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LETTER TO EDITOR
Year : 2012  |  Volume : 60  |  Issue : 3  |  Page : 346-348

Langerhans cell histiocytosis of the clivus: An unusual cause of a destructive central skull base mass in a child


1 Department of Radiodiagnosis, Christian Medical College, Vellore, Tamil Nadu, India
2 Division of Neuropathology, Department of Pathology and Neurological Sciences, Christian Medical College, Vellore, Tamil Nadu, India

Date of Submission20-Mar-2012
Date of Decision20-Mar-2012
Date of Acceptance31-May-2012
Date of Web Publication14-Jul-2012

Correspondence Address:
Munawwar Ahmed
Department of Radiodiagnosis, Christian Medical College, Vellore, Tamil Nadu
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/0028-3886.98538

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How to cite this article:
Ahmed M, Sureka J, Koshy CG, Chacko BR, Chacko G. Langerhans cell histiocytosis of the clivus: An unusual cause of a destructive central skull base mass in a child. Neurol India 2012;60:346-8

How to cite this URL:
Ahmed M, Sureka J, Koshy CG, Chacko BR, Chacko G. Langerhans cell histiocytosis of the clivus: An unusual cause of a destructive central skull base mass in a child. Neurol India [serial online] 2012 [cited 2021 Oct 19];60:346-8. Available from: https://www.neurologyindia.com/text.asp?2012/60/3/346/98538


Sir,

We report a rare case of Langerhans cell histiocytosis (LCH) of the clivus, a rare cause of destructive central skull base.

A 6-year-old girl presented with a 1½ month history of hoarseness of voice and deviation of the angle of mouth to the left side, and slurring of speech, difficulty in swallowing, and decreased hearing for 20 days. Examination revealed right 6 th, 7 th, 8 th, 9 th, 10 th, and 12 th cranial nerve palsy. Magnetic resonance imaging (MRI) revealed a large homogeneously enhancing mass involving the clivus with extension into the prepontine cistern, petrous apex, sphenoid sinus, and the right nasopharynx [Figure 1]a-e. Biopsy of the friable mass seen in the nasopharynx was done under endoscopic guidance. Histopathology showed a tumor composed of large cells with grooved nuclei and eosinophilic cytoplasm, which were CD1a positive [Figure 1]f and g. Admixed with them were lymphocytes, plasma cells, and eosinophils. A diagnosis of LCH was made. Bone scans did not reveal any other lesion in the rest of the body. She was given chemotherapy for 12 weeks and showed a good response with reduction in the size of the mass [Figure 1]h and clinical improvement.
Figure 1: T1-weighted sagittal MR image (a) shows a hypointense mass (arrow) involving the clivus with extension into the nasopharynx and sphenoid sinus. Post-gadolinium T1W sagittal image (b) shows intense enhancement in the mass (arrow). T2-weighted axial image (c) shows hyperintense mass (arrow) extending into the right petrous and prepontine cistern. DWI (d) and ADC map image (e) show restricted diffusion in the mass (arrow). (f) Photomicrograph illustrating the histiocytes with grooved nuclei (black arrows) (H and E, ×100). (g) Immunohistochemistry for CD1a (white arrows) (avidin peroxidase, ×400). (h) Axial post-gadolinium T1W image shows reduction in the size of the lesion (arrow)

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LCH is a rare disease of neoplastic proliferation of monocyte-macrophage system. The lesions are characterized by Langerhans cells which are bone marrow derived cells of dendritic cell line with antigen presenting and processing properties. [1] Although LCH can affect almost any organ, solitary involvement of the clivus is extremely rare [2],[3] and can present a diagnostic dilemma because of its rarity at this location. LCH usually presents as solitary or multiple bony lesions, commonly in the flat bones of the skull, ribs, pelvis, mandible, and tubular bones. In the skull base, petrous ridge of the temporal bone is the most common site. Clinical presentation of clival LCH depends on the size and extent of the lesion. The commonest presentation is with 6 th nerve palsy with or without retro-orbital, post-auricular or neck pain, decreased vision, and raised intracranial pressure. [2] Multiple cranial nerve palsy is a rare presentation as seen in the present case.

