Olfactory neuroblastoma (ONB, also called esthesioneuroblastoma) is a rare malignant tumor of neuroectodermal olfactory cells. We report a case of an undifferentiated ONB with unfavorable histology arising ectopically in the nasopharynx. The patient was a 15-year-old male who presented with a right-sided painful neck mass, nasal obstruction, and weight loss. Awareness of the ectopic ONBs, although exceedingly rare, is important when considering differential diagnoses of sinonasal tumors as treatment and prognosis may differ from other lesions.
Background: Mesencephalic astrocyte-derived neurotrophic factor (MANF) is an endoplasmic reticulum (ER) stress response protein that plays an important role in pancreatic functions. As both alcohol and ER stress response proteins are involved in the pathogenesis of pancreatitis, we sought to investigate the expression of MANF in chronic alcoholic pancreatitis (CAP) and chronic non-alcoholic pancreatitis (CNP). Methods: A cohort of chronic pancreatitis tissues was gathered from routine surgical pathology (n = 77) and autopsy (n = 10) cases and tissue microarrays were created. Sampled tissues were reviewed and designated as representing CAP (n = 15), CNP (n = 58), or normal pancreatic tissue (NPT) (n = 27). MANF immunohistochemistry (IHC) and digital image analysis were performed to obtain an estimation of tissue fibrosis and an optical density (OD) of MANF IHC in ducts and acini for each case. The averaged values for these variables among histologic designations were compared. Results: The amount of fibrous tissue of the combined CAP and CNP group (chronic alcoholic and non-alcoholic pancreatitis, CANP) exceeded that of the NPT group (70% vs. 34%, p < 0.0001). The MANF OD in ducts of CANP was significantly higher than that of NPT (0.19 vs. 0.10, p < 0.05). The MANF OD in ducts of CAP was significantly higher than that of CNP (0.27 vs. 0.17, p < 0.05). The MANF OD in acini of CAP was significantly lower than that in CNP (0.81 vs. 1.05, p < 0.05). Finally, there was a statistically significant positive relationship between the amount of fibrosis and MANF OD in ducts (p < 0.001). Conclusions: MANF expression was higher in ducts of CAP than CNP. In contrast, MANF expression in acini was lower in CAP than CNP and NPT. There was a positive correlation between fibrosis and MANF levels in the ducts.
Mucinous cystic neoplasms (MCNs) are uncommon cystic lesions that arise in the liver and biliary system (MCN-LBS) and the pancreas (MCN-P) and rarely arise from the extrahepatic biliary system. Histologically, these lesions are defined by the presence of variably mucin-producing epithelium with ovarian-like, hypercellular mesenchymal stroma. Herein, we present a case of extrahepatic MCN-LBS in a 51-year-old woman. This lesion arose from the cystic duct and was removed via laparoscopic cholecystectomy. Histologic examination confirmed the diagnosis. To our knowledge, this is the third case report of an MCN-LBS arising from the cystic duct in the English literature. In this article, we review clinical and histologic characteristics of MCNs and present two other reports of MCN-LBS of the cystic duct.
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