Nephrotic syndrome after extended thymectomy for thymoma with myasthenia gravis; report of a case

A 35-year-old man visited our hospital complaining of blepharoptosis and fatigability of the left arm. Under a diagnosis of myasthenia gravis (Osserman Ila), ambenonium was administered and improvements were noted. At 40-year-old, the patient underwent extended thymectomy due to the development of thymoma, which invaded the lung and pericardium (Masaoka stage Ill). Then ADOC therapy (doxorubicin hydrochloride+cisplatin+vincristine sulfate+cyclophosphamide) was initiated. At 47-year-old, we found recurrence of disseminated thymoma in thoracic cavity. Because of multiple metastatic lesions, radiotherapy in combination with chemotherapy was chosen and these lesions were reduced in size. Facial and foot edema developed at 50-year-old. Chest X-ray revealed bilateral pleural effusion. He was diagnosed as minimal change nephrotic syndrome and steroid therapy was started. The changes in cellular immunity due to thymoma is considered to be causative in the development of nephrotic syndrome. We report the clinical course of our case and discuss with reference to the literature.     

Recurrent thymoma in patients with myasthenia gravis

One hundred sixty-six patients underwent operation for myasthenia gravis between 1977 and 1989. Thirty-eight patients had associated thymoma, registering stages I (n = 17), II (n = 9), III (n = 11), and IVa (n = 1) according to the classification of Masaoka and colleagues. Extended thymectomy was performed on 128 patients without thymoma; thymothymectomy, with resection of the anterior mediastinal fat and tissues adherent to the tumor, was performed in all patients with thymoma. There were no instances of early or late death. Neuromuscular function improved, and clinical myasthenic symptoms stabilized in almost all patients except 2 patients in stage III and 1 patient in stage IVa, who had an exacerbation of the myasthenic symptoms associated with recurrence of thymoma. All the recurrent tumors were on the pleura and could be resected. The suspected cause of recurrence is either dissemination of tumor cells as a result of operative manipulation or undetected disseminated foci that existed at the time of the first operation. The resections of the recurrent invasive thymomas localized on the pleura were easily performed and improved the myasthenic symptoms.     

Autoimmune-like pancreatitis in thymoma with myasthenia gravis

A patient with thymoma and myasthenia gravis admitted for surgery presented increased serum levels of pancreatic amylase and lipase. Suspecting a thymoma-related autoimmune disorder, autoantibody serum titers were determined: increased autoantibody titers to acetylcholine receptors, thyroglobulin, thyroperoxidase and pancreatic insulin were detected. After thymectomy the serum levels of pancreatic enzymes decreased rapidly. Myasthenia gravis symptoms also improved. To the best of our knowledge no similar cases have been reported in the literature.     

Primary intrapulmonary thymoma associated with myasthenia gravis

Primary intrapulmonary thymomas are very rare. So far, research in the field has identified only 31 cases. In all databases, a total of two published articles describing primary intrapulmonary thymoma with myasthenia gravis were encountered between 1950 and 2010. We admitted a 58-year-old male patient with a mass in the right lower lobe of his lung. The tumor was excised, and histological findings were found to be consistent with Type AB thymoma. The patient was intubated due to respiratory distress during the postoperative period, and his acetylcholine receptor antibody was determined positive. He was diagnosed with myasthenia gravis. Pyridostigmine therapy and plasmapheresis were scheduled; yet, we could not begin therapy because of rapid deterioration of the patient's respiratory status due to myasthenia gravis and subsequently resulting in intubation-associated pneumonia. The patient's health rapidly worsened, and he died.     

Extended thymectomy and intraoperative-intrapleural perfusion hyperthermo-chemotherapy for stage IVa invasive thymoma with myasthenia gravis

A 37-year-old woman diagnosed with ocular myasthenia gravis was referred to our department. Chest computed tomography (CT) showed anterior mediastinal tumor and right pleural dissemination. Extended thymectomy and right intraoperative-intrapleural perfusion hyperthermo-chemothrapy (IPHC) were performed. Pathological diagnosis was invasive thymoma type B2 and stage IVa based on Masaoka's classification. The post operative course was uneventful. The patient underwent 4 cycles of adjuvant chemotherapy with doxorubicin, cisplatin, vincristine, and cyclophosphamide (ADOC), and is free from recurrence at 12 months postoperatively.     

