Cerebral Syphilitic Gumma Mimicking a Brain Tumor in the Relapse of Secondary Syphilis in a Human Immunodeficiency Virus-Negative Patient
A 59-year-old woman was admitted to the emergency department with a 20-day history of speech disturbance. She had a medical history of diabetes mellitus and hypertension, controlled well with medication at a local clinic.\nVital signs at admission were stable. She was alert but had cognitive disturbance (Glasgow Coma Scale 15 and Mini-Mental Status Examination 16/30) and dysarthria. Both pupils showed normal light reflex. There were no abnormal neurological findings for muscle strength and deep tendon reflexes in the extremities.\nComputed tomography (CT) scanning of the brain revealed a mass-like lesion at the left frontal lobe and severe cerebral swelling. Brain magnetic resonance imaging (MRI) revealed an irregular enhancing mass with central necrosis that measured 2.8×2.7×2.6 cm in size (). There was severe cerebral edema around the enhancing mass (). The mass was adjacent to the enhanced dura in the left frontal lobe ().\nMalignant brain tumors including glioblastoma, metastatic brain tumor or primary CNS lymphoma were suspected with the need to rule out inflammatory conditions. To confirm the diagnosis, the mass was surgically resected with frontal craniotomy. On intraoperative findings, the mass was severely adhered to the dura. It was yellow in color and had a hard consistency (). En bloc resection was performed and the intra-axial mass was completely removed. During the first admission, cerebrospinal fluid (CSF) examination was not performed, because lumbar puncture is contraindicated in the presence of increased intracranial pressure. On histopathological examination, the mass was first diagnosed as chronic inflammation originating from an unknown cause. Without any postoperative complications, she fully recovered from the cognitive defects and speech disturbance. She was discharged from the hospital 14 days after operation.\nHowever, she was readmitted for high fever to the Department of Infectious Diseases 2 months after discharge. She had experienced non-itchy erythematous papules and macules over her whole body for three days (). She had a history of sex with a partner who had multiple sex partners two years prior. She had an episode of primary syphilis at that time which was spontaneously resolved without any treatment. On admission, her initial vital signs were a body temperature of 39.5℃, blood pressure of 100/70 mm Hg, pulse rate of 91 beats/minute and respiratory rate of 22 times/minute. Physical examination revealed no generalized lymphadenopathy. On follow-up brain MRI, there was no enhancing mass lesion or severe edema at the cerebral parenchyma (). Chest CT scans revealed an infiltration with interstitial pattern on the right lower lobe of the lung without respiratory symptoms (). Results of a complete set of blood tests were as follows : white blood cell (WBC) count, 8200/µL, erythrocyte sedimentation rate, 42/mm3, and C-reactive protein, 187 mg/dL. Repeated blood and sputum cultures were negative. Serum mycoplasma antibody testing, including a follow-up test, were also negative. At that time, her serum Venereal Disease Research Laboratory (VDRL) titer was 1 : 16, and fluorescent treponemal antibody absorbed (FTA-ABS) test IgM and IgG were reactive. Histopathological examination of the skin tissue revealed heavy lymphoplasmacytic infiltration consistent with lymphomatoid papulosis. Analysis of the cerebrospinal fluid yielded the following findings : 0 white cells/dL, 1 erythrocyte/mm2, glucose level of 74 mg/dL, protein level of 16.8 mg/dL, negative VDRL test, and negative T. pallidum polymerase chain reaction (PCR) test. However, FTA-ABS IgG in the CSF was reactive. Cardiovascular involvement was not noted with chest CT scans and transthoracic echocardiography. Ophthalmic fundoscopic examination also revealed no abnormal findings. Histopathological reassessment of the previous brain tissue revealed intraluminal obliteration of a large blood vessel just below the meninges, suggesting endarteritis (). Spirochetes were not identified in this tissue by Warthin-Starry stain. Necrotic tissue in the neighboring brain parenchyma was also seen (). Around the occluded vessel, parenchymal infiltration of lymphocytes and plasma cells was found (). Other immunohistochemical examinations were negative for the possibility of malignant lymphoma, glioma and inflammatory pseudotumor.\nThe patient was finally diagnosed with cerebral gumma accompanied by probable pulmonary involvement in tertiary syphilis concomitant with relapse of secondary syphilis, on the evidence of the histopathological findings and positive FTA-ABS IgG in the serum and the CSF. Based on this diagnosis, ceftriaxone was intravenously administrated at a daily dose of 2 g for 14 days. Thereafter, the fever and rash subsided in two days, and an infiltration with interstitial pattern on subsequent chest X-ray showed marked improvement in three days. On April 9, 2012, her follow-up VDRL titer was 1 : 4. Her symptoms associated with syphilis fully resolved without relapse and she was doing well fifteen months after hospital discharge.