Introduction: Langerhans cell histiocytosis (LCH) is a group of idiopathic disorders characterized by the proliferation of specialized bone marrow-derived Langerhans cells (LCs) and mature eosinophils, resulting in solitary or few, indolent and chronic, lesions of bone or other organs called eosinophilic granulomas.Calvarial LCH is quite rare and an underappreciated differential etiology of skull lesions. We present a most unusual case of a young child with hyperacutely symptomatic langerhans histiocytosis of the skull. Method: A 7-year-old male presented with a history of increasing (progressive) frontal headaches of 8 days duration, unaccompanied by associated nausea, vomiting, or diplopia. His only additional complaint was a hard bump on his forehead. MRI and CT done in the ER identified a right fronto-parietal lesion with associated skull erosion. Nuclear medicine and SPECT studies confirmed an erosive skull lesion without significant metabolic activity. A right frontal craniectomy and excision was performed. Results: A soft, rubbery well-circumcised mass coming from the diploic layer of the skull with involvement of bone was identified.The mass had eroded both the outer and inner table of the skull, and the involved area of the right frontal bone was resected. Intra-operative histo-pathologic analysis of the lesion revealed Langerhans cell histiocytosis without involvement of the dura. The patient experienced no neurological worsening as a result of the resection. He was discharged home in stable condition. Conclusion: LCH lesions of the skull are common findings, however, this focal hyperacute symptomatic presentation is most rare and should not deter us from anticipating an erosive bony tumor and planning timely surgical management.
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P. Kaloostian, H. Chen, F. Westhout and E. Marchand, "Unusual Acute Onset of Symptomatic Langerhans Cell Histiocytosis of the Skull: Case Report and Literature Review," Open Journal of Modern Neurosurgery, Vol. 3 No. 3, 2013, pp. 44-47. doi: 10.4236/ojmn.2013.33010.
 A. M. Stark, T. Eichman and H. M. Mehdorn, “Skull Metastases: Clinical Features, Differential Diagnosis, and Review of the Literature,” Surgical Neurology, Vol. 60, No. 3, 2003, pp. 219-225.
 G. Alessi, M. Lemmerling, L. Vereecken and L. De Waele, “Benign Metastasizing Leiomyoma to Skull Base and Spine: A Report of Two Cases,” Clinical Neurology and Neurosurgery, Vol. 105, No. 3, 2003, pp. 170-174.
 J. McLelland, J. Pritchard and A. C. Chu, “Current Controversies: Histiocytosis-X,” Hematology/Oncology Clinics of North America, Vol. 1, No. 1, 1987, pp. 147-162.
 A. Shahla, V. Parvaneh and H. D. Hossein, “Langerhans Cells Histiocytosis in One Family,” Pediatric Hematology-Oncology, Vol. 21, No. 4, 2004, pp. 313-320.
 P. Kleihaus and W. K. Cavenee, “Pathology and Genetics: Tumours of the Nervous System—WHO Classification of Tumours, International Agency for Research on Cancer,” IARC Press, Lyon, 2000, p. 204.
 C. Hadjigeorgi, C. Parpounas, P. Zarmakoupis and S. Lafoyianni, “Eosinophilic Granuloma of the Temporal Bone: Radiological Approach in the Pediatric Patient,” Pediatric Radiology, Vol. 20, No. 7, 1990, pp. 546-549.
 M. A. Leahy, S. M. Krejci, M. Friednash, S. S. Stockert, H. Wilson, J. C. Huff, et al., “Human Herpesvirus 6 Is Present in Lesions of Langerhans Cell Histiocytosis,” Journal of Investigative Dermatology, Vol. 101, 1993, pp. 642-645. doi:10.1111/1523-1747.ep12371669
 C. L. Willman, L. Busque, B. B. Griffith, B. E. Favara, K. L. McClain, M. H. Duncan, et al., “Langerhans’ Cell Histiocytosis (Histiocytosis-X)—A Clonal Proliferative Disease,” The New England Journal of Medicine, Vol. 331, 1994, pp. 154-160.
