IgG4-related inflammatory pseudotumor of the brain parenchyma: a case report and literature review.

A 29-year-old male with a 4-month history of binocular temporal visual field defect, 1-month history of memory loss and 5-day history of headache was admitted to our hospital. Brain MRI showed progressive signal abnormalities in the brain parenchyma. His laboratory tests showed elevated serum IgG4 and liver dysfunction. Abdominal CT, ultrasound and liver biopsy showed chronic liver disease. According to the diagnostic criteria of IgG4-related disease, IgG4-related inflammatory pseudotumor of brain parenchyma was considered. After methylprednisolone treatment, his symptoms improved and brain lesion reduced. This is the second reported case of a tumefactive lesion of the brain with serum IgG4 elevation, which was responsive to steroid treatment. Coincidentally, his mother had similar medical history and imaging findings, and was subspecialty diagnosed with the same disease, but without pathological and immunohistochemical confirmation. This suggests that the disease may be hereditary. It is important to recognize IgG4-related inflammatory pseudotumors of the brain parenchyma so that patients do not undergo unnecessary surgical or other procedures.

Ultrasound findings in asymptomatic patients with modular metal on metal total hip arthroplasty.

OBJECTIVE: The use of metal-on-metal and modular total hip arthroplasty is associated with potentially serious local and systemic complications. The primary aim of this study was to identify the prevalence of a pseudotumor in asymptomatic patients with a particular metal-on-metal hip prosthesis after a minimum follow-up of 5 years using ultrasound evaluation. A secondary purpose was to identify associations between the presence of pseudotumor and serum metal ion levels following implantation. METHODS: We prospectively evaluated data collected from 36 asymptomatic patients who underwent implantation of a Profemur Z metal-on-metal total hip arthroplasty from January 2004 to January 2010. Serum metal ion levels were collected in 2012 and 2015. Hip ultrasounds were performed in 2015. RESULTS: Pseudotumors were found in 7/36 patients (19.4%). The average pseudotumor size measured 38.2 cm3 (range 7.35 cm3-130.81 cm3). Elevated metal ion levels were found in all patients at all time points. No statistical correlation was found between the presence of pseudotumor and patient age, age of the implant, component design, and any of the serum metal ion levels or ratios. CONCLUSIONS: One in every five asymptomatic patients with metal-on-metal implants was found to have a periarticular pseudotumor. There was no dose-dependent relationship found between elevated serum metal ion levels and the development of a pseudotumor. Our findings suggest that in patients with known elevated metal ion levels, continued monitoring of ion levels may not be a reliable predictor of pseudotumor formation, and ultrasound surveillance can and should be routinely used to document the presence and progression of pseudotumor.

Sensitivity and Specificity of Metal Ion Levels in Predicting "Pseudotumors" due to Taper Corrosion in Patients With Dual Taper Modular Total Hip Arthroplasty.

BACKGROUND: Currently, no serum metal ion threshold exists to identify adverse tissue reactions in total hip arthroplasty (THA) patients with taper corrosion. Our study aims to investigate the sensitivity and specificity of serum metal ions in detecting taper corrosion related pseudotumors in patients with dual taper modular THA. METHODS: A total of 148 patients with dual taper modular THA were investigated: (1) 90 patients with pseudotumors detected with metal artifact reduction sequence-magnetic resonance imaging (MARS-MRI) and (2) 58 patients without pseudotumors on MARS-MRI. Receiver operating characteristic curves were constructed to determine the sensitivity and specificity using different metal ion thresholds. The severity of intraoperative tissue damage was correlated with preoperative metal ion levels. RESULTS: Pseudotumor was associated with higher cobalt (5.0 µg/L vs 3.7 µg/L, P < .01) and Co/Cr ratio (6.0 vs 3.7, P < .01). The sensitivity and specificity for cobalt level of 2.8 µg/L and Co/Cr ratio of 3.8 in detecting taper corrosion-related pseudotumors on MARS-MRI was 88% and 32% and 70% and 50%, respectively. Higher intraoperative tissue damage grades demonstrated significantly higher Co/Cr ratios (8.6 vs 3.4, P = .03). CONCLUSION: Although metal ion levels alone should not be relied on as the sole parameter to determine revision surgery, cobalt level >2.8 µg/L and the Co/Cr ratio >3.8 are useful clinical diagnostic adjuncts in the systematic clinical evaluation for taper corrosion-related adverse tissue reactions in patients with dual modular taper THA.

