. Chemotherapy is also used as a component of multimodality treatment in the neoadjuvant setting with the aim of increasing the chances of achieving a complete surgical resection. In this article we outline various clinical trials that have been performed to evaluate the role of chemotherapy in the treatment of thymic malignancies.     

Cardiac metastasis from gallbladder carcinoma

INTRODUCTIONPrimary gallbladder carcinoma is a rare aggressive neoplasm of elderly with poor prognosis. The tumour is often unresectable at the time of diagnosis. Metastasis to heart is rare and only 6 cases have been reported in the indexed literature. We herein report a case of gallbladder carcinoma metastasizing to heart.PRESENTATION OF CASEA 54 year old female presented with dyspnoea and chest pain with past history of radical cholecystectomy and palliative chemotherapy for adenocarcinoma of gallbladder. Chest X-ray showed cardiomegaly and 2-D ECHO revealed features of tumour deposits on the surface of myocardium and malignant pericardial effusion. Mini-thoracotomy and pericardial window procedure was done to relieve distressing symptoms and biopsy of pericardial tissue revealed metastatic adenocarcinoma. In spite of intensive care, patient succumbed to disease in the post-operative period.DISCUSSIONPrimary adenocarcinoma of gallbladder is the most common malignancy of biliary tract and fifth most common malignancy of gastro-intestinal system with dismal prognosis. It most commonly spreads to liver and regional lymph nodes, very rarely distant metastasis occurs to kidney, adrenal, thyroid and bones as reported in the literature. Metastasis to heart presents with symptoms of cardiac failure due to pericardial effusion. Even with intensive care patients will invariably succumb to the disease.CONCLUSIONMetastatic spread to heart from carcinoma of gallbladder is very rare. Should a patient be suspected of or an operated case of gallbladder carcinoma present with symptoms of congestive heart failure and massive pericardial effusion, cardiac metastasis should be considered.     

Nonmalignant pericardial effusion associated with thymic cancer

We report a case of thymic carcinoma with massive pericardial effusion in a 74-year-old man. This patient with dyspnea was referred to our hospital because of pericardial effusion detected by echocardiography. A chest computed tomography and magnetic resonance imaging showed an anterior mediastinal mass measuring 8.0 × 5.0 cm with massive pericardial effusion. The mass lesion was suggestive of thymic carcinoma or invasive thymoma. Initially, he underwent pericardial drainage. The collected fluid was serous and yellow, and cytological examination found no malignant cells. The tumor with partial pericardium was resected. Histopathological findings confirmed the lesion to be squamous cell carcinoma of the thymus. The etiology of a massive nonmalignant pericardial effusion associated with thymic carcinoma warrants further studies. The patient is alive without recurrence and without pericardial effusion at 3 years to date after the operation. Not all pericardial effusion associated with thymic cancer involves malignant effusion.     

A prospective trial of subxiphoid pericardiotomy in the diagnosis and treatment of large pericardial effusion. A follow-up report.

OBJECTIVE: This study was designed to determine the cause of large pericardial effusions and evaluate the efficacy of subxiphoid pericardiotomy. SUMMARY BACKGROUND DATA: Despite great advances in the techniques used to diagnose pericardial effusions, much controversy remains concerning their cause and the optimal treatment of these effusions. METHODS: In a prospective consecutive case series, 57 patients underwent a thorough preoperative evaluation followed by a subxiphoid pericardiotomy. All tissue and fluid was exhaustively evaluated. Postoperatively, all patients were followed for a least 1 year. RESULTS: Surgery was performed under local anesthesia in 77% of patients, and the complications of surgery were minimal. Pericardial tissue and fluid established or aided in establishing a diagnosis in 81% of patients. Infection and malignancy were the leading causes; the condition in only 4 patients remained undiagnosed. Follow-up revealed recurrent effusion in nine (16%) patients, but only five (9%) required further surgery. The mortality rate at 30 days was 12%, and at 1 year, it was 37%. Fourteen of the 21 deaths occurred in patients with malignancies. CONCLUSIONS: These data show that the cause of most large pericardial effusions can be determined by a thorough evaluation accompanied by subxiphoid pericardiotomy. In addition, subxiphoid pericardial biopsy and window creation is safe and effective in the treatment of these effusions.     

