There are many Clinical Trials that are being conducted for Thymic Carcinoma.
Enclosed is an article about Immunotherapy and whether this treatment is right for you. This article is from the NY Times.
A hopeless journey ends with much success with Immunotherapy. Watch Shannon Semple's story on her journey with Thymic Carcinoma
The on-going fight with a determined Wendy Chioji against Thymic Carcinoma.
Many of the doctors and people talk about our cancer as being as rare as a Unicorn. So welcome Beppe the Thymicorn our new mascot for Thymic Carcinoma
The story of Michael Delaney's fight against Thymic Carcinoma and his journey to beat Thymic Carcimona.
Received 2015 Oct 13; Accepted 2015 Nov 16.
The rarity of thymic epithelial tumors (TETs) creates unique challenges in conducting translational research and developing newer paradigms of treatment (1). Nevertheless, steady progress in genome research, the development of thymic cell lines, and an increase in collaborative efforts between various institutions have helped in fostering an increase in the understanding of the biology of TETs and associated paraneoplastic syndromes and given an impetus to the development of newer treatment options for patients with these rare tumors.
Within the last few years, a number of unique genomic changes have been described in TETs (2). Thymic carcinomas have been found to frequently harbor mutations in epigenetic regulatory genes (3). A specific mutation in GTF2I on chromosome 7 is present at a high frequency in World Health Organization (WHO) subtype A and AB thymomas (4). A nine-gene signature has been developed to predict the metastatic behavior of thymomas (5). The ongoing study of thymoma as part of The Cancer Genome Atlas (TCGA) project promises to add significantly to the existing body of knowledge about genomic alterations in TETs.
The association between thymomas and autoimmune paraneoplastic disorders is well recognized (6). Some patients with thymoma are also more susceptible to infectious complications due to underlying immune dysregulation (7). Various pathogenic mechanisms implicated in the development of immune dysfunction include decreased expression of the autoimmune regulator (AIRE) gene and the presence of anti-cytokine antibodies (8–13). These discoveries have influenced the screening and management paradigms for patients with thymoma.
Treatment strategies for newly diagnosed and recurrent TETs have also evolved over time. Surgery is considered the cornerstone of management of early stage TETs and complete resection of the tumor has a major impact on prognosis (14). For locally advanced disease, thymectomy with en bloc removal of all involved structures is indicated (15). Active areas of investigation focusing on the surgical management of TETs include an evaluation of minimally invasive surgery and an assessment of the role of surgery for recurrent TETs (16–19). Though of unproven benefit and with some controversy, post-operative radiotherapy is recommended after resection of stage III and IVA TETs, and can be considered for stage II thymic carcinoma and cases of stage II thymoma at high risk for recurrence (WHO B3 histology; extensive transcapsular invasion) (15, 20, 21). Chemotherapy is used for induction therapy in cases of stage III/IVA TETs and for treatment of unresectable and recurrent disease (15). There is scant evidence to support the use of chemotherapy after resection of TETs (22).
Contributions to the research topic on “Novel Treatments for Thymoma and Thymic Carcinoma” highlight recent developments in the understanding of the biology of TETs and review various aspects of management of TETs. Huang and colleagues describe previously unreported changes in the expression of apoptosis-related genes in WHO subtype B3 thymomas and thymic squamous cell carcinomas (23). These changes include up-regulation of the anti-apoptotic gene BIRC-3, overexpression of the BIRC-3 protein, and reduced expression of the pro-apoptotic gene, MTCH2 in thymic squamous cell carcinomas, and reduced expression of the pro-apoptotic gene, PMAIP1/NOXA in WHO subtype B3 thymomas. These discoveries have potential therapeutic implications since drugs targeting BIRC-3 and PMAIP-1 are in development (24).
