Encyclopedia of Cancer

2011 Edition
| Editors: Manfred Schwab

Thymoma

Reference work entry
DOI: https://doi.org/10.1007/978-3-642-16483-5_5802

Definition

An epithelial neoplasm derived from the thymic epithelium.

Characteristics

Thymomas are among the most common epithelial tumors of the thymic gland. The tumor may occur at any age, but it is very unusual for this neoplasm to occur in the pediatric age group. The tumor is most likely to occur in adult individuals of over 40 years of age. The most common anatomic site is in the anterior mediastinum. However, in rare instances, thymomas may also arise in the lung, pleura, and neck. Clinically, patients with thymomas may be completely asymptomatic, and their tumors may become apparent during a routine physical examination. Radiological studies will disclose the presence of an anterior mediastinal mass. On the other hand, thymomas may be associated with other medical conditions such as Myasthenia Gravis. In some series, myasthenia gravis has been associated with thymomas as high as 30–60% of the cases. Although myasthenia gravis is often associated with thymomas, a long and wide spectrum of medical conditions has also been associated with this neoplasm, including neuromuscular, hematologic, immune deficiency, collagen vascular, dermatologic, metabolic, and endocrine processes.

Diagnosis

The two most important parameters in the evaluation of thymomas are whether the tumor is encapsulated or invasive. If the tumor is invasive, pathological staging of the tumor needs to be determined by analyzing the extent of the invasion and/or whether the tumor is limited to the mediastinal region or involves structures such as the pleura, pericardium, lung, or other extrathoracic areas. Although, from the histopathological point of view, the diagnosis of thymoma may not represent a challenge, there is very limited information that can be provided in a small biopsy. The same can be said for fine needle aspirate cytology in which one cannot determine the invasion or encapsulation of the tumor. Thus, final diagnosis is best provided after complete surgical resection of the tumor. Histologically, the tumor may not pose a problem in diagnosis, and the vast majority of neoplasms will show similar histopathological features, namely the presence of epithelial cells in a background of lymphocytes, which recapitulates to some extent the normal cellular composition of the thymic gland. The presence of lymphocytes in thymomas may vary, and, in some cases, there is a predominance of lymphocytes, while in other cases, the lymphocytes are more inconspicuous. Thick fibrous bands composed of fibroconnective tissue separating the cellular proliferation are usually present giving the appearance of a nodular type of growth pattern. However, the essential component that must be evaluated with more detail is the tissue that is surrounding the tumor. In the great majority of tumors, there is a thick fibroconnective boundary, which is denominated “capsule.” If the tumor is confined within the boundaries of that capsule, then the tumor is diagnosed as encapsulated; in cases in which there is a breach of the capsule by the cellular proliferation, then the tumor is diagnosed as invasive. Although as stated above, the majority of tumors will display the characteristic histology, there is a group of tumors that may show unusual characteristics such as the presence of plasma cells, muscle cells, among others. In this setting, it is very important to be entirely familiar with these neoplasms in order to avoid an equivocal diagnosis. In terms of cytologic atypia and mitotic figures, thymomas do not display marked cellular atypia or increased mitotic activity. In some cases, areas of cystic degeneration, hemorrhage, or necrosis may be present, but it is still the cellular characteristics of the tumor and the presence of capsular integrity that are more significant in predicting outcome.

Classification

Over the years, thymomas have been the subject of numerous classification systems, which have the goal of predicting their pathologic behavior. Unfortunately, none of the current schemes appears to provide an unequivocal proof regarding the behavior that these neoplasms may follow. Essentially, there is still no cytologic feature that will determine which tumors are more likely to behave aggressively. The most important parameter in the evaluation of the tumors remains the capsular integrity. It has been demonstrated that tumors that are invasive from the outset are more likely to follow an aggressive course, while those tumors that are encapsulated are more likely to follow an indolent course. Thus, most of the classification schemes that have been presented derived from the need to provide clues as to the clinical course of the tumor. The most common schemes on the classification of thymomas are presented in Table 1.
Thymoma. Table 1

Histological schemes proposed for the classification of thymomas

WHO

Traditional

Muller–Hermelink

Suster and Moran

Type A

Spindle cell

Medullary

Thymoma

Type AB

Mixed

Thymoma

Type B1

Predominantly cortical

Thymoma

Type B2

Lymphocyte rich

Cortical

Thymoma

Type B2

Lymphoepithelial

Cortical

Thymoma

Type B3

Epithelial rich

Well-differentiated carcinoma

Atypical thymoma

Treatment and Prognosis

In cases of thymomas, surgical resection is the treatment of choice. However, if the determination is that the tumor is invasive or that the tumor is not completely resected, adjuvant radiation or chemotherapy may be indicated. In cases in which the tumor is large and involves important adjacent thoracic structures, the use of chemotherapy may be necessary prior to surgical resection. The prognosis of patients with thymomas depends largely on the determination of the tumor being invasive or encapsulated. Three main features should be considered in predicting prognosis in patients with thymomas: encapsulation, invasiveness, and resectability of the tumor at the time of diagnosis.

References

  1. 1.
    Suster S, Moran CA (2006) Thymoma classification: current status and future trends. Am J Clin Pathol 125:542–554PubMedCrossRefGoogle Scholar
  2. 2.
    Suster S, Moran CA (2005) Problems and inconsistencies in the WHO classification of thymoma. Semin Diagn Pathol 22:188–197PubMedCrossRefGoogle Scholar
  3. 3.
    Suster S, Moran CA (1999) Thymoma, atypical thymoma, and thymic carcinoma: a novel conceptual approach to the classification of neoplasms of thymic epithelium. Am J Clin Pathol 111:826–833PubMedCrossRefGoogle Scholar
  4. 4.
    Rieker RJ, Hoegel J, Morresi-Hauf A et al (2002) Histologic classification of thymic epithelial tumors: comparison of established classification schemes. Int J Cancer 98:900–906PubMedCrossRefGoogle Scholar
  5. 5.
    Marx A, Strobel P, Zettl A et al (2004) Thymomas. In: Travis WD, Brambilla E, Muller-Hermelink K et al (eds) Pathology and genetics. Tumours of the lung, pleura, thymus, and heart. International Agency for Research on Cancer (IARC), Lyon, pp 152–171Google Scholar

Copyright information

© Springer-Verlag Berlin Heidelberg 2011

Authors and Affiliations

  1. 1.Department of PathologyM D Anderson Cancer CenterHoustonUSA