Pediatric Thymoma Treatment & Management

  • Author: Cecilia P Mikita, MD, MPH; Chief Editor: Harumi Jyonouchi, MD   more...
 
Updated: Aug 12, 2010
 

Medical Care

  • Postoperative radiotherapy has been used for invasive thymoma and incompletely resected thymoma.
    • Commonly, radiotherapy has treated T tumors demonstrated to be unresectable on CT scan or with supraclavicular extension.
    • Primary radiotherapy in unresectable stage III or stage IVa disease has controlled local disease with a 5-year survival rate of 45-50%.[2]
    • Combination chemotherapy using cisplatin has been reported to have a response rating of 70-80%. Doxorubicin, vincristine, and cyclophosphamide have been used in combination chemotherapy.
  • The acquired immunodeficiency associated with thymoma should be treated with monthly replacement immunoglobulin (Ig) therapy. Replacement Igs may be intravenously or subcutaneously administered. Doses of intravenous Igs should be 300-400 mg/kg every 3 weeks or 400-500 mg/kg every 4 weeks with dose adjustment to maintain trough IgG levels above 500-600 mg/dL. In addition, prophylactic antibiotics may be required in addition to Ig replacement therapy to prevent bacterial infections in immunodeficient patients. Aggressive and long-term antibiosis is often required to treat bacterial infections in these patients.[21]
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Surgical Care

  • Because thymoma is usually well encapsulated and characterized by local spread, thymectomy can be curative in the early stages.
    • Encapsulated (stage I) tumors can usually be completely excised, and the local relapse rate is less than 5%. The relapse rate increases in more invasive stages.
    • Surgery can be challenging because of the tendency of the tumor to surround blood vessels, bronchi, and other mediastinal structures. Excessive bleeding can complicate thymectomy of large tumors that have become vascular or lacunar.
    • Tumor recurrence can occur even after complete resection.[29]
  • Eighty-five percent of patients with myasthenia gravis (MG) have some histologic abnormality of the thymus.
    • Thymectomy is considered a routine treatment for MG. Reportedly, resection of the thymoma is associated with improvement in weakness in 25% of patients, and almost one half of patients without thymoma improve after thymectomy.[13]
    • A recent study of 153 patients by Werneck et al compared thymectomy with conservative treatment groups in paired patients at similar stages and found no statistical difference between the conservative treatment and thymectomy groups.[30]
    • Thymectomy is believed to improve muscle weakness in 25% of individuals with MG and thymoma and in 50% of patients with MG without thymoma.
  • Thymectomy results in resolution of red cell aplasia in 30% of persons with this disorder.
  • The acquired immunodeficiency phenotype does not improve after thymectomy, and, in some persons, immunodeficiency has occurred years after resection of a thymoma.[31]
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Contributor Information and Disclosures
Author

Cecilia P Mikita, MD, MPH  Associate Program Director, Allergy-Immunology Fellowship, Associate Professor of Pediatrics and Medicine, Uniformed Services University of the Health Sciences; Hospital Intern Director, Staff Allergist/Immunologist, Walter Reed Army Medical Center

Cecilia P Mikita, MD, MPH is a member of the following medical societies: American Academy of Allergy Asthma and Immunology, American Academy of Pediatrics, American College of Allergy, Asthma and Immunology, and Clinical Immunology Society

Disclosure: Nothing to disclose.

Coauthor(s)

Richard A Bickel, MD  Fellow in Allergy/Immunology, Walter Reed Army Medical Center

Richard A Bickel, MD is a member of the following medical societies: American Academy of Pediatrics

Disclosure: Nothing to disclose.

Specialty Editor Board

Terry W Chin, MD, PhD  Associate Director, Pediatric Allergy/Immunology/Pulmonology, Miller Children's Hospital, Long Beach Memorial Medical Center; Associate Professor, Department of Pediatrics, University of California, Irvine, School of Medicine

Terry W Chin, MD, PhD is a member of the following medical societies: American Academy of Allergy Asthma and Immunology, American Association of Immunologists, American College of Allergy, Asthma and Immunology, American College of Chest Physicians, American Thoracic Society, California Thoracic Society, Clinical Immunology Society, and Western Society for Pediatric Research

Disclosure: Nothing to disclose.

