Malignant Primary and Metastatic Cardiac Tumors: A Single-Center 27-Year Case Review.

BACKGROUND: Malignant primary cardiac tumors are exceedingly rare, and despite surgical exeresis or chemotherapy, their prognosis remains poor. Cardiac invasion by metastatic tumors, while more common, also entails an unsatisfactory outcome. This study aimed to review patients diagnosed with malignant primary and secondary cardiac tumors in a tertiary center between 1995 and 2022. METHODS: Clinical data, echocardiographic, computed tomography, and magnetic resonance assessments of tumor location and morphology, histology, treatment, and survival were retrospectively analyzed. RESULTS: Sixty malignant cardiac tumors were diagnosed: 17 primary (A) and 43 metastatic (B) tumors. A: the most common types were angiosarcoma (41%), undifferentiated sarcoma (23%), and fibrosarcoma (18%). Patients with primary tumors were younger than patients with metastatic tumors (41 ± 13 years vs. 57 ± 18 years, p = 0.001), with no significant gender difference. The most frequent presentations were heart failure (59%) and arrhythmia (23%). The most prevalent tumor location was the right heart chambers (71%), mostly in the right atrium (35%). 47% were submitted to tumor resection, and 29% received chemotherapy. The mortality rate was 82% with a median survival of 6.0 (interquartile range: 1.0-11.8) months after diagnosis (minimum of 12 days and maximum of 19 years). One patient with fibrosarcoma underwent heart transplantation and was still alive and well after 19 years. B: regarding metastatic cardiac invasion, the most common primary tumor sites were lung carcinomas (38%), thymomas (17%), and lymphomas (14%). Presentation with pericardial effusion was common (33%). The mortality rate was 72%, with a median survival of 3.6 (1.0-13.4) months (minimum of 7 days, maximum of 5 years). CONCLUSION: Diagnosis of metastatic cardiac tumors was more common than that of malignant primary tumors, both with a dismal prognosis. When radical exeresis is not possible, heart transplantation can be an option with a favorable outcome in carefully selected patients with sarcomas.

Surgical resection of Masaoka stage III thymic epithelial tumours with great vessels involvement: a retrospective multicentric analysis from the European Society of Thoracic Surgeons thymic database.

OBJECTIVES: The aim of this study was to analyse the outcomes of an international cohort of patients affected by Masaoka stage III thymic epithelial tumours with vascular involvement and treated by surgery. METHODS: Study design was the observational multicentre retrospective cohort study. Data were extracted from the European Society of Thoracic Surgeons thymic database; additional variables were collected. Inclusion criteria were as follows: stage III (Masaoka-Koga) thymic epithelial tumours; surgery with radical intention; clinical or pathological great vessels involvement; and radiologically suspected or diagnosed intraoperatively. Outcome items were analysed. RESULTS: Sixty-five patients submitted to surgery from 2001 to 2017 fulfilled inclusion criteria. Thymoma and thymic carcinoma patients did not differ for demographics and clinical characteristics. The majority of great vessel treated were superior vena cava or innominate veins (72.3%). Eleven patients (16.9%) had postoperative cardiopulmonary complications; vascular stenosis was observed in 3 patients (4.6%). The multivariable Cox analysis for disease-free survival showed an increased hazard of recurrence for thymic carcinoma (hazard ratio = 3.59; 95% confidence interval: 1.66-7.78, P = 0.001). The 1-, 3-, 5- and 10-year overall survival rates were 0.86, 0.84, 0.81, and 0.53, respectively. There was no significant difference in overall survival according to resection status or between thymoma and thimic carcinoma. The univariable Cox regression model did not show an increased hazard of death for myasthenic patients considering all resection status and for patients who received neoadjuvant therapy. CONCLUSIONS: We observed that clinical outcomes of patients treated for stage III thymic epithelial tumours with vascular involvement are satisfactory suggesting to increase the confidence in dealing with these complex surgeries. Complete resection should be achieved, even though extensive vascular reconstructions are required.

Thymomectomy plus total thymectomy versus simple thymomectomy for early-stage thymoma without myasthenia gravis: a European Society of Thoracic Surgeons Thymic Working Group Study.