Martinez and Browne review immunological deficiencies associated with thymoma and suggest a paradigm for comprehensive immunological evaluation in patients with thymoma, which should include an assessment of quantitative immunoglobulins, lymphocyte phenotyping, a vaccine challenge in patients suspected to have antibody deficiency and detection of anti-cytokine antibodies, whenever possible (25). Possible therapeutic interventions include immunoglobulin replacement in patients experiencing recurrent sinopulmonary infections due to immunoglobulin deficiency, and use of topical or systemic antifungal drugs in patients susceptible to chronic mucocutaneous candidiasis due to the presence of IL-17 or IL-22 antibodies (25).
Shapiro and Korst discuss the role of surgery for thymic tumors with pleural involvement (26). Surgical approaches that can be considered in this setting include metastasectomy for patients with a limited number of pleural lesions and extrapleural pneumonectomy for patients with more extensive pleural involvement. Data supporting the potential utility of intraoperative, hyperthermic, intrathoracic chemotherapy are also discussed and the need for prospective clinical trials to firmly establish the role of surgery for the management of stage IVA TETs is highlighted.
The role of radiation therapy in the management of thymic epithelial tumors is reviewed by Komaki and Gomez (27). The indications for adjuvant and definitive radiation therapy are discussed as well as techniques to deliver radiation and the long-term effects of mediastinal radiation therapy.
Finally, the review by Chen and colleagues focuses on the latest advances in systemic therapies for TETs (28). Results from clinical trials evaluating novel biological therapies including histone deacetylase inhibitors, insulin-like growth factor inhibitors, and multikinase inhibitors are discussed, and ongoing phase II trials for TETs are highlighted.
The aforementioned manuscripts provide a snapshot of important research efforts related to TETs. Continued advances in the field have resulted in an ever increasing stream of data that offer newer insights into the biology of these rare tumors and support the use of newer paradigms of management for patients with thymoma and thymic carcinoma.
This article is taken from the PMC US National Library of Medicine National Institutes of Health. To go to the article please click on Find out More below:
Information Provided by Dr. Giuseppe Giaccone M.D., Ph.D.
Internationally recognized expert in the field of lung cancer and developmental therapeutics.
Tumors of the thymus have been classified based on the morphology and percentage of lymphocyte cells versus tumor epithelial cells present in the mass. The most widely accepted classification is the 2004 WHO (World Health Organization) histological classification, which recognizes several types of thymomas (type A, AB, B1, B2 and B3) and thymic carcinomas .
There is a gradual increase in aggressiveness of these tumors, ranging from types A and AB with a 100% survival at 10 years (essentially cured by surgery only), to B types (intermediate prognosis) and thymic carcinoma (50% 5-year survival). Thymic carcinomas are somewhat more difficult to diagnose and differentiate from tumors that originate in other organs, because the morphology of the cells and the structure of the tumor does no longer resemble that of the thymus gland. Overall, thymic carcinomas can give raise to metastasis much more frequently than thymomas, and that is the reason for a shorter survival (less often operable, higher proliferation and metastases in important organs)1.
Thymic carcinomas are different from thymomas also in that they are very rarely if ever associated with autoimmune disorders, such as myasthenia gravis, pure red cell aplasia, and others, which in contrast are relatively common in thymomas.
Because these are rare tumors (about 600 new cases each year in the USA), there is not much known about the biology. However, recently research has shed some light to what are the more common genetic abnormalities in these tumors, and a unique mutation in a transcription factor (GTF2I) has been identified, which is not present in other tumor types and is observed almost exclusively in the less aggressive thymomas (type A and AB)2. The other genetic alterations are not typical of these tumors and are relatively infrequent, compared to many other cancer types (3). A large initiative sponsored by the National Cancer Institute (TCGA) is about to complete a study on over 100 tumors, where extensive data will become available in the near future.