Mary L Windle, PharmD  Adjunct Associate Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference

Disclosure: Nothing to disclose.

David J Valacer, MD  Consulting Staff, Hoffman La Roche Pharmaceuticals

David J Valacer, MD is a member of the following medical societies: American Academy of Allergy Asthma and Immunology, American Academy of Pediatrics, American Association for the Advancement of Science, American Thoracic Society, and New York Academy of Sciences

Disclosure: Nothing to disclose.

David Pallares, MD  Clinical Assistant Professor, Department of Pediatrics, Division of Allergy and Immunology, University of Louisville School of Medicine

David Pallares, MD is a member of the following medical societies: American Academy of Allergy Asthma and Immunology

Disclosure: Nothing to disclose.

Chief Editor

Harumi Jyonouchi, MD  Associate Professor, Division of Pulmonary, Allergy/Immunology, and Infectious Diseases, Department of Pediatrics, University of Medicine and Dentistry of New Jersey-New Jersey Medical School

Harumi Jyonouchi, MD is a member of the following medical societies: American Academy of Allergy Asthma and Immunology, American Academy of Pediatrics, American Association of Immunologists, American Medical Association, Clinical Immunology Society, New York Academy of Sciences, Society for Experimental Biology and Medicine, Society for Mucosal Immunology, and Society for Pediatric Research

Disclosure: Nothing to disclose.

Additional Contributors

The authors and editors of eMedicine gratefully acknowledge the contributions of previous author Marion Johnson, MD, to the development and writing of this article.

References

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Table 1. Comparison of the Different Classifications of Thymic Epithelial Tumors[26]
Clinicopathologic ClassificationWHO TypeTerminology of the Histogenetic Classification for the Histologic Subtypes of Thymic Epithelial Tumors
Benign thymomaA



AB



Medullary thymoma



Mixed thymoma



Malignant thymomas,



Category I



B1



B2



B3



Predominantly cortical thymoma



Cortical thymoma



Well-differentiated thymic carcinoma



Malignant thymomas,



Category II



CEpidermoid keratinizing (squamous cell) carcinoma



Epidermoid nonkeratinizing carcinoma



Lymphoepithelioma-like carcinoma



Sarcomatoid carcinoma (carcinosarcoma)



Clear cell carcinoma



Mucoepidermoid carcinoma



Undifferentiated carcinoma



Table. Macchiarini et al (1991)[34]
Cisplatin75 mg/m2 on day 13 courses repeated q3wk
Epirubicin100 mg/m2 on day 1
Etoposide120 mg/m2 on days 1, 3, and 5
Surgery and radiation



in patients with



complete or partial



response to chemotherapy



4500 cGy if complete



resection



6000 cGy if incomplete



resection



Table. Loehrer et al (1997)[35]
Cisplatin50 mg/m22-4 cycles q3wk
Doxorubicin50 mg/m2
Cyclophosphamide500 mg/m2
Followed by radiation54 Gy to the primary tumor and lymph nodes
Table. Venuta et al (1997)[36]
Cisplatin75-100 mg/m2 on day 1Repeated q3wk 3 times before surgery and 2 or 3 times after surgery
Epirubicin hydrochloride100 mg/m2 on day 1
Etoposide120 mg/m2 on days 1, 3, and 5
Postoperative radiation in patients with radical resection30 GyDelivered in 3 wk with 5 fractions per wk
Postoperative radiation in



patients with incomplete resection



50 GyDelivered in 5 wk with 5 fractions per wk
Table. Palmieri et al (1999)[37]
Octreotide1.5 mg/d SCIn patients shown to have



somatostatin receptors



Lanreotide30 mg/d SC q14dSwitch to this longer-acting



somatostatin analogue or depot form of octreotide if short-acting octreotide



is well tolerated



Prednisone0.6 mg/kg/d PO



for 3 mo, then



decreasing to 0.2 mg/kg



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