RESUMEN
Background: Hepatic Inflammatory Pseudotumor (IPT) is an infrequent condition often masquerading as a malignant tumor, resulting in misdiagnosis and unnecessary surgical resection. The emerging concept of IgG4-related diseases (IgG4-RD) has gained widespread recognition, encompassing entities like IgG4-related hepatic IPT. Clinically and radiologically, corticosteroids and immunosuppressive therapies have proven effective in managing this condition. Case Presentation: A 3-year-old Chinese boy presented to the clinic with an 11-month history of anemia, fever of unknown origin, and a tender hepatic mass. Blood examinations revealed chronic anemia (Hb: 6.4 g/L, MCV: 68.6 fl, MCH: 19.5 pg, reticulocytes: 1.7%) accompanied by an inflammatory reaction and an elevated serum IgG4 level (1542.2 mg/L). Abdominal contrast-enhanced computed tomography unveiled a 7.6 cm low-density mass in the right lateral lobe, while magnetic resonance imaging demonstrated slight hypointensity on T1-weighted images and slight hyperintensity on T2-weighted images, prompting suspicion of hepatic malignancy. A subsequent liver biopsy revealed a mass characterized by fibrous stroma and dense lymphoplasmacytic infiltration. Immunohistochemical analysis confirmed the presence of IgG4-positive plasma cells, leading to the diagnosis of IgG4-related hepatic IPT. Swift resolution occurred upon initiation of corticosteroid and mycophenolate mofetil therapies. Conclusion: This study underscores the diagnostic approach to hepatic IPT, utilizing histopathology, immunostaining, imaging, serology, organ involvement, and therapeutic response. Early histological examination plays a pivotal role in clinical guidance, averting misdiagnosis as a liver tumor and unnecessary surgical interventions.
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Granuloma de Células Plasmáticas , Enfermedad Relacionada con Inmunoglobulina G4 , Inmunoglobulina G , Humanos , Masculino , Granuloma de Células Plasmáticas/diagnóstico , Granuloma de Células Plasmáticas/inmunología , Granuloma de Células Plasmáticas/tratamiento farmacológico , Preescolar , Inmunoglobulina G/sangre , Inmunoglobulina G/inmunología , Enfermedad Relacionada con Inmunoglobulina G4/diagnóstico , Hepatopatías/diagnóstico , Hepatopatías/inmunología , Diagnóstico Diferencial , Hígado/patología , Hígado/diagnóstico por imagen , Hígado/inmunología , Tomografía Computarizada por Rayos X , Biopsia , Inmunosupresores/uso terapéuticoRESUMEN
IgG4-related inflammatory pseudotumors are very uncommon and are characterized histologically by the presence of inflammatory swellings with increasing IgG4-positive plasma cells and lymphocytes infiltrating the tissues. As reports of intracranial IgG4-related pseudotumors are very rare, we report a case of an IgG4-related inflammatory pseudotumor involving the clivus mimicking meningioma. A 46-year-old male presented with intermittent headache for 2 years and a sudden onset of dysphagia and dysphonia of 7 days' duration along with lower limb weakness. Enhanced magnetic resonance imaging (MRI) of the skull base revealed an isointense signal on T1- and T2-weighted images from an enhanced mass located at the middle of the upper clivus region, for which a meningioma was highly suspected. Then, an endoscopic transsphenoidal approach was adopted and the lesion was partially resected, as the subdural extra-axial lesion was found to be very tough and firm, exhibiting fibrous scarring attaching to the brain stem and basal artery. After the surgery, brain stem and posterior cranial nerve decompression was achieved, and the patient's symptoms, such as dysphagia, dysphonia and lower limb weakness, improved. Pathological findings showed many IgG4-positive plasma cells and lymphocytes surrounded by collagen-rich fibers. The patient was sent to the rheumatology department for further glucocorticoids after the diagnosis of an IgG4-related inflammatory pseudotumor was made. This case highlights the importance of considering IgG4-related inflammatory pseudotumors as a differential diagnosis in patients with lesions involving the clivus presenting with a sudden onset of symptoms of dysphagia and dysphonia along with lower limb weakness when other more threatening causes have been excluded. IgG4-related inflammatory pseudotumors are etiologically enigmatic and unpredictable, and total resection might not be warranted. Glucocorticoids are usually the first line of treatment after diagnosis.
