The sarcomatous guise of cervical ectopic hamartomatous thymoma

BACKGROUND: Ectopic hamartomatous thymoma is a rare benign neoplasm occurring in the deep soft tissues adjacent to the sternoclavicular joint. Although clinical presentation and diagnostic imaging can be consistent with a malignant lesion such as a sarcoma, recognition of pathologic features can readily exclude such a diagnosis. However, this remains a challenge caused by their unusual histologic features and diverse composition. Recognition of this tumor is, however, important, because it follows a benign clinical course, and conservative surgical excision is the therapy of choice. METHODS: A literature review of all reports of ectopic hamartomatous thymoma was undertaken and compared with our indexed case. RESULTS: From this review, we identified 26 previous reports. Most patients were men, with a 4.5:1 predominance. There was a wide range of ages at presentation, from 26 to 79 years. All tumors were located in the lower neck, with the exception of one arising presternally. Treatment predominantly consisted of tumor resection, with no reported recurrences on follow-up. CONCLUSIONS: We conclude that ectopic hamartomatous thymoma is an extremely rare neoplasm usually presenting in the lower neck. Correct identification of this tumor is important, because it follows a benign course, and surgical excision is adequate therapy.     

Paraneoplastic Extralimbic Encephalitis Associated with Thymoma: A Case Report

Here we a report a rare case of extralimbic encephalitis associated with thymoma. A 66-year-old woman was admitted to our hospital with cramping in her right leg and inability to walk. Magnetic resonance imaging of the brain showed multifocal high intensity signals on T2 flare images in the cerebral cortex, and chest computed tomography showed a 5-cm anterior mediastinal mass, which was considered to be a thymoma. We speculated that she had paraneoplastic encephalitis associated with thymoma. She underwent a thymectomy and was diagnosed with type B1 thymoma. On postoperative day 6, her neurological symptoms began to improve. On postoperative day 31, she was discharged without complications. Limbic encephalitis is a paraneoplastic neurological syndromeassociated with thymoma, but extralimbic encephalitis has been described in the literature very rarely. We report the case of extralimbic encephalitis associated with thymoma along with a literature review.     

滤泡树突状细胞肉瘤并发副肿瘤性天疱疮1例并文献复习

目的报告1例滤泡树突状细胞肉瘤并发副肿瘤性天疱疮的临床表现及临床治疗,从而提高对该疾病多样临床表现的认识,避免误诊、漏诊的发生并完善相关诊治。方法分析1例滤泡树突状细胞肉瘤并发副肿瘤性天疱疮的临床表现及临床治疗并查阅国内外文献资料。结果此例是来自淋巴结外部的滤泡性树突状细胞肉瘤,因其细胞边界不清,明显的异型和有丝分裂而具有较高的肿瘤恶性程度,手术后患者的状态逐渐恶化。结论对于局限性病变,手术切除是首选治疗方式。但单纯手术具有较高的局部复发率,巩固性放疗可降低手术患者的局部复发率,并延长其无病生存期。对全身播散性FDCS(多处淋巴结肿大)、有巨大肿块或手术未能根治的患者,需要进行有效的联合化疗。     

Paraneoplastic neurological and hematological syndromes associated with prostate cancer

Paraneoplastic neurological syndromes are defined as the remote effects of cancer on the nervous system. Here we report a 68-year-old man who initially presented with worsening paresthesia in the lower extremities. Although the culprit lesion remained to be identified, he coincidentally had diagnosis of prostate cancer by an annual prostate-specific antigen examination. Leukocytosis and elevated granulocyte colony-stimulating factor in serum were also detected. Neurological symptoms and leukocytosis improved after initiation of androgen-deprivation therapy followed by external beam radiotherapy. A total of 9 months after treatment, the patient showed no evidence of cancer recurrence or neurological signs. Paraneoplastic neurological syndromes are rare in prostate cancer and therefore have received little attention. We should be aware that when paraneoplastic neurological syndromes occur, they usually occur as the first sign of or during progression of prostate cancer. Furthermore, we should take into account the existence of malignancy when the cause of neurological symptoms cannot be specified.     

