Nephrogenic systemic fibrosis with multiorgan involvement in a teenage male after lymphoma, Ewing's sarcoma, end-stage renal disease, and hemodialysis.
Nephrogenic systemic fibrosis (NSF) is a rare condition that always occurs after acute or chronic renal failure with or without dialysis. The vast majority of cases in the literature are adults, and postmortem findings have been reported in only 5 cases. We report a 15-year-old male who developed NSF with multiorgan involvement after successful treatment of renal lymphoma and a subsequent sacral Ewing's sarcoma, and end-stage renal disease treated with hemodialysis. At autopsy, he was found to have diffuse dural osseous metaplasia, transmural bronchiolar fibrosis, diaphragmatic central tendon fibrosis, and fibrous plaques of the mitral valve. These previously unreported findings expand the spectrum of multiorgan involvement in NSF providing additional evidence that it is an emerging systemic disorder.
Krous
Henry F
HF
Pathology Department, Rady Children's Hospital--San Diego, San Diego, CA, USA. hkrous@chsd.org
Breisch
Eric
E
Chadwick
Amy E
AE
Pinckney
Lee
L
Malicki
Denise M
DM
Benador
Nadine
N
D000293:Adolescent; D000970:Antineoplastic Agents / Q000009:adverse effects; D001842:Bone and Bones / Q000473:pathology; D002114:Calcinosis / Q000209:etiology / Q000473:pathology; D004388:Dura Mater / Q000473:pathology; D005355:Fibrosis; D006801:Humans; D007676:Kidney Failure, Chronic / Q000139:chemically induced / Q000150:complications* / Q000628:therapy; D007680:Kidney Neoplasms / Q000188:drug therapy / Q000532:radiotherapy; D016030:Kidney Transplantation; D008171:Lung Diseases / Q000209:etiology / Q000473:pathology; D016403:Lymphoma, Large B-Cell, Diffuse / Q000188:drug therapy / Q000473:pathology* / Q000532:radiotherapy; D008297:Male; D008679:Metaplasia; D008943:Mitral Valve / Q000473:pathology; D016609:Neoplasms, Second Primary / Q000473:pathology; D011878:Radiotherapy / Q000009:adverse effects; D006435:Renal Dialysis / Q000009:adverse effects; D012512:Sarcoma, Ewing / Q000188:drug therapy / Q000473:pathology*; D012871:Skin Diseases / Q000209:etiology* / Q000473:pathology
D000970:Antineoplastic Agents