MRI appearance of clival LCH includes abnormal marrow signal with homogeneously or heterogeneously enhancing destructive soft tissue mass and variable local extension. It is hyperintense on T2 and hypointense on T1-weighted images. [2] Diffusion restriction as seen in the present case is probably due to the increased cellularity of the lesion and may also correlate with the aggressive nature of the LCH. Imaging differentials of an aggressive lesion involving the clivus or central skull base in a pediatric patient would include chordoma, lymphoma, metastatic neuroblastoma, and rhabdomyosarcoma. Chordoma is uncommon in the pediatric age and is usually a heterogeneous destructive mass with areas of hemorrhages, cystic changes, and calcifications. It shows little or no enhancement on post-contrast study. Calcifications and hemorrhage are uncommon features in LCH and synchronous characteristic lytic lesions in other areas of the skeleton would favor LCH. Lymphoma of central skull base is another uncommon tumor in pediatric patients, but the most common malignancy in the head and neck region. It presents characteristically as T1-/T2-weighted isointense or hypointense mass with homogeneous enhancement and lack of calcifications and hemorrhage. [4] It is usually hyperdense on plain computed tomography (CT). [4] Furthermore, the presence of enlarged lymph nodes in the neck would favor lymphoma. Neuroblastoma can present for the first time with features related to extra-abdominal metastases and cross-sectional imaging of the abdomen may reveal the primary lesion. MRI features of metastatic neuroblastoma are nonspecific, and it presents as a homogenously or heterogeneously enhancing mass which is hypointense on T1 and hyperintense on T2-weighted MRI, with or without calcifications and cystic changes. [5] Lymphoma and neuroblastoma can be associated with multiple metastatic bone lesions however lesions are mottled or permeative unlike the characteristic punched-out or bevelled lesions of LCH. Rhabdomyosarcoma arising in the nasopharynx and invading the sphenoid and clivus can present as a mass in the central skull base. [5] MRI features of rhabdomyosarcoma are not specific [6] and may be difficult to differentiate from LCH in the absence of characteristic lytic skeletal lesions at other sites.

LCH is a rare but possible cause of a clival mass in the pediatric population. Since the imaging features are non-specific, it is often difficult to differentiate this from other similar masses. Biopsy is often required to confirm the diagnosis. It would be useful to keep the above-mentioned points in mind while approaching the diagnosis of a destructive mass involving the clivus.

 
 » References Top

1.Azouz EM, Saigal G, Rodridgez MM, Podda A. Langerhans' cell histiocytosis: Pathology, imaging and treatment of skeletal involvement. Pediatr Radiol 2005;35:103-15.  Back to cited text no. 1
    
2.Hurley ME, O'Meara A, Fogarty E, Hayes R. Langerhans' cell histiocytosis of the clivus: Case report and literature review. Pediatr Radiol 2004;34:267-70.  Back to cited text no. 2
[PUBMED]  [FULLTEXT]  
3.Krishna H, Behari S, Pal L, Chhabra AK, Banerji D, Chhabra DK, et al. Solitary Langerhans-cell histiocytosis of the clivus and sphenoid sinus with parasellar and petrous extensions: Case report and a review of literature. Surg Neurol 2004;62:447-54.  Back to cited text no. 3
[PUBMED]  [FULLTEXT]  
4.Choi HK, Cheon JE, Kim IO, Youn BJ, Jung AY, Shin SM, et al. Central skull base lymphoma in children: MR and CT features. Pediatr Radiol 2008;38:863-67.  Back to cited text no. 4
[PUBMED]  [FULLTEXT]  
5.Lui YW, Dasari SB, Young RJ. Sphenoid masses in children: Radiologic differential diagnosis with pathologic correlation. Am J Neuroradiol 2011;32:617-26.  Back to cited text no. 5
[PUBMED]  [FULLTEXT]  
6.Freling NJ, Merks JH, Saeed P, Balm AJ, Bras J, Pieters BR, et al. Imaging findings in craniofacial childhood rhabdomyosarcoma. Pediatr Radiol 2010;40:1723-38.  Back to cited text no. 6
[PUBMED]  [FULLTEXT]  


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