Treatment of thymic tumors is surgical whenever possible. This is usually possible in the less aggressive tumors, despite the dimension of the tumor. Much less commonly is surgery complete in thymic carcinomas, where additional treatment may be indicated (radiation). If tumors are too large to be resected or are spread to other areas, then chemotherapy is the standard treatment for these situations1. Chemotherapy is relatively active in thymomas with significant tumor shrinkage seen in more than 50% of patients, but less active in thymic carcinoma. Chemotherapy can induce long lasting responses, especially in thymomas and remains the first form of treatment for tumors that are inoperable. Chemotherapy can shrink tumors and make them resectable. Operations are not excluded even when the tumors are not completely resectable, because a reduction of tumor volume may be beneficial in reducing the symptoms and potentially increasing survival.
The standard treatment of advanced thymic tumors is the PAC regimen (cisplatin, doxorubicin and cyclophosphamide) and very old regimen, with substantial side effects4. An alternative, especially for thymic carcinomas is the carboplatin-taxol regimen, which is less toxic and has a response rate in thymic carcinomas of about 30% (similar to the PAC regimen)5.
Unfortunately, chemotherapy alone is not curative in this disease, although it may take sometimes years for the tumor to come back. At that point, other forms of treatment will be available, which range from more chemotherapy (sometimes the same chemotherapy, if it was successful the first time and there was a long interval), to experimental treatments. In thymic carcinomas, sunitinib, an oral targeted drug, has been shown to reduce significantly the tumor size in about 25% of patients (6).
Because of the rarity of the disease, there are only a very limited number of studies that are performed in this disease. One study is currently looking at pembrolizumab (a PD-1 antibody that has been approved for the treatment of melanoma and lung cancer) in patients with advanced thymic carcinoma. Because this treatment stimulates the immune system, there is the potential risk of developing autoimmune disorders as a side effect of the treatment and this was the reason to select only patients with thymic carcinomas, who usually do not have an associate autoimmune disorder.
 Kelly RJ, Petrini I, Rajan A, Wang Y, Giaccone G: Thymic malignancies: from clinical management to targeted therapies. J Clin Oncol 2011, 29:4820-7.
 Petrini I, Meltzer PS, Kim IK, Lucchi M, Park KS, Fontanini G, Gao J, Zucali PA, Calabrese F, Favaretto A, Rea F, Rodriguez-Canales J, Walker RL, Pineda M, Zhu YJ, Lau C, Killian KJ, Bilke S, Voeller D, Dakshanamurthy S, Wang Y, Giaccone G: A specific missense mutation in GTF2I occurs at high frequency in thymic epithelial tumors. Nat Genet 2014, 46:844-9.
 Wang Y, Thomas A, Lau C, Rajan A, Zhu Y, Killian JK, Petrini I, Pham T, Morrow B, Zhong X, Meltzer PS, Giaccone G: Mutations of epigenetic regulatory genes are common in thymic carcinomas. Sci Rep 2014, 4:7336.
 Loehrer PJ, Sr., Chen M, Kim K, Aisner SC, Einhorn LH, Livingston R, Johnson D: Cisplatin, doxorubicin, and cyclophosphamide plus thoracic radiation therapy for limited-stage unresectable thymoma: an intergroup trial. J Clin Oncol 1997, 15:3093-9.
 Lemma GL, Lee JW, Aisner SC, Langer CJ, Tester WJ, Johnson DH, Loehrer PJ, Sr.: Phase II study of carboplatin and paclitaxel in advanced thymoma and thymic carcinoma. J Clin Oncol 2011, 29:2060-5.
 Thomas A, Rajan A, Berman A, Tomita Y, Brzezniak C, Lee MJ, Lee S, Ling A, Spittler AJ, Carter CA, Guha U, Wang Y, Szabo E, Meltzer P, Steinberg SM, Trepel JB, Loehrer PJ, Giaccone G: Sunitinib in patients with chemotherapy-refractory thymoma and thymic carcinoma: an open-label phase 2 trial. Lancet Oncol 2015, 16:177-86.