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Fosa Craneal Posterior/patología , Granuloma de Células Plasmáticas/diagnóstico , Inmunoglobulina G/inmunología , Inflamación/patología , Neoplasias Meníngeas/diagnóstico , Meningioma/diagnóstico , Fosa Craneal Posterior/inmunología , Diagnóstico Diferencial , Granuloma de Células Plasmáticas/inmunología , Granuloma de Células Plasmáticas/cirugía , Humanos , Inflamación/inmunología , Masculino , Neoplasias Meníngeas/inmunología , Neoplasias Meníngeas/cirugía , Meningioma/inmunología , Meningioma/cirugía , Persona de Mediana Edad , PronósticoAsunto(s)
Colitis/virología , Infecciones por Citomegalovirus/virología , Hemorragia Gastrointestinal/etiología , Granuloma de Células Plasmáticas/virología , Huésped Inmunocomprometido , Infecciones Oportunistas/virología , Anciano , Colectomía , Colitis/diagnóstico , Colitis/inmunología , Colitis/cirugía , Colonoscopía , Infecciones por Citomegalovirus/diagnóstico , Infecciones por Citomegalovirus/inmunología , Infecciones por Citomegalovirus/cirugía , Resultado Fatal , Granuloma de Células Plasmáticas/diagnóstico , Granuloma de Células Plasmáticas/inmunología , Granuloma de Células Plasmáticas/cirugía , Humanos , Masculino , Infecciones Oportunistas/diagnóstico , Infecciones Oportunistas/inmunología , Infecciones Oportunistas/cirugía , Resultado del TratamientoAsunto(s)
Granuloma de Células Plasmáticas/inmunología , Enfermedad Relacionada con Inmunoglobulina G4/inmunología , Hepatopatías/inmunología , Hígado/inmunología , Células T Auxiliares Foliculares/inmunología , Biopsia , Granuloma de Células Plasmáticas/diagnóstico , Granuloma de Células Plasmáticas/cirugía , Humanos , Enfermedad Relacionada con Inmunoglobulina G4/diagnóstico , Enfermedad Relacionada con Inmunoglobulina G4/cirugía , Inmunohistoquímica , Hígado/patología , Hígado/cirugía , Hepatopatías/diagnóstico , Hepatopatías/cirugía , Masculino , Persona de Mediana Edad , Receptor de Muerte Celular Programada 1/análisisAsunto(s)
Pólipos del Colon/diagnóstico , Sarcoma de Células Dendríticas Foliculares/diagnóstico , Granuloma de Células Plasmáticas/diagnóstico , Herpesvirus Humano 4/inmunología , Inmunoglobulina G/inmunología , Colon/patología , Colon/virología , Pólipos del Colon/inmunología , Pólipos del Colon/patología , Pólipos del Colon/virología , Sarcoma de Células Dendríticas Foliculares/inmunología , Sarcoma de Células Dendríticas Foliculares/patología , Sarcoma de Células Dendríticas Foliculares/virología , Femenino , Granuloma de Células Plasmáticas/inmunología , Granuloma de Células Plasmáticas/patología , Granuloma de Células Plasmáticas/virología , Humanos , Persona de Mediana Edad , Células Plasmáticas/inmunología , Células Plasmáticas/patología , Células Plasmáticas/virologíaRESUMEN
INTRODUCTION: Inflammatory pseudotumor (IPT) in the sinonasal cavity and skull base region is benign non-neoplastic inflammatory process. However, IPT can mimic malignant tumor or infectious disease and there are difficulties in confirmation of diagnosis. The aim of study is to evaluate the clinical significance of immunoglobulin G4 (IgG4) in IPT in terms of steroid response and differential diagnosis with other skull base infiltrative lesions. METHODS: Medical records were reviewed retrospectively from 1998 to 2016. Subjects diagnosed with IPT by surgical biopsy were enrolled. IgG4 positivity was defined as IgG4/IgG ratio > 0.4. Additionally, IgG4/IgG ratio was calculated in eight skull base osteomyelitis (SBO) patients. RESULTS: Twenty-six IPT patients were included and the average age was 52.3 years, and 57.7% were male and 42.3% were female. Most lesions were involved in the sinuses (88.5%) and the incidence of extension beyond the sinuses itself was as follows: the cheek/hard palate/parapharynx (15.4%), orbit (61.5%), skull base (57.7%), and dura or brain (23.1%). All IPT cases revealed IgG4 + plasma cells and IgG4/IgG ratio over 0.4 was detected in 42.3% (11/26) of cases. In case of SBO, no patients had IgG4/IgG ratio exceed 0.4. Main treatment modality was systemic steroids (61.5%) and other modalities were used: surgery (3.8%), immunosuppressant (7.7%), radiotherapy (30.8%), or a combination of these modalities (15.4%). Steroid responses were not significantly different, but IgG4-positive group tended to have better response to steroid therapy. CONCLUSIONS: IgG4-positive and IgG4-negative IPT patients revealed no differences in involvement sites, clinical course, and steroid responses. However, IgG4/IgG ratio and IgG4 + plasma cell count can provide a diagnostic clue for infiltrative skull base lesions such as IPT and a differential diagnosis of SBO.
