ABSTRACT
Intraductal papillary neoplasm of the bile duct (IPNB) is a rare bile duct neoplasm mostly found in far eastern nations where hepatolithiasis and clonorchiasis infections are endemic. In western countries, it is very rare and the etiology is unknown. In this article, we report the first IPNB patient we encountered in our clinic and a literature review. The patient is a 38-year-old female with a history of choledocholithiasis who presented with obstructive jaundice. She was found to have a papillary mass at the junction of the right hepatic duct and common hepatic duct with six masses in the liver parenchyma. The immunophenotypic and histologic features of the tumor are consistent with IPNB, gastric subtype. The patient had a partial hepatectomy and has been receiving palliative chemotherapy. In a search of PubMed database, we collected 354 IPNB patients reported in 22 articles. In these patients, 52.8% were from Japan and 27.7% were from western countries including the United States (11.0%). The age of the patients ranged from 35 to 80 years old with an average of 64.6. Male/female ratio was 1.5. Macroscopically, 57.5% of the tumors were in the left lobe and 29.5% were in the right lobe. The average size of the tumor were 4.2 cm at the time of diagnosis. Histologically, pancreato-biliary subtype accounted for 41.8%, intestinal 28.0%, gastric 13.5% and oncocytic 16%. An invasive component is most often present in the pancreato-biliary and gastric subtypes. Despite recent advanced technologies, diagnosis of IPNB is still challenging, especially in western countries due to its rarity. Defined clinico-pathologic features are in demand for the accurate diagnosis and proper treatment.
Subject(s)
Adenocarcinoma, Papillary/pathology , Bile Duct Neoplasms/pathology , Hepatic Duct, Common/pathology , Neoplasms, Multiple Primary/pathology , Adenocarcinoma, Papillary/chemistry , Adenocarcinoma, Papillary/therapy , Adult , Bile Duct Neoplasms/chemistry , Bile Duct Neoplasms/therapy , Biomarkers, Tumor/analysis , Biopsy , Chemotherapy, Adjuvant , Female , Hepatectomy , Hepatic Duct, Common/chemistry , Hepatic Duct, Common/surgery , Humans , Immunohistochemistry , Magnetic Resonance Imaging , Neoplasm Invasiveness , Neoplasms, Multiple Primary/chemistry , Neoplasms, Multiple Primary/therapy , Palliative Care , Tomography, X-Ray Computed , Treatment OutcomeABSTRACT
Nasolabial cysts are interesting, relatively uncommon benign extraosseous maxillary lesions. We review current knowledge about epidemiology, symptoms, imaging modalities, pathogenesis, histopathologic and ultrastructural features, treatment options, and prognosis. Nasolabial cyst lining epithelium is characteristically composed of a basal layer of cuboidal cells and a luminal layer of columnar secretory cells with interspersed mucous goblet cells. In addition, areas of multilayered epithelium and squamous metaplasia may be seen. The cyst stroma is characterized by collagen-rich fibrovascular tissue with variably admixed chronic inflammatory cells. Furthermore, to our knowledge, we report the first example of immunohistochemical protein expression profiling of nasolabial cyst lining epithelium, discovering that basal layer cells express p63 and cytokeratin 5/6, while goblet cells express MUC-2 and MUC-5AC mucins, supporting the notion that nasolabial cysts can be understood as hamartomatous, locally expansile remnants of distal nasolacrimal duct development.
