Case Author(s): Zhiyun Yang, M.D., William Holloway, M.D. and Jerold, Wallis, M.D. , 03/16/05 . Rating: #D2, #Q3

Diagnosis: Langerhan's cell histiocytosis.

Brief history:

40-year-old male with thoracic spine pain.

Images:

Anterior and posterior images

View main image(bs) in a separate image viewer

View second image(bs). Spot images

View third image(mr). Axial image of thoracic MRI

View fourth image(ct). CT of the spine

Full history/Diagnosis is available below

Diagnosis: Langerhan's cell histiocytosis.

Full history:

40-year-old male with thoracic spine pain.

Radiopharmaceutical:

20.85 mCi Tc-99m MDP i.v.

Findings:

Delayed whole-body scintigrams were obtained. There is markedly increased activity at the T4 vertebral body extending into the posterior elements.

MRI shows an enhancing mass within the T4 vertebrae which obliterates the left T4-5 neural foramen and abuts the spinal canal.

CT of thoracic spine demonstrated that expansile lytic lesion within the L4 vertebral body extending into the posterior elements.

Discussion:

Langerhan's Cell Histiocytosis (LCH, also known as Histiocytosis X) is a rare disease. It is not really a cancer, though it may behave like one in some respects. Histiocytes are normal cells found throughout the body, in this disease abnormally large numbers are found. LCH is a clonal, myeloproliferative disorder of the Langerhans cell with variable clinical behavior. LCH is more common in children but it is often seen in adults too. The disease will be classed as either single-system or multi-system depending on how many of the body's systems are effected.

In a study of 314 people treated for LCH at the Mayo Clinic (Howarth et al, Cancer 1999;85:2278-90) 69% had single-system disease and 31% had multi-system LCH. Approximately half of those treated at Mayo were under 25 years old at diagnosis. Of people with single-system disease the system involved was bone (52%),pulmonary (lung) (40%), skin/mucous membrane (7%) and other sites (1%). Bone involvement was more common in younger patients while pulmonary involvement was mostly seen in those over 15 years old at diagnosis.

Localized pulmonary Langerhans'cell histiocytosis (also sometimes referred to as pulmonary eosinophoic granuloma) is a rare pulmonary disease that occurs predominantly in young adults.

The cause of LCH is unknown; most cases occur spontaneously.

The symptoms of LCH will depend upon which part of the body it is affecting and if the disease is affecting more than one part of the body.

Single-system LCH usually disappears on its own without any treatment. This may occur following a biopsy. In a small number of children, treatment will be needed and low-dose radiotherapy, surgery and steroids may be used. Multi-system disease is usually treated with chemotherapy and steroids.

80% of children who develop LCH will recover from it. The older the child, the higher the chance of recovery. Sometimes the disease can recur and therefore long-term followup is advised

References: Web Sites.

Followup:

Spine, T4, laminectomy with biopsy: -- Langerhan's cell histiocytosis (histiocytosis X)

Differential Diagnosis List

Compression fracture, metastases, osteoarthritc degeneration, osteomyelitis, osteoporotic collapse, bone island, hemangioma, osteoid osteoma, paget's disease, malignant melanoma, tuberculous spondylitis.

ACR Codes and Keywords:

References and General Discussion of Bone Scintigraphy (Anatomic field:Spine and Contents, Category:Neoplasm, Neoplastic-like condition)

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Case number: bs149

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