RESUMEN
Immunoglobulin (Ig) G4-related disease (IgG4RD) is a chronic autoimmune disorder characterized by dense lymphoplasmacytic infiltrations and fibrosis of storiform pattern. The most typical manifestations include major salivary or lacrimal gland involvement, autoimmune pancreatitis, and retroperitoneal fibrosis. While the increase in IgG4 is the typical feature of the disease, hypercalcemia has been rarely reported in IgG4RD so far, only one of these cases has been shown parathyroid gland involvement (isolated involvement). In this study, we present a 43-year-old female patient with weight loss, pancreatic mass, lymphadenopathy, nodular lesion in the lung, hypercalcemia, and also increased level of serum IgG4. Histopathological investigation following parathyroidectomy revealed a dense lymphoplasmacytic infiltrate with an IgG4 to IgG ratio of > 50% in the fat tissue surrounding the parathyroid gland, particularly at the perivascular areas. This is the first systemic IgG4RD case in combination with hypercalcemia in the literature who was detected to have parathyroid adenoma. Our aim in this review is to emphasize that, although rarely, IgG4RD may be accompanied by hypercalcemia and parathyroid gland may be one of its target sites.
Asunto(s)
Enfermedades Autoinmunes , Hipercalcemia , Enfermedad Relacionada con Inmunoglobulina G4 , Fibrosis Retroperitoneal , Adulto , Enfermedades Autoinmunes/complicaciones , Enfermedades Autoinmunes/diagnóstico , Femenino , Humanos , Hipercalcemia/complicaciones , Inmunoglobulina G , Enfermedad Relacionada con Inmunoglobulina G4/complicaciones , Enfermedad Relacionada con Inmunoglobulina G4/diagnósticoRESUMEN
Immunoglobulin (Ig) A vasculitis (IgAV), formerly known as Henoch-Schonlein purpura (HSP), is a relatively uncommon form of vasculitis primarily targeting the skin, gastrointestinal system, and the kidneys. Although the pathogenesis has not yet been well identified, several triggering factors, such as infections, drugs, have been implicated in the development of IgAV. Tuberculosis (TB), albeit rare, may precipitate IgAV. Herein, we have presented a case manifested by purpuric skin rash and proteinuria 6 weeks following diagnosis of pulmonary tuberculosis while receiving anti-TB drugs. The case was diagnosed as having active tuberculosis and TB-related IgA vasculitis with multi-organ involvement. In this case-based review, we recruited cases with TB-related Ig A vasculitis from the literature and discussed the features of tuberculosis that mimic vasculitides and vice versa. We also discussed the difficulties in diagnosis and the therapeutic approach in the light of the literature.
Asunto(s)
Vasculitis por IgA/diagnóstico , Tuberculosis Pulmonar/complicaciones , Adulto , Anciano , Anticuerpos Anticitoplasma de Neutrófilos/sangre , Antituberculosos/administración & dosificación , Antituberculosos/efectos adversos , Niño , Humanos , Vasculitis por IgA/etiología , Masculino , Persona de Mediana Edad , Tomografía Computarizada por Rayos X , Tuberculosis Pulmonar/diagnóstico por imagen , Tuberculosis Pulmonar/tratamiento farmacológicoRESUMEN
OBJECTIVE: The aim of the present study was to analyze the prognostic significance of tumor budding in muscle-invasive urothelial carcinomas of the urinary bladder, and also to determine an optimal threshold value in evaluation. PATIENTS AND METHODS: The study included 108 patients diagnosed with muscleinvasive conventional urothelial carcinoma between 2010 and 2020. Tumor budding was evaluated on H&E-stained slides. The critical tumor budding number was determined with the "receiver operating characteristics (ROC)" curve. Cases with a tumor budding number of ≤6 were categorized as low, and cases with >6 as high tumor budding. RESULTS: The univariate Cox proportional hazards regression model for recurrence-free survival showed that lymphovascular invasion (P = 0.001), tumor budding (P = 0.012), pT stage (T4 vs. T2) (P = 0.005), and lymph node metastasis (P = 0.009) were significantly associated with recurrence-free survival. The multivariate Cox proportional hazards regression model utilizing backward stepwise (wald) method revealed that only LVI (P = 0.001) was independent risk factor for recurrence-free survival. The univariate Cox analysis showed that lymphovascular invasion (P = 0.001), tumor budding (P = 0.004), pT stage (T4 vs. T2) (P = 0.003), and lymph node metastasis (P = 0.001) were significantly associated with overall survival. The multivariate Cox analysis (backward stepwise (wald) method) revealed that tumor focality (P = 0.018), pT stage (T4 vs. T2) (P = 0.015), and lymphovascular invasion (P = 0.002) were independent factors for overall survival. CONCLUSIONS: Our findings suggested that the evaluation of tumor budding may be a useful parameter for predicting outcome in patients with muscle-invasive bladder cancer.
