Biliary papillomatosis.

Biliary papillomatosis (BP) is a rare disorder of the biliary tract characterized by the presence of multiple papillary adenomas spread along the biliary tree. Although benign, it carries a significant risk of malignant transformation. Due to low sensitivity and specificity of conventional radiologic modalities, the diagnosis as well as estimation of disease extent is difficult. Endoscopic ultrasound (EUS) and endoscopic retrograde cholangiopancreaticography (ERCP) are superior although direct peroral cholangioscopy (POC) is currently the most accurate diagnostic method. Mainly because it provides more detailed information and makes targeted histological diagnosis possible. The treatment of biliary papillomatosis consists of surgical resection, liver transplantation (LT) or a combination of both. Unfortunately, the recurrence rate after radical surgery without LT remains high due to the diffuse distribution of the disease.

Intraductal Papillary Neoplasm of the Bile Duct: Radiological Diagnosis of a Rare Entity: Case Series.

Background: Intraductal papillary neoplasm of the bile duct (IPNB) is a rare bile duct neoplasm characterized by an intraluminal papillary growth pattern in bile ducts and is considered a biliary counterpart of intraductal papillary mucinous neoplasm of the pancreas. Case description: We report here two cases. (1) A case of a 34-year-old woman who presented with complaints of pain in the abdomen, jaundice, and pruritus. Further radiological investigations revealed the possibility of an IPNB, which was confirmed on histopathology. (2) A case of a 61-year-old man who was a known case of Barrett's esophagus and presented with complaints of right upper abdomen and jaundice. Radiological investigations and histopathology further confirmed the diagnosis of an IPNB involving the extrahepatic bile ducts. The purpose of this article is to highlight the role of computed tomography (CT) and magnetic resonance imaging in reaching this challenging diagnosis. Conclusion: Although the diagnosis is not straightforward, imaging plays a great role in raising the possibility of an IPNB. The correct preoperative diagnosis is necessary for accurate surgical planning and resection. How to cite this article: Jain K. Intraductal Papillary Neoplasm of the Bile Duct: Radiological Diagnosis of a Rare Entity: Case Series. Euroasian J Hepato-Gastroenterol 2023;13(1):28-31.

Successful cholangioscopic electrocoagulation for biliary papillomatosis: Report covering six cases (with video) / Electrocoagulación colangioscópica exitosa para la papilomatosis biliar: Informe de 6 casos (con vídeo)

Background: Biliary papillomatosis is a rare type of papillomatosis. Depending on the location of the disease, radical surgical resection or liver transplants are the treatment of choice. These radical surgical options may be unrealistic in patients who are not surgical candidates or who are unwilling to consider surgery. Aims: To evaluate the effectiveness of endoscopic electrocoagulation for the biliary papillomatosis. Methods: In this case series, we report six patients with unresectable biliary papillomatosis who underwent cholangioscopic electrocoagulation using needle knife and their clinic follow up information. Results: After patients received cholangioscopy with electrocoagulation of the residual biliary papilloma, the daily T-tube drainage volume increased to 200–400ml with improvement in the drainage content and significant relief of clinical symptoms, such as jaundice and abdominal pain. Conclusion: This method of using electrocoagulation to directly target and destroy tumor tissue is a safe and effective alternative for those with unresectable disease, and this method has shown to enhance T-tube drainage volume and improve patients’ overall clinical symptoms.(AU)

Antecedentes: La papilomatosis biliar es un tipo raro de papilomatosis. Dependiendo de la localización de la enfermedad, la resección quirúrgica radical o los trasplantes de hígado son el tratamiento de elección. Estas opciones quirúrgicas radicales pueden ser poco realistas en pacientes que no son candidatos a la cirugía o que no están dispuestos a considerarla. Objetivos: Evaluar la eficacia de la electrocoagulación endoscópica para la papilomatosis biliar. Métodos: En esta serie de casos, informamos de 6 pacientes con papilomatosis biliar irresecable que fueron sometidos a electrocoagulación colangioscópica mediante bisturí de aguja, y de su información de seguimiento clínico. Resultados: Después de que los pacientes recibieran la colangioscopia con electrocoagulación del papiloma biliar residual, el volumen de drenaje diario del tubo en T aumentó a 200-400ml con mejora del contenido del drenaje y alivio significativo de los síntomas clínicos, como la ictericia y el dolor abdominal. Conclusión: Este método de uso de la electrocoagulación para apuntar directamente y destruir el tejido tumoral es una alternativa segura y eficaz para aquellos con enfermedad no resecable, y este método ha demostrado mejorar el volumen de drenaje del tubo T y mejorar los síntomas clínicos generales de los pacientes.(AU)

Unusual presentation of gallbladder papillomatosis without bile duct involvement: A case report.