Does a titanium sleeve reduce the frequency of pseudotumors in metal-on-metal total hip arthroplasty at 5-7years follow-up?

INTRODUCTION: Little is known about pseudotumor frequency and risk factors for pseudotumor formation among different types of metal-on-metal (MoM) hip arthroplasties. A lower release of chromium and cobalt have been reported in MoM hip arthroplasties with a titanium sleeve compared to MoM designs without a titanium sleeve, but yet it is unknown whether a titanium sleeve reduces the pseudotumor frequency. We conducted a cross-sectional study to investigate: 1) pseudotumor frequency, 2) risk factors of pseudotumor formation 3) and correlations between pseudotumors, serum metal-ions, implant position, and clinical symptoms. HYPOTHESIS: We expected a lower pseudotumor frequency in MoM hip articulation with a titanium sleeve than reported in MoM hip articulation designs using chromium-cobalt sleeve. MATERIALS AND METHOD: A consecutive series of 41 patients/49 hips (31 males), mean age 52 (28-68) years, participated in a 5.5±0.5 (4-6.5) year follow-up study of their M2a_Magnum hip articulation (Biomet Inc., Warsaw, Indiana, USA). Patients were evaluated with magnetic resonance imaging (MRI), measurements of serum metal-ions, plain radiographs, and clinical outcome measures of Harris Hip Score (HHS) and Oxford Hip Score (OHS). RESULTS: Eighteen of 47 hips (38%) had MRI-verified pseudotumors, all cystic, with a mean dimension of 10.6×25.6×41mm. Digital measurements on plain radiographs revealed a higher cup anteversion in patients with a pseudotumor of mean 28.4°±5.05° compared to mean 23.5°±6.5° in patients without a pseudotumor (P=0.009). Serum metal-ion concentrations, acetabular cup inclination and measures of HHS and OHS were similar between patients with and without a pseudotumor (P>0.46). CONCLUSION: At 5.5±0.5years after surgery, MRI-verified cystic pseudotumors were frequently observed in M2a_Magnum hip articulations despite the use of titanium sleeves. The pseudotumors were related to high cup anteversion angles but not related to high serum metal-ions or clinical symptoms. LEVEL OF EVIDENCE: IV: cross-sectional study.

No association between pseudotumors, high serum metal-ion levels and metal hypersensitivity in large-head metal-on-metal total hip arthroplasty at 5-7-year follow-up.

OBJECTIVE: The relationship between metal wear debris, pseudotumor formation and metal hypersensitivity is complex and not completely understood. The purpose of this study was to assess the prevalence of pseudotumor formation in a consecutive series of metal-on-metal (MoM) total hip arthroplasty (THA) and to investigate its relationship to serum metal-ion levels and hypersensitivity to metal. METHODS: Forty-one patients (31 males), mean age 52 (28-68) years, with a total of 49 large-head MoM THA participated in a 5-7-year follow-up study. Patients underwent ultrasonography, serum metal-ion concentrations were measured, metal allergy and atopic dermatitis were evaluated, and the questionnaires of the Oxford Hip Score (OHS), Harris Hip Score (HHS) and the Short-Form Health Survey (SF-36) were completed. RESULTS: Pseudotumors were found in eight patients, but they were asymptomatic and their serum metal-ion levels were similar to those observed in patients with no pseudotumors (p > 0.36). The capsule-stem distance of mean 8.6 mm (SD 3.82, 95% CI: 5.40-11.79) was wider (p = 0.02) in patients with pseudotumours than in patients without pseudotumors of mean 5.6 mm (SD 2.89, 95% CI: 4.68-6.58). Positive patch test reactions were seen in three patients. Higher serum metal-ion levels of chromium and cobalt were significantly correlated with steeper cup inclination and smaller femoral head sizes, and were associated with female gender (p < 0.04). CONCLUSION: We found no association between pseudotumor formation, serum metal-ion levels, metal patch test reactivity, and atopic dermatitis. However, clinicians should be aware of asymptomatic pseudotumors, and we advise further exploration into the mechanisms involved in the pathogenesis of pseudotumors.

