Intraductal Papillary Adenocarcinoma Of The Bile Duct With Associated Invasive Neuroendocrine Component: Case Report

Casestudy Intraductal papillary neoplasm of the bile duct (IPNB) is a variant of bile duct carcinoma that is characterized by intraductal growth and better outcomes compared with common cholangiocarcinoma. IPNBs are mainly found in patients from Far Eastern countries, where hepatolithiasis and clonorchiasis are endemic. Approximately 40%-80% of IPNBs are associated with invasive carcinoma. The invasive carcinomas are most often tubular or mucinous type. Invasive neuroendocrine component has not been previously described. We present a case of 69 year-old African American male who presented with worsening abdominal pain and jaundice over few months. Computed tomography showed 4 cm mass near the porta hepatis with massive ductal dilatation. A fine needle aspiration of the mass was performed and a diagnosis of adenocarcinoma was made. Subsequently, the patient underwent Right Trisegmentectomy. Conclusion Histologic sections revealed intraductal papillary neoplasm in the bile duct lumina. The papillae were lined by columnar epithelial cells with delicate fibrovascular cores. The tumor cell nuclei were slightly elongated, hyperchromatic and basally located without significant multilayering. The cytoplasm was eosinophilic with occasional intestinal differentiation. Frank invasion of the stalk and underlying periductular tissues was identified. Interestingly; the invasive component had different cytological features than the intraductal component, it was composed of small to medium sized cells with granular cytoplasm. The nuclei were uniform and round with “salt and pepper” chromatin. The invasive component stained positively for the neuroendocrine markers (Synaptophysin, Chromogranin and CD56), whereas the intraductal component stained positively for CK7, CK20 and CDX2 and negative for neuroendocrine markers. Three years later, a recurrent tumor was found in the left lobe on imaging. Biopsy of the lesion showed metastatic neuroendocrine carcinoma. The intraductal papillary neoplasm with invasive neuroendocrine component is very unusual tumor, we think more studies are warranted to best evaluate the treatment options and the prognosis of such neoplasms.

Clinical outcomes of previously untreated patients with unresectable intrahepatic cholangiocarcinoma following proton beam therapy

Background The effectiveness of proton beam therapy (PBT) as initial treatment for patients with unresectable intrahepatic cholangiocarcinoma (ICC) is unclear, particularly as related to ICC histological subtypes. We performed this study to address this gap in knowledge. Methods Thirty-seven patients with unresectable ICC who underwent PBT as their initial treatment were evaluated. Twenty-seven patients had Child-Pugh class A liver function, 11 exhibited jaundice, and 10 had multiple tumors. Nineteen, 7, and 11 tumors were classified as mass forming (MF), periductal infiltrating (PI), and intraductal growth (IG) types, respectively, based on gross appearance in imaging studies. Patients were classified into the curative group (n = 25) and palliative group (n = 12) depending on whether the planning target volume covered all the macroscopic tumors. Results The 1- and 2-year overall survival rates were 60.3, and 41.4%, respectively; the median survival time (MST) was 15 months for all patients. The MSTs for curative and palliative groups were 25 and 7 months, respectively. Curative treatment and adjuvant chemotherapy significantly improved overall survival, while the presence of periductal infiltrating type tumors was a negative prognostic factor. In the curative group, the 1- and 2-year local control rates were 100 and 71.5%, respectively, while the 1-, and 2-year progression-free survival rates were 58.5, and 37.6%, respectively. No severe acute toxicities were observed. Three patients experienced grade 3 biliary tract infection, although it was unclear whether this was radiotherapy-related. Conclusion PBT may yield to improve survival and local tumor control among patients with unresectable ICC.

Resection for a Recurrent Mucus-Producing Cholangiocarcinoma After Surgery: a Case Report

Introduction Intraductal papillary neoplasm of the bile duct (IPNB) is a variant of bile duct carcinoma characterized by intraductal growth. There are few case reports and papers discussing treatment policy and prognosis for cases of postoperative relapse. Case Presentation The case subject was a female in her 40s. Due to IPNB with the primary tumor in hepatic left lobe, the patient underwent hepatic left lobe, caudate lobe, and bile duct resection, and biliary tract reconstruction at another institution. Four years later, near the cholangiojejunostomy, a localized recurrence was indicated. Though systemic metastasis was examined with CT, FDG-PET, distant metastasis was not confirmed. As it was believed that resection of the recurrent focal could lead to a long-term survival, the recurrent focal was resected. The resected specimen was a cystic mass with a film, and in a pathologic examination, lymph node tissue was not confirmed in the specimen, while intracystic papilloma of attached liver tissue without continuity was confirmed. The patient has not seen a relapse for 5 years and 5 months after the second surgery. Conclusion In a case of localized recurrence after the IPNB surgery, resection should be actively considered.

Mass-forming cholangiocarcinoma

Cholangiocarcinoma is the second most common primary liver cancers. It is arising from epithelial cells of the biliary tract. It has been categorized to intrahepatic and extrahepatic. The Intrahepatic orperipheral cholangiocarcinoma can be presented as mass-forming, periductal infiltrating and intraductal growth. Many patients of mass-forming cholangiocarcinoma have symptoms such as abdominal pain about 85% but some patients don’t have any symptoms. This is the difficult cancer to diagnose. While patients were having any symptom, the disease was an advanced stage (unresectable). The diagnostic tools for assess this disease are imaging modalities include ultrasound (US), computed tomography (CT) with contrast, magnetic resonance imaging (MRI) with contrast. However, the goal standard for confirm diagnosis is tissue pathology. This article showed a case presentation and reviewed the imaging appearance of mass-forming cholangiocarcinoma. Figure 1 Axial non-contrast (A), axial contrast enhanced in arterial phase (B), axial contrast enhanced in portal venous phase (C) and axial contrast enhanced in 5-minute delay phase (D) CT scans show a large ill-defined hypodense mass at hepatic segment 7/8, about 7.0x7.0x5.0 cm in APxLxH diameter, which has poor enhancement on arterial phase with gradual progressive enhancement on portal venous and 5-minute delay phase. Coronal contrast enhanced in portal venous phase CT scan (E) shows mass confined in peripheral area of right hepatic lobe with hepatic vein abutment.