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Case report |
Department of Radiology, Clemens Hospital Muenster, Düesbergweg 124, D-48153 Muenster, Germany
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Abstract |
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Introduction |
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Sarcoidosis is characterized by an enhanced cellular immune processes at the sites of disease activity; in CNS involvement, a leptomeningeal inflammation accompanied by typical changes of the cerebrospinal fluid is usually found. Space-occupying sarcoid parenchymal lesions of the brain are less common, occurring in less than 1% of patients with neurosarcoidosis. Because no part of the nervous system is protected from sarcoidosis, clinical and neuroradiological manifestations vary greatly. In the acute phase of the disease CNS involvement has a favourable prognosis, while chronic courses respond poorly to medical treatment. Cerebral irradiation may be considered as a possible effective treatment modality, as shown previously by several investigators [2, 4–6].
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Case report |
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The MRI of the head showed a large single parenchymal lesion in the left temporoparietal region with clear signs of malignancy, possibly a high-grade glioma. Neurosurgical resection revealed neurosarcoidosis, as an isolated recurrence of the known pulmonary sarcoidosis. Additional long-term treatment with prednisolone was started.
Dizziness with ataxia and a progressive visual disturbance developed 5 years later. Clinical and radiological examination revealed an isolated recurrence of the previous neurosarcoidosis without any further signs of systemic sarcoidosis. The MRI scan showed a large cystic tumour mass in the left temporoparietal region of the hemisphere and in the left-sided basal ganglia. In comparison with the findings 3 years previously, it was multilocular, confluent and larger (Figure 1
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The whole brain was irradiated using a 4 MV linear accelerator with a total dose of 20 Gy in 10 fractions over 2 weeks, with a daily midplane dose of 2 Gy. The region of the whole brain and the meninges was covered by parallel opposed lateral 19 x 21 cm2 fields, identical to the recommendations for the cranial fields for prophylactic cranial irradiation in acute lymphoblastic leukaemia (caudal margin at the bottom of the second cervical vertebra and lead shielding sparing the anterior globe and lens as well as the face (so-called German Helmet fields)). Radiotherapy was well tolerated without any severe side effects. The neurological symptoms resolved only partially and the MRI scans showed no change.
8 months after radiotherapy, the patient developed a systemic recurrence of extraneurological sarcoidosis with left-sided flank pain and progressive dyspnoea on exertion. Neurological examination and MRI scans still showed no change in clinical findings relating to the neurosarcoidosis. Therefore, immunosuppressive therapy with azathioprine was given, achieving stable disease in this steroid-resistant patient.
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Discussion |
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The systemic administration of steroids is the mainstay of therapy for sarcoidosis, regardless of the organ of involvement. Therefore, treatment of neurosarcoidosis is usually initiated with corticosteroids, although this medication has less effect in patients with primary CNS disease than in patients with classic pulmonary or ocular involvement. Patients responding to this medical treatment have a good prognosis with 55% reported showing complete recovery. Unfortunately, during long-term treatment about 40% of the patients develop symptoms. Others may suffer from glucose intolerance, cataracts, and obesity, which are adverse side effects of long-time high dose steroids. The site of neurosarcoidosis relapse is often the same as the initial disease, and there has been some suggestion that patients with intracranial neurosarcoidosis, especially with mass lesion or hydrocephalus, have a more malignant disease and a higher probability of relapse [2, 3, 10].
Various reports in the literature suggest that alternative treatments including chemotherapeutic agents, like methotrexate or cyclophosphamide, immunosuppressive drugs like azathioprine or cyclosporin A, as well as whole brain radiotherapy may be useful in these situations after an initial good response to corticosteroid treatment. However, there have been no controlled trials of these alternative treatments for neurosarcoidosis and no established guidelines for the use of any additional immunosuppressive agents or radiotherapy [5, 6, 12, 13].
