Eosinophilic granuloma is characterized by lesions that arise predominantly in bones. Treatment for eosinophilic granuloma depends on several factors and usually includes a combination of surgery, chemotherapy, radiation therapy, and corticosteroids. However, for some patients, close observation alone may be the appropriate course of action.
Eosinophilic granuloma is the most common and the mildest subtype of a group of diseases known as Langerhans cell histiocytosis—disorders in which there is an overproliferation of Langerhans cells.
Usually found in the skull bones, eosinophilic granuloma can also arise in other bones, such as the vertebrae, ribs, mandibles, and bones in the limbs. This disorder can sometimes arise in the lungs, skin and stomach too.
Eosinophilic granuloma most frequently appears as a single lesion, but multiple lesions are also possible.
Common symptoms include pain, swelling and tenderness around the affected bone; headache; and fever.
In most instances, a neurological examination is performed to evaluate symptoms. This exam may include assessments of sensation, motor function, hearing, sense of smell, ability to swallow, eye movements, balance and coordination.
Diagnosis relies largely on imaging studies. X-rays, bone scans, computed tomography (CT) scans or magnetic resonance imaging (MRI) scans may be used to see the lesion. CT and MRI scans can be performed with or without contrast enhancement.
Other diagnostic tests may be conducted, including a water deprivation test to identify diabetes insipidus and blood tests to obtain a complete blood count, assess liver function or measure hormone levels.
Biopsy of the lesion is generally needed to confirm a diagnosis. Also, the tissue sample acquired during the biopsy is often used to identify a BRAF mutation and in turn guide treatment decisions.
The cause of eosinophilic granuloma is unknown.
Eosinophilic granuloma is not inherited but rather arises in some cases as a result of spontaneous mutations that have been found in the MAP Kinase pathway. The mutated genes include BRAF and MAPK2, and the mutations lead to the accidental triggering of the gene ERK.
Children of parents who were exposed to benzene have an increased risk for developing eosinophilic granuloma.
This disorder can occur at any age but is most common among children over 5 and young adults. It is more common among males than females.
At Columbia, our neurosurgeons use the latest surgical techniques to treat eosinophilic granuloma that arises in the skull or spine and offer the best possible results.
Some patients may not need treatment. Instead, close monitoring and observation with imaging tests may be recommended. Sometimes a lesion may resolve on its own, or the action of inserting a biopsy needle into the lesion can prompt self-healing.
For individuals who need surgery, the standard treatment for eosinophilic granuloma is usually excision of the skull bone lesion from the surface of the bone. Though all surgery carries some risk, this procedure is relatively straightforward.
Treatments that may be used instead of surgery, or sometimes in addition to surgery, include chemotherapy, radiation therapy, corticosteroids, or a combination. Our neurosurgeons customize a treatment plan that is best for each patient.