Thymoma in Myasthenia Gravis: From Diagnosis to Treatment

Romi, Fredrik

doi:10.4061/2011/474512

Cited by 104 publications

(110 citation statements)

References 43 publications

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“…Moran and Suster (1) reported 25 cases of thymoma with prominent hemorrhagic and necrotic changes, without a detailed description of tumor regression. Thymoma is generally asymptomatic (1) and the majority of the patients with thymoma commonly present with an abnormal chest shadow, while 15% of the cases with thymoma are associated with myasthenia gravis (11). By contrast, all the reported patients with SR of thymoma have presented with symptoms, such as fever or chest pain (Table II).…”

Section: Discussionmentioning

confidence: 99%

Thymoma exhibiting spontaneous regression in size, pleural effusion and serum cytokeratin fragment level: A case report

Furuya

Isobe

Sano

et al. 2015

Molecular and Clinical Oncology

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Abstract.A 30-year-old man was admitted to Toho University Omori Medical Center for assessment of right chest pain and fever. Chest computed tomography (CT) revealed an anterior mediastinal tumor sized 11.0x6.0x5.0 cm, with right pleural effusion. The laboratory analysis revealed elevated white blood cell count (11,000/µl), C-reactive protein (4.1 mg/dl) and cytokeratin fragment (CYFRA; 12.7 ng/ml; normal, <2 ng/ml). The level of CYFRA in the pleural effusion was also markedly elevated (143 ng/ml). On the first day after admission (6 days after the initial CT), there was a mild regression on CT (10.0x5.5x4.4 cm; reduction rate, 26.7%), with decrease of the pleural effusion volume. A CT-guided needle biopsy was performed, but the findings were not conclusive, as most of the tissue was necrotic. Seven days later (13 days after the initial CT), a CT revealed further regression (9.5x5.4x4.2 cm; reduction rate, 34.7%) with disappearance of the pleural effusion. The patient was followed up on an outpatient basis. At 35 days after the initial CT, the tumor continued to shrink without treatment (8.0x3.6x3.0 cm; reduction rate, 73.8%) and the serum CYFRA level had decreased to 0.8 ng/ml, although it had not returned to normal levels. At 62 days after the initial CT, the patient underwent surgical resection. The resected specimen was diagnosed as thymoma (World Health Organization type B2; Masaoka classification, stage II), with prominent degeneration and necrosis. One possible cause of the spontaneous regression may be increased internal pressure, probably associated with rapid tumor growth, leading to massive necrosis with resulting chest pain, inflammatory reaction with pleural effusion and subsequent tumor regression. The serum CYFRA level may be a useful marker for the evaluation of the clinical course of thymoma with extensive necrosis. IntroductionThymoma is derived from thymic epithelial cells and is one of the most common neoplasms in the anterior mediastinum (1). The overall incidence of thymoma is 0.15 cases per 100,000 individuals and the majority of the patients with thymoma or thymic carcinoma are aged 40-60 years (2,3). The World Health Organisation (WHO) classification is based on histological analysis of epithelial cell atypia and the degree of infiltration of non-neoplastic lymphocytes (4). Although the great majority of these tumors exhibit more conventional histological characteristics, unusual types have also been described, such as sclerosing thymoma (5). Spontaneous regression (SR) of thymoma without therapy has been reported, although its incidence is rare (6)(7)(8). This is the case report of a patient with thymoma exhibiting SR and disappearance of the pleural effusion. Case reportA 30-year-old man was admitted to Toho University Omori Medical Center with right chest pain and low-grade fever. The patient was a never-smoker and had no medical history. On physical examination, the temperature was 37.8˚C and the respiratory sounds were clear. A chest X-ray revealed a mass shadow in the right hilum a...

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Section: Discussionmentioning

confidence: 99%

Thymoma exhibiting spontaneous regression in size, pleural effusion and serum cytokeratin fragment level: A case report

Furuya

Isobe

Sano

et al. 2015

Molecular and Clinical Oncology

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“…Antibodies against these intracellular autoantigens occur in 90% of TAMG and 50% of LOMG patients, have prognostic value, but are of uncertain pathogenicity. However, occurrence of anti-RyR and anti Kv1.4 potassium channel autoantibodies have been associated with more severe myasthenic symptoms and cardiac dysfunction (reviewed in [77,78]). Striational antibodies are absent in patients with congenital AIRE deficiency [39,40], and rare in EOMG patients [79,80], although thymic myoid cells express AChR and titin and are involved in the intrathymic inflammation that is typical of EOMG [17].…”

Section: Autoantigens and Autoantibodies In Tamgmentioning

confidence: 99%

Thymoma related myasthenia gravis in humans and potential animal models

Marx

Porubský

Belharazem

et al. 2015

Experimental Neurology

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“…Although the aetiology of thymoma is still unknown, it has been suggested to be associated with autoimmune diseases, such as myasthenia gravis (MG) (Fujii 2013). About 30-50% of thymoma patients have been reported to develop MG, while the frequency of thymoma was 10-20% among the patients with MG (Romi 2011;Marx et al 2015). Surgery has been well-established as the standard treatment for thymoma.…”

Section: Introductionmentioning

confidence: 99%

Therapeutic Potential of Docetaxel plus Cisplatin Chemotherapy for Myasthenia Gravis Patients with Metastatic Thymoma

Liu

Hua

et al. 2017

Tohoku J. Exp. Med.

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The prognosis of myasthenia gravis (MG) in association with invasive or metastatic thymoma is usually worse, and therapeutic options are quite limited. Here, we retrospectively reported the therapeutic effect of docetaxel plus cisplatin (docetaxel/cisplatin) chemotherapy in 7 MG patients with metastatic thymoma. Previously, all patients underwent thymectomy at the first onset of thymoma. After the metastasis of thymoma, none of the patients received thymectomy due to unresectable conditions after surgeon's evaluation for great risk of myasthenic crisis (n = 5) or patients' refusal (n = 2). All patients received docetaxel (75 mg/m 2 ) and cisplatin (70 mg/m 2 ) on day 1 (d1) every 21 days, with the cycle ranging from 1 to 4. After docetaxel/cisplatin chemotherapy, one patient achieved partial response, and 6 with stable disease of the tumors. The clinical symptoms of MG were alleviated in all patients, 2 with complete remission and the other 5 with marked improvement. Myelosuppression was the major adverse event, occurring in 2 patients (grade II and IV). MG relapse occurred in one patient during the follow-up. Our study presented a series of MG patients with metastatic thymoma who underwent docetaxel/cisplatin chemotherapy. Besides the improved/stabilized thymoma, markedly improvement of MG with the tolerable adverse events was achieved. Docetaxel/cisplatin chemotherapy appears to be an effective treatment for selected patients with MG in association with unresectable metastatic thymoma. Further follow-up of these patients and additional subjects will be needed to determine whether the therapeutic benefits are durable.

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Thymoma in Myasthenia Gravis: From Diagnosis to Treatment

Cited by 104 publications

References 43 publications

Thymoma exhibiting spontaneous regression in size, pleural effusion and serum cytokeratin fragment level: A case report

Thymoma exhibiting spontaneous regression in size, pleural effusion and serum cytokeratin fragment level: A case report

Thymoma related myasthenia gravis in humans and potential animal models

Therapeutic Potential of Docetaxel plus Cisplatin Chemotherapy for Myasthenia Gravis Patients with Metastatic Thymoma

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