Hailiang Tang1, Guanfu Ding2, Ji Xiong3, Hongda Zhu1, Lingyang Hua1, Qing Xie1, Ye Gong4. 1. Department of Neurosurgery, Huashan Hospital, Fudan University, Shanghai, China. 2. Department of Neurosurgery, 1st Hospital Affiliated to Gannan Medical College, Jiangxi Province, China. 3. Department of Pathology, Huashan Hospital, Fudan University, Shanghai, China. 4. Department of Neurosurgery, Huashan Hospital, Fudan University, Shanghai, China. Electronic address: drgongye@163.com.
Abstract
BACKGROUND: Immunoglobulin G4 (IgG4)-related disease is a more recently recognized syndrome. It is characterized by increasing IgG4-positive plasma cells and lymphocyte infiltration. Reports of intracranial pseudotumors associated with IgG4-related disease are very rare. We report a rare case of clivus pseudotumor associated with IgG4-related disease mimicking meningioma. CASE DESCRIPTION: A 50-year-old man presented with abducens paralysis of the right eye. Brain magnetic resonance imaging revealed a uniformly enhanced mass located in the right upper clivus area, and meningioma was highly suspected. The tumor was totally resected via a retrosigmoid approach craniotomy, which achieved sufficient decompression of the right abducens nerve. Postoperative pathology revealed IgG4-related disease with IgG4 strongly positive on immunohistochemical staining. The patient had no history of autoimmune disease. Postoperative laboratory data revealed no elevation of either IgG (6.94 g/L) or IgG4 (0.131 g/L). Follow-up contrast MRI showed the lesion had disappeared. CONCLUSIONS: Although several cases of intrasellar hypophysitis have been reported, reports of intracranial pseudotumors associated with IgG4-related disease are rare. Special attention is required when the differential diagnosis includes meningioma. Preoperative diagnosis is very important because inflammatory pseudotumors associated with IgG4-related disease could be treated with steroid therapy, avoiding surgery.
BACKGROUND: Immunoglobulin G4 (IgG4)-related disease is a more recently recognized syndrome. It is characterized by increasing IgG4-positive plasma cells and lymphocyte infiltration. Reports of intracranial pseudotumors associated with IgG4-related disease are very rare. We report a rare case of clivus pseudotumor associated with IgG4-related disease mimicking meningioma. CASE DESCRIPTION: A 50-year-old man presented with abducens paralysis of the right eye. Brain magnetic resonance imaging revealed a uniformly enhanced mass located in the right upper clivus area, and meningioma was highly suspected. The tumor was totally resected via a retrosigmoid approach craniotomy, which achieved sufficient decompression of the right abducens nerve. Postoperative pathology revealed IgG4-related disease with IgG4 strongly positive on immunohistochemical staining. The patient had no history of autoimmune disease. Postoperative laboratory data revealed no elevation of either IgG (6.94 g/L) or IgG4 (0.131 g/L). Follow-up contrast MRI showed the lesion had disappeared. CONCLUSIONS: Although several cases of intrasellar hypophysitis have been reported, reports of intracranial pseudotumors associated with IgG4-related disease are rare. Special attention is required when the differential diagnosis includes meningioma. Preoperative diagnosis is very important because inflammatory pseudotumors associated with IgG4-related disease could be treated with steroid therapy, avoiding surgery.