scholarly journals Spontaneous regression of infantile dural-based non-Langerhans cell histiocytosis after surgery: case report

2015 ◽  
Vol 15 (4) ◽  
pp. 372-379 ◽  
Author(s):  
Yohei Miyake ◽  
Susumu Ito ◽  
Mio Tanaka ◽  
Yukichi Tanaka
Keyword(s):  
Langerhans Cell Histiocytosis ◽  
Spontaneous Regression ◽  
Histopathological Examination ◽  
Residual Tumor ◽  
Giant Cells ◽  
Langerhans Cell ◽  
Juvenile Xanthogranuloma ◽  
Review Of The Literature ◽  
Unique Case ◽  
Postoperative Course

The authors report the case of a large left occipital mass lesion in an 8-month-old boy who presented with seizure. Neuroimaging demonstrated an approximately 5-cm extraaxial tumor, and the patient underwent partial resection. The tumor was strongly attached to the tentorium and falx. In the postoperative course the residual lesion regressed spontaneously, and after 5 years only a slight residual tumor remained along the tentorium. Histopathological examination of the tumor revealed non-Langerhans cell histiocytosis (non-LCH). However, the tumor was not diagnosed as juvenile xanthogranuloma (JXG) because it lacked Touton giant cells. Hence, the authors described this lesion as a fibroxanthogranuloma. Most intracraniospinal non-LCHs have been reported as JXG; however, several cases of xanthomatous tumors with histopathological features resembling those of JXG have been described as fibrous xanthoma, xanthoma, fibroxanthoma, and xanthogranuloma. Among JXG and the xanthomatous tumors, a review of the literature revealed several cases of dural-based tumors; these dural-based tumors have had favorable courses, including the case described in this report. In addition, the patient in the present case experienced spontaneous regression of the residual tumor. The authors report this unique case and review the literature on isolated intracraniospinal non-LCHs, especially in cases of dural-based lesion.

scholarly journals Unifocal Granuloma of Femur due to Langerhans' Cell Histiocytosis: A Case Report and Review of the Literature

2010 ◽  
Vol 2010 ◽  
pp. 1-3 ◽  
Author(s):  
Harpreet Singh ◽  
Satnam Kaur ◽  
P. Yuvarajan ◽  
Nishant Jain ◽  
Lalit Maini
Keyword(s):  
Case Report ◽  
Clinical Data ◽  
Case History ◽  
Langerhans Cell Histiocytosis ◽  
Eosinophilic Granuloma ◽  
Pediatric Population ◽  
Langerhans Cell ◽  
Long Bones ◽  
Review Of The Literature ◽  
Osteolytic Lesions

The radiological diagnosis of osteolytic lesions of the long bones in pediatric population constitutes a challenge when the case history and clinical data are uncharacteristic. We believe that the description of few clinically and histologically proven cases to verify the existence of radiological signs useful for diagnosis may be of interest. Here, we describe a case of Langerhans' cell histiocytosis (LCH) presenting as unifocal eosinophilic granuloma of femur along with a brief review of the literature.

Fulminant Multisystem Non–Langerhans Cell Histiocytic Proliferation With Hemophagocytosis

2005 ◽  
Vol 129 (2) ◽  
pp. e39-e43 ◽  
Author(s):  
R. Nagarjun Rao ◽  
Chung-che Chang ◽  
Nevin Uysal ◽  
Kenneth Presberg ◽  
Vinod B. Shidham ◽  
...  
Keyword(s):  
Langerhans Cell Histiocytosis ◽  
Clinical Course ◽  
Langerhans Cell ◽  
Variant Form ◽  
Malignant Histiocytosis ◽  
Unique Case ◽  
Multiple Organs ◽  
Biopsy Specimens ◽  
Extensive Involvement ◽  
Erdheim Chester

Abstract Hemophagocytosis (HP), a feature seen in malignant histiocytosis and infection- and lymphoma-associated disorders, has not been previously emphasized in Erdheim-Chester disease (ECD). Generally, ECD is recognized as a rare, systemic, non–Langerhans cell histiocytosis with a variable clinical course. Herein, we describe a unique case of multisystem non–Langerhans cell histiocytic proliferation with a fulminant clinical course (death occurred within 3 months of presentation) that showed prominent HP and extensive involvement of multiple organs, including the lungs, resulting in respiratory failure. Hemophagocytosis led to severe anemia that required transfusion and thrombocytopenia. Antemortem lung and bone marrow biopsy specimens revealed involvement by a histiocytic infiltrate with features highly suggestive of ECD and HP. Furthermore, the autopsy documented the presence of HP and the histiocytic infiltrate in multiple other organs. This case is best categorized as a variant form of ECD. Recognizing this variant has the following important implications: (1) HP may be a marker for fulminant clinical course in ECD, (2) the presence of HP does not exclude a diagnosis of ECD, and (3) ECD should be considered in the differential diagnosis of HP.

scholarly journals Vemurafenib in Langerhans cell histiocytosis: report of a pediatric patient and review of the literature

Oncotarget ◽  
2018 ◽  
Vol 9 (31) ◽  
pp. 22236-22240 ◽  
Author(s):  
Anne Heisig ◽  
Jan Sörensen ◽  
Stefanie-Yvonne Zimmermann ◽  
Stefan Schöning ◽  
Dirk Schwabe ◽  
...  
Keyword(s):  
Pediatric Patient ◽  
Langerhans Cell Histiocytosis ◽  
Langerhans Cell ◽  
Review Of The Literature

An ‘eruptive’ variant of juvenile xanthogranuloma associated with langerhans cell histiocytosis

2008 ◽  
Vol 35 ◽  
pp. 50-54 ◽  
Author(s):  
Don T. Tran ◽  
Gregory M. Wolgamot ◽  
John Olerud ◽  
Stan Hurst ◽  
Zsolt Argenyi
Keyword(s):  
Langerhans Cell Histiocytosis ◽  
Langerhans Cell ◽  
Juvenile Xanthogranuloma

Childhood Histiocytoses

2020 ◽  
pp. 162-168
Author(s):  
Maurizio Aricò ◽  
Cor van den Bos ◽  
Sheila Weitzman
Keyword(s):  
Targeted Therapy ◽  
Langerhans Cell Histiocytosis ◽  
Langerhans Cell ◽  
Clinical Spectrum ◽  
Juvenile Xanthogranuloma ◽  
Erk Pathway ◽  
Therapeutic Approaches ◽  
Activating Mutations ◽  
Rosai Dorfman Disease ◽  
Genetic Abnormalities

This chapter summarizes the clinical spectrum of the histiocytic disorders—Langerhans cell histiocytosis (LCH), haemophagocytic lymphohistiocytosis (HLH), and some uncommon histiocytic disorders, including juvenile xanthogranuloma (JXG) and Rosai–Dorfman disease—as well as the current diagnostic and therapeutic approaches in these diseases. Multiple activating mutations in the RAS–RAF–MEK–ERK pathway have recently been described in LCH. Their role in the pathophysiology of the disorder and in targeted therapy is reviewed. This chapter explains the differences between primary and secondary HLH, and reviews the genetic abnormalities playing a role in both forms of HLH.

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