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120829a - THYMIC CARCINOMA

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Paper written by Professor Omar Hasan Kasule Sr.


The thymus gland is in the upper part of the chest found in children and grows reaching maximum size at puberty then it starts decreasing in size until it virtually disappears. Its function is confined to childhood. It seeds the immune system with the thymus-derived lymphocytes called T cells. The T cells are the main stay of body defence against viral infections, cancer, and other diseases.

Thymic carcinomas are extremely rare[1] being found in 3 out of 100,000 (0.003%). They were first reported about 30 years ago[2]. They arise from embryonic tissue[3] that normally has disappeared by adulthood and are therefore sometimes called orphan tumors[4].  Histological diagnosis (examining the tissue under the microscope) is not easy because this group of tumors resembles several other tumors of the chest area. They present in different clinical and histological manifestations. They are indolent and slow growing. They are silent with no obvious clinical signs and symptoms. About 30% percent of cases are diagnosed by chance when doctors are making diagnostic work-ups for something else. They can also be aggressive and spread inside and outside the chest cavity. Research is limited because of the rarity of the tumor in clinical practice.

The treatment is multimodal[5] (involving surgery and chemotherapy). The role of radiotherapy is unclear[6],[7]. Surgery followed by chemotherapy is used for the aggressive forms of the disease[8].

Research results show the efficacy multimodal therapy[9]. A 59 year old with locally advanced disease had successful pre-operative chemotherapy followed by surgery and was free of disease after 46 months[10]. Combination chemotherapy with doxorubicin, vincristine, cyclophosphamide, and platinum compounds was an effective and well-tolerated treatment for unresectable advanced thymic carcinoma and side effects were mild [11]. Cisplatin and irinotecan combination chemotherapy appeared to be acceptable for advanced thymic carcinoma with disease control achieved in 88.9% of patients[12]. Thymic carcinoma was found sensitive to platinum-based chemotherapy and that amrubicin appeared to have significant activity against thymic carcinoma[13]. Combined paclitaxel+carboplatin therapy exhibited activity in patients with unresectable thymic carcinoma[14]


Disease prognosis depends on the size, stage, and histology of the tumor. Small tumors with low stages with limited spread are easier to treat. Some tumors are histologically more aggressive while others are more amenable to treatment.


Clinical trials have shown long term survival after treatment of recurrent or progressive disease[15]. Computer tomography enabled detection of early disease and early surgical treatment resulting in long term survival[16]. Good prognosis and satisfactory 5 year survival for low stage disease[17]. Survival can be as high as 93.8%[18].


Research on thymic carcinoma is limited. The Foundation for Thymic Cancer Research (FTCR) was founded in 2003 to foster research on this little known disease. Its mailing address is: Foundation for Thymic Cancer Research 6 Cold Spring Court Mount Kisco, NY 10549. Contacts are: 1. Administrative 85 Lincoln Avenue Ardsley, NYjschwartz@thymic.org  2. Requests for patient contact and literature: P.O. Box 474 Longport NJ 08403 Tel (609) 214-8616 info@thymic.org. The Foundation has resources for patients to get more information about the disease and also to share stories for mutual moral support. The foundation’s website is www.thymic.org.


NOTES

[4] Okuma Y. Chemotherapy for thymic carcinoma. Gan To Kagaku Ryoho. 2012 May;39(5):702-7.  [Original Article in Japanese].
[17] Hosaka Y, Tsuchida M, Toyabe S, Umezu H, Eimoto T, Hayashi J. Masaoka stage and histologic grade predict prognosis in patients with thymic carcinoma. Ann Thorac Surg. 2010 Mar;89(3):912-7.