Nephrotic syndrome associated with meningioma
A 58-year-old man presented to the nephrology clinic in September 2011 with a history of progressive pedal and scrotal edema of two weeks’ duration. In 2005, he had undergone surgical resection of a left frontal meningioma and follow-up till 2007 was unremarkable. Since the last 12 months, he had gradually increasing proptosis on the left side with decreasing vision, which had significantly worsened since the last two weeks. There was no history of any drug intake during the last 12 months.\nPhysical examination revealed massive pedal and scrotal edema, left orbital proptosis with mechanical restriction of left-eye movement, mainly elevation and abduction, and chemosed conjunctiva. There were no other neurological deficits; other systems were normal. Direct fundoscopy revealed a large mass indenting almost half the globe from above with multiple choroidal folds Passing through the fovea with dilation and tortuosity of retinal veins, predominantly in the superior half with disc edema on the left side.\nHe had normal serum creatinine (0.6 mg/dl), hypoalbuminemia (1.5 gm/dl), hypercholesterolemia (300 mg/dl) and proteinuria (4+) with no abnormality on urine microscopy. 24-h urine protein excretion was 12.672 mg. Hemogram, blood sugars, and serum complements were normal. HBsAg and anti-HCV (ELISA) was negative. Ultrasonogram of the abdomen showed normal-sized kidneys with bilateral, raised cortical echo texture. X-ray chest was normal. A diagnosis of nephrotic syndrome, probably membranous nephropathy was entertained. Computed tomography (CT) scan of head showed a large-mass lesion involving the frontal and intra-orbital regions with extensive bony involvement []. As per the imaging characteristics, the lesion was very suggestive of frontal meningioma.\nThe patient was initially treated with diuretics and albumin/plasma transfusions. A renal biopsy was planned; however, he developed recurrent focal seizures. He was started on appropriate anti-convulsants. Despite being on anti-convulsants, he had an episode of status epilepticus, consequent to which he had to be ventilated. In view of the recurrent seizures, he had to be taken up for an emergency surgery. He underwent left craniotomy, and total excision of the tumor was done. Histology confirmed features of atypical meningioma (WHO grade 2) with clear cell areas and invasion into the orbital tissues. Immunohistochemistry revealed strong epithelial membrane antigen (EMA) positivity and high Ki 67 index, which suggested increased possibility for recurrence. Post-operative course was uneventful and there were no further seizure episodes. Ocular movements recovered gradually. He was discharged two weeks after the surgery. Within four weeks of resection of the tumor, edema disappeared and serum albumin started to improve. At three months after surgery, urine albumin was negative, serum albumin was 4.1 gm/dl, and serum cholesterol was 170 mg/dl. Follow-up MRI scan three months after surgery showed mild gliotic changes []. Six months after surgery, he is doing well and his nephrotic state is in remission.