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Granuloma de Células Plasmáticas/inmunología , Inmunoglobulina G/sangre , Neoplasias Nasales/inmunología , Neoplasias de la Base del Cráneo/inmunología , Adulto , Anciano , Biopsia , Recuento de Células Sanguíneas , Niño , Diagnóstico Diferencial , Femenino , Granuloma de Células Plasmáticas/diagnóstico , Granuloma de Células Plasmáticas/patología , Humanos , Imagen por Resonancia Magnética , Masculino , Persona de Mediana Edad , Neoplasias Nasales/diagnóstico , Neoplasias Nasales/patología , Estudios Retrospectivos , Base del Cráneo/patología , Neoplasias de la Base del Cráneo/diagnóstico , Neoplasias de la Base del Cráneo/patologíaRESUMEN
Metal-on-metal hip resurfacing arthroplasties (MoMHRAs) have a high failure rate due to pseudotumour formation. It is not certain whether pseudotumours in bilateral MoMHRAs form on the basis of an adverse reaction to metal debris (ARMD) that is entirely due to a local innate and adaptive immune response to Cobalt-Chrome (Co-Cr) wear particles. To determine if there is a systemic component to ARMD in bilateral MoMHRAs, we examined the histology of ARMD in unilateral and bilateral MoMHRAs revised for pseudotumour and determined whether implant survival differed between these two groups. Periprosthetic tissue specimens from 119 hips revised for pseudotumour were examined. These were derived from 109 patients including 10 patients with bilateral MoMHRAs and 99 with sunilateral MoMHRAs including a cohort of 20 patients with bilateral MoMHRAs that had undergone only one MoMHRA revision for pseudotumour. The mean time to revision for pseudotumour of unilateral and bilateral MoMHRAs was determined. The histology of periprosthetic tissue was examined for evidence of the innate and adaptive immune response and scored semi-quantitatively. There was no significant difference in histological features of the innate / adaptive response between Group 1 bilateral pseudotumours and Group 2 and Group 3 unilateral pseudotumours. Histological features, including ALVAL scores, were similar in the periprosthetic tissues of right and left hips in Group 1 bilateral MoMHRAs. The mean time to first revision for pseudotumour of bilateral MoMHRAs (6.59 years) was not decreased compared with unilateral MoMHRAs (5.66 years) or bilateral MoMHRAs that had only one revision (7.05 years). Right and left hip pseudotumours in bilateral MoMHRAs exhibit similar histological features of the innate and adaptive immune response. Mean implant survival is not decreased in bilateral compared with unilateral MoMHRA cases. The findings suggest that pseudotumour formation is due more to a local than a systemic innate /adaptive immune response to components of metal wear.
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Artroplastia de Reemplazo de Cadera/efectos adversos , Análisis de Falla de Equipo , Granuloma de Células Plasmáticas/etiología , Granuloma de Células Plasmáticas/patología , Prótesis de Cadera/efectos adversos , Prótesis Articulares de Metal sobre Metal/efectos adversos , Inmunidad Adaptativa/fisiología , Adulto , Anciano , Femenino , Granuloma de Células Plasmáticas/inmunología , Humanos , Inmunidad Innata/fisiología , Masculino , Persona de Mediana Edad , Diseño de Prótesis , Falla de Prótesis , ReoperaciónRESUMEN
BACKGROUND: Once touted as the future of hip arthroplasty, metal-on-metal (MoM) bearing surfaces have fallen sharply from favor with the emergence of a strong body of evidence demonstrating unacceptably high premature implant failure rates. The previously unpredictable development of adverse local tissue reactions (ALTRs) has been a substantive contributor to this. Although the underlying pathophysiology of these so-called "pseudotumors" is now well understood, the fundamental predisposing patient risk factors have remained elusive. QUESTIONS/PURPOSES: The aim of this research, as a clinical-genotype correlation analysis, was to identify specific alleles (genes) associated with the development of ALTRs in patients with in situ MoM THAs. METHODS: A