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									Radiological Category: Neuroradiology            Principal Modality :   CT

                                         Case Report # 775

    Submitted by:            Sireesha Yedururi , M.D.

    Faculty reviewer:        Scott Serlin, M.D., The University of Texas Medical School at
    Date accepted:           11 January, 2011
                                Case History

39y male presents with neck pain, but his most concerning symptom is severe
   muscle spasm of the neck and shoulders. No significant medical/surgical
   history is present.
                       Radiological Presentations

Lateral view of Cervical Spine            Mid sagittal reformatted CT image
                                          of Cervical Spine
              Radiological Presentations

Sagittal T2 and post contrast T1 weighted image weighted images
             Radiological Presentations

Axial post contrast T1 weighted images through C5 vertebra
                             Test Your Diagnosis

Which one of the following is your choice for the appropriate diagnosis? After
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 • Langerhans Cell Histiocytosis
 • Multiple myeloma
 • Osteomyelitis/ Tuberculosis
 • Hemangioma
 • Pathological fracture in an aneurysmal bone cyst
                          Findings and Differentials
Lateral view of the Cervical Spine and mid sagittal reformatted CT image through the
cervical spine: Marked flattening and compression of C5 vertebral body
with near complete loss of height and mild retropulsion consistent with vertebra plana.

Sagittal T2, sagittal and axial post contrast T1 weighted MRI : In addition to near
complete collapse of C5 vertebral body MR images also demonstrate small ventral
epidural soft tissue component effacing the thecal sac without cord compression.
Small prevertebral and paravertebral soft tissue components are also present.

     • Metastasis
     • Lymphoma/leukemia
     • Langerhans Cell Histiocytosis
     • Aneurysmal bone cyst with pathologic fracture
     • Osteomyelitis
     • Sarcoidosis

The patient underwent vertebrectomy and decompression at C5 with
reconstruction. Histopathological examination confirmed a final diagnosis of
Langerhans Cell Histiocytosis (LCH).

Langerhans Cell Histiocytosis is a rare disorder characterized by idiopathic
proliferation of Langerhans cells/histiocytes with either localized or multifocal
involvement. It occurs most commonly in the first two decades of life and is
extremely rare in adults. The most frequently reported anatomic sites are those in
the skull, vertebra, ribs, upper and lower jaw, and bones of extremities. In the
spine, LCH mainly involves the vertebral bodies, with a predilection for the
thoracic spine, followed by the lumbar and cervical spine. Sharply-circumscribed
osteolytic lesions are seen on radiographs and CT examinations. In the spine
usually a single vertebral body is involved, although rarely more than one vertebra
can be involved. The disc space is usually preserved and vertebral involvement is
usually confined to the body and results in anterior wedging or, more commonly,
near collapse with a characteristic “vertebra plana” appearance.

The most common classical radiological differential diagnosis for vertebra plana
are LCH and leukemia/lymphoma. However, these two conditions are much more
prevalent in children than adults. In adults, the other potential differential
considerations include metastasis, multiple myeloma and sarcoidosis. Since LCH
is very rare in adults, biopsy is required to differentiate LCH from the above
conditions. No primary neoplasm was identified and metastasis is unlikely.

The intervertebral disc is preserved and so osteomyelitis is considered unlikely.
Aneurysmal bone cyst is less likely as there are no fluid-fluid levels on T2
weighted sagittal images. Patient did not report any history of trauma. The classic
striated appearance of bone trabeculae is absent to consider hemangioma. No
other lytic areas are present to suggest multiple myeloma.
Langerhans Cell Histiocytosis of the Cervical spine
Bertram C, Madert J, Eggers C: Eosinophilic granuloma of the cervical spine. Spine
27:1408-1413, 2002

Langerhans cell histiocytosis of the cervical spine in an adult: a case report. Sayhan S,
Altinel D, Erguden C, Kizmazoglu C, Guray M, Acar U. Turk Neurosurg. 2010

From the archives of the AFIP. Langerhans Cell Histiocytosis of bone. Stull MA,
Kransdorf MJ, Denaney KO. Radiographics. 1993 (12):801-823

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