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Post Thymectomy Myasthenia Gravis Osserman III In Patient Diagnosed With Thymic Carcinoma FREE TO VIEW

Odeson Estabillo, MD; Gene Philip Louie Ambrocio, MD; Joel Santiaguel, MD
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Philippine General Hospital, Manila, Philippines

Chest. 2014;146(4_MeetingAbstracts):279A. doi:10.1378/chest.1994121
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SESSION TITLE: Critical Care Global Case Reports

SESSION TYPE: Global Case Report

PRESENTED ON: Tuesday, October 28, 2014 at 01:30 PM - 02:30 PM

INTRODUCTION: Thymic carcinoma is a rare type of thymus gland cancer, thymic epithelial tumor that exhibits clear-cut cytologic atypia and histologic features no longer specific to the thymus. Thymic carcinomas are rare and have been reported to account for only 0.06% of all thymic neoplasms. Post operative myasthenia gravis or post thymectomy MG (PTMG) is defined as occurrence of MG after total removal of the thymus tumor, more commonly thymoma. Commonly myasthenia gravis is associated with thymoma and a few thymoma patients without myasthenia gravis have been observed to develop MG after total removal of the thymoma with and incidence of 1.5% but association of thymic carcinoma with autoimmune disorders are very rare like MG, only 3 cases have been documented. No local and international report encountered on Post thymectomy MG Osserman III in patient with thymic carcinoma.

CASE PRESENTATION: A case of 54 year old female who complained of shortness of breath. The present condition started 2 months prior to admission she complained of persistent fever and shortness of breath, workups revealed a pulmonary mass on the left apical region. FNAB, Thoracoscopy and excision of mediastinal mass thymectomy were done which on histopath revealed high grade malignant potential favoring thymic carcinoma. She was advised to undergo radiotherapy but due to persistent shortness of breath, she was admitted after 52 days post operatively. On the second hospital day patient had severe dyspnea and was intubated, no associated fever nor cough, with initial consideration of pneumonia. On the 5th hospital day patient noted to have bilateral ptosis. Repetitive nerve stimulation test was done and findings were compatible with Myasthenia gravis. Pyridostigmine and Prednisone started. Plasmapheresis was done for a total of 5 sessions, and patient was extubated after the 4th session of plasmapheresis, patient was discharged improved

DISCUSSION: The thymus plays a key role in the immunologic status of an individual, and disease of the thymus can be associated with autoimmune disorders. MG first appearing many years following the removal of a thymoma reportedly have an incidence of 0.9-20%. Thymic carcinomas have a greater propensity to capsular invasion and metastases than thymomas. Patients with thymic carinoma were treated with radiation therapy or chemotherapy. The current hypothesis of the pathogenic mechanism of PTMG includes: thymoma recurrence; surgical exposure to larval MG; and activation of peripheral lymphocytes from thymoma after surgery. To differentiate PTMG with myasthenic crisis, Myasthenic crisis is a life-threatening condition, which is defined as weakness from acquired myasthenia gravis (MG) that is severe enough to necessitate intubation or to delay extubation. The respiratory failure is due to weakness of respiratory muscles may be precipitated by a variety of factors, most often a concurrent infection. It can also follow a surgical intervention, pregnancy, childbirth, or tapering of immunosuppressive medications.

CONCLUSIONS: Thymic carcinoma is a rare, fatal disease that can occur in adult individual, thymectomy is the most common recommendation for patients with thymic tumors namely thymoma and thymic carcinoma. Post thymectomy myasthenia gravis although also rare has been reported, 1 case report has been documented for post thymectomy myasthenia gravis presenting with Osserman III. In this report we showed two rare separate cases that are associated with each other. Definitive treatment and pathophysiology of such case is still unclear hence, awareness of this rare condition can help us in early diagnosis and prompt treatment in order to prevent long term complications and possible morbidity

Reference #1: Kuwata, et. al, Post-thymectomy myasthenia gravis with an episode of Osserman stage III

Reference #2: Veitez et. Al, Myasthenia gravis associated with carcinoma of the thymus.

Reference #3: Kondo, et. al, Myasthenia gravis appearing after thymectomy

DISCLOSURE: The following authors have nothing to disclose: Odeson Estabillo, Gene Philip Louie Ambrocio, Joel Santiaguel

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