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      Nephrotic syndrome associated with metastatic thymoma treated with chemotherapy

      case-report

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          Abstract

          Rationale:

          Nephropathy with concurrent invasive thymoma is a type of paraneoplastic syndrome.

          Patient concerns and Diagnoses:

          We report a 32-year-old female with nephrotic syndrome that was first diagnosed along with invasive thymoma and treated by means of cisplatin-based chemotherapy for the thymoma. The patient initially presented with dyspnea and generalized edema. Chest radiography and computed tomography scans revealed right pleural effusion and a mass in the right middle lung field, which were confirmed by a percutaneous lung biopsy as metastatic invasive thymoma. Severe hypoalbuminemia, heavy proteinuria, hyponatremia, and hypercholesterolemia were features of the nephrotic syndrome. A kidney needle biopsy suggested focal segmental glomerulosclerosis.

          Interventions and Outcomes:

          All of the symptoms of nephrotic syndrome were resolved simultaneously during the first 2 cycles of chemotherapy. The patient was on regular follow-up with no specific treatment for nephrotic syndrome and underwent successful resection of the left pleura and anterior thymoma. The patient has shown no evidence of recurrence for 2 years.

          Lessons:

          We conclude that chemotherapy for invasive thymoma is an effective treatment for nephrotic syndrome accompanying the thymoma.

          Related collections

          Most cited references18

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          Therapy for thymic epithelial tumors: a clinical study of 1,320 patients from Japan.

          Surgery remains the mainstay of treatment for thymic epithelial tumors, and radiation and chemotherapy also have been applied widely as adjuvant and palliative procedures. We compiled records of 1,320 patients with thymic epithelial tumors who were treated from 1990 to 1994 in 115 institutes certified as special institutes for general thoracic surgery by The Japanese Association for Chest Surgery. Patients with stage I thymoma were treated with only surgery, and patients with stage II and III thymoma and thymic carcinoid underwent surgery and additional radiotherapy. Patients with stage IV thymoma and thymic carcinoma were treated with radiation or chemotherapy. The Masaoka clinical stage is an excellent predictor of the prognosis of thymoma and thymic carcinoma, but not thymic carcinoid. In stage III and IV thymoma, the 5-year survival rates of total resection, subtotal resection, and inoperable groups were 93%, 64%, and 36%, respectively. On the other hand, in thymic carcinoma, the 5-year survival rates of total resection, subtotal resection, and inoperable groups were 67%, 30%, and 24%, respectively. Prophylactic mediastinal radiotherapy could not prevent local recurrences effectively in patients with totally resected stage II and III thymoma. Adjuvant therapy including radiation or chemotherapy did not improve the prognosis in patients with totally resected III and VI thymoma and thymic carcinoma. Total resection is the most important factor in the treatment of thymic epithelial tumors. There is value in debulking surgery in invasive thymoma, but not in thymic carcinoma. We doubt that adjuvant therapy is valuable for patients with totally resected invasive thymoma and thymic carcinoma.
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            Pathogenesis of lipoid nephrosis: a disorder of T-cell function.

            J Shalhoub (1974)
            Clinical observations suggest that lipoid nephrosis is produced by a systemic abnormality of T-cell function resulting in the secretion of a circulating chemical mediator toxic to an immunologically innocent glomerular basement membrane. The lack of evidence of a humoral antibody response, remission induced by measles which modifies cell-mediated immunity, the therapeutic benefits of steroids and cyclophosphamide which also abate cell-mediated responses, and the occurrence of this syndrome in Hodgkin's disease support this hypothesis. The susceptibility of untreated patients to pneumococcal infections may be of primary or secondary pathogenetic importance. Taken together, the data suggest that this syndrome is a clinical expression of a self-limited primary immune-deficiency disease.
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              Malignant thymoma in the United States: demographic patterns in incidence and associations with subsequent malignancies.

              The cause of thymoma is unknown. No population-based study has described demographic patterns of thymoma incidence. Previous reports have linked thymoma with diverse subsequent malignancies, but these associations are uncertain. We used Surveillance, Epidemiology and End Results (SEER) data to study the incidence of malignant thymoma by sex, age and race in the United States (1973-1998). Incidence was modeled with joinpoint regression (for age) and Poisson regression. We also used SEER data to compare malignancies following thymoma diagnosis with those expected from general population rates, calculating the standardized incidence ratio (SIR, observed/expected cases) to measure risk. The overall incidence of malignant thymoma was 0.15 per 100000 person-years (849 cases). Thymoma incidence increased into the 8th decade of age and then decreased. Incidence was higher in males than females (p=0.007) and was highest among Asians/Pacific Islanders (0.49 per 100000 person-years). Following thymoma, there were 66 malignancies (SIR 1.5, 95%CI 1.2-1.9). The most notable excess risk for subsequent malignancy was for non-Hodgkin's lymphoma (B immunophenotype) where the SIR was 4.7 (95%CI 1.9-9.6, 7 cases). There were also excess digestive system cancers (SIR 1.8, 95%CI 1.1-2.9) and soft tissue sarcomas (SIR 11.1, 1.3-40.1). No other cancers were increased after thymoma. In conclusion, malignant thymoma is extremely rare. The peak in late adulthood deserves further study. Variation in incidence by race suggests a role for genetic factors. Our study did not demonstrate broadly increased risk for malignancies following thymoma. Copyright 2003 Wiley-Liss, Inc.
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                Author and article information

                Journal
                Medicine (Baltimore)
                Medicine (Baltimore)
                MEDI
                Medicine
                Wolters Kluwer Health
                0025-7974
                1536-5964
                January 2017
                10 January 2017
                : 96
                : 1
                : e5408
                Affiliations
                [a ]Department of Internal Medicine, Seoul National University Hospital, Seoul
                [b ]Division of Hemato-medical Oncology, Department of Internal Medicine, Seoul National University Bundang Hospital, Gyeong-gi
                [c ]Division of Hematology-Oncology, Department of Internal Medicine, Gyeongsang Institute of Health Science, Gyeongsang National University Hospital, Gyeongsang National University School of Medicine, Jinju
                [d ]Department of Pathology, Gyeonsang National University College of Medicine, Jinju, Korea.
                Author notes
                []Correspondence: Se Hyun Kim, Department of Internal Medicine, Seoul National University Bundang Hospital, 82 Gumi-ro 173, Bundang-gu, Seongnam, Korea (e-mail: sehyunkim@ 123456snubh.org)
                Article
                MD-D-16-05041 05408
                10.1097/MD.0000000000005408
                5228645
                28072685
                0a95e9b0-e4f1-4e8c-bb1f-f465d99b09c6
                Copyright © 2017 the Author(s). Published by Wolters Kluwer Health, Inc.

                This is an open access article distributed under the terms of the Creative Commons Attribution-Non Commercial-No Derivatives License 4.0 (CCBY-NC-ND), where it is permissible to download and share the work provided it is properly cited. The work cannot be changed in any way or used commercially without permission from the journal. http://creativecommons.org/licenses/by-nc-nd/4.0

                History
                : 4 August 2016
                : 26 October 2016
                : 27 October 2016
                Categories
                5700
                Research Article
                Clinical Case Report
                Custom metadata
                TRUE

                case reports,nephrotic syndrome,paraneoplastic syndromes,thymoma

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