Pseudotumour incidence, cobalt levels and clinical outcome after large head metal-on-metal and conventional metal-on-polyethylene total hip arthroplasty: mid-term results of a randomised controlled trial.

We compared the incidence of pseudotumours after large head metal-on-metal (MoM) total hip arthroplasty (THA) with that after conventional metal-on-polyethylene (MoP) THA and assessed the predisposing factors to pseudotumour formation. From a previous randomised controlled trial which compared large head (38 mm to 60 mm) cementless MoM THA with conventional head (28 mm) cementless MoP THA, 93 patients (96 THAs: 41 MoM (21 males, 20 females, mean age of 64 years, standard deviation (sd) 4) and 55 MoP (25 males, 30 females, mean age of 65 years, sd 5) were recruited after a mean follow-up of 50 months (36 to 64). The incidence of pseudotumours, measured using a standardised CT protocol was 22 (53.7%) after MoM THA and 12 (21.8%) after MoP THA. Women with a MoM THA were more likely to develop a pseudotumour than those with a MoP THA (15 vs 7, odds ratio (OR) = 13.4, p < 0.001). There was a similar incidence of pseudotumours in men with MoM THAs and those with MoP THAs (7 vs 5, OR = 2.1, p = 0.30). Elevated cobalt levels (≥ 5 microgram/L) were only associated with pseudotumours in women with a MoM THA. There was no difference in mean Oxford and Harris hip scores between patients with a pseudotumour and those without. Contrary to popular belief, pseudotumours occur frequently around MoP THAs. Women with a MoM THA and an elevated cobalt level are at greatest risk. In this study, pseudotumours had no effect on the functional outcome after either large head MoM or conventional MoP THA.

Do patients with a failed metal-on-metal hip implant with a pseudotumor present differences in their peripheral blood lymphocyte subpopulations?

