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LEFT TEMPORAL BONE LANGERHANS CELL HISTIOCYTOSIS
Authored By: Keith Fischer and Chad Wright.
Patient: 8 year old male
History:

8-year-old boy with no significant past medical history who presents after being struck in the head by a dodge ball approximately 2 months ago.

 

On physical exam, there is a 2 x 2 cm area of swelling around the left mastoid in the post-auricular region with no active drainage from the left ear.

 

The patient has no difficulty with hearing, no fever or other signs of infection.

Image Size:[small][as-submitted]

Fig. 1
AP Skull Radiograph

Fig. 2
MRI of the Brain

Fig. 3
Tc-99m MDP Bone Scintigraphy - Whole Body

Fig. 4
Tc-99m MDP Bone Scintigraphy - Spot Views
Image Size:[small][as-submitted]

Findings:

Skull Radiograph:

There is a destructive lesion in the left mastoid region.

 MRI of the Brain:

There is a multilobulated lesion in the left mastoid with avid enhancement and significant bony destruction. 

 

Whole Body Bone Scintigraphy: 

There is increased tracer uptake in the left mastoid which corresponds to the lesion on MRI.

DDx:

Differential considerations include:

 Rhabdomyosarcoma

Langerhans' cell histiocytosis

Metastasis

Lymphoma

Diagnosis: An excisional biopsy of a postauricular lymph node as well as a left temporal bone biopsy revealed Langerhans' cell histiocytosis. 
General Discussion:

Langerhans’ cell histiocytosis (LCH): 

In children, LCH can range from a solitary lesion to multifocal lesions.  The primary presentation may be repetitive otitis with otorrhea and otalgia or simply a temporal mass.  In 60% of cases, LCH affects the head and neck (i.e., skull base).  The temporal bone is involved in 19—25% of cases with bilateral involvement in 1/3 of all cases.  Cervical lymph nodes can also be involved in isolated organ disease or as part of a multisystem process.

References: Saliba I, Sidani K, El Fata F, Arcand P, Quintal MC, and Abela A.  Langerhans' cell histiocytosis of the temporal bone in children.  International Journal of Pediatric Otorhinolaryngology.  Volume 72.  Issue 6.  Pages 775-786.
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Additional Details:

Case Number: 372671Owner(s): Keith Fischer and Chad WrightLast Updated: 02-07-2013
Anatomy: Cranium and Contents   Pathology: Hematological
Modality: Conventional Radiograph, MR, Nuc MedAccess Level: Readable by all users, writable by NucMed Certifiers
Keywords: temporal bone, langerhans cell histiocytosis

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Certified by Keith Fischer on 01-23-2013

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