A case of nephrotic syndrome associated with myasthenia gravis and malignant thymoma]

A 26-year-old woman who presented facial and lower leg edema associated with massive proteinuria was admitted to our hospital in February 1992. Nine months before this admission, she exhibited myasthenia gravis and malignant thymoma, and underwent total thymectomy. On admission, there was no symptom of myasthenia gravis. She was diagnosed as having nephrotic syndrome and the first renal biopsy was performed. The histological findings showed membranous nephropathy. Immunofluorescent microscopy revealed that IgG and C3 were stained in a granular pattern in the periphery, and subepithelial deposits were observed in the basement membrane of the glomerulus by electron microscopy. With the administration of prednisolone, proteinuria disappeared and the nephrotic syndrome remitted. She was admitted again in January 1993 due to proteinuria and lower leg edema following cystitis. The findings of the second renal biopsy were unremarkable. She was administered cyclosporin A to improve the nephrotic syndrome and to reduce the side effects of prednisolone. The proteinuria disappeared again and this effect was dependent on the dose of cyclosporin A. Since the first administration, no symptoms of myasthenia gravis or malignant thymoma have been observed. The relationships among myasthenia gravis, malignant thymoma and nephrotic syndrome were examined. Although the first renal biopsy findings showed membranous nephropathy, from the therapeutic responses of both prednisolone and cyclosporin A, the main course of proteinuria in this case may have been due to minimal change nephrotic syndrome. We consider this case of nephrotic syndrome to be important considering its etiology and the relationship between the histological findings and its clinical course.     

Management of myasthenia gravis in association with thymoma

From June 1975 to March 2002, we experienced 339 patients with myasthenia gravis (MG). Ninety-four patients (81 generalized MG and 13 ocular type) had associated with thymoma. Extended thymectomy including thymoma was performed in all patients. The thymomas were classified as stage I (n = 46), II (n = 31), III (n = 14), and IV a (n = 3). Histopathological findings of the thymoma indicated polygonal cell type in 75 cases, mixture of polygonal and spindle cell type in 14, and spindle cell type in 3, respectively. Three cases in stage II, 12 in III, and 3 in IV a received postoperative radiation therapy. Twenty-two patients required prolonged respirator management for respiratory crisis. Complete remission of MG was seen in 15 cases (17%), and good therapeutic results were obtained in 55 cases (58%) with combined corticosteroid therapy. On the other hand, recurrences of the invasive thymoma were seen in 12 cases (13%), and six of them (6%) died of the tumor. In conclusion, early extended thymectomy including thymoma is markedly effective therapy for MG associated with thymoma, although careful attention should be paid for recurrence of the invasive thymoma.     

Invasive thymoma with osseous metaplasia and cystic change in a case of myasthenia gravis: a rare presentation

A 35-year-old woman, a known case of myasthenia gravis, was found to have an anterior mediastinal mass, which was surgically removed. The preoperative clinical and radiologic diagnosis was that of a thymoma, but foci of calcification and prominent cystic change suggested the remote possibility of a teratoma. Histopathologically, it was confirmed to be a type B3 invasive thymoma with intratumoral ossification. Up to the present, three cases of thymoma with osseous metaplasia, including only one with myasthenia gravis, have been reported in the English literature. The present case report highlights the rare occurrence of osseous metaplasia in thymomas and the diagnostic challenge that it can pose, especially if it is associated with cystic degeneration.     

The thymus, thymoma and myasthenia gravis

Myasthenia gravis is an autoimmune disease. An autoantibody directed toward acetylcholine receptor (AChR) causes the destruction of the postsynaptic membrane and a reduction of the number of AChRs at neuromuscular junctions. A very puzzling, but interesting characteristic of myasthenia gravis is that many of the patients have an abnormality in their thymus. Many have a hyperplastic thymus with germinal centers, while others have a thymic tumor. How is the abnormality of the thymus related to myasthenia gravis? This review will summarize the existing evidence and try to find the missing link between the thymus and myasthenia gravis. The review will also comment on two distinct populations of myasthenia gravis patients without thymoma. The autoimmunity found in elderly patients is nonspecific and initiated via a different mechanism from the initiation of myasthenia gravis in younger patients.     