BACKGROUND: Early adverse tissue reactions around metal-on-metal (MoM) hip replacements, especially pseudotumors, are a major concern. Because the causes and pathomechanisms of these pseudotumors remain largely unknown, clinical monitoring of patients with MoM bearings is challenging. QUESTIONS/PURPOSES: The purpose of this study was to compare the lymphocyte subpopulations in peripheral blood from patients with a failed MoM hip implant with and without a pseudotumor and patients with a well-functioning MoM hip implant without a pseudotumor. Potential differences in the systemic immune response are expected to reflect local differences in the periprosthetic tissues. METHODS: Consenting patients who underwent a revision of a failed MoM hip implant at The Ottawa Hospital (TOH) from 2011 to 2014, or presented with a well-functioning MoM hip implant for a postoperative clinical followup at TOH from 2012 to 2013, were recruited for this study, unless they met any of the exclusion criteria (including diagnosed conditions that can affect peripheral blood lymphocyte subpopulations). Patients with a failed implant were divided into two groups: those with a pseudotumor (two hip resurfacings and five total hip arthroplasties [THAs]) and those without a pseudotumor (10 hip resurfacings and two THAs). Patients with a well-functioning MoM hip implant (nine resurfacings and three THAs) at 5 or more years postimplantation and who did not have a pseudotumor as demonstrated sonographically served as the control group. Peripheral blood subpopulations of T cells (specifically T helper [Th] and cytotoxic T [Tc]), B cells, natural killer (NK) cells, memory T and B cells as well as type 1 (expressing interferon-γ) and type 2 (expressing interleukin-4) Th and Tc cells were analyzed by flow cytometry after immunostaining. Serum concentrations of cobalt and chromium were measured by inductively coupled plasma-mass spectrometry. RESULTS: The mean percentages of total memory T cells and, specifically, memory Th and memory Tc cells were lower in patients with a failed MoM hip implant with a pseudotumor than in both patients with a failed implant without a pseudotumor and patients with a well-functioning implant without a pseudotumor (memory Th cells: 29% ± 5% [means ± SD] versus 55% ± 17%, d = 1.8, 95% confidence interval [CI] [1.2, 2.5] and versus 48% ± 14%, d = 1.6, 95% CI [1.0, 2.2], respectively; memory Tc cells: 18% ± 5% versus 45% ± 14%, d = 2.3, 95% CI [1.5, 3.1] and versus 41% ± 12%, d = 2.3, 95% CI [1.5, 3.1], respectively; p < 0.001 in all cases). The mean percentage of memory B cells was also lower in patients with a failed MoM hip implant with a pseudotumor than in patients with a well-functioning implant without a pseudotumor (12% ± 8% versus 29% ± 16%, d = 1.3, 95% CI [0.7, 1.8], p = 0.025). In addition, patients with a failed MoM hip implant with a pseudotumor had overall lower percentages of type 1 Th cells than both patients with a failed implant without a pseudotumor and patients with a well-functioning implant without a pseudotumor (5.5% [4.9%-5.8%] [median with interquartile range] versus 8.7% [6.5%-10.2%], d = 1.4, 95% CI [0.8, 2.0] and versus 9.6% [6.4%-11.1%], d = 1.6, 95% CI [1.0, 2.2], respectively; p ≤ 0.010 in both cases). Finally, serum cobalt concentrations in patients with a failed MoM hip implant with a pseudotumor were overall higher than those in patients with a well-functioning implant without a pseudotumor (5.8 µg/L [2.9-17.0 µg/L] versus 0.9 µg/L [0.6-1.3 µg/L], d = 2.2, 95% CI [1.4, 2.9], p < 0.001). CONCLUSIONS: Overall, results suggest the presence of a type IV hypersensitivity reaction, with a predominance of type 1 Th cells, in patients with a failed MoM hip implant with a pseudotumor. CLINICAL RELEVANCE: The lower percentages of memory T cells (specifically Th and Tc) as well as type 1 Th cells in peripheral blood of patients with a failed MoM hip implant with a pseudotumor could potentially become diagnostic biomarkers for the detection of pseudotumors. Although implant design (hip resurfacing or THA) did not seem to affect the results, as suggested by the scatter of the data with respect to this parameter, future studies with additional patients could include the analysis of implant design in addition to correlations with histological analyses of specific Th subsets in periprosthetic tissues.

Pseudotumor formation and serum ions after large head metal-on-metal stemmed total hip replacement. Risk factors, time course and revisions in 706 hips.