Myasthenia gravis with thymoma associated with occult thyroid carcinoma

We report herein a case of myasthenia gravis associated with thymoma and occult thyroid carcinoma with metastasis to a cervical lymph node, which was treated in two stages by a total thymectomy and radical dissection of the bilateral neck after a total thyroidectomy. A 48 year old woman was admitted with right blepharoptosis and hypodynamia of the proximal muscles. After various examinations, a diagnosis of myasthenia gravis was made. The association of thymoma was clarified upon CT scanning and a total thymectomy performed. However, after surgery, swelling of the cervical lymph nodes became apparent and a biopsy revealed metastasis of thyroid carcinoma in the lymph nodes. A diagnosis of occult thyroid carcinoma with metastasis to a cervical lymph node was subsequently made and a total thyroidectomy and radical dissection of the bilateral neck performed 37 days after the total thymectomy. The patient's postoperative course was uneventful and for the time being, no recurrence is expected. It is considered better to perform two-stage operations for easier and safer postoperative management when myasthenia gravis associated with occult thyroid carcinoma is treated, although it depends on the development of thyroid carcinoma being occult.     

Ectopic primary pleural thymoma: Report of a case

We herein report an 83-year-old man who presented with a pleural tumor in the right thorax. The tumor was surgically resected and histopathologically diagnosed as ectopic primary pleural thymoma lacking any evidence of a mediastinal tumor. The clinicopathological aspects of this unusual thymoma are also discussed.     

Malignant thymoma associated with liposarcoma of the mediastinum —A case report—

Malignant thymoma occurring concurrently with mediastinal liposarcoma in a 49-year-old man is described. The patient underwent an incomplete resection of the mediastinal mass followed by irradiation therapy and additional chemotherapy. He died about 9 months after the detection of a mediastinal mass on chest X-ray films and the immediate cause of death was superior vena cava syndrome. The incidence of primary liposarcoma of the mediastinum alone is extremely rare. Only a few more than 20 with such a lesion have been docummented in Japan. A review of the literature of patients with mediastinal liposacoma and thymoma associated with malignancies revealed no case of an association of thymoma and liposacoma.     

Paraneoplastic neurologic syndrome associated with occult breast cancer: a case report and review of literature

We report a case of occult breast cancer presenting as paraneoplastic sensory and motor neuropathy. The paraneoplastic antibody panel was negative and no other unknown identifiable antibody could be detected in the serum. Systemic treatment with four cycles of Adriamycin and cyclophosphamide was given with no significant improvement in the neurologic condition. We reviewed the literature of the last 10 years and identified 31 reported cases of paraneoplastic neurologic syndrome due to breast cancer. Paraneoplastic antibodies could be found in only 36% of patients, no identifiable antibodies in 32% of patients, and unknown antibodies in 16% of patients. The status of the paraneoplastic antibodies was not reported in 16% of patients. Only 29% of patients responded to chemotherapy with improvement in neurologic deficits. The role of systemic treatment in the progression of neurologic impairment is unclear.     

Right-side congenital pericardial defect associated with ischemic heart disease

We report an unusual right-side congenital pericardial defect with herniation of the right atrium to the right thoracic cavity found intraoperatively in a 73- year-old man undergoing coronary artery bypass grafting for triple-vessel coronary artery disease. The right atrial wall showed fibrous changes due to contact with the defect edge. We suspected that the right coronary artery was obstructed by chronic strangulation of the right atrium. We repaired the defect with a polytetrafluoroethylene patch to prevent it from compressing the bypass graft and coronary arteries.     

Multiple thymoma with myotonic dystrophy

A case of multiple thymoma associated with myotonic dystrophy is reported. The patient was a 42-year-old man who had two separate encapsulated thymoma in the anterior mediastinum, at 3 cm in diameter on the right side, and at 4 cm in diameter on the left. Extended thymo-thymomectomy was performed. Microscopically, the tumor in the right thymic lobe was predominantly mixed type, and that in the left predominantly epithelial type. Neuromuscular disease appeared to develop, with severe sputum retention in the larynx and he was referred to neurology at 6 months after surgery. On examination, he presented a characteristic hatchet face, muscle atrophy, muscle weakness, percussion myotonia and grip myotonia, and diagnosis was revised to be multiple thymoma associated with myotonic dystrophy. This association is extremely rare. It is difficult to clarify whether this association was a syndrome or coincidence.     