 B. Källén, O. Finnström, A. Lindam, E. Nilsson, K. G. Nygren and P. O. Olausson, “Cancer Risk in Children and Young Adults Conceived by in Vitro Fertilization,” Pediatrics, Vol. 126, 2010, pp. 270-276.
 J. D. Wirtschafter, M. Nesbit, P. Anderson and K. McClain, “Intralesional Methylprednisolone for Langerhans’ Cell Histiocytosis of the Orbit and Cranium,” Journal of Pediatric Ophthalmology and Strabismus, Vol. 24, No. 4, 1987, pp. 194-197.
 G. A. Kaltsas, T. B. Powles, J. Evanson, P. N. Plowman, J. E. Drinkwater, P. J. Jenkins, et al., “Hypothalamo-Pituitary Abnormalities in Adult Patients with Langerhans Cell Histiocytosis: Clinical, Endocrinological, and Radiological Features and Response to Treatment,” The Journal of Clinical Endocrinology & Metabolism, Vol. 85, No. 4, 2000, pp. 1370-1376. doi:10.1210/jc.85.4.1370
 M. Oliveira, P. Steinbok, J. Wu, N. Heran and D. Cochrane, “Spontaneous Resolution of Calvarial Eosinophilic Granuloma in Children,” Pediatric Neurosurgery, Vol. 38, 2003, pp. 247-252. doi:10.1159/000069828
 M. J. Katati, J. M. Martin, J. Pastor and V. Arjona, “Isolated Primary Langerhans’ Cell Histiocytosis of Central Nervous System,” Neurocirugía, Vol. 13, No. 6, 2002, pp. 477-478.
 D. Belen, A. Colak and O. E. Ozcan, “CNS Involvement of Langerhans Cell Histiocytosis: Report of 23 Surgically Treated Cases,” Neurosurgical Review, Vol. 19, No. 4, 1996, pp. 247-252. doi:10.1007/BF00314840
 K. M. Fung, J. M. Schwalb, H. A. Riina, J. S. Kurana, J. M. Mindaxy, M. S. Grady, et al., “February 2002: 29-Year-Old Woman with a Skull Mass for 2 Months,” Brain Pathology, Vol. 12, No. 3, 2002, pp. 393-394.
 A. Taranath and E. Onikul, “Fluid-Fluid Levels in Transcalvarial Langerhans’ Cell Histiocytosis,” Pediatric Radiology, Vol. 34, No. 4, 2004, p. 363.
 V. Debois and R. van den Bergh, “Benign Tumors of the Cranial Vault, a Report of 12 Cases,” Clinical Neurology and Neurosurgery, Vol. 81, No. 1, 1979, pp. 1-12.
 S. I. Basha, J. B. Kinsella and S. S. Napier, “Unifocal Langerhans’ Cell Histiocytosis and Frontal Sinus Agenesis: Report of a Rare Case,” Ear, Nose & Throat Journal, Vol. 81, No. 11, 2002, pp. 795-799.
 A. Isoo, K. Ueki, T. Ishida, T. Yoshikawa, T. Fujimaki, I. Suzuki, et al., “Langerhans Cell Histiocytosis Limited to the Pituitary-Hypothalamic Axis—Two Case Reports,” Neurologia Medico-Chirurgica, Vol. 40, No. 10, 2000, pp. 532-535. doi:10.2176/nmc.40.532
 L. I. Malis, “Titanium Mesh and Acrylic Cranioplasty,” Neurosurgery, Vol. 25, No. 3, 1989, pp. 351-355.
 J. L. Brisman, N. A. Feldstein, N. J. Tarbell, D. Cohen, A. L. Cargan, J. Haddad Jr. and J. N. Bruce, “Eosinophilic Granuloma of the Clivus: Case Report, Follow-Up of Two Previously Reported Cases, and Review of the Literature on Cranial Base Eosinophilic Granuloma,” Neurosurgery, Vol. 41, No. 1, 1997, pp. 273-278.
 S. C. Mayou, A. C. Chu, D. D. Munro and N. Plowman, “Langerhans-Cell Histiocytosis—Excellent Response to Etoposide,” Clinical and Experimental Dermatology, Vol. 16, No. 4, 1991, pp. 292-294.