Nephrotic syndrome associated with metastatic thymoma treated with chemotherapy

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.

Autoimmune disorders and paraneoplastic syndromes in thymoma

“…Both, non-proliferative and proliferative glomerulopathies were associated with systemic autoimmune features in thymomas, namely minimal change disease, membranous nephropathy and focal segmental glomerulosclerosis as well as membranoproliferative glomerulonephritis and crescentic glomerulonephritis (rapidly progressive glomerulonephritis) as outlined in a former review by Bacchetta et al (90) and in recent case reports (91)(92)(93)(94)(95). Pronounced in the nonproliferative glomerulopathies, nephrotic syndrome is frequently encountered as complication which itself bears the risk of secondary thrombosis or infections due to renal protein loss.…”

“…Pronounced in the nonproliferative glomerulopathies, nephrotic syndrome is frequently encountered as complication which itself bears the risk of secondary thrombosis or infections due to renal protein loss. Responses to immunosuppressive agents in combination with thymoma-specific treatments were described as quite favourable (90)(91)(92)(93)(94)(95). The underlying pathophysiologies are still inadequately understood but seem to involve different mechanisms of AID as well as PNS eliciting the distinct glomerulopathy variants (96).…”

Thymoma-associated nephrotic syndrome: report of seven cases

“…A short combination therapy, insu cient dosage of steroid and/or immunosuppressants and persistent existence of the primary etiology may be the fail reason of case 2, 4 and 7. On the other hand, Shin Hye Yoo reported a patient with metastatic thymoma accompanied by NS (surgical staging: Masaoka stage IVa, World Health Organization type B3), which is the same WHO classi cation stage of case 4, with 6 cycles of chemotherapy and removal of left diaphragm, left pleura and anterior thymomectomy, the patient achieved complete remission, and no signs of recurrence of thymoma and NS were observed after that [20] .…”

Secondary membranous nephropathy in a patient with myasthenia gravis without thymic disease, and partial remission induced by adrenocorticotropic hormone therapy

“…See Table 1 for previously reported cases of MG with or without thymic disease and glomerular disease. 1,8,18,19,22,23,26–39 A newer case report by Bolz et al 40 was added where the MGN could be classified as primary in setting of anti-PLA2R antibody positivity. There are 11 other cases with similar presentation to our currently presented case (total of 12 with current case): nine cases with MG and concomitant MGN and three cases with thymus disease (benign or malignant), MGN, but no active MG. A variety of other nephropathies that present with MG and both benign and malignant thymomas are also listed and totaled 70 cases.…”