The papillomatosis is a very rare benign pathology diagnosed histologically with a significant potential for malignant transformation. We report a case a 60-year-old female without comorbidity present the gallbladder papillomatosis without involvement of the intra or extra hepatic biliary tract. The interest in knowing the radiological aspect of this pathology and make the early diagnosis in order to oriented treatment.

Intraductal Papillary Neoplasm of the Bile Duct: A Rare Case of Intrahepatic Space-Occupying Lesion.

Intraductal papillary neoplasm of the bile duct (IPNB) is a rare tumor and is considered one of the precursor lesions for cholangiocarcinoma. Though relatively common in the far east countries, it is uncommon in the Indian population. A 67-year-old gentleman presented with vague upper abdominal pain with no history of fever, jaundice, melena, or hematemesis. An abdominal ultrasound showed a solid cystic lesion in the left lobe of the liver with upstream dilatation of bile ducts. Computed tomography and magnetic resonance imaging showed similar findings. With a differential diagnosis of intrahepatic cholangiocarcinoma, intraductal papillary neoplasm, and biliary cystadenoma, he underwent robotic-assisted left hepatectomy. Histopathology was suggestive of IPNB. Following surgery, he had an uneventful recovery and was advised for follow-up visits every six months. A clinical, radiological, and pathological profile of this rare tumor has been described here with a review of the existing literature.

Successful cholangioscopic electrocoagulation for biliary papillomatosis: Report covering six cases (with video).

BACKGROUND: Biliary papillomatosis is a rare type of papillomatosis. Depending on the location of the disease, radical surgical resection or liver transplants are the treatment of choice. These radical surgical options may be unrealistic in patients who are not surgical candidates or who are unwilling to consider surgery. AIMS: To evaluate the effectiveness of endoscopic electrocoagulation for the biliary papillomatosis. METHODS: In this case series, we report six patients with unresectable biliary papillomatosis who underwent cholangioscopic electrocoagulation using needle knife and their clinic follow up information. RESULTS: After patients received cholangioscopy with electrocoagulation of the residual biliary papilloma, the daily T-tube drainage volume increased to 200-400ml with improvement in the drainage content and significant relief of clinical symptoms, such as jaundice and abdominal pain. CONCLUSION: This method of using electrocoagulation to directly target and destroy tumor tissue is a safe and effective alternative for those with unresectable disease, and this method has shown to enhance T-tube drainage volume and improve patients' overall clinical symptoms.

Living donor liver transplantation for diffuse biliary papillomatosis with malignant change: A case report with 10-year follow-up.

Biliary papillomatosis is a rare disease of the biliary tract. In this study, we present a case of a male patient with biliary papillomatosis with malignant change who underwent living donor liver transplantation (LDLT). The 49-year-old man was admitted due to intrahepatic ductal mass, which was finally diagnosed as biliary papillomatosis. It was initially treated by photodynamic therapy, but LDLT was planned due to low efficacy of photodynamic therapy. LDLT was performed with a right liver graft from his son. The pathology report revealed intraductal papillary neoplasm with associated invasive carcinoma. He recovered uneventfully. Although the distal bile duct resection margin was free of tumor, the risk of tumor recurrence at the remnant intrahepatic bile duct still remained. Therefore, staged pylorus-preserving pancreatoduodenectomy was planned. However, after 3 months, he was readmitted due to retropancreatic abscess. It took 6 months to control the intractable retropancreatic abscess. Due to late development of retropancreatic abscess and its slow recovery, staged pancreatoduodenectomy was no longer considered. The patient recovered complete at 1 year posttransplant without any evidence of disease recurrence. After 5 years posttransplant, lung metastasis developed and pulmonary metastasectomy was performed three times. No further recurrence developed during the last 3 years to date, and the patient currently is alive, 10 years after the LDLT. Experience on our case and literature review suggest that patients with biliary papillomatosis with or without malignant transformation are selectively indicated for liver transplantation.

Surgical treatment of malignant biliary papillomatosis invading adjacent organs: A case report.

BACKGROUND: Biliary papillomatosis (BP) is a rare disease characterized by multiple papillary adenomas in the intrahepatic and extrahepatic biliary tree and has a high risk of malignant transformation. Early diagnosis and treatment for BP are challenges, as it spreads along the biliary tract. A radical resection with an adequate resection margin is advocated in patients with malignant BP. CASE SUMMARY: We report a case of BP with malignant transformation in a 52-year-old female patient who presented with emaciation, severe malnutrition, and a T-tube for biliary drainage that was placed for almost 2 years at the time she visited us. The enhanced magnetic resonance imaging of the upper abdomen revealed a neoplasm with a diameter of 15 cm located in the gallbladder area surrounding the common bile duct where a drainage tube was placed. It was the first case with malignant BP involving multiple organs and the abdominal wall and receiving a radical resection. Plastic surgeons helped close the incision by transferring the rectus muscle and external oblique muscle flap. A diagnosis of papillary carcinoma with diffuse malignant papillomatosis of the biliary duct was given. The postoperative course was uneventful and she was asymptomatic and in good physical condition at 35 mo postoperatively with appropriate chemotherapy. CONCLUSION: Aggressive surgical treatment with appropriate chemotherapy is advocated for patients with malignant BP even if multiple organ invasion is present.

