Isolated Intraspinal Juvenile Xanthogranuloma in an Infant Presenting as Acute Paraplegia

Abstract

Juvenile xanthogranuloma is a histiocytic proliferative disease that predominantly affects the skin. Extracutaneous involvement is rare. We present the case of a 6-month-old infant with acute paraplegia. Magnetic resonance imaging showed an intraspinal extradural mass at midthoracic level with marked compression of the spinal cord. Complete tumor removal was achieved by emergency surgery and was followed by complete neurologic recovery. Histologic examination led to the diagnosis of a juvenile xanthogranuloma. To the best of our knowledge, an isolated intraspinal juvenile xanthogranuloma in the first 12 months of life has not been described before.

Keywords

juvenile xanthogranuloma - children - paraplegia - spinal cord

• References

• 1 Adamson HG. Congenital xanthoma multiplex. Society intelligence: The Dermatological Society of London. Br J Dermatol 1905; 17: 222
• 2 Castro-Gago M, Gómez-Lado C, Alvez F, Alonso A, Vieites B. Juvenile xanthogranuloma of the cauda equina. Pediatr Neurol 2009; 40 (2) 123-125
• 3 Dehner LP. Juvenile xanthogranuloma in the first two decades of life: a clinicopathologic study of 174 cases with cutaneous and extracutaneous manifestations. Am J Surg Pathol 2003; 27 (5) 579-593
• 4 Inoue H, Seichi A, Yamamuro K, Kojima M, Kimura A, Hoshino Y. Dumbbell-type juvenile xanthogranuloma in the cervical spine of an adult. Eur Spine J 2011; 20 (Suppl. 02) S343-S347
• 5 Janssen D, Harms D. Juvenile xanthogranuloma in childhood and adolescence: a clinicopathologic study of 129 patients from the Kiel pediatric tumor registry. Am J Surg Pathol 2005; 29 (1) 21-28
• 6 Sun LP, Jin HM, Yang B, Wu XR. Intracranial solitary juvenile xanthogranuloma in an infant. World J Pediatr 2009; 5 (1) 71-73
• 7 Cao D, Ma J, Yang X, Xiao J. Solitary juvenile xanthogranuloma in the upper cervical spine: case report and review of the literature. Eur Spine J 2008; 17 (Suppl. 02) S318-S323
• 8 Ashley Jr WW, Narayan P, Park TS, Tu PH, Perry A, Leonard JR. Incidental pediatric intraparenchymal xanthogranuloma: case report and review of the literature. J Neurosurg 2005; 102 (3, Suppl) 307-310
• 9 Zvulunov A, Barak Y, Metzker A. Juvenile xanthogranuloma, neurofibromatosis, and juvenile chronic myelogenous leukemia. World statistical analysis. Arch Dermatol 1995; 131 (8) 904-908
• 10 Iwasaki Y, Hida K, Nagashima K. Cauda equina xanthogranulomatosis. Br J Neurosurg 2001; 15 (1) 72-73
• 11 Kim DS, Kim TS, Choi JU. Intradural extramedullary xanthoma of the spine: a rare lesion arising from the dura mater of the spine: case report. Neurosurgery 1996; 39 (1) 182-185
• 12 Kitchen ND, Davies MS, Taylor W. Juvenile xanthogranuloma of nerve root origin. Br J Neurosurg 1995; 9 (2) 233-237
• 13 Rampini PM, Alimehmeti RH, Egidi MG , et al. Isolated cervical juvenile xanthogranuloma in childhood. Spine 2001; 26 (12) 1392-1395
• 14 Shimosawa S, Tohyama K, Shibayama M, Takeuchi H, Hirota T. Spinal xanthogranuloma in a child: case report. Surg Neurol 1993; 39 (2) 138-142
• 15 Fulkerson DH, Luerssen TG, Hattab EM, Kim DL, Smith JL. Long-term follow-up of solitary intracerebral juvenile xanthogranuloma. Case report and review of the literature. Pediatr Neurosurg 2008; 44 (6) 480-485