OBJECTIVES: Resection of thymic tumours including the removal of both the tumour and the thymus gland (thymothymectomy; TT) is the procedure of choice and is recommended in most relevant articles in the literature. Nevertheless, in recent years, some authors have suggested that resection of the tumour (simple thymomectomy; ST) may suffice from an oncological standpoint in patients with early-stage thymoma who do not have myasthenia gravis (MG) (non-MG). The goal of our study was to compare the short- and long-term outcomes of ST versus TT in non-MG early-stage thymomas using the European Society of Thoracic Surgeons thymic database. METHODS: A total of 498 non-MG patients with pathological stage I thymoma were included in the study. TT was performed in 466 (93.6%) of 498 patients who had surgery with curative intent; ST was done in 32 (6.4%). The completeness of resection, the rate of complications, the 30-day mortality, the overall recurrence and the freedom from recurrence were compared. We performed crude and propensity score-adjusted comparisons by surgical approach (ST vs TT). RESULTS: TT showed the same rate of postoperative complications, 30-day mortality and postoperative length of stay as ST. The 5-year overall survival rate was 89% in the TT group and 55% in the ST group. The 5-year freedom from recurrence was 96% in the TT group and 79% in the ST group. CONCLUSION: Patients with early-stage thymoma without MG who have a TT show significantly better freedom from recurrence than those who have an ST, without an increase in postoperative morbidity rate.

Severe Intraoperative Complications During VATS Anatomical Ressections and their Surgical Resolution in a Portuguese Thoracic Surgery Centre - A 9-Year Review.

INTRODUCTION: Many studies have demonstrated that video-assisted thoracoscopic surgery (VATS) is not only feasible and safe but is actually the approach chosen for an increasing number of pulmonary anatomic resections. There are however few studies reporting on severe intraoperative complications during VATS anatomical ressections and their resolution. OBJECTIVE: Our aim is to analyse the incidence of severe intraoperative complications during VATS anatomical ressections, at our department, in the past nine years, and describe their technical resolution during the surgery. METHODS: We performed the retrospective analysis of the patients submitted to lobectomy, bilobectomy or segmentectomy by VATS or VATS converted to thoracotomy at Hospital de Santa Marta, between May 2008 and September 2017. Severe intraoperative complications were defined as an event that results in a life threatening situation or an injury to a proximal airway, blood vessel or organ that would lead to an unplanned additional anatomical resection. RESULTS: A total of 151 patients were submitted to anatomical ressections, 90,7% (n=137) of them for a primary lung cancer, other indications were metastatic disease 6%(n=9) and benign disease in 3,3% (n=5). The surgery was a lobectomy in 94% of the cases (n=142), a segmentectomy in 5% (n=8), and one bilobectomy. The conversion rate to thoracotomy was 12% (n=18), most of which were for technical/ oncological reasons (n=11), and 7 others were to control bleeding. Four (2,6%) severe intraoperative complications were identified. Three of them (2%) were erroneous transections of bronchovascular structures (left main bronchus, left main pulmonary artery and both left pulmonary veins); and one was a membranous airway injury proximal to the staple line. There were no intraoperative deaths. The three patients with erroneous bronchovascular transection were converted to thoracotomy and the bronchial or vascular re-anastomosis was performed, therefore avoiding a left pneumonectomy. In the patient with the membranous airway injury, the bronchoplastic suture was performed by VATS. All four patients were primary lung cancer patients. In all these cases the patients were discharged alive and well and are undergoing their follow-up program with no signs of disease recurrence. CONCLUSION: Albeit rare, severe complications during VATS Lobectomy can occur but when they happen the thoracic surgeon has to be ready to solve them with the minimal repercussion for the patient.

Major Complications of Videomediastinoscopy and their Resolution - a 5 year experience.

INTRODUCTION: Videomediastinoscopy is an invasive procedure for mediastinal assessment, with low rates of morbidity and mortality. Despite the low risk of complications, they can be potentially lethal if not immediately controlled. OBJECTIVE: The goal of this study is to analyse the overall incidence of complications of videomediastinoscopies, performed in the last 5 years at our department, as well as their resolution and outcomes. METHODS: A retrospective review of all videomediastinoscopies performed at a single institution during a 5-year period was performed. Major complications were defined as life-threatening events. RESULTS: During the study period, from July 2012 to July 2017, were performed 160 mediastinoscopies, 67 were diagnostic and 93 for staging. There were 3 major complications (1.87%), of which a severe haemorrhage from a bronchial artery, a tracheal rupture, and a massive haemorrhage from an innominate artery laceration. In this 3 cases, the diagnosis were lung cancer in 2 patients and lymphoma in the other one. There were no intraoperative deaths. One patient died in the postoperative period due to mediastinitis and disease progression. The patient who suffered innominate artery laceration, had a stroke due to dissection of the right carotid artery. During follow-up, one patient died from progression of oncologic disease, and the other one is alive 4 years later. CONCLUSION: Although mediastinoscopy has a low rate of complications, these can be potentially lethal and the thoracic surgeon should be able to resolve them rapidly. Due to the scarcity of publications on this subject, it is important to describe potential complications of this surgical procedure and their clinical resolution.

