Nephrogenic Systemic Fibrosis with Multiorgan Involvement in a Teenage Male after Lymphoma, Ewing's Sarcoma, End-Stage Renal Disease, and Hemodialysis

Abstract

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.

Nephrogene systemische Fibrose

“…Innerhalb von Tagen bis Wochen entwickeln sich so Bewegungseinschränkungen, die zu Kontrakturen führen können. Neben der Hautbeteiligung ließen sich darüber hinaus fibrotische Veränderungen in anderen Organen wie dem Bronchialsystem, dem Zwerchfell und den Herzklappen nachweisen [4]. Todd et al berichteten in einer Arbeit über eine signifikant erhöhte Mortalität von NSF-Patienten gegenüber einer Kontrollgruppe von Patienten mit chronischer Niereninsuffizienz im Stadium 5 [5].…”

CT and MR Imaging of Progressive Dural Involvement by Nephrogenic Systemic Fibrosis

“…10 Initially, NSF was believed to involve the skin and subcutaneous tissues, but subsequent reports documented involvement of the myocardium, muscle, lung, pericardium, pleura, kidney, bone, testis, and dura. [4][5][6][7][8] The diagnosis of NSF is made by a combination of clinical, laboratory, and histopathologic findings. 2,7 Prognosis is variable, but NSF may be fatal.…”

“…4,6 Both spinal and intracranial dural involvement have been described. 4,6,8 Microscopic examination demonstrates fibrosis with a spindle-cell proliferation, areas of calcification, collections of CD68-positive mononuclear cells, and occasional multinucleated giant cells. 4 The histologic changes in the dura mater of our patient were similar to those previously reported.…”

“…4 The histologic changes in the dura mater of our patient were similar to those previously reported. 4,6,8 The differential diagnosis for dural thickening and enhancement is extensive and includes sarcoidosis, tuberculosis, Wegener granulomatosis, intracranial hypotension, lymphoma, and metastatic disease. The differential diagnosis for dural calcifications includes physiologic calcifications, previous hemorrhage, previous infection, pseudoxanthoma elasti-cum, hyperparathyroidism, basal cell nevus syndrome, and idiopathic calcification.…”

“…[4][5][6][7] Dural involvement has been described at autopsy; however, the depiction of the progressive imaging changes has not been documented. 4,6,8 We report a patient with progressive dural calcification, thickening, and enhancement as a manifestation of NSF. The initial brain MR imaging examination in September 2004 was performed for elevated serum prolactin levels.…”