RESUMO
AIM: To retrospectively evaluate the characteristic clinicopathological spectrum in patients with suspicion of IgG4-related disease (IgG4RD). METHODS: Winpath histology database from January 2011 to April 2018 identified all suspected IgG4RD cases wherein IgG4 immunohistochemistry was performed. The histology slides were reviewed to categorise cases into Boston criteria groups-highly suggestive of IgG4RD, probable IgG4RD and insufficient evidence. Information regarding clinical data, treatment received, follow-up and serum IgG4 levels was obtained from medical records and AllScripts Patient Administration System (APAS) clinical database. RESULTS: The study included 204 patients and the most common sites of biopsy/resection were pancreas and duodenum. The most common clinical presentation was fibroinflammatory lesion or mass/lump. On histology, 54/204 (26.47%) cases showed typical storiform fibrosis, 65/204 (32.64%) had >10 IgG4+ plasma cells per high power field and only one case showed thrombophlebitis (0.49%). There were 14/204 (6.78%) cases categorised as highly suggestive of IgG4RD; 8 of these showed high serum IgG4 levels and were managed clinically as true IgG4RD. CONCLUSION: Histological diagnosis of IgG4RD remains challenging, as not all characteristic features are always present especially in small biopsies. Due to the novelty of its experience, fear of over diagnosis in the context of malignancy and features overlapping with diseases of similar clinical scenario, diagnosis of IgG4RD has become more puzzling. Further multicentre clinical trials/studies are advisable.
Assuntos
Doenças Autoimunes , Doença Relacionada a Imunoglobulina G4 , Humanos , Doença Relacionada a Imunoglobulina G4/diagnóstico , Doença Relacionada a Imunoglobulina G4/patologia , Estudos Retrospectivos , Biópsia , Imunoglobulina G , Reino Unido/epidemiologia , Doenças Autoimunes/diagnósticoRESUMO
Malignancy arising within a gastric duplication cyst (GDC) is extremely rare; only 15 cases have been reported in the literature. We present a 70-year-old woman who was referred with a history of vague postprandial abdominal discomfort. Subsequent imaging identified a gastric cystic mass. A laparoscopic sleeve gastrectomy of a 90 × 60 × 60-mm cystic mass was performed. Histopathological examination showed the presence of a sarcomatoid carcinoma arising within a GDC. The patient, unfortunately, died 5 months after surgery with metastatic disease. To the best of our knowledge, this is the first case of sarcomatoid carcinoma arising within a GDC.
