Thymic tumor

Thymomas account for the vast majority of thymic neoplasms and are often associated with autoimmune or neurologic syndromes, most commonly myasthenia gravis.

About one third of thymomas are asymptomatic and identified as an incidental radiographic finding. A chest computed tomography (CT) scan with intravenous contrast in the appropriate demographic can be highly suggestive of thymoma.

Small, encapsulated tumors are resected for both pathologic diagnosis and treatment. For anterior mediastinal tumors with a substantial chance of representing lymphoma, core needle biopsy is recommended. Positron emission tomography (PET)/CT scan can be very useful in differentiating thymoma from lymphoma prior to biopsy as lymphoma has a higher maximum standardized uptake value (SUVmax) compared to thymoma.

Thymic malignancies that are locally advanced at presentation are usually treated with neoadjuvant therapy (chemotherapy ± radiation therapy) followed by reassessment for surgery. Patients with myasthenia gravis should have myasthenic symptoms optimized before surgery.

Thymomas are relatively indolent tumors with high cure rates; however, all thymomas are considered malignant. Prognosis depends largely on completeness of resection, stage, and WHO histology. Thymic carcinomas and neuroendocrine tumors of the thymus (NETT) are generally more aggressive.

Treatment for locally advanced and metastatic tumors should involve a multidisciplinary team of medical oncologists, thoracic surgeons, radiation oncologists, pathologists, and radiologists who have experience and expertise in thymic malignancies.

Thymic tumors are rare, although they are the most common anterior mediastinal tumors in adults.[1]Hsu CH, Chan JK, Yin CH, et al. Trends in the incidence of thymoma, thymic carcinoma, and thymic neuroendocrine tumor in the United States. PLoS One. 2019;14(12):e0227197. https://journals.plos.org/plosone/article?id=10.1371/journal.pone.0227197 http://www.ncbi.nlm.nih.gov/pubmed/31891634?tool=bestpractice.com ​​[2]Girard N, Ruffini E, Marx A, et al. Thymic epithelial tumours: ESMO Clinical Practice Guidelines for diagnosis, treatment and follow-up. Ann Oncol. 2015;26(suppl 5):v40-v55. https://www.sciencedirect.com/science/article/pii/S0923753419471779 http://www.ncbi.nlm.nih.gov/pubmed/26314779?tool=bestpractice.com Thymomas account for the majority of thymic neoplasms. Although smaller tumors are typically asymptomatic and identified incidentally on imaging (unless associated with myasthenia gravis), large tumors can present with chest discomfort, cough, and/or dyspnea, with or without myasthenia gravis symptoms.[3]Detterbeck FC. Clinical value of the WHO classification system of thymoma. Ann Thorac Surg. 2006;81:2328-34. https://www.annalsthoracicsurgery.org/article/S0003-4975(05)02367-2/fulltext http://www.ncbi.nlm.nih.gov/pubmed/16731193?tool=bestpractice.com About one third of thymomas are diagnosed as an incidental finding.

In-depth management of myasthenia gravis is beyond the scope of this topic. See Myasthenia gravis.