Surgical treatment of stage III and IV thymoma

During past 15 years, 39 cases of thymoma were underwent surgical intervention. In these cases, invasive type, so called stage III and IV in Masaoka's classification were 19 cases. This report documents the results of extended operation in 19 patients treated for malignant thymoma. All patients had neoplasm which invaded adjacent structures; superior vena cava, pericardium, and lung. Eight patients had disseminated lesions in the pleural or pericardial cavities. All patients were underwent surgical exploration through median sternotomy (18 patients) or left thoracotomy (1 patient). Our surgical management to malignant thymoma is to have complete resection, even if tumor invades the great veins. Of 8 patients, superior vena cava and left innominate vein were resected with tumor and reconstructed with ringed PTFE. Mediastinal pleura and pericardium should be widely opened and intrapericardial or intrapleural disseminated lesions should be removed as far as possible. Malignant thymoma could be resected completely applying technique of vascular surgery. Good results were expected when tumor was resected with invading adjacent structures completely. Reoperation to the recurrent tumor is also important.     

Extrapleural pneumonectomy for thoracic malignancies

Extrapleural pneumonectomy has been used by thoracic surgeons in the treatment of malignant pleural mesothelioma as well as other pleural diseases, such as tuberculous empyema. Recently, this operative procedure has been also sporadically applied for the treatment of carcinomatous pleuritis of lung cancer and/or invasive thymoma in some institutions. We performed this operation in 24 patients with thoracic malignancy: 15 patients with carcinomatous pleuritis of primary lung adenocarcinoma (6 patients with T4N0 disease, 2 with T4N1, and 7 with T4N2), 3 patients with stage IV a thymoma, and 6 patients with malignant pleural mesothelioma (2 patients with stage II disease, 3 with stage III, and 1 with stage IV). All patients survived the operation and were discharged from the hospital. Major complications were intrathoracic hemorrhage and empyema in 2 patients each. The median survival time and 5-year survival rate of lung cancer patients were 34 months and 45.5%, respectively. All patients with thymoma are alive now without disease 9 to 133 months after operation. All patients with malignant pleural mesothelioma except 1, who lately underwent this treatment, died of disease from 15 to 27 months after surgery. Our results indicate that carefully selected patients with carcinomatous pleuritis of lung cancer and thymoma may be candidates for extrapleural pneumonectomy for cure. Nevertheless, the ultimate value of this surgical treatment should be ascertained in a prospective study with a large number of patients.     

Pericardial catheter sclerosis versus surgical procedures for pericardial effusions in cancer patients

BACKGROUND: Approximately 21% of patients with advanced malignancies have cardiac or pericardial involvement with tumor. Controversy exists regarding the optimal approach to the pericardial space when hemodynamic compromise due to effusions occurs. METHODS: A six-year retrospective review of 59 cancer patients with pericardial effusions. RESULTS: Thirty-six patients had subxiphoid pericardial window (SXPW) alone (Group A), 5 had pericardial catheter drainage (PCD) followed by a SXPW (Group B), 10 had PCD with sclerosis (Group C), 5 had PCD alone (Group D), 2 had PCD with pericardial-pleural window (Group E), and one had pericardial-peritoneal window (Group F). The method of procedure, complications, number of hospital and ICU days, cytological or pathologic evidence of malignancy, solid versus hematological tumors, and survival were analyzed. The median survival for those patients in group C was one month compared to 4 months for Group A and 6 months for Group B. Essentially, results were similar regardless of method performed with the exception that professional and hospital charges averaged $4830 for SXPW compared to $1625 for PCD. CONCLUSIONS: Pericardial catheter drainage and sclerosis provides a viable option for the treatment of pericardial effusions in selected cancer patients at markedly reduced cost and patient discomfort.     

