Most thymomas are diagnosed and staged at the time of surgical intervention. Surgical resection is the preferred treatment for patients who can tolerate surgery and have a mediastinal mass that is suspected of being a thymoma. A total thymectomy with complete resection of all tumor tissue can be achieved in nearly all early stage patients. Later stages are associated with more morbidity and mortality, and postoperative radiation therapy is generally employed in these cases. Most thymomas in the later stages can only rarely be resected completely, and patients are usually offered debulking surgery and postoperative radiation therapy as palliative measures.
The likelihood of long-term survival depends on the completeness of surgical resection. (Seven-year survival following a complete resection is about 70%.) Resection of the pericardium and accompanying lung parenchyma is often required to achieve a complete resection with histologically negative margins. Radiation therapy may be useful in the management of patients with microscopic or macroscopic residual thymoma or thymic carcinoma after an incomplete surgical resection, as an adjuvant following complete resection of an invasive tumor, and for those with locally advanced or metastatic unresectable disease. Radiation is also useful in recurrent disease.
In patients with locally invasive tumor or large bulky masses, immediate surgical resection may not be technically feasible. Combination chemotherapy followed by radical resection with or without postoperative adjuvant therapy is considered experimental at this point. The most effective agents against thymoma are cisplatin, doxorubicin, cyclophosphamide, ifosfamide, and steroids. Single-agent and combination therapy have been used in adjuvant and neoadjuvant treatment, but combination therapy is more effective; combination therapy that includes cisplatin is perhaps the most effective chemotherapy.
Patients with thymoma are also at risk for the development of other malignancies, which have been reported in 17%-28% of patients following thymectomy. In a series of 849 cases of thymoma identified through the Surveillance Epidemiology and End Results (SEER) database, the risk was significantly increased for B-cell non-Hodgkin lymphoma, gastrointestinal cancers, and soft-tissue sarcomas (standardized incidence ratios [SIR] 4.7, 1.8, and 11.1, respectively, compared with the number of cases expected in the general population).
In this case, a multilobular tumor was present in the anterior superior section of the mediastinum, which is usually occupied by the thymic cortex. The mass had occasional calcifications surrounded by small tumor nodules. The pericardial infiltration measured up to 3 cm from the base of the heart to the apex area. The lungs and the abdomen were free of tumor infiltrations. The patient was transferred to the nearby university hospital and was admitted to the intensive care unit of the cardiology department, wherein pericardiocentesis was performed; 500 mL of bloody pericardial effusion were drained, samples of which were sent for cytology. A CT scan of the thorax and the abdomen was performed, which showed that the pericardial effusion had almost completely regressed. Serum alpha-fetoprotein and beta–human chorionic gonadotropin findings were negative.
Because of the tumor's location, a fine-needle aspiration biopsy or a bronchoscopy (with transbronchial biopsy) was not feasible; therefore, a video-assisted thoracic surgical biopsy under general anesthesia was performed. Further pertinent lab results included a urine electrophoresis with no trace of Bence-Jones proteinuria and a plasma electrophoresis showing signs of an inflammatory process, with normal immunoglobulins and no M components. The cellular analysis of the pericardial effusion showed few lymphoid cells associated with an inflammatory process. The biopsy confirmed the presence of a thymoma.
The patient was transferred to the pulmonary medicine department with a combined treatment plan of chemotherapy, radiation treatment, and surgery. At the time of this report, the patient had completed the third chemotherapy cycle with cisplatin, doxorubicin, and cyclophosphamide. The next planned step was surgical extraction of the tumor.
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