case-control study of patients who received a large-head, primary MoM THA between 2005 and 2008 was performed with a minimum followup of 5 years. Twenty-six patients who had undergone revision of a primary MoM THA secondary to symptomatic ALTRs were recruited. The mean timeframe from primary MoM THA to symptomatic revision was 5.5 years (range, 1-10 years). Twenty-eight control subjects were randomly selected asymptomatic patients with no evidence of ALTRs on protocol-specific screening. Baseline demographics and high-resolution genotype (human leukocyte antigen [HLA] Class II) were collected for all patients. Cohorts were similar with respect to age at the time of primary MoM THA (mean, 54.8 versus 54.9 years, p = 0.95) and serum cobalt (mean, 5.5 versus 8.5 µg/L, p = 0.09) and chromium concentrations (mean, 2.9 versus 4.2 µg/L, p = 0.27). The association between genotype and revision surgery secondary to ALTRs was determined with gender as a covariate. RESULTS: The prevalence of the risk genotype was 30% (16 of 54) among the entire cohort. Adjusting for sex, the odds of revision were 6.1 times greater among patients with the risk genotype present than among patients without (95% confidence interval [CI], 1.5-25.4; p = 0.01). Among females, the specificity of the risk genotype was 1.0 (95% CIexact, 0.5-1.0; pexact = 0.03), and for males, it was 0.8 (95% CIexact, 0.6-0.9; pexact < 0.01). CONCLUSIONS: The findings of this study suggest that, among patients with a primary MoM THA, allelic variation within the HLA Class II loci may be a strong, independent risk factor associated with the need for subsequent revision surgery secondary to pseudotumor formation. CLINICAL RELEVANCE: Given the hypothesis-generating nature of this novel undertaking, confirmatory prospective clinical studies are required to further elucidate this correlation and to explore the clinical utility of targeted genetic screening in this specific population. This research may, however, represent a key missing piece in the puzzle that is metal ion-induced pseudotumor formation.
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Artroplastia de Reemplazo de Cadera/efectos adversos , Artroplastia de Reemplazo de Cadera/instrumentación , Granuloma de Células Plasmáticas/genética , Articulación de la Cadera/cirugía , Prótesis de Cadera , Antígenos de Histocompatibilidad Clase II/genética , Prótesis Articulares de Metal sobre Metal , Falla de Prótesis , Anciano , Colombia Británica/epidemiología , Estudios de Casos y Controles , Femenino , Frecuencia de los Genes , Estudios de Asociación Genética , Predisposición Genética a la Enfermedad , Granuloma de Células Plasmáticas/epidemiología , Granuloma de Células Plasmáticas/inmunología , Granuloma de Células Plasmáticas/cirugía , Articulación de la Cadera/fisiopatología , Antígenos de Histocompatibilidad Clase II/inmunología , Humanos , Masculino , Persona de Mediana Edad , Prevalencia , Diseño de Prótesis , Reoperación , Medición de Riesgo , Factores de Riesgo , Factores de Tiempo , Resultado del TratamientoRESUMEN
IgG4-related disease (IgG4-RD) is a multi-system fibro-inflammatory disorder with classical histopathological findings, often in the context of elevated serum IgG4 levels. The thoracic manifestations of IgG4-RD are numerous and can mimic several common and better known conditions. The objective of this study was to outline the frequency and nature of thoracic involvement in a prospective cohort of IgG4-RD patients who met defined diagnostic criteria. Over 40% of IgG4-RD patients had clinicoradiological and/or histological evidence of thoracic involvement, predominantly mediastinal lymphadenopathy, the majority associated with multi-system disease outside the chest. Thoracic involvement was associated with a higher serum IgG4 level, potentially representing greater disease activity or spread. Our data highlight the diverse nature of thoracic IgG4-RD, and the importance of knowledge and recognition of the condition among respiratory physicians who are likely to encounter this disease entity on an increasing basis.
