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Abstract: Background: Primary cardiac and pericardial tumours are rare with a prevalence of between 0.001% and 0.3%. Thus, general pathologists are not familiar with them. Modern advances in cardiac imaging have increased the number of patients identified with a primary cardiac tumour in its early stage and also improved prognosis. At the Royal Brompton Hospital, London, we did a retrospective study to investigate the pathological features of primary cardiac and pericardial tumours and compared our findings to other cardiac centres. Methods: All pathologic records at the Royal Brompton Hospital between 1990 and 2008 were reviewed to identify patients with a confirmed diagnosis of primary cardiac tumours. A total of 94 patients with a histological diagnosis of primary cardiac and pericardial tumours were identified and formed the study population. Results: The majority (n=67, 71.3%) of cases were benign cardiac tumours. Myxoma was the most common histologic type accounting for 27 cases. Among cases with primary malignant tumours (n=27, 28.7%), unclassified sarcoma (n=11), leiomyosarcoma (n=5), and lymphoma (n=4) were the most common histologic types. Conclusion: This study, primarily from an adult setting (n=78, 83%) demonstrates a large spectrum of cardiac tumours seen in recent cardiologic practice. Myxoma is still the most common tumour but more fibroelastomas are being diagnosed due to increased imaging. [ABSTRACT FROM AUTHOR]

Study objectives: To determine whether the new classification system for thymic carcinoid tumors/neuroendocrine carcinomas provides prognostic data, and to study the presentation, diagnosis, treatment, and prognostic factors of these rare tumors. Design: Retrospective analysis. Setting: Royal Brompton Hospital, London, UK. Results: Eight men and four women with a median age of 58 years. Complete excision was possible in nine patients. Postoperative staging revealed two stage I tumors, two stage II tumors, three stage III tumors, one stage IVA tumor, and four stage IVB tumors. All tumors demonstrated the histopathologic features of neuroendocrine tumors, which were confirmed by positive immunohistochemical staining for chromogranin A in 11 of 12 tumors and for CD56 in 12 of 12 tumors, and the presence of dense core granules on ultrastructural analysis in 9 of 9 tumors. All 12 tumors did not stain positively for somatostatin receptors. Three tumors were grade 1, six cases were grade 2, and three eases were grade 3. Follow-up was available in all patients. One patient died 1 month postoperatively. Distant metastasis developed in nine patients (82%). Local recurrence was evident in six patients, of whom five had not received postoperative radiotherapy. Seven patients died of distant metastasis (22 to 83 months after surgery). Two are alive and disease-free (at 67 and 81 months), and two are alive with disease (at 60 and 86 months) Conclusions: Neither grading as neuroendocrine carcinomas nor any individual histologic parameter showed a significant association with prognosis. Initial aggressive treatment, including complete surgical excision and adjuvant radiotherapy, appears to offer the best hope for prolonged survival. Adjuvant chemotherapy also should be considered, since the incidence of distant relapse is high. [ABSTRACT FROM AUTHOR]