Glioblastoma multiforme disguised as sarcoidosis: pitfalls in diagnosis.
To the Editor: Sarcoidosis is a multisystem granulomatous disease that can involve any part of the nervous system. About 5 to 15% of patients with sarcoidosis will have clinical involvement of the nervous system. Neurologic features of sarcoidosis are nonspecific and include cranial neuropathy, aseptic meningitis, increased intracranial pressure increased intracranial pressure Intracranial hypertension, see there , hydrocephalus, mass lesions, white matter disease, and peripheral neuropathy. Cerebrospinal fluid (CSF) abnormalities are nonspecific. We describe a patient with active pulmonary sarcoidosis that developed neurologic symptoms. Although neurosarcoidosis was felt to be the diagnosis, clinical and cerebral imaging showed progression of disease on prednisone and methotrexate therapy. A brain biopsy showed glioblastoma multiform multiform /mul·ti·form/ (mul´ti-form) polymorphic.
Occurring in or having many forms or shapes; polymorphic. .
A 45-year-old man presented with dyspnea on exertion dyspnea on exertion Cardiology Shortness of breath which occurs with effort, often a sign of heart failure or ischemia and dry cough for 2 months. A chest x-ray showed bilateral upper lobe infiltrates. A pulmonary function testing was done that showed restrictive physiology with moderately reduced diffusion capacity. A computed tomography scan Computed tomography scan (CT scan)
A specialized type of x-ray imaging that uses highly focused and relatively low energy radiation to produce detailed two-dimensional images of soft tissue structures, particularly the brain. of the chest also showed bilateral pulmonary infiltrates. There was no hilar hi·lar
Of or relating to a hilum. or mediastinal lymph node enlargement. Bronchoscopy with transbronchial lung biopsy transbronchial lung biopsy A biopsy from the lung by an endoscopically-guided forceps, used to diagnose benign–eg, interstitial fibrosis, sarcoidosis and malignant–eg, cancer, lymphoma–lesions. See Transbronchial needle aspiration biopsy. was done. Bronchoalveolar lavage cultures were negative. Biopsy showed a granulomatous pneumonitis consistent with sarcoidosis. The patient was a nonsmoker. He had no history of exposure to chemicals. The patient's past medical history was unremarkable. Physical examination on presentation was normal. Prednisone 40 mg daily was started. One month after presentation, the patient's respiratory symptoms improved. Prednisone was tapered to 20 mg daily. Six months after presentation, the patient developed generalized seizure. Cerebral magnetic resonance imaging magnetic resonance imaging (MRI), noninvasive diagnostic technique that uses nuclear magnetic resonance to produce cross-sectional images of organs and other internal body structures. (MRI) showed diffuse meningeal me·nin·ge·al
Of, relating to, or affecting the meninges.
pertaining to the meninges.
meningeal hemorrhage enhancement and periventricular white matter lesions. Cerebral MRI also showed small 1.5 cm lesion in suprasellar region. CSF analysis was normal. Neurosarcoidosis was suspected. Treatment with prednisone 100 mg daily and methotrexate 5 mg weekly were initiated. Methotrexate dose was gradually increased to 12.5 mg weekly. Seven months after presentation, the patient developed diplopia diplopia /di·plo·pia/ (di-plo´pe-ah) the perception of two images of a single object.
binocular diplopia , left hemiparesis, left 3rd cranial nerve palsy, left facial nerve palsy facial nerve palsy Facial palsy, see there , and memory loss. Repeat cerebral MRI again showed diffuse meningeal enhancement and periventricular white matter lesions. The suprasellar mass showed marked enlargement (Fig.). There was also a new left temporal cortical abnormality. Treatment with methylprednisolone 1000 mg daily for 5 days for presumed refractory neurosarcoidosis did not result in any improvement. Biopsy of new temporal lesions showed glioblastoma multiform.
The clinical, radiographic, and CSF manifestations of neurosarcoidosis are nonspecific and overlap with other central nervous system pathologies. Diagnosis of neurosarcoidosis is usually considered in patients with pulmonary or extrapulmonary sarcoidosis and neurologic findings compatible with neurosarcoidosis. The pathology of meningeal and brain involvement in neurosarcoidosis shows characteristic non-necrotizing granulomas. Although brain biopsy is not routinely recommended, in selected cases it is extremely helpful to include or exclude neurosarcoidosis. The presence of intracranial mass or progression of lesions on therapy should alert the clinicians to an alternative diagnosis.
Bobbak Vahid, MD
Sandra Weibel, MD
Department of Pulmonary and Critical
Thomas Jefferson University Hospital