Thymolipoma in association with myasthenia gravis; report of a case

An 18-year-old man was admitted with easy fatigue and muscle weakness. A diagnosis of myasthenia gravis was made. Chest X-ray showed no mediastinal abnormality, however, chest computed tomography (CT) showed a soft tissue mass in the thymus. The patient underwent extended thymectomy and small tumor (2 x 2 x 2 cm) was resected. On histological examination the tumor proved to be a thymolipoma composed of mature adipose elements containing cords and nests of thymic tissue. Symptoms of myasthenia gravis were dramatically improved after the operation. This case is the 12th in the world literature in which a thymolipoma is associated with symptoms of myasthenia gravis.     

Clinical use of atracurium in myasthenia gravis: a case report

A patient with myasthenia gravis was anaesthetized for thymectomy utilizing atracurium for maintenance of muscle relaxation. The patient was not taking anticholinesterase medications before surgery. Neuromuscular function was monitored by recording the train-of-four response to supramaximal stimulation of the ulnar nerve. Anaesthesia was induced with thiopentone and maintained with isoflurane and N2O in oxygen. Following complete recovery from a succinylcholine block for intubation, the patient developed complete neuromuscular block within two minutes after atracurium (5 mg) was administered. Evidence of recovery commenced 12 minutes later and was complete at 72 minutes. This rate of recovery is consistent with the recovery rate from atracurium during isoflurane anaesthesia in normal patients. Atracurium appears to offer an advantage over other nondepolarizing muscle relaxants in patients with myasthenia gravis due to its shorter duration and less cumulative effect at the neuromuscular junction.     

Colon carcinoma after thymectomy for myasthenia gravis: report of a case

A 74-year-old Japanese man was admitted to our hospital with anemia, 4 years after a thymectomy for thymoma associated with myasthenia gravis. A diagnosis of sigmoid colon carcinoma was confirmed, followed by surgical resection. This case is presented to reinforce that physicians should bear in mind the possibility of extrathymic malignancies in patients with thymoma. Received: June 28, 2001 / Accepted: January 8, 2002     

The effect of thymectomy on myasthenia gravis,thrombocytopenia, and granulocytopenia associated with thymoma: Report of a case

We report the case of a 47-year-old woman with thymoma who developed myasthenia gravis, thrombocytopenia, and granulocytopenia, simultaneously, the concurrent association of these four disorders being extremely rare. Thymectomy was performed, and, during the post-thymectomy course, there were surprising findings concerning the recovery of not only the myasthenia gravis but also of the hematologic disorders. Immediately after thymectomy, the myasthenic symptoms completely disappeared, and the granulocyte and platelet counts recovered to within the normal range within a few days. The laboratory data revealed no difference between pre- and post-thymectomy in the release of cytokines (tumor necrosis factor; TNF, interleukin; IL-2, and IL-6), anti-acetylcholine receptor antibody, or platelet-associated IgG. On the other hand, the serum level of anti-neutrophil cytoplasmic antibody (p-ANCA), against the myeloperoxidase of the granulocytes was dramatically decreased, after thymectomy, showing a significant correlation with the granulocyte count. According to our survey of the literature, this is the first report to show that the removal of a thymoma led to the dramatic resolution not only of myasthenia gravis but also of other associated diseases. It is possible that p-ANCA may be regulated by thymoma, thus causing severe granulocytopenia.     

胸腔镜胸腺切除术治疗重症肌无力(附2例报告)

目的:探讨胸腔镜胸腺切除术治疗重症肌无力的可行性。方法:2例重症肌无力合并胸腺增生(胸腺瘤)的患者行胸腔镜胸腺切除术。结果:2例患者胸腺均在胸腔镜下完整切除,无中转开胸及并发症。术后通过4个月电话随访,症状都有明显改善。结论:胸腔镜胸腺切除术具有创伤小,术后疼痛轻,并发症少,住院时间短,恢复快等优点,是一种非常有前途的技术。