INTRODUCTION: The incidence and natural course of pseudotumors in metal-on-metal total hip arthroplasties is largely unknown. The objective of this study was to identify the true incidence and risk factors of pseudotumor formation in large head metal-on-metal total hip arthroplasties. MATERIALS AND METHODS: Incidence, time course and risk factors for pseudotumor formation were analysed after large femoral head MoM-THA. We defined a pseudotumor as a (semi-)solid or cystic peri-prosthetic soft-tissue mass with a diameter ≥2 cm that could not be attributed to infection, malignancy, bursa or scar tissue. All patients treated in our clinic with MoM-THA's were contacted. CT scan, metal ions and X-rays were obtained. Symptoms were recorded. RESULTS: After median follow-up of 3 years, 706 hips were screened in 626 patients. There were 228 pseudotumors (32.3 %) in 219 patients (35.0 %). Pseudotumor formation significantly increased after prolonged follow-up. Seventy-six hips (10.8 %) were revised in 73 patients (11.7 %), independent risk factors were identified. Best cutoff point for cobalt and chromium was 4 µg/l (68 and 77 nmol/l). CONCLUSIONS: This study confirms a high incidence of pseudotumors, dramatically increasing after prolonged follow-up. Risk factors for pseudotumors are of limited importance. Pain was the strongest predictor for pseudotumor presence; cobalt chromium and swelling were considered poor predictors. Cross-sectional imaging is the main screening tool during follow-up.

Outcome of Birmingham hip resurfacing at ten years: role of routine whole blood metal ion measurements in screening for pseudotumours.

PURPOSE: Emerging concern has arisen because of recent papers reporting a high prevalence of pseudotumours (PTs), even in patients with surface arthroplasties with a good clinical track record. The aim of our study was to establish the ten year survivorship of Birmingham hip resurfacing (BHR), to investigate whole blood (WB) metal ion levels and prevalence of adverse reactions to metal debris (ARMeD) and to determine the association of blood metal ion levels and symptoms with ARMeD in patients operated on with BHR at our institution. METHODS: Between May 2001 and May 2004, 261 consecutive BHRs were implanted in 219 patients. All living, nonrevised patients underwent a systematic screening programme consisting of clinical examination, WB cobalt and chromium measurements and targeted cross-sectional imaging. RESULTS: The ten year survival for the entire cohort was 91% (89-93%), with any revision as the endpoint. Prevalence of ARMeD was 6.9% in male and 8.8% in female patients. Symptomatic patients with elevated metal ion levels evinced highest prevalence (63%) of PTs compared with asymptomatic patients with elevated metal ion levels (42%) and symptomatic patients with nonelevated metal ions (11%). CONCLUSIONS: Contradicting the current international guidelines, our results suggest that it seems beneficial to combine routine metal ion measurement with clinical assessment, even in patients with well-functioning BHRs. Further follow-up will reveal whether new PTs will develop in these patients and BHR survivorship in the longer term.

Pseudotumours and IgG4-related disease: a case report.

We report a case of a patient presenting with abdominal pain, weight loss and CT imaging showing mass lesions in the chest and abdomen associated with lymphadenopathy. He was diagnosed as having IgG4-related disease and responded well to steroid treatment.

Fast growing pseudotumour in a hairdresser after metal-on-metal hip resurfacing: a case report.

A 44-year-old female hairdresser who underwent metal-on-metal hip resurfacing (MOMHR) for hip osteoarthritis developed a benign pelvic pseudotumour. Elevated levels of chromium and cobalt ions were detected in the blood. Patch testing after pseudotumor formation, showed positive skin reactions to cobalt and nickel. Marked hypereosinophilia was noted, as well as the presence of eosinophils in the pseudotumor mass. A revision to a ceramic-on-ceramic implant was performed. Radiographs showed no implant loosening or bone resorption. We hypothesized that a steep cup positioning as well as hypersensitivity response to the metal nanoparticles and ion release may have induced pseudotumour development. Currently there is no evidence that negative patch testing reduces the probability to develop an adverse reaction to metal debris therefore we suggest to carefully investigate patient medical history regarding occupation exposure and daily contact with jewellery, beauty and cleaning products before implanting MOMHR. The main challenge is to identify a sensitive patient candidate to MOMHR never suspected to be.

High metal ion levels after use of the ASR™ device correlate with development of pseudotumors and T cell activation.

