Cases reported "Thymus Neoplasms"

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1/355. thymoma associated with myasthenia gravis and minimal lesion nephrotic syndrome.

    A nephrotic syndrome has been observed rarely in association with thymoma. In most of the reported cases, it occurs when the thymoma is in remission; histological examination generally shows minimal change disease. We report a case of a 43-year-old man presenting with minimal lesion nephrotic syndrome at the time of the diagnosis of thymoma and myasthenia gravis, which persists despite remission of the thymoma. The role of a disorder of T-cell function and of circulating cytokines is discussed.
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2/355. Spindle epithelial tumor with thymus-like differentiation (SETTLE) of the thyroid with prominent mitotic activity and focal necrosis.

    Spindle epithelial tumor with thymus-like differentiation (SETTLE) is a rare, apparently low-grade spindle cell tumor of the thyroid gland occurring in young individuals and thought to be derived from thymic or branchial pouch remnants. Spindle epithelial tumor with thymus-like differentiation has little to no mitotic activity, and focal necrosis has been reported in one case. We present a case of SETTLE in a 29-year-old man that was initially evaluated by fine-needle aspirate biopsy and ultimately found to be consistent histologically with SETTLE. In this case, there were numerous mitotic figures among the spindle cells and focal necrosis. Spindle epithelial tumor with thymus-like differentiation has been considered to be a tumor of low malignant potential with metastases developing some years after diagnosis. This is the first case in which prominent mitotic activity and necrosis is reported perhaps representing an aggressive variant.
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3/355. Angiomatoid neuroendocrine carcinoma of the thymus: report of a distinctive morphological variant of neuroendocrine tumor of the thymus resembling a vascular neoplasm.

    Three cases of primary thymic neuroendocrine tumors characterized by prominent angiomatoid features that resembled a vascular neoplasm are presented. The patients were all men between 52 and 59 years of age who presented with chest pain and shortness of breath attributable to a large anterior mediastinal mass. The lesions ranged in size from 6 cm to 15 cm in greatest diameter, and were grossly soft and well circumscribed, but not encapsulated. The cut surface was remarkable for multiple blood-filled cyst-like spaces admixed with focal solid, hemorrhagic areas. Histologically, the tumors contained multiple cystically dilated spaces filled with blood which imparted the lesion with a striking angiomatoid appearance. The walls of the cysts were lined by a monotonous proliferation of round to oval cells with distinct cell borders, round central nuclei, and abundant eosinophilic cytoplasm. Mitotic activity was present in all cases and varied from 3 to 8 mitoses per 10 high-power fields. Immunohistochemical studies performed in two cases showed positivity of the tumor cells for keratin, Leu 7, and synaptophysin, and focal chromogranin positivity in one. Follow-up information obtained in two patients showed that both had died of tumor 4 and 8 years after initial diagnosis. The present cases show an unusual morphological appearance of thymic neuroendocrine tumors that may be mistaken for a vascular neoplasm. Immunohistochemical stains may be of importance in such instances in arriving at the correct diagnosis.
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4/355. Multifocal polyradiculoneuropathy and carcinoma of the thymus.

    We studied a patient with polyradiculoneuropathy with anaplastic carcinoma of the thymus. Motor manifestations dominated. Postmortem examinations indicated that the primary changes were in the spinal nerve roots, peripheral nerves and, possibly, the spinal anterior horn cells. The posterior funiculi and posterior root ganglia were also affected, implying multifocal and multiphasic degeneration. This unusual polyradiculoneuropathy is a form of carcinomatous neuropathy.
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5/355. A long-term survival case of thymic squamous cell carcinoma, performed complete extirpation with vascular reconstruction of the superior vena cava.

    A 59-year-old woman who complained of anterior chest pain exhibited an abnormal shadow on chest X ray and was admitted to our hospital. The chest X ray showed a demarcated tumor at the anterosuperior mediastinum and she was diagnosed as having a mediastinal tumor. After the midsternotomy was performed, the mediastinal tumor derived from the thymic tissues was discovered to have invaded the right upper lung, pericardium and superior vena cava. After excising the tumor, anastomosis between the right brachiocephalic vein and superior vena cava, followed by that between the left brachiocephalic vein and right cardiac auricle was performed using expanded polytetrafluoroethylene-ringed vascular grafts (phi 10 mm) for reconstruction. The tumor was diagnosed as a thymic carcinoma (squamous cell carcinoma) pathologically. After surgery, she was treated by cobalt irradiation. One month and again 3 months after the operation, venography showed patency. The patient has not demonstrated recurrence for 9 years and 6 months.
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6/355. Granulocytic sarcoma of the thymus in a nonleukaemic patient.

    We report a case of granulocytic sarcoma arising from the thymus in a 17-year-old nonleukaemic patient. The patient presented with an anterior mediastinal tumour and underwent surgical resection. Histological examination showed a diffuse infiltrate of immature round cells in the thymus. Tumour cells were diffusely peroxidase positive, but naphthol AS-D chloroacetate esterase negative. Immunohistochemical staining revealed expression of CD34 and terminal deoxynucleotidyl transferase (TdT), but not of CD13 and CD33. Ultrastructurally, electron-dense or medium-density granules were present in the cytoplasm. Four months after successful autogenic bone marrow transplantation, pleural and pericardial fluid contained tumour cells with azurophilic granules, which expressed CD13 and CD33, but not CD34 and TdT. The patient died of the disease 18 months after clinical manifestation, but still without developing leukaemia. The granulocytic sarcoma in the present case may have originated from myeloid precursors in the thymus and remained within the extramedullary site despite the differentiation into a more committed myeloid lineage at the relapse.
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7/355. Pigmented thymic carcinoids: a clinicopathological and immunohistochemical study of two cases.

