TY - CHAP M1 - Book, Section TI - Mediastinal Disease A1 - Plantmason, Lee A1 - Lui, Natalie A1 - Theodore, Pierre A2 - Yuh, David D. A2 - Vricella, Luca A. A2 - Yang, Stephen C. A2 - Doty, John R. PY - 2014 T2 - Johns Hopkins Textbook of Cardiothoracic Surgery AB - EpidemiologyThymic tumors are the most common primary neoplasm of the anterior mediastinum, accounting for about 20 percent of anterior mediastinal masses. Their peak incidence is in middle-aged adults between the ages of 30 and 50 years. Although lymphomas are the most common neoplasm of the mediastinum, primary mediastinal lymphomas are rare and account for less than 10 percent of lymphomas. Mediastinal germ cell tumors, including both seminomas and nonseminomas, account for about 20 percent of anterior mediastinal masses and, with the exception of teratomas, occur almost exclusively in males. All of these tumors have a peak incidence in young adults in their third decade of life. In general, extragonadal germ cell tumors are rarely primary tumors, with the mediastinum frequently being a site of metastasis.PathophysiologyPathophysiologic processes range from the antibody-mediated effects of myasthenia gravis (MG) seen with thymoma to the mass effects of tumors as noted in superior vena cava (SVC) syndrome. The primary mediastinal lymphomas include Hodgkin, non-Hodgkin, and lymphoblastic lymphomas. Seminomas, which occur exclusively in men, are generally malignant but highly responsive to treatment, whereas nonseminomas can range from benign teratomas that have a very low percentage of malignant transformation (8 cm) often receive neoadjuvant chemotherapy, and patients with Masaoka Stage III or IV often receive comprehensive treatment with chemotherapy, radiotherapy, and surgery. Treatment of MG includes immunosuppression with prednisolone, cyclosporine, and azathioprine; rituximab and cyclophosphamide for severely drug-resistant patients; cholinesterase inhibitors such as pyridostigmine; plasma exchange; and thymectomy. Treatment of lymphomas includes chemotherapy, radiotherapy, and immune modulators such as rituximab. Treatment of teratomas is surgical resection. Treatment of seminomas is cisplatin-based chemotherapy. For patients suffering from relapse, high-dose chemotherapy with autologous stem cell transplantation is suggested. In general, surgical resection is not performed for relapse due to the excessive morbidity from the desmoplastic reaction induced by chemotherapy. Treatment of nonseminomas includes chemotherapy and surgical excision for residual disease, given the poor results noted with salvage chemotherapy.Outcomes/PrognosisMost patients with thymoma can expect a favorable outcome, while patients with concomitant MG can expect a normal life expectancy ... SN - PB - McGraw-Hill Education CY - New York, NY Y2 - 2024/04/19 UR - accesssurgery.mhmedical.com/content.aspx?aid=1104586761 ER -