Solitary Cranial Langerhans Cell Histiocytosis: Two case reports.

Langerhans cell histiocytosis (LCH) is a proliferation of Langerhans cells intermixed with inflammatory cells, in particular eosinophils, that may manifest as a unisystem (unifocal or multifocal) or multisystem disease. We describe the clinical and histologic spectrum of LCH of the orbit and skull in our two cases. Both cases had unifocal erosive skull lesions with a history of trauma. Typical histologic features included numerous histiocytes with varying degrees of giant cell formation and scattered eosinophilic granulocytes. The presence of Langerhans cells was confirmed by CD1a and S100 immunohistochemistry. LCH has an excellent prognosis when treated with surgical resection, steroids and radiotherapy or chemotherapy. One of our patients is disease free at 7 year follow-up and one patient had regression of lesion on follow-up.

[1]  G. Harris,et al.  Langerhans Cell Histiocytosis of the Orbit: Spectrum of Disease and Risk of Central Nervous System Sequelae in Unifocal Cases , 2016, Ophthalmic plastic and reconstructive surgery.

[2]  C. Allen,et al.  Treatment of Langerhans cell histiocytosis: it is time to learn from the past: response to Minkov and Rodriguez‐Galindo , 2015, British journal of haematology.

[3]  K. Hayashi,et al.  Interleukin-1 loop model for pathogenesis of Langerhans cell histiocytosis , 2015, Cell Communication and Signaling.

[4]  J. Jonas,et al.  Ocular trauma as the first presentation of Langerhans cell histiocytosis. , 2013, Eye science.

[5]  J. Rootman.  Diseases of the orbit , 2013 .

[6]  H. Grossniklaus,et al.  Langerhans cell histiocytosis of the orbit: five clinicopathologic cases and review of the literature. , 2013, Survey of ophthalmology.

[7]  F. Söylemezoğlu,et al.  Langerhans Cell Histiocytosis of the Orbit , 2013, European journal of ophthalmology.

[8]  G. Alexiou,et al.  Cranial unifocal Langerhans cell histiocytosis in children. , 2009, Journal of pediatric surgery.

[9]  S. Karimi,et al.  Craniofacial and intracranial manifestations of langerhans cell histiocytosis: report of findings in 100 patients. , 2008, AJR. American journal of roentgenology.

[10]  R. Egeler,et al.  Langerhans cell histiocytosis: update for the pediatrician , 2008, Current opinion in pediatrics.

[11]  N. Shinton.  WHO Classification of Tumors of Hematopoietic and Lymphoid Tissues , 2007 .

[12]  M. Binning,et al.  Novel Multidisciplinary Approach for Treatment of Langerhans Cell Histiocytosis of the Skull Base , 2007, Skull base : official journal of North American Skull Base Society ... [et al.].

[13]  H. Wagner,et al.  Acute brain injury triggers MyD88-dependent, TLR2/4-independent inflammatory responses. , 2007, The American journal of pathology.

[14]  U. Pötschger,et al.  Risk factors for diabetes insipidus in langerhans cell histiocytosis , 2006, Pediatric blood & cancer.

[15]  P. Hogendoorn,et al.  Langerhans-cell histiocytosis 'insight into DC biology'. , 2003, Trends in immunology.

[16]  D. Prayer,et al.  Central nervous system disease in Langerhans cell histiocytosis. , 1998, Hematology/oncology clinics of North America.

[17]  A multicentre retrospective survey of Langerhans' cell histiocytosis: 348 cases observed between 1983 and 1993. The French Langerhans' Cell Histiocytosis Study Group. , 1996, Archives of disease in childhood.

[18]  R. Zimmerman,et al.  Langerhans cell histiocytosis: presentation and evolution of radiologic findings with clinical correlation. , 1995, Radiographics : a review publication of the Radiological Society of North America, Inc.

[19]  A. W. Craft,et al.  Globe luxation in histiocytosis X. , 1988, The British journal of ophthalmology.

[20]  D. Taylor,et al.  Histiocytosis X: an ophthalmological review. , 1985, The British journal of ophthalmology.

[21]  N. Jaffe,et al.  Radiation therapy in patients with histiocytosis: management of diabetes insipidus and bone lesions. , 1979, International journal of radiation oncology, biology, physics.

[22]  S. Batnitzky,et al.  Coexistent Intra-and Extracranial Mass Lesions : An Unusual Manifestation of Histiocytosis X Eosinophilic granuloma , 2013 .

[23]  K. Woo,et al.  Eosinophilic granuloma of the orbit: a paradox of aggressive destruction responsive to minimal intervention. , 2003, Transactions of the American Ophthalmological Society.

[24]  R. Warrier,et al.  Langerhans' cell histiocytosis. , 1996, The Orthopedic clinics of North America.

[25]  S. Batnitzky,et al.  Coexistent intra- and extracranial mass lesions: an unusual manifestations of histiocytosis X. , 1991, AJNR. American journal of neuroradiology.