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Enfermedades Autoinmunes/inmunología , Granuloma de Células Plasmáticas/inmunología , Inmunoglobulina G/inmunología , Enfermedades Pulmonares Intersticiales/inmunología , Linfadenopatía/inmunología , Mediastinitis/inmunología , Enfermedades Pleurales/inmunología , Adulto , Anciano , Anciano de 80 o más Años , Enfermedades Autoinmunes/complicaciones , Enfermedades Autoinmunes/fisiopatología , Femenino , Fibrosis , Granuloma de Células Plasmáticas/etiología , Granuloma de Células Plasmáticas/fisiopatología , Humanos , Enfermedades Pulmonares Intersticiales/etiología , Enfermedades Pulmonares Intersticiales/fisiopatología , Linfadenopatía/etiología , Linfadenopatía/fisiopatología , Masculino , Mediastinitis/etiología , Mediastinitis/fisiopatología , Mediastino/patología , Persona de Mediana Edad , Enfermedades Pleurales/etiología , Enfermedades Pleurales/fisiopatología , Reino Unido , Adulto JovenRESUMEN
OBJECTIVES: There is marked inconsistency in reporting the key features of IgG4-related inflammatory pseudotumor (IPT) cases. We aimed to analyze the various aspects of IgG4-related IPTs and to test the performance of the consensus criteria for their diagnosis. METHODS: PubMed database was searched for IgG4-related IPT cases. The data regarding patient demographics, clinical presentation, laboratory findings, histopathological features, and treatment response are extracted and are presented here in a descriptive manner. RESULTS: The study included 40 papers describing the clinicopathological features of 83 IPTs in 80 patients. Seventeen cases were diagnosed on biopsies; while remaining were diagnosed on excision specimens. Among these, 50 cases were categorized as highly suggestive and 24 cases as probable for IgG4RD; while nine cases had insufficient histopathological evidence of IgG4RD. Two cases diagnosed on biopsies having insufficient evidence of IgG4RD showed partial or no response to steroids; while 12/14 cases (85.71%) diagnosed on biopsies that were histologically suggestive or probable for IgG4RD showed prompt response to steroids. CONCLUSION: Many reports have not specifically mentioned the full histopathological findings of IgG4-related IPTs that may hinder in refining the diagnostic criteria of IgG4RD. The IgG4-related IPTs diagnosed on biopsies with requisite features showed prompt response to steroids indicating specificity of histopathological findings in predicting treatment response.
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Granuloma de Células Plasmáticas/patología , Adolescente , Adulto , Anciano , Anciano de 80 o más Años , Niño , Femenino , Granuloma de Células Plasmáticas/inmunología , Humanos , Inmunoglobulina G/inmunología , Masculino , Persona de Mediana Edad , Células Plasmáticas/patologíaAsunto(s)
Pared Abdominal , Enfermedades Autoinmunes/diagnóstico , Granuloma de Células Plasmáticas/diagnóstico , Inmunoglobulina G/sangre , Pared Abdominal/diagnóstico por imagen , Pared Abdominal/patología , Pared Abdominal/cirugía , Enfermedades Autoinmunes/sangre , Enfermedades Autoinmunes/inmunología , Enfermedades Autoinmunes/cirugía , Biomarcadores/sangre , Biopsia , Diagnóstico Diferencial , Granuloma de Células Plasmáticas/sangre , Granuloma de Células Plasmáticas/inmunología , Granuloma de Células Plasmáticas/cirugía , Humanos , Inmunohistoquímica , Masculino , Persona de Mediana Edad , Tomografía Computarizada por Tomografía de Emisión de Positrones , Valor Predictivo de las PruebasRESUMEN
One of the pathogenic causes of cutaneous inflammatory pseudotumors is chronic localized fibrosing leukocytoclastic vasculitis (CLFLCV), a vasculitic reaction pattern seen in granuloma faciale (GF), a localized vasculitis, and erythema elevatum diutinum (EED), a generalized vasculitis. Patients with myelodysplastic syndromes (MDSs) are at risk for a diverse spectrum of cutaneous neutrophilic dermatoses such as EED. Herein, we report a 74-year-old man who presented with a large ulcerative, fungating tumor affecting the right flexor ankle caused by CLFLCV. During his workup and management, MDS and Philadelphia chromosome-negative chronic myeloid leukemia was diagnosed. Surgical excision of the inflammatory mass promptly triggered tumor recurrence, whereas antineutrophil therapy (dapsone coupled with hydroxyurea, and prednisone) lead to tumor regression. Histopathologic examination revealed an eosinophilic-rich small-vessel neutrophilic vasculitis associated with storiform and angiocentric fibrosis (GF-like). In the regions of fibrosis, dilated lymphatic and vascular spaces were numerous, some of which were congested with small CD3-positive lymphocytes (intralymphatic and intravascular lymphocytosis). These findings indicate coexisting localized lymphedema. By direct immunofluorescence, IgM and C4d vessel deposits were detected. The pathogenesis of the fibrotic nodules and plaques of CLFLCV is suspected to be due to recurring bouts of immune-complex vasculitis, creating a cycle of vessel damage followed by reparative granulation tissue. Poor lymphatic drainage may be the underlying factor initiating and maintaining recurrent, localized episodes of immune-complex vasculitis and progressive fibrosis. Although his tumor histopathology resembled GF-eosinophilic rich CLFLCV-the clinical context points to a solitary and paraneoplastic case of EED.
