Thymic Carcinoma With Multiple Paraneoplastic Disorders
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Thymic carcinoma
Thymus Neoplasm
Thymic carcinoma
Thymus Neoplasm
Surgical oncology
Neuroendocrine carcinoma
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Thymomas and thymic carcinomas are rare neoplasms derived from the epithelial tissue of thymus, very infrequently developing in young adults and children. The estimation of thymomas' invasiveness has been the matter of discussion for many years reflected by numerous clinical and histological classifications. In 1999 the WHO classification was created, joining all the most important issues present in previously used systems. It is believed that histological structure is the most important prognostic factor in thymic carcinomas while in less aggressive types of thymomas the clinical stages influence the outcome. Staging of thymomas is most commonly based on the Masoka classification. Independent evaluation of the stage and histological aggressiveness are necessary to predict the clinical course and outcome in thymomas. Thus the term 'malignant thymoma' has been replaced by 'invasive thymoma' in clinical practice. The treatment strategy depends on the clinical stages of thymoma. Complete resection of the tumour is the treatment of choice with supplementing radiotherapy in more advanced clinical stages. Chemotherapy in invasive thymomas has been reported to play an increasingly important role as induction, supplementing and palliative therapy. It has been proved that combined treatment improves the outcome in invasive thymomas, especially in thymic carcinomas. This paper reviews the literature data concerning the histology, clinical issues and treatment of thymomas and thymic carcinomas. The clinical data on nine children with thymic carcinomas treated between 1992 and 2006 in the Polish oncological and surgical centres were also analysed and presented. Based on multicentre data we were able to conclude the following: 1. Thymic carcinomas in children are very rare and that is why early diagnosis is often difficult. 2. At diagnosis most cases are already inoperable, which results in poorer prognosis. 3. Complex adjuvant chemo- and radiotherapy in childhood thymic carcinomas seem to prolong overall survival. 4. Further detailed analysis in all the cases of thymic carcinomas in children is recommended in order to estimate the optimal strategy of treatment.
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We have compared clinical, immunological and radiological data in 20 patients with myasthenia gravis and thymoma and in 21 patients with myasthenia gravis and thymic atrophy. The median age at onset was 54 years in the thymoma group and 63 years in the thymic atrophy group (p = 0.04). The severity of the disease was similar in the two groups, and there was no significant difference in the concentration of acetylcholine receptor antibodies. CA antibodies were demonstrated in 17/20 thymoma patients and in 6/21 with thymic atrophy, while 19/20 thymoma patients had antibodies to titin, compared with 9/21 among those with thymic atrophy. The diagnosis and treatment of patients with myasthenia gravis is based upon an evaluation of clinical, immunological and radiological data.
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Thymus Neoplasm
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Myasthenia gravis and thymoma are often presented in association with ∼10% of myasthenic cases having concomitant thymoma. Thymic carcinoma is one of the rarest/aggressive human epithelial tumors and has no correlation with myasthenia gravis hitherto. Here is provided a clinical case and review of literature on a very rare association of thymic carcinoma (with no sign of thymoma) and myasthenia gravis (antiacetylcholine receptor antibody positive). Two years after thymectomy, clinical evolution was satisfactory. This clinical case elicits hypothesis that thymic carcinoma may be related with myasthenia gravis, what may have good prognostic from oncologic and neurologic perspectives.
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Thymectomy
Concomitant
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The five-stage classification system of thymoma (I, II, III, IVa, IVb) is now adopted widely. As the cases with lymphogenous or hematogenous metastasis are included in IVb, it is suggested that IV b includes various groups with different prognostic factors. This facilitates establishment of TNM classification of thymic epithelial tumours (thymoma, thymic cancer and thymic carcinoid). T factors correspond with the stages, I: T1N0M0, II: T2N0M0, III: T3N0M0, and IV a : T4 N0M0. N and M factors are as follows: N1 : restricted to the anterior mediastinal nodes; N2 : intrathoracic nodes; N3 : supraclavicular nodes; and M1 : hematogenous metastasis and/or extrathoracic nodes excluding supraclavicular nodes. Such criteria were used to classify IVb into any T N(1,2,3) M0, and any T any NM1. N criteria could divide the cases into the groups with proper populations in IV b thymoma, thymic carcinoma, and thymic carcinoid, respectively. The possible relationships between survival and TNM were investigated in thymic carcinoma cases. T, N, and M have a relationship with survival, respectively. This TNM classification is not yet authorized, but it is used in many clinics in Japan to determine the selection of therapy and estimate the therapeutic effect.
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Thymoma and thymic carcinoma are known as thymic epithelial tumors (TETs) given their shared origin as epithelial neoplasms arising from the thymus. The understanding of the relationship between thymoma and thymic carcinoma has evolved over time.
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Everolimus
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Thymic epithelial tumors (TETs) are generally categorized as those that originate from thymic epithelial cells (thymoma and thymic carcinoma) and those that originate from neuroendocrine cells (thymic carcinoid, germ-cell tumor, and thymic cyst).
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MUCH has been written concerning the relation of myasthenia gravis to the thymus. There are fewer articles, however, describing myasthenia gravis with thymoma, and fewer still on its relation to malignant thymoma. In 1942 only 5 such cases had been collected.1 Castleman and Norris2 stated that thymoma occurred in 10 to 15 per cent of cases of myasthenia gravis and that "if implantation connotes malignancy, then the thymoma may be carcinomatous in a very small percentage of cases (between 5–10%)." Most authors agree with this figure of the association of thymoma with myasthenia gravis3 4 5 6; in 1951 an estimated 130 . . .
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Thymomas and thymic carcinomas are rare epithelial tumors of thymus (TETs) associated with paraneoplastic autoimmune (PN/AI) disorders, including myasthenia gravis (MG), pure red cell aplasia, hypogammaglobulinemia, encephalitis, and acquired neuromyotonia (1,2). Patients affected by these disorders are usually screened for thymoma at diagnosis, and they frequently improve with thymoma treatment (2).
Hypogammaglobulinemia
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Autoimmune encephalitis
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