There are few reports in the literature showing objective responses to cranial irradiation by clinical and radiological evaluation. The dose of radiation to the whole brain varied between 12 Gy and 30 Gy, with some case reports suggesting that dose and duration may be a prognostic factor in its efficacy. The inclusion of the meninges appears to be necessary in view of the leptomeningeal involvement that is usually found. However, the use of radiation therapy in the reported neurosarcoid cases has been limited to patients in whom steroid therapy was no longer effective. The evaluation of radiotherapy response was difficult because of the broad variety of presenting symptoms, prior and concurrent medical treatment, and reported clinical outcomes. With regard to this and the temporal relationship of radiotherapy and improvement of symptoms, there may be a significant role of radiotherapy in the management of neurosarcoid in some patients [4, 6, 14].
These are a few reports indicating objective responses with the use of radiotherapy in a series of sarcoid lesions in atypical sites. In particular, radiation therapy has been used in a limited number of patients with extracerebral sarcoidosis, most often in those with uncontrolled, progressive disease after failure of medical treatment [15].
For example, Fogel et al [16] reported a case of a laryngeal sarcoidosis. The patient became refractory to corticosteroids and was treated locally with megavoltage radiotherapy as an alternative to tracheostomy. A gradual and complete clinical recovery was observed 16 months after treatment with 30 Gy. Whittaker et al [17] reported a case of sarcoidosis of the penis which initially caused ulceration around the external urethral meatus. After an immediate response to steroid therapy, the disease recurred locally in spite of continuing therapy, and involved other parts of the penis. Partial amputation of the penis was performed but further ulceration occurred at the cut ends of the corpora cavernosa in the penile stump. This ulceration healed after local radiotherapy with 30 Gy as well. Cutaneous manifestations of sarcoidosis, which frequently respond to medical management, can occasionally reappear following the discontinuation of medication, ultimately resulting in the scarring and disfigurement of the patient's skin. Such a patient can be treated successfully with radiation therapy also, with considerable improvement in the acute disease [18].
A similar experience with radiotherapy is reported for other granulomatous disorders, such as histiocytosis X (Langerhans cell histiocytosis (LCH)). LCH is a proliferative histiocytic disorder of unknown aetiology, and may involve one or multiple organ systems or tissues, such as bone, lung, skin, lymph nodes and liver [19]. The clinical course of LCH varies from generalized and fulminant to localized and curable forms which only need to be treated locally. In particular, the group of patients with a solitary bone lesion without extraosseous LCH can be treated very successfully by local radiotherapy only, or local radiation therapy after surgical excision. Usually, low doses in the range of 5 Gy to 20 Gy in 1.5–2.0 Gy fractions achieve long-term local control [20–22].
Although the evidence for the aetiology of granulomatous disorders like sarcoidosis is consistent with the concept of a disease resulting from an exaggerated cellular immune response to a limited class of antigens or auto-antigens, the fundamental pathophysiology of sarcoidosis remains unknown and therefore, definitive treatments are difficult to describe [1, 15, 19]. However, two possible mechanisms for radiation effects on granulomatous tissue can be suggested: (1) radiation induced direct cytotoxicity in the macrophages, lymphocytes, and plasma cells that make up the granulomatous lesions; and (2) radiation-induced phenotypic alterations within the cellular matrix that inhibit the autocrine and paracrine signals [14]. Whatever the mechanism, modern theories can only be extrapolated from current knowledge of the radiobiological effects on normal and neoplastic tissue. Therefore, the definitive mechanism of radiation in granulomatous disorders like sarcoidosis can only be clarified once the pathophysiology of sarcoid lesions is understood [6].
In the present case, an isolated neurosarcoidosis recurred after previous successful surgical removal and adjuvant treatment with corticosteroids. In this very unfavourable situation, the CNS involvement became stable after whole-brain irradiation with 20 Gy without changes in the enhancing brain lesions seen on MRI follow-up scans. Unfortunately, the patient's clinical symptoms improved only partially. However, we conclude that if primary medical treatment fails to induce a remission in neurosarcoidosis, radiation therapy is an appropriate treatment, with minimal adverse sequelae.
Received for publication February 24, 2003. Revision received November 24, 2003. Accepted for publication December 10, 2003.
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This article has been cited by other articles:
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  G. Tamagno, E. De Carlo, C. Martini, and G. Murialdo Neurosarcoidosis J R Soc Med, October 1, 2005; 98(10): 443 - 443. [Full Text] [PDF]
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