BACKGROUND: Pseudotumors and immunologic alterations are reported in patients with elevated metal ion levels after resurfacing arthroplasty of the hip. A direct association of increased cobalt and chromium concentrations with the development of pseudotumors has not been established. QUESTIONS/PURPOSES: We hypothesized that (1) patients with higher blood cobalt and chromium concentrations are more likely to have pseudotumors develop, (2) elevated cobalt and chromium concentrations correlate with increased activation of defined T cell populations, and (3) elevated metal ion levels, small implant size, cup inclination angle, and patient age are risk factors for the development of pseudotumors. METHODS: A single-surgeon cohort of 78 patients with 84 Articular Surface Replacement(®) implants was retrospectively investigated. Between 2006 and 2010, we performed 84 THAs using the Articular Surface Replacement(®) implant; this represented 2% (84/4950) of all primary hip replacements performed during that period. Of the procedures performed using this implant, we screened 77 patients (99%) at a mean of 43 months after surgery (range, 24-60 months). Seventy-one patients were investigated using ultrasound scanning, and cobalt and chromium concentrations in whole blood were determined by high-resolution inductively coupled plasma mass spectrometry. Differential analysis of lymphocyte subsets was performed by flow cytometry in 53 patients. Results of immunologic analyses were investigated separately for patients with and without pseudotumors. Pseudotumors were found in 25 hips (35%) and were more common in women than in men (p = 0.02). Multivariable regression analysis was performed to identify risk factors for the development of pseudotumors. RESULTS: Cobalt and chromium concentrations were greater in patients with pseudotumors than in those without (cobalt, median 8.3 versus median 1.0 µg/L, p < 0.001; chromium, median 5.9 versus median 1.3 µg/L, p < 0.001). The percentage of HLA-DR(+)CD4(+) T cells was greater in patients with pseudotumors than in those without (p = 0.03), and the proportion of this lymphocyte subtype was positively correlated with cobalt concentrations (r = 0.3, p = 0.02). Multivariable regression analysis indicated that increasing cobalt levels were associated with the development of pseudotumors (p < 0.001), and that patients with larger implants were less likely to have them develop (p = 0.04); age and cup inclination were not risk factors. CONCLUSIONS: We found a distinct association of elevated metal ion concentrations with the presence of pseudotumors and a correlation of increased cobalt concentrations with the proportion of activated T helper/regulator cells. Thus, the development of soft tissue masses after metal-on-metal arthroplasty could be accompanied by activation of T cells, indicating that this complication may be partly immunologically mediated. Further investigations of immunologic parameters in larger cohorts of patients with metal-on-metal arthroplasties are warranted. LEVEL OF EVIDENCE: Level III, therapeutic study. See the Instructions for Authors for a complete description of levels of evidence.

Supraglottic immunoglobulin-G4 related plasma cell granuloma: case report and literature review.

BACKGROUND: Granuloma of the upper aerodigestive tract is a rare presentation of immunoglobulin-G4 (IgG4)-related disease. Since the disease process was defined in 2003, only 2 cases affecting the laryngopharynx have been reported in the literature. METHODS AND RESULTS: A 62-year-old white man presented with persistent productive cough of brown sputum, globus symptoms, dysphagia, odynophagia, dysphonia, otalgia, and general malaise over a period of 2 months. Investigations revealed IgG4-positive plasma cell granuloma of the supraglottic region. This was successfully treated with oral corticosteroids. CONCLUSION: Because IgG4 testing is not performed routinely, management recommendations have been poorly defined. We reviewed the literature and discuss herein the clinical characteristics, pathology, diagnosis, and management. The authors theorize that IgG4-related disease involving the larynx and pharynx may be more common than suggested by the paucity of reported cases.

Treatment of pseudotumors after metal-on-metal hip resurfacing based on magnetic resonance imaging, metal ion levels and symptoms.

