Annals of Indian Academy of Neurology
CASE REPORT
Year
: 2014  |  Volume : 17  |  Issue : 4  |  Page : 429--432

Langerhans cell histiocytosis of atlantoaxial joint in a middle-aged man presenting with deafness as first symptom and soft-tissue mass at neck showing excellent response to radiotherapy alone: Report of an extremely rare and unusual clinical condition and review of literature


Dodul Mondal1, PK Julka1, Manisha Jana2, Ritika Walia3, Tamojit Chaudhuri4 
1 Department of Radiation Oncology, DRBRAIRCH, All India Institute of Medical Sciences, New Delhi, India
2 Department of Radiodiagnosis, DRBRAIRCH, All India Institute of Medical Sciences, New Delhi, India
3 Department of Pathology, DRBRAIRCH, All India Institute of Medical Sciences, New Delhi, India
4 Department of Radiation Oncology, SGPGIMS, Lucknow, India

Correspondence Address:
Dodul Mondal
Department of Radiation Oncology, DRBRAIRCH, All India Institute of Medical Sciences, Ansari Nagar, New Delhi - 110 029
India

Langerhans cell histiocytosis (LCH) is a disorder of clonal proliferation of dendritic cell mainly occurring in children. Spine involvement is rare. This usually presents with pain and torticollis when neck is involved. Histopathology with immunohistochemistry is confirmatory. Local curative therapy with excision or curettage is used for localized disease. Radiotherapy is usually reserved for selected cases. Systemic chemotherapy is the treatment of choice for widespread systemic disease. In this article, we present an unusual presentation of atlantoaxial LCH with mastoid involvement resulting in hearing loss as the first symptom and quadruparesis in a middle aged male patient, which was also associated with soft-tissue mass at the nape of the neck and deafness. The patient was treated with radical radiotherapy, which provided excellent response to the disease. Involvement of atlantoaxial joint and temporal bone associated with soft-tissue mass neck and deafness in a middle-aged man is an extremely rare clinical situation.


How to cite this article:
Mondal D, Julka P K, Jana M, Walia R, Chaudhuri T. Langerhans cell histiocytosis of atlantoaxial joint in a middle-aged man presenting with deafness as first symptom and soft-tissue mass at neck showing excellent response to radiotherapy alone: Report of an extremely rare and unusual clinical condition and review of literature.Ann Indian Acad Neurol 2014;17:429-432


How to cite this URL:
Mondal D, Julka P K, Jana M, Walia R, Chaudhuri T. Langerhans cell histiocytosis of atlantoaxial joint in a middle-aged man presenting with deafness as first symptom and soft-tissue mass at neck showing excellent response to radiotherapy alone: Report of an extremely rare and unusual clinical condition and review of literature. Ann Indian Acad Neurol [serial online] 2014 [cited 2020 Aug 15 ];17:429-432
Available from: http://www.annalsofian.org/article.asp?issn=0972-2327;year=2014;volume=17;issue=4;spage=429;epage=432;aulast=Mondal;type=0