Langerhans Cell Histiocytosis

Clinical Cases 25.02.2001
Scan Image
Section: Musculoskeletal system
Case Type: Clinical Cases
Patient: 27 years, male
Authors: T. Weits, G.J.I.M. van der Werf
icon
Details
icon
AI Report

Clinical History

Progressive low back pain. Personal medical history revealed intermittent low back pain for several years, but no trauma nor other chronic diseases. On physical examination, a slight kyphosis of the thoracolumbar spine was found. Neurological and laboratory examinations were normal.

Imaging Findings

A 27-year-old man was admitted to the orthopedic department of the hospital because of progressive low back pain. Personal medical history revealed intermittent low back pain for several years, but no trauma nor other chronic diseases. On physical examination, a slight kyphosis of the thoracolumbar spine was found. Neurological and laboratory examinations were normal.

Discussion

Langerhans cell histiocytosis or histiocytosis X is an uncommon disease with multisystemic presentation. Pathohistologically, it is characterized by a granulomatous proliferation of the reticulum cells at one or several sites in the reticuloendothelial system. The etiology is unknown. It is possibly related to prior infection. The disease may occur in the skeleton, spleen, thymus, lymph nodes and skin. There are three major manifestations of histiocytosis X: eosinophilic granuloma, Hand-Schüller-Christian disease and Letterer-Siwe disease. It is believed that these three disorders are expression of the same basic pathological process. The role of MRI in the evaluation of Langerhans cell histiocytosis is twofold: identification of the lesion and staging, i.e. definition of location and extent. On MRI the skeletal lesions are often well defined, isointense or hypointense on T1-weighted images and definitely hyperintense compared to bone marrow on fat saturated T2-weighted images. The lumbar and thoracic spine are predominantly affected. Involvement of one or several adjacent vertebrae is often seen. Intervertebral discs usually remain normal, but a paraspinal mass may be evident, simulating a soft-tissue abscess. The imaging findings of skeletal histiocytosis resemble those of osteomyelitis, Ewing’s sarcoma, lymphoma or multiple myeloma. Therefore, these diseases should be considered in the differential diagnosis.

Differential Diagnosis List

Langerhans cell histiocytosis

Final Diagnosis

Langerhans cell histiocytosis

Liscense

Figures

Conventional radiography of the lumbar spine

icon
Conventional radiography of the lumbar spine

Radiography of the chest

icon
Radiography of the chest

MRI of the lumbar spine

icon
MRI of the lumbar spine
icon
MRI of the lumbar spine

MRI of the cervico-thoracic spine

icon
MRI of the cervico-thoracic spine
icon
MRI of the cervico-thoracic spine