Peri-prosthetic pseudotumor formation can be a severe complication following Metal-on-Metal hip resurfacing arthroplasty (MoMHRA), with limited data on the optimal management of this complication. The aims of this study were (1) to evaluate the prevalence and severity of pseudotumors in a consecutive cohort of 248 MoMHRA (214 patients, mean follow-up 4.6 years, range: 1 - 8.2), and (2) to present a clinical guideline for their treatment based on severity grading with Metal Artefact Reduction Sequence Magnetic Resonance Imaging, metal ion levels and symptoms. Pseudotumor prevalence was 36.3%: 61 mild, 25 moderate and four were graded severe. Five revisions followed, all in symptomatic patients with elevated metal ion levels. Pseudotumor severity grading allowed us to be conservative with revision surgery for mild and moderate MoM disease.

Asymptomatic pseudotumours after metal-on-metal hip resurfacing show little change within one year.

The aim of this study was to establish the natural course of unrevised asymptomatic pseudotumours after metal-on-metal (MoM) hip resurfacing during a six- to 12-month follow-up period. We used repeated metal artefact reduction sequence (MARS)-magnetic resonance imaging (MRI), serum metal ion analysis and clinical examination to study 14 unrevised hips (mean patient age 52.7 years, 46 to 68, 5 female, 7 male) with a pseudotumour and 23 hips (mean patient age 52.8 years, 38 to 69, 7 female, 16 male) without a pseudotumour. The mean post-operative time to the first MARS-MRI scan was 4.3 years (2.2 to 8.3), and mean time between the first and second MARS-MRI scan was eight months (6 to 12). At the second MRI scan, the grade of severity of the pseudotumour had not changed in 35 hips. One new pseudotumour (Anderson C2 score, moderate) was observed, and one pseudotumour was downgraded from C2 (moderate) to C1 (mild). In general, the characteristics of the pseudotumours hardly changed. Repeated MARS-MRI scans within one year in patients with asymptomatic pseudotumours after MoM hip resurfacing showed little or no variation. In 23 patients without pseudotumour, one new asymptomatic pseudotumour was detected. This is the first longitudinal study on the natural history of pseudotumours using MARS-MRI scans in hip resurfacing, and mirrors recent results for 28 mm diameter MoM total hip replacement.

Hepatic inflammatory pseudotumor with elevated serum CA19-9 level mimicking liver metastasis from rectal cancer: report of a case.

Inflammatory pseudotumor (IPT) of the liver is rare, and differential diagnosis from malignant tumors is difficult. We herein report a case of IPT of the liver with marked elevation of carbohydrate antigen 19-9 (CA19-9). A 72-year-old woman, who underwent low anterior resection for rectal cancer, had low-grade fever and epigastric pain. The patient has well-controlled idiopathic thrombocytopenic purpura (ITP) with steroid maintenance treatment. Abnormal laboratory findings included serum carcinoembryonic antigen of 15.9 ng/mL and CA19-9 up to 14,632 U/mL. Computed tomography revealed a low-density lesion, and magnetic resonance imaging demonstrated a high intensity lesion on T1, T2-weighted, and diffusion-weighted images (55 × 22 mm in the Couinaud's segment 4 of the liver). However, 1 month after the appearance of the symptom, serum level of CA19-9 suddenly decreased to 37 U/mL. The tumor decreased spontaneously to 20 × 10 mm. Seven months later, the tumor had reduced to 18 × 11 mm. With a diagnosis of IPT, the patient remains well without regrowth of the tumor at 1 year follow-up after CA19-9 decrease to normal limits.

IgG4-related inflammatory pseudotumors mimicking multiple meningiomas.

IgG4-related disease is an emerging clinicopathologic entity. Hypophysitis, diffuse thickening of dura, and enlargement of the trigeminal nerve are well-known intracranial involvements of IgG4-related disease. This report of a case of systemic IgG4-related disease is the first to present neuroimaging of apparent supratentorial meningioma-like lesions and thickening and contrast enhancement of the walls of the intracranial internal carotid arteries. It is important to recognize IgG4-related intracranial pseudotumors so that patients do not undergo unnecessary surgical procedures.