Asunto(s)
Carcinoma de Células Transicionales/patología , Metástasis Linfática/patología , Invasividad Neoplásica/patología , Neoplasias de la Vejiga Urinaria/patología , Adulto , Anciano , Anciano de 80 o más Años , Carcinoma de Células Transicionales/diagnóstico , Cistectomía/métodos , Femenino , Humanos , Masculino , Persona de Mediana Edad , Estadificación de Neoplasias/métodos , Pronóstico , Vejiga Urinaria/patología , Neoplasias de la Vejiga Urinaria/diagnósticoRESUMEN
Systemic sclerosis (SSc) may overlap frequently with other rheumatic diseases, including systemic lupus erythematosus (SLE), which can require high-dose steroids depending on the clinical presentation. We present a case involving this overlap in which the patient concomitantly developed lupus nephritis and Coombs (+) hemolytic anemia, which was confused with scleroderma renal crisis. In this condition, assessing for lupus nephritis with timely renal biopsy and lupus serology can aid in guiding the appropriate treatment. We discuss the clinical features and challenging management of this case with a review of the English-language literature for SSc and SLE overlap with glomerulonephritis.
Asunto(s)
Anemia Hemolítica/diagnóstico , Glomerulonefritis/diagnóstico , Adulto , Anemia Hemolítica/complicaciones , Diagnóstico Diferencial , Femenino , Glomerulonefritis/complicaciones , Humanos , Nefritis Lúpica/diagnóstico , Esclerodermia Localizada/diagnósticoRESUMEN
Mucinous cystic neoplasm of the liver (MCN-L) is a rare tumor which accounts for less than 5% of all liver cysts. Although they are considered to be "benign cysts" radiologically and clinically because of their slow growth, they are considered as premalignant. We present two radiologically misdiagnosed cases that operated in a short time range, in order to increase awareness for these rare tumors. A 47-year-old female patient who had no active complaints 58 x 40 mm cystic lesion was detected in the liver, which was diagnosed hydatid cyst radiologically. The pathological examination showed multiloculated cysts which was covered by low-grade mucinous epithelium and ovarian-type stroma on the cyst wall. A 50-year-old female patient presented with abdominal distention. The radiographical screening revealed a 204 x 140 mm cystic lesion that completely fills left lobe of liver which interpreted in favor of hydatid cyst. Histopathologically, the inner surface of the cyst was covered with low grade mucinous epithelium. Ovarian-type stroma was detectable only by immunohistochemistry due to significant bleeding and edema on the wall. The diagnosis of both of our cases was low grade MCN-L. Since cysts were not intact at the time of gross examination, we could not make any comment about surgical margins or total excision. MCN-L is a tumor that creates difficulty in presurgical differential diagnosis because of its rarity and lack of specific radiologic features. Although the prognosis is excellent as a result of total excision in the benign group, relapses have also been reported.
RESUMEN
Metabolic myopathies are among the treatable causes of rhabdomyolysis and myoglobinuria. Carnitine palmitoyl transferase 2 (CPT II) deficiency is one of the most common causes of recurrent myoglobinuria in adults. It is an inherited disorder of fatty acid oxidation pathway, commonly associated with elevated acylcarnitine levels. In this case report, we present a 49-year-old male patient who developed acute kidney injury after rhabdomyolysis and was thus diagnosed with CPT2 deficiency after his first episode of rhabdomyolysis. Inborn errors of metabolism should be kept in mind in patients with rhabdomyolysis. Acylcarnitine profile may be normal in CPT II deficiency, even during an acute attack, and molecular genetic diagnostics should be applied if there is high index of clinical suspicion.