[Anorexia and Weight Loss as First Symptoms of a Solitary Fibrous Tumor of the Pleura]. / Anorexia e Perda Ponderal, Os Primeiros Sintomas de Um Tumor Fibroso Solitário da Pleura.

We report the clinical case of a 66 year-old woman with anorexia and weight loss, in whom a giant mass was found in the left pulmonary field. A computorized tomography guided fine-needle biopsy established the diagnosis of a solitary fibrous tumor of the pleura. Surgical resection of a 2655 grams of tumor mass was performed and the pathological examination confirmed the diagnosis. Nine months after surgery, the patient remained free of symptoms and with no evidence of disease recurrence. Solitary fibrous tumor of the pleura is a rare entity with mesenchymal origin. Although most solitary fibrous tumors of the pleura are benign, they possess a malignant potential and thus should be totally excised. Diagnosis is generally incidental, being the majority of the patients asymptomatic. However, extrinsic compression of the solitary fibrous pleural tumor on lung parenchyma may cause symptoms, from which the commonest are cough, dyspnea, and chest pain. The clinical presentation, suggestive of a consumptive and malignant disease reinforced the heterogeneity of this disease and the singularity of this clinical case, thus justifying its presentation.

[Reconstructive surgery of the large intrathoracic veins, four case reports]. / Cirurgia Reconstrutiva das Grandes Veias Intratorácicas, a Propósito de Quatro Casos Clínicos.

The resection and reconstruction of the large venous vessels of the chest is a procedure intended to treat the symptoms of venous hypertension caused by the Superior Vena Cava Syndrome and to allow resection of mediastinal tumors that invade the superior vena cava (SVC) and the left and right innominate veins. We report four clinical cases of mediastinal tumors involving the large intrathoracic venous vessels, submitted to surgery between 2010 and 2013. In all cases our purpose was to completely resect the tumor. We intended to evaluate the surgical results in terms of improvement of symptoms, complications of the procedures, permeability of the bypasses in the short and medium term and mortality rates. We used ringed ePTFE grafts to perform the following vascular reconstructions: - Y configuration bypass from the left subclavian vein and the left internal jugular vein to the left inominate vein; - two bypasses from the top of the left innominate vein to the right atrial appendage; - bypass from the left innominate vein to the right atrial appendage and a bypass from the right innominate vein to the SVC. All patients were discharged, and all the bypasses were patent at discharge and after 30 days . There were two cases of late thrombosis, but patients remained asymptomatic. Our series shows the feasibility of these technically complex surgeries, which are an excellent example of the benefits of multidisciplinary collaboration between vascular and thoracic surgeons.

[Videothoracoscopic anatomic pulmonary segmentary: an initial single-center experience]. / Segmentectomia Pulmonar Anatómica por Videotoracoscopia: Experiência Inicial de um Centro.

The use of minimally invasive surgery for the treatment of lung cancer has been growing worldwide. Between May 2008 and November 2012, we performed 24 videothoracoscopic anatomical lung resections in our department. This includes 22 lobectomies and 2 anatomic segmentectomies, which is known to be a more complex surgery, since it demands a finer dissection of sub-lobar structures. We report the clinical cases of two patients who underwent anatomic segmentectomies. The first one was a 63 year old woman, smoker and with a history of breast cancer 20 years earlier. An incidental 9 mm node was found in the lingula. The patient underwent an anatomic lingulectomy and the frozen section was suggestive of a primary lung cancer. Therefore, we proceeded to a full lymphadenectomy. The final pathology evaluation showed a typical carcinoid tumour (pT1aN0). The second patient was a 50 year old woman, a smoker and with a heavy family history of lung cancer. In a screening CT scan a 8 mm ground glass opacity was identified in the left lower lobe (segment VI). After a VATS wedge resection of the node the frozen section evaluation was compatible with adenocarcinoma. We then proceeded to an anatomic segmentectomy with lymphadenectomy. The definitive pathology evaluation confirmed that it was a pT1a N0 bronchioloalveolar adenocarcinoma. The patients now have 5 and 2 months of follow up respectivelly and neither of them has signs of recurrence and the surgical incision showed a good aesthetic result. Anatomic segmentectomy is the indicated surgery especially in patients with low grade tumours, in early stage lung cancers or in patients without pulmonary function for a lobar resection, and it can be done safely using VATS.

[Resection of bronchogenic cyst by video-mediastinoscopy]. / Ressecção de quisto broncogénico por vídeomediastinoscopia.

