IgG4-related disease (IgG4-RD) is a systemic autoimmune disorder that has been defined in various organs. The disease is characterized by typical clinicopathological features including a dense lymphoplasmacytic infiltrate rich in IgG4 positive plasma cells, storiform fibrosis, obliterative phlebitis, and often an elevated serum IgG4 level. Esophageal IgG4-RD is rare, and its presentation as a solid mass is even more rare. Only 15 previous cases of IgG4-related esophageal disease have been described. We herein present a case of giant IgG4-related pseudotumor of the esophagus resected with endoscopic submucosal dissection (ESD) and a review of the literature. The patient was a 67-year-old man who was admitted to our hospital for assessment of progressive dysphagia. Upper gastrointestinal endoscopy revealed a 9 cm mass in the cervical esophagus. In the previous two hospitals, the patient’s mass could not be diagnosed despite repeated biopsies. Because of concerns regarding malignancy, endoscopic submucosal dissection was performed. Histopathological examination showed dense lymphoplasmacytic infiltration with predominant IgG4-positive plasma cells on a sclerotic background. The patient was diagnosed with IgG4-RD. During the follow-up, no residual mass was detected but the patient was diagnosed with lung adenocarcinoma. We present a unique case of giant IgG4-related pseudotumor of the esophagus. Resection with ESD of such a big mass of IgG4-RD in the esophageal region has never been reported before in the literature.
Neoplasms of the appendix are very rare. They usually show glandular or neuroendocrine differentiation, and when they both occur in the same area, it is called a "collision tumor." Low-grade mucinous neoplasms associated with appendiceal diverticula are also uncommon. The appendectomy specimen of a 60-year-old man contained dense and mucoid luminal content on the distal tip, and similarly a solid, yellow, lumen-obscuring tumor with a diameter of 1.5 cm at the base of the appendix was detected. Microscopically, there were three diverticula that comprised herniation of the mucosal layer through the appendiceal wall. Interestingly, all of the diverticula and the normal-appearing appendiceal wall were lined by adenomatous epithelium. The luminal portion had pools of mucin-containing, rare clusters of low-grade epithelium that gave rise to the diagnosis of a "low-grade mucinous neoplasm." The solid-appearing tumor was diagnosed as a "neuroendocrine neoplasm," and there was no transition zone between these two types of tumors. There are some cases that have been reported as low-grade mucinous neoplasms associated with appendicular diverticula and collision tumors consisting both mucinous neoplasms and carcinoid tumors in the literature; our case has a unique appearance with two different types of tumors both in the appendix wall and within multiple diverticula.
Neoplasms of the appendix are very rare. They usually show glandular or neuroendocrine differentiation, and when they both occur in the same area, it is called a “collision tumor.” Low-grade mucinous neoplasms associated with appendiceal diverticula are also uncommon. The appendectomy specimen of a 60-year-old man contained dense and mucoid luminal content on the distal tip, and similarly a solid, yellow, lumen-obscuring tumor with a diameter of 1.5 cm at the base of the appendix was detected. Microscopically, there were three diverticula that comprised herniation of the mucosal layer through the appendiceal wall. Interestingly, all of the diverticula and the normal-appearing appendiceal wall were lined by adenomatous epithelium. The luminal portion had pools of mucin-containing, rare clusters of low-grade epithelium that gave rise to the diagnosis of a “low-grade mucinous neoplasm.” The solid-appearing tumor was diagnosed as a “neuroendocrine neoplasm,” and there was no transition zone between these two types of tumors. There are some cases that have been reported as low-grade mucinous neoplasms associated with appendicular diverticula and collision tumors consisting both mucinous neoplasms and carcinoid tumors in the literature; our case has a unique appearance with two different types of tumors both in the appendix wall and within multiple diverticula.
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