mycophenolic-acid and Granuloma--Plasma-Cell

Inflammatory pseudotumor of the temporal bone is a benign, idiopathic inflammatory process that is locally invasive and a cause of significant morbidity. This study reviews our experience with seven patients and is currently the largest series to date.. Retrospective review from January 1, 2014 to January 1, 2016.. Single tertiary medical center.. There were five male and two female (n = 7) subjects with a diagnosis of temporal bone inflammatory pseudotumor. The mean age at presentation was 41 years old. The most common presenting symptoms were hearing loss (7/7) and headache (4/7). Four patients demonstrated an inflammatory aural polyp. Two patients experienced facial nerve paralysis.. Seven patients underwent computed tomography and six underwent magnetic resonance imaging. Corticosteroids and antibiotics were the initial treatment of choice. Five patients also underwent surgery. As adjuvant therapy, two patients received Rituximab, one patient received radiation, and one received mycophenolate mofetil.. Clinical courses were followed with focus on symptoms, disease recurrence, duration, and treatment. Mean follow-up was 17.8 months.. The primary lesions demonstrated T2 hypo-intensity and enhancement as well as diffuse dural thickening on magnetic resonance imaging in five of six patients. Histopathology demonstrated chronic inflammation in the setting of hyalinized fibrosis (7/7). All the patients are currently symptomatically stable.. Inflammatory pseudotumor of the temporal bone can cause devastating effects on neurological function and quality of life. Recognition of characteristic imaging and histopathology can expedite appropriate treatment. Patients may require chronic steroid therapy. Adjunctive therapy with radiation and immuno-modulation are currently being explored.

Topics: Adolescent; Adult; Aged; Bone Diseases; Child; Female; Granuloma, Plasma Cell; Humans; Immunosuppressive Agents; Male; Middle Aged; Mycophenolic Acid; Quality of Life; Retrospective Studies; Rituximab; Temporal Bone; Tomography, X-Ray Computed; Treatment Outcome; Young Adult

Orbital apex and skull base masses often present with neuro-ophthalmic signs and symptoms. Though the localization of these syndromes and visualization of the responsible lesion on imaging is typically straightforward, definitive diagnosis usually relies on biopsy. Immunohistochemistry is important for categorization and treatment planning. IgG4-related disease is emerging as a pathologically defined inflammatory process that can occur in multiple organ systems. We present two patients with extensive inflammatory mass lesions of the central nervous system with immunohistochemistry positive for IgG4 and negative for ALK-1 as examples of meningeal based IgG4-related inflammatory pseudotumors. In both patients, there was treatment response to mycophenolate mofetil.

Topics: Adult; Central Nervous System; Female; Granuloma, Plasma Cell; Humans; Immunoglobulin G; Immunosuppressive Agents; Meningitis; Middle Aged; Mycophenolic Acid

Topics: Anti-Inflammatory Agents; Child, Preschool; Glomerulonephritis, Membranous; Granuloma, Plasma Cell; Humans; Immunosuppressive Agents; Male; Mycophenolic Acid; Prednisone; Skin Diseases