RESUMO
OBJECTIVES: To evaluate whether the grade of IgG4+ plasma cell infiltration in biopsies is associated with clinical or serologic outcomes in IgG4-RD. METHODS: We included 57 patients with biopsy proven IgG4-RD according to the Comprehensive Diagnostic Criteria and/or the 2019 ACR/EULAR Classification Criteria. We collected histological, clinical (disease duration, phenotype, remission and relapses) and serological variables. RESULTS: 29 (50.9%) patients were men, mean age 49.9 years, with a median disease duration of 22 months. The distribution among clinical phenotypes were 14% pancreato-hepato-biliary, 12.3% retroperitoneal/aortic, 29.8% head and neck-limited, 29.8% Mikulicz/systemic and 14% undefined. Thirty-nine patients had a proliferative and 18 a fibrotic phenotype. Most biopsies were from lacrimal gland, lymph node, pancreas, orbit, kidney, retroperitoneum and thyroid gland. Thirty-nine (68.4%) patients had <100 IgG4+ plasma cells/HPF and 18 (31.6%) ≥100 IgG4+ plasma cells/HPF. Patients with ≥100 IgG4+ plasma cells/HPF were more likely to belong to the pancreato-hepato-biliary and the proliferative phenotypes, had fewer relapses and a higher remission rate. On multivariate analysis, the OR for remission at last follow-up was 6.7, 95% CI 1.1-4.42, p=0.03. The log-rank test showed a difference in relapse-free survival between the two groups (HR 2.6, 95% CI 1.2-5.6, p=0.01). According to the ROC analysis, patients with more than 61 IgG4+ plasma cells were less likely to relapse. CONCLUSIONS: A count of ≥100 IgG4+ plasma cells/HPF may identify patients with a proliferative phenotype, fewer relapses and a higher remission rate.
Assuntos
Amiloidose/etiologia , Doença Relacionada a Imunoglobulina G4/complicações , Amiloidose/tratamento farmacológico , Amiloidose/imunologia , Feminino , Glucocorticoides/uso terapêutico , Humanos , Doença Relacionada a Imunoglobulina G4/tratamento farmacológico , Fatores Imunológicos/uso terapêutico , Ácido Micofenólico/uso terapêutico , Prednisona/uso terapêutico , Rituximab/uso terapêutico , Adulto JovemRESUMO
Objective: To evaluate if major salivary gland enlargement in patients with IgG4-related disease (IgG4-RD) is associated with systemic involvement.Methods: We included 47 patients with diagnosis of IgG4-RD. We retrospectively collected demographics, organ involvement, disease activity and damage assessed by the IgG4-RD Responder Index (IgG4-RD RI) and treatment.Results: 25 patients (53%) were men, mean age 50.8 years and median disease duration 27 months. Most frequently anatomic sites affected were lymph nodes 55%, pancreas 51% and lacrimal glands 43%. We observed major salivary gland involvement in 22 (46.8%) patients. When we compared patients with (n = 22) vs. without (n = 25) salivary gland enlargement, the first group had a higher number of affected organs, a higher prevalence of lacrimal glands, lymph nodes, and lung involvement, rheumatoid factor positivity, azathioprine and prednisone use, as well as a higher baseline IgG4-RD RI and a longer delay in diagnosis. At logistic regression analysis we found an association of major salivary gland enlargement with the basal IgG4-RD RI (OR 1.37, 95%CI 1.09-1.61, p = .001) and with lacrimal gland involvement (OR 34.7, 95%CI 4.6-258, p = .001).Conclusion: Our study highlights the systemic nature of IgG4-RD. Patients with major salivary gland enlargement should be routinely screened for multi-organ disease.
Assuntos
Doença Relacionada a Imunoglobulina G4/complicações , Doenças das Glândulas Salivares/etiologia , Glândulas Salivares/patologia , Feminino , Humanos , Doença Relacionada a Imunoglobulina G4/diagnóstico , Masculino , Pessoa de Meia-Idade , Tomografia por Emissão de Pósitrons , Estudos Retrospectivos , Doenças das Glândulas Salivares/diagnóstico , Índice de Gravidade de Doença , Tomografia Computadorizada por Raios X , UltrassonografiaRESUMO
Phosphaturic mesenchymal tumor (PMT) is a morphologically heterogeneous soft tissue and bone neoplasm, producing a paraneoplastic syndrome due to phosphate wasting. These tumors produce fibroblast growth factor 23, which is implicated in renal tubule phosphate loss. Medical records of patients seen from 1999 to 2013 with osteomalacia associated or not with a tumor were reviewed. Clinical and laboratory data, radiographic studies, and follow-up of 8 patients were tabulated. Histologic features and the immunoprofile of the tumors were analyzed. There were 208 patients with osteomalacia, but only 8 (3.84%) had osteomalacia associated with a tumor. The median age of the patients was 40 years. The tumor size ranged from 1.5 to 4 cm. Five were located in soft tissues and skin; and 3, in bones. Osteomalacia symptoms lasted from 2 to 14 years with a median of 6 years. Laboratory data showed hypophosphatemia and phosphaturia in all patients. All tumors were histologically benign. Histologically, the salient features were a hemangiopericytoid pattern, chronic hemorrhage, and microcystic areas. All neoplasms were diffusely positive for vimentin and focally positive for epithelial membrane antigen, CD34, and S-100 protein. Ki-67 was positive in approximately 10% of neoplastic cells in 2 cases and less than 1% in the remainder. We report 8 cases of PMTs producing osteomalacia, from a single third-level Mexican medical institution. These tumors occurred in soft tissues, skin, and bones. All tumors were benign, small, not easily detected by physical examination and diagnosed due to the metabolic abnormalities.