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Granuloma de Células Plasmáticas/patología , Leucemia Mieloide Crónica Atípica BCR-ABL Negativa/patología , Linfedema/patología , Linfocitosis/patología , Síndromes Paraneoplásicos/patología , Vasculitis Leucocitoclástica Cutánea/patología , Anciano , Protocolos de Quimioterapia Combinada Antineoplásica/uso terapéutico , Biopsia , Dapsona/uso terapéutico , Resultado Fatal , Granuloma de Células Plasmáticas/inmunología , Granuloma de Células Plasmáticas/terapia , Humanos , Hidroxiurea/uso terapéutico , Leucemia Mieloide Crónica Atípica BCR-ABL Negativa/tratamiento farmacológico , Leucemia Mieloide Crónica Atípica BCR-ABL Negativa/genética , Leucemia Mieloide Crónica Atípica BCR-ABL Negativa/inmunología , Linfedema/inmunología , Linfedema/terapia , Linfocitosis/inmunología , Linfocitosis/terapia , Masculino , Síndromes Paraneoplásicos/inmunología , Síndromes Paraneoplásicos/terapia , Pentoxifilina/uso terapéutico , Prednisona/uso terapéutico , Inducción de Remisión , Resultado del Tratamiento , Vasculitis Leucocitoclástica Cutánea/inmunología , Vasculitis Leucocitoclástica Cutánea/terapiaRESUMEN
A 50-year-old woman presented with epigastralgia. Computed tomography (CT) of the abdomen revealed a 6-cm well-enhanced mass extending from the left lobe of the liver to the abdominal wall, suggestive of cholangiocarcinoma. Liver and skin mass biopsies did not provide evidence of hepatic malignancy but were rich in plasma cells and sclerotic lesions. Subsequent detection of elevated serum immunoglobulin G4 (IgG4) led to a diagnosis IgG4-related inflammatory pseudotumor (IPT) of the liver. Treatment with systemic corticosteroids resulted in rapid clinical improvement. This case is the first report of an IgG4-related hepatic IPT invading the abdominal wall.
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Pared Abdominal/patología , Granuloma de Células Plasmáticas/diagnóstico por imagen , Inmunoglobulina G/sangre , Hepatopatías/diagnóstico por imagen , Neoplasias de los Conductos Biliares/diagnóstico , Biopsia , Colangiocarcinoma/diagnóstico , Diagnóstico Diferencial , Femenino , Granuloma de Células Plasmáticas/inmunología , Granuloma de Células Plasmáticas/patología , Humanos , Hígado/patología , Hepatopatías/inmunología , Hepatopatías/patología , Imagen por Resonancia Magnética , Persona de Mediana Edad , Piel/patología , Tomografía Computarizada por Rayos X , UltrasonografíaRESUMEN
BACKGROUND: Hepatic inflammatory pseudotumor (IPT) is classified into 2 types based on IgG4 stain: IgG4-related and non-IgG4-related; the two types differ not only in their pathological characteristics, but also in the clinical features. This study aimed to investigate the MR character of hepatic IPT, and differentiate the IgG4-related IPT from the non-IgG4-related IPT. METHODS: Twenty-five patients with 27 histologically proven hepatic IPTs were retrospectively analyzed. Ten lesions were diagnosed as IgG4-related IPT, and the other 17 as non-IgG4-related IPT. The MR signal features on T1, T2-weighted, dynamic-enhanced, and diffusion-weighted imaging were evaluated and compared. RESULTS: The dominant lesions were subcapsularly distributed (n=17, 63.0%) with clear boundary (n=20, 74.1%), and showed progressive enhancement pattern (n=21, 77.8%) with diffuse homogeneous (n=12, 44.4%) or heterogeneous (n=8, 29.6%) hyperintensity, accompanied by delayed capsule-like enhancement (n=17, 63.0%) and central nonenhanced areas (n=18, 66.7%). Morphological features (P>0.05) were not sufficient to differentiate IgG4-related IPT from non-IgG4-related IPT; the wash-out pattern was only found in 2 IgG4-related IPT, while the progressive enhancement pattern was more common in the non-IgG4-related lesions (n=16) (P=0.022). During portal and delayed phases, iso-/hypoenhanced lesions were only seen in 3 IgG4-related IPT, and circular-enhanced lesions (n=5) existed exceptionally in the non-IgG4-related group with significant differences (P=0.029 and 0.027). Most IgG4-related IPTs had lower apparent diffusion coefficient compared with the liver parenchyma (n=6), while most non-IgG4-related IPTs had higher apparent diffusion coefficient value (n=13) (P=0.046). CONCLUSIONS: Although MR images of hepatic IPT have certain characteristics, they are not enough to differentiate IgG4-related IPT from non-IgG4-related IPT. The enhancement pattern, signal features on portal and delayed phases, and the apparent diffusion coefficient value of the lesion may be helpful for the diagnosis.
