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Abstract: PO1868

Unusual Case of Histiocytic Glomerulopathy in the Setting of Sarcomatoid Malignancy

Session Information

Category: Trainee Case Report

  • 1203 Glomerular Diseases: Clinical, Outcomes, and Trials


  • Al faris, Faris, Albany Medical Center, Albany, New York, United States
  • Al-Ahmad, Majd, Albany Medical Center, Albany, New York, United States
  • Foulke, Llewellyn A., Albany Medical Center, Albany, New York, United States
  • Monrroy, Mauricio, Albany Medical Center, Albany, New York, United States
  • Beers, Kelly H., Albany Medical Center, Albany, New York, United States

We present a case of histiocytic glomerulopathy and pauci-immune ANCA negative glomerulonephritis (GN) in the setting of sarcomatoid malignancy.

Case Description

An 83-year-old female presented to our hospital for evaluation of abdominal pain. Her vital signs were normal and her physical exam was only significant for mid-abdominal tenderness on palpation. Aortic angiography revealed occlusion of the superior mesenteric artery, celiac artery and left renal artery, in addition to a mass engulfing the celiac artery. She later developed acute kidney injury (AKI) with creatinine rising to 1.5mg/dl from baseline of 1.2mg/dl. Work up reveled bland urine sediment and 24-hour urine protein of 3.4g/day. Further laboratory studies revealed anti myeloperoxidase of 19.4u/ml, anti-proteinase 3 of 10.3u/ml, negative C-ANCA and P-ANCA, and normal complement levels.
Renal biopsy showed focal crescentic and endocapillary proliferative GN comprised of mostly of CD68-positive foamy histiocytes, pauci-immune, with segmental C3 staining, as well as severe arterial fibrointimal hyperplasia with scattered intimal histiocytes and lymphocytes, consistent with arteritis and acute tubular injury with foci of prominent tubular vacuolization. Contrast induced nephropathy was thought to be the cause of AKI. Celiac mass biopsy showed sarcomatoid malignancy.


Histiocytic glomerulopathy poses a diagnostic challenge as it has many possible underlying causes with different pathophysiology. Kaur and Sethi described 5 entities with histiocytic/foamy glomerular change on the kidney biopsy: crystal-storing histiocytosis, histiocytic glomerulopathy associated with macrophage-activating syndrome, thrombotic microangiopathy, lecithin-cholesterol acyltransferase deficiency and lipoprotein glomerulopathy. Our patient does not fulfill criteria for either of these entities.
Small and large vessel vasculitis is well described as neoplastic and paraneoplastic phenomena, including both immune complex–mediated and ANCA-associated (pauci-immune).
Pauci-immune GN is a rare and aggressive cause of AKI with 10% of the cases being ANCA-negative. Few case reports linked ANCA-negative pauci-immune GN to non-small cell lung cancer.
To our knowledge this is the first case of histiocytic glomerulopathy, ANCA-negative pauci-immune GN and arteritis in the setting of sarcomatoid malignancy.