A bronchogenic cyst is a congenital malformation originating from the ventral primitive gut. It may be located in the mediastinum or in the lung parenchyma. Its location depends on the stage of gestation in which it developed. Despite being a histological benign tumor, many authors recommend its complete excision in order to obtain histological confirmation and to prevent future complications. The traditional approaches for excision are thoracotomy or video-assisted thoracoscopic surgery (VATS). However, a minimally invasive approach through vídeomediastinoscopy constitutes a valid alternative in selected cases. The authors present a case of a 23 years old female patient, admitted to the emergency department with retrosternal pain, dyspnea at rest, tachycardia, polypnea, infra-clavicular accessory muscle contraction, jugular vein distention and hypoxemia. A Chest CT revealed a large cystic lesion of the middle mediastinum, with compression of the vascular structures, deviation of the tracheobronchial tree and reduction in the diameter of the main bronchi. The patient was referred for surgical treatment. A mini-cervicotomy incision was made, and with elevation of the sternum a video-mediastinoscope was introduced. With bimanual instrumentation, complete excision of a large mediastinal cyst of the middle mediastinum was performed. The cyst was located in the subcarinal and pre-tracheal space, had contact with the left and right main bronchi, esophagus, roof of the left atrium, pulmonary artery and superior pulmonary veins. The histological evaluation revealed a bronchogenic cyst. There were no immediate postoperative complications. The patient remains asymptomatic after two years of follow-up, and without recurrence.

Primary angiosarcoma of the pericardium: case report and review of the literature.

Primary cardiac tumours are rare entities and angiosarcoma is the most frequent primary cardiac malignant tumour. Mean survival is six months and the tumour responds poorly to chemotherapy. We present the case of a 50 year-old patient with localised pericardial angiosarcoma who survived 23 months after diagnosis with a combined approach of chemotherapy and surgery.

[Pulmonary metastasis in a cardiac angiosarcoma - case report and discussion]. / Metastização pulmonar na apresentação de angiossarcoma cardíaco - Caso clínico e discussão.

We present a case report of a 35 year-old male without any relevant former pathology admitted to the emergency room with atypical chest pain, cough and sputum with ECG changes suggesting pericarditis. He was initially admitted to the cardiology ward and experienced clinical improvement after initiating anti- inflammatory treatment. As intercurrence he had bacterial origin pneumonia complicated by pleural effusion (PE). After discharge patient was referred to a pulmonology appointment where aetiological investigation of the PE was instigated. Investigation revealed changes in the cardiac cavities and multiple lung nodules, suggesting subacute endocarditis with septic pulmonary embolism. Admitted to the pulmonology unit patient underwent videothoracsopy which diagnosed cardiac angiosarcoma with pulmonary metastisation. Within a few days there was a rapid evolution of the clinical picture, with cardiac failure and death of the patient without radiotherapy or adjuvant chemotherapy being started.

[Mediastinal teratoma with malignant transformation of the somatic component. Clinical report]. / Teratoma de mediastino com transformação maligna do componente mesenquimatoso: a propósito de um caso clínico.

Mediastinal germ cell tumours (M-GCT) are rare forms of neoplasms compared with other tumours of the same location. They are classified in seminomas, malignant non-seminomatous GCT and teratomas. The malignant transformation of the somatic component of the teratoma, with sarcomatous or carcinomatous degeneration, is even more uncommon. We report the clinical case of a 32 year old man who presented with severe chest pain on the right hemithorax. The image exams revealed the existence of a large heterogeneous lesion with a diameter of 7.7 cm, with areas of lipomatous density and a calcic image with the appearance of a tooth, in the right projection of the anterior mediastinum, in the vicinity of the large vessels, compatible with teratoma. The transthoracic biopsy (CT guided) showed morphologic aspects of sarcoma. The patient was operated on with the en bloc resection of the mediastinal mass, right lung, a segment of the pericardium and the thymus. The pathological studies showed a teratoma with malignant transformation of the mesenquimatous component, with muscular differentiation into leiomiosarcoma and rabdomiosarcoma. After surgery, the patient was treated with a scheme of doxorubicin and ifosfamide. The most prominent concepts related to this clinical entity, as well as its treatment, are debated in this article, based on the most recent publications dedicated to the subject.

[Post-partum spontaneous coronary artery dissection. Clinical report]. / Dissecção coronária espontânea pós-parto. A propósito de um caso clínico.

The authors report the clinical case of a 32 years old woman who suffered a spontaneous dissection of the common trunk, anterior descending, intermediary and circumflex coronary arteries, on the 6th post partum day. The diagnosis of acute myocardial infarction was made, through ECG and enzymatic studies and the patient was transferred to a Coronary Unit. Coronary angiography disclosed those lesions and the patient underwent a triple coronary bypass graft, under extracorporeal circulation. The post operative course was uneventful. Eight months after the operation she was found in good condition, free of symptoms and with a normal pattern of life.