An immunohistochemical study of thymic epithelial tumors. III. The distribution of interdigitating reticulum cells and S-100 beta-positive small lymphocytes

We report an immunohistochemical study of the distribution and number of interdigitating reticulum cells (IDC) and S-100 beta-positive small lymphocytes (S-100 beta + lymphocytes) in 53 thymomas and 11 thymic carcinomas. All 53 thymomas showed the presence of IDC in the tumor parenchyma. In most cases of predominantly lymphocytic and mixed-type thymoma, IDC clustered in areas, that corresponded to locations that had medullary differentiation and contained accumulated mature lymphocytes. By contrast, in most of the predominantly epithelial-type thymomas, IDC were scattered rather than forming clusters. The distribution and number of IDC were correlation with the histological type of thymoma but not with invasiveness. In thymic carcinomas, IDC were scattered in tumor nests. In 47 of the 53 thymomas (89%), infiltrating S-100 beta + lymphocytes were readily recognized. The remaining six cases without S-100 beta + lymphocytes were noninvasive thymoma. We conclude that the degree of S-100 beta + lymphocyte infiltration is correlated with the stage of thymoma and may be a marker of thymoma malignancy.     

Therapy for thymic epithelial tumors

Thymoma is the most common tumor of the anterior mediastinum. This tumor is associated with unique paraneoplastic syndromes. The rarity of this tumor has somewhat obscured the optimal treatment for this disease. The World Health Organization classification system, which published in 1999, appears to be an advance in our understanding of thymoma. The Masaoka classification is now the most widely accepted and is an excellent predictor of the prognosis of thymoma. Now the International Thymic Malignancy Interest Group is currently engaged in the development of a validated formal TNM classification system for thymic malignancies. The optimal treatment of thymoma is performed according to its clinical stage. Surgery remains the mainstay of treatment for thymic epithelial tumors. Minimally invasive surgery including thoracoscopic surgery and robotic surgery for stage I and II thymomas is increasing now. The value of postoperative radiotherapy in completely resected stage II or III tumors is questionable. As thymomas have a moderate response rate to chemotherapy or radiotherapy, multimodality therapy involving surgery, chemotherapy and radiotherapy appears to increase the rate of complete resection and survival in the advanced (stage III and IV) thymomas.     

A case of refractory uremic pleuropericarditis--successful corticosteroid treatment

We report the case of a patient with uremic pleuropericarditis who showed a marked improvement following corticosteroid therapy. A 66-year-old man who had been on hemodialysis therapy for 13 years was admitted to our hospital presenting with increases in bilateral pleural effusions and pericardial effusion. Repeated thoracentesis showed hemorrhagic and exudative findings. Pleural and pericardial fluid cytologic examination, bacterial culture and acid-fast staining showed negative findings. Despite the administration of antibiotics and antituberculosis drugs, low-grade fever continued and C-reactive protein level remained high. A pleural biopsy revealed fibrinous pleuritis without infectious disease or malignancy. He was diagnosed as having uremic pleuropericarditis on the basis of the clinicopathological features, but had been unresponsive to conventional treatments including repeated thoracentesis and the continuance of hemodiafiltration using nafamostat mesylate. Ultimately, both pleural and pericardial effusions were controlled after the treatment with prednisolone at an initial dose of 50 mg per day. In conclusion, corticosteroid therapy seems to be useful for treating patients with conventional therapy-resistant uremic pleuropericarditis.     

The Subxiphoid Approach to Pericardial Disease

During the 36-month period from July, 1978, through July, 1981, 25 patients underwent a subxiphoid pericardial window procedure for diagnosis and therapy. Twelve patients were operated on for uremic pericarditis, 6 for malignancy, and 7 for etiological diagnosis of the pericarditis. All 12 patients with renal failure had enlarging effusions, despite aggressive dialysis. Eleven of the 12 are alive, free from recurrence, 3 to 36 months postoperatively. Six patients were operated on for suspected pericardial malignancy with hemodynamic compromise. Histological diagnosis was made from the pericardial tissue in all patients; only 1 patient lived more than 43 days following the procedure.In the group of 7 patients operated on for diagnosis, 4 were thought preoperatively to have tuberculous pericarditis. All 4 were treated with antituberculosis chemotherapy and are asymptomatic, without evidence of calcification, 12 to 31 months postoperatively.This diverse group of patients demonstrates that the subxiphoid pericardial window is an effective approach for relief of uremic effusions and may adequately treat effusive tuberculous pericarditis when combined with multidrug chemotherapy. Patients with suspected malignant pericardial disease and hemodynamic compromise need to be carefully studied before an operative procedure is considered as a means of diagnosis and therapy.     