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Imagen de Difusión por Resonancia Magnética , Granuloma de Células Plasmáticas/diagnóstico por imagen , Inmunoglobulina G/análisis , Hepatopatías/diagnóstico por imagen , Hígado/diagnóstico por imagen , Autoinmunidad , Biomarcadores/análisis , Biopsia , Diagnóstico Diferencial , Femenino , Granuloma de Células Plasmáticas/inmunología , Humanos , Inmunohistoquímica , Hígado/inmunología , Hepatopatías/inmunología , Masculino , Persona de Mediana Edad , Valor Predictivo de las Pruebas , Estudios RetrospectivosAsunto(s)
Enfermedades Autoinmunes/diagnóstico , Granuloma de Células Plasmáticas/diagnóstico , Inmunoglobulina G/inmunología , Hepatopatías/diagnóstico , Administración Oral , Enfermedades Autoinmunes/tratamiento farmacológico , Enfermedades Autoinmunes/inmunología , Biopsia con Aguja Gruesa , Glucocorticoides/administración & dosificación , Granuloma de Células Plasmáticas/tratamiento farmacológico , Granuloma de Células Plasmáticas/inmunología , Humanos , Inmunohistoquímica , Hepatopatías/tratamiento farmacológico , Hepatopatías/inmunología , Masculino , Persona de Mediana Edad , Tomografía Computarizada por Tomografía de Emisión de Positrones , Prednisolona/administración & dosificación , Inducción de Remisión , Factores de Tiempo , Resultado del TratamientoRESUMEN
Granulomatosis with polyangiitis (Wegener disease) is a rare antineutrophil cytoplasmic antibody (ANCA)-positive vasculitis that seldom involves brain and meninges. We present a case of a 35-year-old woman with granulomatosis with polyangiitis involving the brain and the meninges. The histologic diagnosis required a complete resection of the lesion. Despite the possibility of postoperative wound infection, surgery was mandatory to ascertain the diagnostic and to treat locally.
Asunto(s)
Encefalopatías/cirugía , Granuloma de Células Plasmáticas/cirugía , Granulomatosis con Poliangitis/cirugía , Base del Cráneo/cirugía , Adulto , Anticuerpos Anticitoplasma de Neutrófilos/sangre , Encefalopatías/diagnóstico , Encefalopatías/inmunología , Craneotomía , Diagnóstico Diferencial , Progresión de la Enfermedad , Femenino , Estudios de Seguimiento , Granuloma de Células Plasmáticas/diagnóstico , Granuloma de Células Plasmáticas/inmunología , Granulomatosis con Poliangitis/diagnóstico , Granulomatosis con Poliangitis/inmunología , Humanos , Imagen por Resonancia Magnética , Tomografía Computarizada por Rayos XRESUMEN
BACKGROUND: Hepatic inflammatory pseudotumor (IPT) is a rare disease which often mimics a malignant tumor and is therefore often misdiagnosed and surgically resected. Recently, a concept of IgG4-related diseases (IgG4-RD) has been proposed that is becoming widely recognized and includes IgG4-related hepatic IPT. Corticosteroids are widely accepted as the standard treatment. CASE PRESENTATION: A 72-year-old Japanese man, who had been followed for ten years after surgery and chemotherapy for treatment of hilar and lower bile duct cancers, developed intermittent fever and abdominal pain and visited this hospital. Blood examinations revealed an inflammatory reaction, worsened glucose intolerance, and an increased level of serum IgG4 (137 mg/dL). Computed tomography (CT) revealed a 5 cm-sized mass in hepatic segment 7. Because of his cancer history, not only was a benign mass suspected, but there was also the possibility of a recurrent biliary malignancy. Liver biopsy was performed and the histology met the criteria for IgG4-related IPT. Corticosteroid therapy was initiated and his symptoms quickly resolved. However, two months later, a repeat CT demonstrated that the hepatic mass had been replaced by an abscess. The abscess was initially refractory, despite tapering corticosteroid treatment, controlling diabetes by intensive insulin therapy, administration of antibiotics, and percutaneous abscess drainage. Finally, after six months, the condition resolved. CONCLUSION: The diagnosis of hepatic IPT is sometimes difficult. To differentiate it from a malignant tumor, histological examination is necessary. Although corticosteroids are recognized as the standard therapy, unexpected and critical complications can develop in cases of IgG4-related hepatic IPT.