Asunto(s)
Lesión Renal Aguda , Carnitina O-Palmitoiltransferasa , Carnitina , Errores Innatos del Metabolismo Lipídico , Errores Innatos del Metabolismo , Enfermedades Mitocondriales , Enfermedades Musculares , Mioglobinuria , Rabdomiólisis , Humanos , Masculino , Persona de Mediana Edad , Lesión Renal Aguda/complicaciones , Carnitina/uso terapéutico , Carnitina/análogos & derivados , Carnitina O-Palmitoiltransferasa/genética , Carnitina O-Palmitoiltransferasa/deficiencia , Enfermedades Musculares/complicaciones , Mioglobinuria/complicaciones , Rabdomiólisis/etiología , Rabdomiólisis/complicacionesRESUMEN
BACKGROUND: Mesothelioma is a malignant neoplasm with a poor survival rate. We aimed to investigate the importance of BAP1, MTAP (IHC), and p16/CDKN2A homozygous deletion (FISH) in cytologic material obtained from pleural effusion sampling, which is a less invasive procedure in the diagnosis of mesothelioma. METHODS: Our study discussed pleural cytology samples of cases with histopathologically proven mesothelioma diagnoses between 2017 and 2022. As the control group, materials that had pleural effusion sampling for other reasons and reactive mesothelial hyperplasia were included in the study. Cell blocks prepared from these materials were subjected to fluorescent in situ hybridization for p16/CDKN2A homozygous deletion and immunohistochemistry for BAP1 and MTAP. RESULTS: The specificity of the P16/CDKN2A homozygous deletion in diagnosing mesothelioma is 100%. Its sensitivity is 68.75%. The specificity of BAP1 immunohistochemical nuclear expression loss is 95%, while the sensitivity is 60%. Loss of nuclear expression of MTAP alone has the lowest specificity and sensitivity, with a specificity of 86% and a sensitivity of 43%. The highest sensitivity is reached when BAP1 loss and p16/CDKN2A homozygous deletion are evaluated together, increasing to 81%. The specificity is 95%. CONCLUSION: It has been determined that any marker alone cannot be used for a definitive mesothelioma diagnosis in pleural effusion cytological specimens; however, sensitivity increases in some combinations. The combination of BAP1 immunohistochemistry and p16/CDKN2A homozygous deletion detected by FISH, which has a higher specificity and sensitivity, can be routinely used in the diagnosis of mesothelioma under the guidance of clinical and radiologic information.
Asunto(s)
Mesotelioma Maligno , Mesotelioma , Neoplasias Mesoteliales , Derrame Pleural , Humanos , Citología , Homocigoto , Hibridación Fluorescente in Situ , Eliminación de Secuencia , Mesotelioma/diagnóstico , Mesotelioma/genética , Mesotelioma Maligno/genética , Inhibidor p16 de la Quinasa Dependiente de Ciclina/genética , Proteínas Supresoras de Tumor/genética , Ubiquitina Tiolesterasa/genéticaRESUMEN
OBJECTIVES: Cribriform glands are linked to poorer outcomes in prostate adenocarcinoma. We aimed to assess the prognostic role of the percentage of cribriform glands and the size of the largest invasive cribriform gland in Gleason score 7 prostate adenocarcinomas. METHODS: The presence, percentage, and size of the invasive cribriform glands were investigated and their association with prognostic factors were assessed in 177 Grade Groups 2 and 3 prostate adenocarcinomas. RESULTS: Biochemical recurrence-free survival was statistically significantly lower in cases with a cribriform gland percentage greater than 10% (P < .001) and in cases where the largest invasive cribriform gland size was greater than 0.5 mm (P < .001). Mean largest cribriform gland size and percentage were statistically significant associated with more advanced pT status, lymph node metastasis, biochemical recurrence, and higher preoperative prostate-specific antigen values. CONCLUSIONS: Our findings suggest that the presence of a cribriform pattern, increases in the percentage of such patterns, and increases in the size of the largest cribriform gland within a given tumor are associated with poor prognosis. We suggest that a more aggressive clinical approach may be needed in Grade Group 2 and 3 cases with invasive cribriform glands larger than 0.5 mm and a cribriform gland percentage greater than 10%, especially in prostate needle biopsy specimens.
RESUMEN
OBJECTIVES: To determine the frequency and predictors of peritubular capillaritis (PTCitis) among native kidney biopsies. METHODS: Consecutive native kidney biopsies of 169 patients were reexamined for capturing possible PTCitis according to the Banff Classification. The relation of PTCitis with demographic and clinicopathologic findings was evaluated. Logistic regression analysis was performed to determine predictors of PTCitis. RESULTS: Peritubular capillaritis was captured in 90 (53.3%) patients, with scores of 1, 2, and 3 in 57 (33.7%), 31 (18.3%), and 2 (1.2%) patients, respectively. The highest frequency of PTCitis was observed in pauci-immune glomerulonephritis. In univariate analysis, male sex, the presence of interstitial inflammation, pauci-immune glomerulonephritis, and a higher serum creatinine level were associated with a higher risk of PTCitis, while severe interstitial fibrosis/tubular atrophy was associated with a lower risk. The presence of interstitial inflammation (odds ratio [OR], 5.94 [95% confidence interval (CI), 1.41-25.03]; P = .015), pauci-immune glomerulonephritis (OR, 3.08 [95% CI, 1.01-9.36]; P = .048), and a higher serum creatinine level (per 1 mg/dL) (OR, 1.56 [95% CI, 1.14-2.11]; P = .005) were independent predictors of PTCitis development in a multivariate regression model. CONCLUSIONS: Peritubular capillaritis is common in native biopsies and more likely to be observed in the presence of interstitial inflammation, pauci-immune glomerulonephritis, and a higher serum creatinine level.