Cardiac transplantation in patients with preexisting malignancies.

A history of preexisting malignancy has been considered a contraindication to cardiac transplantation. The reasons for this prejudice include concerns about potentially deficient intrinsic immunomodulation and fear of cancer recurrence (or development of second cancers) because of therapeutic immunosuppression. In the past four years at the Northern Indiana Heart Institute seven patients with preexisting malignancies underwent cardiac transplantation. Their two-year survival rate was 100%, which is comparable to a rate of 81% in non-malignancy patients. After an average 31 months of follow-up (range = 6-56 months), only one patient has had a recurrent tumor (basal cell carcinoma). Statistical comparison of immunosuppression dosages, incidences of rejection, and incidences of infections between patients with preexisting malignancy and those without preexisting malignancy was performed. We found that the only significant difference was an increased number of infections in preexisting malignancy patients. Additionally, we found no difference in the incidence of posttransplant coronary artery disease in the preexisting malignancy group when compared with those patients without preexisting malignancies. This study demonstrates that patients who have been successfully treated for malignancies have no greater incidence of rejection than those patients without preexisting malignancy. Furthermore, preexisting malignancy patients require no significant modulation of immunosuppression. Although preexisting malignancy patients have a higher incidence of infections than patients without preexisting malignancy, their two-year survival is not worse than the patients without preexisting malignancy.     

Usefulness of a program of neoplasia surveillance in liver transplantation. A preliminary report

Abstract:  De novo malignancies are frequent complications after liver transplantation. Aim of the study is to evaluate whether a surveillance program for malignancy may improve patient survival. We have compared the survival after the diagnosis of malignancy (excluding cutaneous and hepatobiliary carcinomas and lymphoproliferative disease) of patients with symptomatic or incidental malignancies with patients with neoplasia diagnosed on screening. Two hundred and eighty patients with a follow-up greater than three months were followed for a median of 77.5 months (total follow-up: 1515 patient-yr). Thirty-three patients developed 41 malignancies. When compared with general population, the entire cohort of liver transplant recipients had a significantly higher risk of malignancy (relative risk: 2.34), gastrointestinal tract (relative risk: 2.52), urological tract (relative risk: 2.94) and head and neck cancer (relative risk: 4.14), and cancer-related death (relative risk: 2.35). All nine patients diagnosed with cancer with active screening are currently alive and free of malignancy after a median follow-up of 25 months. By contrast, 18/24 patients with diagnosis of cancer prompted by symptoms or incidentally diagnosed died as a consequence of the cancer (median survival: 13.5 months). The difference in survival between both groups was significant (p = 0.002). In conclusion, a close surveillance protocol for the diagnosis of malignancy could be life-saving in liver transplant recipients.     

Rheumatology and Oncology: An Updated Review ofRheumatic Manifestations of Malignancy and Anti-NeoplasticTherapy

Objective: Review of the literature addressing the rheumatic manifestations of various malignancies as well as of common chemotherapeutic agents. Methods: A literature search was performed to identify key articles regarding the association of rheumatic disease with malignancy. Results: Our review focused on the association of rheumatic disease with malignancy, paraneoplastic syndromes with rheumatic manifestations, and chemotherapeutic agents related to rheumatic syndromes. We have discussed the importance of a newly described autoantibody that may identify patients at risk for malignancy associated myositis. Conclusion: Based on our literature review, recommendations are suggested regarding who and how patients should be screened for malignancy when presenting with various rheumatic symptoms.