Ectopic cervico-mediastinal thymoma confirmed by flow cytometric analysis of tumor-derived lymphocytes

Ectopic cervical or cervico-mediastinal thymomas are very rare and most of them are asymptomatic, except for the presence of a cervical mass. We present the case of a 71-year-old man with an ectopic cervico-mediastinal thymoma threatening superior vena cava syndrome. He had a slight headache and presented with venous dilatation on the chest wall. A computed tomographic scan and magnetic resonance, imaging of the chest demonstrated a mass extending from the right neck to the hilum, that indented the trachea and compressed and displaced the brachiocephalic veins anteriorly. Under a right hemicollar incision and median sternotomy, the mass was resected en bloc together with the thymus. The resected specimen was an encapsulated mass measuring 11×7×4 cm. The pathological diagnosis was type AB, non-invasive thymoma, confirmed by 3-color flow, cytometry of tumor-derived lymphocytes. Flow cytometry using biopsy material may contribute to the preoperative diagnosis of ectopic thymoma.     

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.     

Malignant thymoma with direct invasion into the peritoneal cavity: Report of a case

(Received for publication on Oct. 9, 1996; accepted on May 12, 1997)     

Invasive thymoma with endobronchial polypoid growth

A rare case of invasive thymoma with endobronchial polypoid growth is presented. A 68-year-old woman presented with coughing and sputum. The chest X-ray and computed tomography (CT) findings demonstrated a large mass with a calcified lesion in the left hilar region. A bronchoscopic examination showed a polypoid tumor in the left B³b bronchus. The tumor was surgically resected and revealed a unique polypoid endobronchial extension. This tumor was pathologically diagnosed to be invasive thymoma.     

Resected invasive thymoma with multiple endocrine neoplasia type 1

We report a rare case of multiple endocrine neoplasia (MEN) type 1 with thymoma. A 57-year-old woman with a chronic duodenal ulcer and hypoglycemia had been seen at a nearby clinic. Abdominal echogram revealed two nodules in the pancreas and she was referred to our hospital for evaluation. Her diagnosis was MEN type 1, gastrinoma and hyperparathyroidism with anterior mediastinal tumor. There were high calcium levels in the blood and urine. Gastrin was quite high. A chest X-ray revealed a retrosternal tumor. Computed tomography revealed an anterior mediastinal tumor without sign of invasion to the surrounding organs, and two small masses in the pancreas. Cervical echogram revealed a few masses in both sides behind the thyroid. From these findings, her preoperative diagnosis was MEN type 1 with thymic carcinoid or thymoma. We performed thymectomy and parathyroidectomy concomitantly. The mediastinal tumor was diagnosed as invasive thymoma. (Jpn J Thorac Cardiovasc Surg 2006; 54:171-173)     

Anaesthetic management of a patient with pemphigus vulgaris for emergency laparotomy

A 45-year-old man with a long-standing history of duodenal ulcer presented with symptoms and signs of perforation peritonitis. He also had lesions of pemphigus vulgaris throughout the body, involving both skin and mucous membranes. Care was taken to avoid pressure and friction during placement of monitoring devices, intravenous and arterial lines. Since the patient had to undergo exploratory laparotomy, intubation was performed in an atraumatic manner after rapid sequence induction. However, there was minor bleeding from the mucous lesions of the oral cavity, which was controlled by a saline adrenaline throat pack. The patient was extubated at the end of the surgery and steroids were continued in the peri-operative period.     

Encapsulated thymoma metastasizing to a pectoralis major muscle

An extensive review of the literature suggests that ours is the first case of encapsulated thymoma metastasizing to a skeletal muscle. A 43-year-old man underwent thymothymectomy for encapsulated Masaoka's stage I thymoma. Four years after complete resection, the tumor metastasized to the left pectoralis major muscle. Although a few reports exist on encapsulated thymoma metastasizing to a distant site, the literature does not describe encapsulated thymoma metastasizing to a skeletal muscle insofar as we could find.     

Cardiac displacement after lobectomy in a patient with a congenital complete left-sided pericardial defect

A 74-year-old man underwent left upper lobectomy for a suspected lung cancer. At thoracotomy, the pericardium was found to be completely absent. The heart was fixed in a central position with an artificial patch to prevent displacement. However, the heart became displaced just after closure of the thoracotomy. The thoracotomy was immediately reopened. The anchor points of the patch on the adventitia of the descending aorta had pulled through. The heart was fixed to the surrounding mediastinal tissues with a larger patch using pledget-reinforced mattress sutures. Because simple closure may result in cardiac herniation due to the cardiac pulsations, congenital pericardial defects should be securely repaired when encountered.