Assuntos
Hipofosfatemia/patologia , Mesenquimoma/patologia , Neoplasias de Tecido Conjuntivo e de Tecidos Moles/patologia , Osteomalacia/patologia , Adulto , Feminino , Humanos , Hipofosfatemia/etiologia , Masculino , Mesenquimoma/complicações , México , Pessoa de Meia-Idade , Neoplasias de Tecido Conjuntivo e de Tecidos Moles/complicações , Osteomalacia/etiologia , Estudos RetrospectivosRESUMO
We report the case of a 26-year-old woman with a 19 cm malignant hepatic neoplasm with morphological features that closely resembled a follicular thyroid carcinoma. Despite this, it was interpreted as a cholangiocarcinoma due to the absence of a primary thyroid tumor and the lack of thyroglobulin and TTF-1 immunoreactivity by the hepatic tumor. The left hepatic lobectomy specimen showed an encapsulated and multinodular gray-white mass with cystic and hemorrhagic areas. Microscopically, it displayed predominant macro and microfolicullar patterns with focal solid, trabecular and insular areas. The small and distended follicles contained a colloid-like secretion and were lined by low cuboidal cells with scant cytoplasm, round or oval hyperchromatic nuclei with fine chromatin. The solid areas, trabecular and insular structures were similar to those of follicular or papillary thyroid carcinomas. In addition, some of the neoplastic cells had clear nuclei with occasional grooves. The tumor was positive for cytokeratin (CK) 7, CK 19 and CD138, and negative for TTF-1, thyroglobulin, Hepar-1, Glypican-3, alpha-fetoprotein and neuroendocrine markers. A thyroid neoplasm was excluded clinically and by ultrasound and computed tomography. Although, the residual hepatic parenchyma was initially not cirrhotic, the patient eventually developed cryptogenic cirrhosis. The patient received adjuvant chemotherapy and died of metastatic disease 18 months after surgery. The thyroid-like pattern broadens the morphologic spectrum of cholangiocarcinoma.
Assuntos
Adenocarcinoma Folicular/patologia , Carcinoma/patologia , Colangiocarcinoma/patologia , Neoplasias Hepáticas/patologia , Neoplasias da Glândula Tireoide/patologia , Adulto , Neoplasias dos Ductos Biliares , Ductos Biliares Intra-Hepáticos , Biomarcadores Tumorais/análise , Carcinoma Papilar , Quimioterapia Adjuvante , Colangiocarcinoma/química , Colangiocarcinoma/tratamento farmacológico , Colangiocarcinoma/cirurgia , Evolução Fatal , Feminino , Hepatectomia , Humanos , Neoplasias Hepáticas/química , Neoplasias Hepáticas/tratamento farmacológico , Neoplasias Hepáticas/cirurgia , Imageamento por Ressonância Magnética , Câncer Papilífero da Tireoide , Resultado do Tratamento , Carga TumoralRESUMO
Hepatic inflammatory myofibroblastic tumors are uncommon low grade malignant neoplasms. They can be confused clinically and by imaging studies with abscess.
Assuntos
Neoplasias Hepáticas , Miofibroblastos , Neoplasias de Tecido Muscular , Actinas/análise , Biomarcadores Tumorais/análise , Biópsia , Feminino , Hepatectomia , Humanos , Imuno-Histoquímica , Neoplasias Hepáticas/química , Neoplasias Hepáticas/diagnóstico por imagem , Neoplasias Hepáticas/patologia , Neoplasias Hepáticas/cirurgia , Miofibroblastos/química , Miofibroblastos/patologia , Neoplasias de Tecido Muscular/química , Neoplasias de Tecido Muscular/diagnóstico por imagem , Neoplasias de Tecido Muscular/patologia , Neoplasias de Tecido Muscular/cirurgia , Tomografia Computadorizada por Raios X , Adulto JovemRESUMO
Autoimmune pancreatitis is part of the spectrum of IgG4-associated diseases. Its diagnostic criteria and histological subtypes have been formally proposed recently and although based on current data it has been suggested that there are differences in clinical presentation among populations, more research is needed to properly establish if this heterogeneity exists. In this paper, we describe 15 cases of autoimmune pancreatitis diagnosed at a Mexican centre of reference, all of them associated to the lymphoplasmocytic sclerosing pancreatitis variant. The mean age at the onset of symptoms was 47.5 ± 14.4 years, and 53% of patients were male. The main manifestations were weight loss (87%), obstructive jaundice (53%), and acute (27%) and chronic (27%) pancreatitis. Only 20% of patients had high IgG4 serum levels at the time of diagnosis. All patients receiving prednisone responded favourably, both in their pancreatic and extrapancreatic manifestations. Clinical manifestations of Mexican patients showed certain differences with respect to those usually reported.