    Two cases of pigmented thymic carcinoids are presented. The patients were two men, 32 and 47 years of age. The two patients were asymptomatic and the tumor was discovered on routine chest radiographic evaluation. The tumors were treated by surgical excision in both patients. Grossly, they presented as tan-white tumors without evidence of necrosis or hemorrhage or any visible pigmentation. Histologically, the tumors were characterized by a monotonous proliferation of tumor cells arranged in a trabecular or nesting pattern. The tumor cells showed moderate amounts of pale eosinophilic cytoplasm, round to oval nuclei, and inconspicuous nucleoli. Mitotic activity varied from three to eight per 10 high-power fields. In addition, both tumors showed prominent areas of intra- and extracellular dark pigment deposition. The pigment reacted positively with the Fontana-Masson stain and was negative for iron stain. Immunohistochemical studies performed in one case showed immunoreactivity of the tumor cells for chromogranin, Leu 7, and keratin. synaptophysin and P53 immunostains were negative. Clinical follow-up was obtained in one patient who was alive and well 10 years after surgical resection. The presence of abundant melanin pigment in thymic carcinoids highlights an important pitfall for diagnosis in these tumors that should be taken into consideration in the evaluation of anterior mediastinal lesions.
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8/355. Thymic Hodgkin's disease--a histological and immunohistochemical study of three cases.

    Thymic Hodgkin's disease (HD) shows some peculiar histological features different from nodal disease which are a result of the interaction with the specific thymic microenvironment. We describe the histological and immunohistochemical findings in three cases presenting as a primary thymic neoplasm both clinically and radiologically. Histological hallmarks were the prominent formation of epithelium-lined cysts, inflammatory changes, a marked proliferation of thymic epithelium in association with Hodgkin- and Reed-Sternberg (RS) cells and the occurrence of the nodular sclerosing subtype in all cases. The immunophenotype of the neoplastic cells was that of classical HD. They expressed CD30, CD15 and lacked CD45. In two cases CD20 expression was observed. All cases were negative for the latent membrane protein (LMP) of the Epstein-Barr virus (EBV). The accompanying inflammatory infiltrate was rich in mature T-cells, but also showed a significant number of B-cells with frequent formation of follicles and proliferation of follicular dendritic cells. Thymic HD develops in a microenvironment with features of thymic medulla as defined by the morphology and pattern of the proliferating epithelial cells and the mature immunophenotype of the admixed thymocytes. These findings, especially the CD20 positivity in Hodgkin and RS-cells, may point to the possible origin of thymic HD from medullary B-cells.
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9/355. Bronchial arterial infusion with cisplatin followed by irradiation successfully treats recurrent stage IVb thymic large cell carcinoma.

    A 48-year-old female was treated with three courses of chemotherapy combined with bronchial arterial infusion (BAI) with cisplatin and intravenous drip infusion with bleomycin and vinblastine for a recurrent thymic large cell carcinoma seen 1 year after the initial operation. The patient had subsequently undergone irradiation. No sign of recurrence has been noted with a follow-up period of 6 years after the initial operation. This is the first reported case of survival from recurrence of thymic large cell carcinoma. BAI combined with irradiation should be considered as one of the treatments of choice in a recurrence like we present herein.
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10/355. Macrocytic anemia and thrombocytosis associated with thymoma: a case report.

    Thymomas are often associated with autoimmune disorders. We report on a 45-year-old female patient with thymoma and hypogammaglobulinemia (Good's syndrome) who developed symptomatic macrocytic anemia (Hb 4.4 g/dl, MCV 112 fl) and thrombocytosis (Plt 442 G/l). Besides hypogammaglobulinemia (IgG 589 mg/dl), an inverted ratio of CD4( )/CD8( ) cells was seen. The bone marrow biopsy showed a slightly hypercellular bone marrow with normal granulopoiesis, normal megakaryopoiesis and a mild dyserythropoiesis without any ring-sideroblasts. The in-vitro stem cell culture from the bone marrow revealed an atypical growth of macroclusters, reduced BFU-E and CFU-GEMM colony growth, whereas the CFU-GM colony growth was within the normal range. The chromosomal analysis showed a normal karyotype. The plasma vitamin B(12) and folate levels were within normal ranges, and we could not detect any autoantibodies. These findings excluded the differential diagnoses pure red cell aplasia (PRCA) and pernicious anemia. After resection of the thymoma of mixed cell type, the macrocytic anemia and thrombocytosis disappeared. The clinical course was complicated by a cerebral palsy and a life-threatening fungal septicemia after surgery. In the third year after thymectomy, hyporegenerative macrocytic anemia and thrombocytosis reappeared and an immunosuppressive treatment with prednisolone (1 mg/kg BW) was started. After initiation of the prednisolone therapy, reticulocyte counts increased and macrocytic anemia as well as thrombocytosis disappeared. The normalization of these laboratory parameters during glucocorticoid therapy suggests that in rare cases the constellation of macrocytic anemia, thrombocytosis and hypogammaglobulinemia may be due to an underlying immunologic mechanism.
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