Asunto(s)
Absceso/inducido químicamente , Corticoesteroides/efectos adversos , Granuloma de Células Plasmáticas/diagnóstico , Granuloma de Células Plasmáticas/tratamiento farmacológico , Inmunoglobulina G/sangre , Hepatopatías/diagnóstico , Hepatopatías/tratamiento farmacológico , Absceso/terapia , Corticoesteroides/uso terapéutico , Anciano , Diagnóstico Diferencial , Granuloma de Células Plasmáticas/inmunología , Humanos , Hepatopatías/inmunología , MasculinoRESUMEN
Since the publication of the first two cases in 1984 by Cotelingam and Jaffe, a hundred of cases were published concerning the splenic inflammatory pseudotumor. The inflammatory pseudotumor is a benign lesion of unknown etiology. It forms a group of solid mesenchymal tumors occurring in different organs mainly in adulthood with female predominance. Symptomatic patients most often manifest fever, abdominal pain and splenomegaly. A substantial proportion of the inflammatory pseudotumors of the spleen are discovered incidentally. In this article we report a case of inflammatory pseudotumor in a 41-year-old male patient. The diagnosis of inflammatory pseudotumor of the spleen is made up by the histological and immunohistochemical examination.
Asunto(s)
Granuloma de Células Plasmáticas/patología , Bazo/patología , Adulto , Granuloma de Células Plasmáticas/inmunología , Humanos , Masculino , Bazo/inmunologíaAsunto(s)
Dolor Abdominal/etiología , Enfermedades Autoinmunes/complicaciones , Dacriocistitis/etiología , Exoftalmia/etiología , Granuloma de Células Plasmáticas/etiología , Inmunoglobulina G/sangre , Enfermedades Renales/etiología , Seudotumor Orbitario/etiología , Pancreatitis/complicaciones , Dolor Abdominal/diagnóstico , Enfermedades Autoinmunes/diagnóstico , Enfermedades Autoinmunes/tratamiento farmacológico , Enfermedades Autoinmunes/inmunología , Biomarcadores/sangre , Biopsia , Dacriocistitis/diagnóstico , Dacriocistitis/tratamiento farmacológico , Dacriocistitis/inmunología , Exoftalmia/diagnóstico , Femenino , Granuloma de Células Plasmáticas/diagnóstico , Granuloma de Células Plasmáticas/tratamiento farmacológico , Granuloma de Células Plasmáticas/inmunología , Humanos , Inmunohistoquímica , Inmunosupresores/uso terapéutico , Enfermedades Renales/diagnóstico , Enfermedades Renales/tratamiento farmacológico , Enfermedades Renales/inmunología , Imagen por Resonancia Magnética , Persona de Mediana Edad , Seudotumor Orbitario/diagnóstico , Seudotumor Orbitario/tratamiento farmacológico , Seudotumor Orbitario/inmunología , Pancreatitis/diagnóstico , Pancreatitis/tratamiento farmacológico , Pancreatitis/inmunología , Esteroides/uso terapéutico , Tomografía Computarizada por Rayos X , Resultado del TratamientoRESUMEN
BACKGROUND: IgG4-related disease is a recently recognized systemic disease characterized by storiform fibrosis with infiltration of IgG4-positive plasma cells. In rare incidences, IgG4-related renal disease can present as a solitary mass lesion at renal pelvis and can pose a diagnostic challenge since these lesions mimic malignancy. Herein, we present a rare case of IgG4-related disease presenting as inflammatory pseudotumor lesion, involving the renal pelvis and also neighboring renal parenchyma. CASE PRESENTATION: A 75-year-old man with no history of IgG4-related disease underwent computed tomography (CT) scan for evaluation of prostatic cancer. The CT scan incidentally revealed a mass lesion located at the right renal pelvis. Radiologic findings were highly suggestive of malignancy. Therefore, the patient underwent right nephroureterectomy. Microscopically, the mass lesion showed storiform fibrosis with diffuse and intense inflammatory cell infiltration. Infiltrating cells were mainly histiocytes and plasma cells. Tubulointerstitium adjacent to the lesion also showed fibrosis with abundant plasmacytic infiltration. Immunohistochemical staining revealed the presence of IgG4-positive plasma cells in both the mass lesion and tubulointerstitium (mean of 94/HPF per field). CONCLUSION: Considering these findings, we diagnosed the mass lesion as IgG4-related inflammatory pseudotumor of the renal pelvis. In patients with renal pelvic masses, IgG4-related inflammatory pesudotumor should be considered in the differential diagnosis to avoid unnecessary surgical intervention.