RESUMO
Well established complications of essential thrombocythemia are multiple thrombohemorrhagic phenomena in various abdominal organs. We describe the case of a 22 year old man with essential thrombocythemia and thrombosis of the mesenteric and splenic veins as well as cavernomatous transformation of the portal vein. The patient also had a splenic infarction and a subphrenic hematoma. Additionally, he developed signs and symptoms of acute cholecystitis which in turn led to an open cholecystectomy. The gallbladder had a markedly thickened wall due to multiple recent and recanalized thrombi predominantly in subserosal veins. Only a few arteries were occluded by thrombi. A marked vascular proliferation in the subserosal connective tissue mimicking a hemangioma was most likely the result of collateral circulation. There was also a mild acute and chronic inflammatory infiltrate and edema in the lamina propria of the gallbladder. Hyperplasia of interstitial cells of Cajal in the lamina propria and between smooth muscle cells and proliferation of nerve trunks in the subserosal connective tissue adjacent to the thrombosed veins and arteries was also noted. To our knowledge this unique gallbladder thrombotic complication of essential thrombocythemia has not been previously reported.
Assuntos
Colecistite Aguda/etiologia , Vesícula Biliar/irrigação sanguínea , Trombocitemia Essencial/complicações , Trombose/etiologia , Colecistectomia , Colecistite Aguda/cirurgia , Vesícula Biliar/patologia , Vesícula Biliar/cirurgia , Doenças da Vesícula Biliar/etiologia , Doenças da Vesícula Biliar/patologia , Humanos , Masculino , Veias Mesentéricas/patologia , Veia Esplênica/patologia , Trombose/patologia , Adulto JovemRESUMO
We report 2 examples of inverted urothelial papillomas with a focal papillary pattern. Both patients, a 43-year-old man and a 13-year-old adolescent boy, presented with hematuria. In addition to the characteristic trabecular endophytic growth pattern and bland cytologic features, the tumors showed focal papillary architecture in the endophytic component. Because of this feature, both tumors were misinterpreted as papillary urothelial carcinoma with inverted pattern. However, the papillary fronds were similar to those of exophytic urothelial papilloma. They were lined by 3 to 8 layers of normal-appearing urothelial cells often covered by a continuous or discontinuous layer of superficial (umbrella) cells. Although follow-up is limited, the 2 patients have remained asymptomatic. The recognition of papillary structures in urothelial inverted papilloma broadens the morphological spectrum of this unusual benign urothelial neoplasm and complicates the microscopic interpretation of urothelial lesions with inverted growth patterns. Surgical pathologists should be aware of this unusual feature of inverted urothelial papilloma of the urinary bladder to avoid misinterpretation with urothelial carcinoma with an inverted pattern.
Assuntos
Carcinoma de Células de Transição/patologia , Papiloma Invertido/patologia , Neoplasias da Bexiga Urinária/patologia , Adolescente , Adulto , Erros de Diagnóstico , Hematúria/etiologia , Humanos , Imuno-Histoquímica , Masculino , Papiloma Invertido/complicações , Papiloma Invertido/metabolismo , Neoplasias da Bexiga Urinária/complicações , Neoplasias da Bexiga Urinária/metabolismoRESUMO
A Thirteen patients with primary endocrine neoplasm of the stomach were studied for 20 years. Six patients were male and 7 female with an age range of 33 to 77, mean age 57 years. Nine cases corresponded to well differentiated carcinoids and four to neuroendocrine carcinomas. Of the former, three were sporadic and six were associated with atrophic gastritis. These two forms of neoplasm showed important differences: those associated with atrophic gastritis had hypergastrinemia, all of the multiple small tumors confined to the corpus and fundus were well differentiated carcinoids associated with intestinal metaplasia and G cell hyperplasia in antrum and ECL cell hyperplasia in corpus and fundus. Tumors were clinically benign, with an excellent prognosis. All patients are currently alive with no evidence of neoplasm. In only one of these cases, antiparietal cell antibodies were documented; in three of them, extensive intestinal metaplasia probably due to Helicobacter pylori infection was found. In contrast, sporadic carcinoids were large isolated tumors originating in the antrum or corpus. Two patients died as a consequence of the neoplasm; all of them were moderately differentiated and in none of the cases we found evidence of endocrine hyperplasia. All were positive for generic endocrine markers and were focally positive to some of the specific hormone markers. Al four neuroendocrine carcinomas had a clinical course similar to that of gastric adenocarcinomas and were poorly differentiated large tumors. We conclude that gastric carcinoids associated with atrophic gastritis have an excellent prognosis. On the other hand, neuroendocrine carcinomas have a very poor prognosis with fatal outcome of patients. Sporadic carcinoids have an intermediate prognosis.