Intraductal papillary neoplasm of the bile duct : case report and review of the literature.

A variant of bile duct carcinoma, intraductal papillary neoplasm of the bile duct (IPNB) is a rare disease mainly found in Eastern Asia which encompasses a spectrum of intraductal papillary growth occurring anywhere along the intrahepatic and/or extrahepatic biliary tree that carries a high potential for malignancy. We report the case of a patient with episodes of recurrent cholangitis that was diagnosed with IPNB, our clinical and diagnostic approach, the radiographic and endoscopic findings, the interventions used, while discussing the therapeutic options.

Biliary papillomatosis with malignant transformation: A case report and review of the literature.

Biliary papillomatosis is a rare benign tumor that possesses a high potential for malignant transformation, and is frequently misdiagnosed. In the present report, a case of biliary papillomatosis with malignant transformation in a 63-year-old female, with a 19-year history of cholelithiasis, is presented. The patient presented with right epigastric pain and magnetic resonance imaging revealed a diffuse mass in the right liver lobe, which extended through the common bile duct. Definitive preoperative diagnosis was unable to be established; however, following surgical resection of the tumor, a diagnosis of biliary papillomatosis with malignant transformation was confirmed by pathological examination. The patient was followed up seven months later and no recurrence was detected. A supplementary review of previously published literature is additionally presented.

Intrahepatic biliary papillomatosis associated with malignant transformation: report of two cases and review of the literature.

Biliary papillomatosis (BP) is a rare disease characterized by multiple numerous papillary adenomas in both the intrahepatic and extrahepatic biliary tree. Due to its high recurrence rate and frequent transformation to malignancy, BP should not be considered a benign disease, and a radical resection with an adequate resection margin is advocated in cases of localized intrahepatic biliary papillomatosis. Since BP is a rare disease and its clinical features and outcomes are not well known, it's really difficult to diagnose the disease before operation. We encountered two cases diagnosed as intrahepatic biliary papillomatosis postoperatively, and herein present the diagnostic difficulties and therapeutic options for this rare disease.

Benign papillomatosis of common bile duct in children: A rare case report.

In children benign neoplasms are rare events in the extrahepatic biliary ducts and scanty literary references on the subject are available. We are reporting a rare case of a 3-year-old female child having biliary papillomatosis in lower common bile duct.

Extrahepatic biliary papillomatosis in a child.

In children, benign neoplasms of extrahepatic biliary ducts are extremely rare. We report a case of 3 year old girl who presented with abdominal pain and jaundice for 6 months. The final diagnosis on histopathology was papillomatosis in lower common bile duct.

Intraductal papillary neoplasm of the bile duct.

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 areas, where hepatolithiasis and clonorchiasis are endemic. According to the immunohistochemical profiles of the mucin core proteins, IPNBs are classified into four types: pancreaticobiliary, intestinal, gastric, and oncocytic. Approximately 40%-80% of IPNBs contain a component of invasive carcinoma or tubular or mucinous adenocarcinoma, suggesting that IPNB is a disease with high potential for malignancy. It is difficult to make an accurate preoperative diagnosis because of IPNB's low incidence and the lack of specificity in its clinical manifestation. The most common abnormal preoperative imaging findings of IPNB are intraductal masses and the involvement of bile duct dilation. Simultaneous proximal and distal bile duct dilation can be detected in some cases, which has diagnostic significance. Cholangiography and cholangioscopy are needed to confirm the pathology and demonstrate the extent of the lesions. However, pathologic diagnosis by biopsy cannot reflect the actual stage in many cases because different foci may be of different stages and because mixed pathologic findings may exist in the same lesion. Surgical resection is the major treatment. Systematic cholangioscopy with staged biopsies and frozen sections is recommended during resection to ensure that no minor tumors are left and that curative resection is achieved. Staging, histologic subtype, curative resection and lymph node metastasis are factors affecting long-term survival.

A Case of Benign Biliary Stricture as a Complication of Photodynamic Therapy for Biliary Papillomatosis / 대한소화기내시경학회지

Biliary papillomatosis is a rare disease with a high risk of recurrence and malignant transformation. Therapeutic options include partial hepatectomy, Whipple's procedure and liver transplantation. If there is no surgical option left due to several reasons, local palliative procedures such as biliary stenting and drainage for the treatment of cholestasis are considered, but tumor growth cannot be influenced. Photodynamic therapy might be a new additional, palliative option for patients with biliary papillomatosis who are not eligible for surgery. Benign biliary stricture is a rare complication of photodynamic therapy. We report here a case of a 63-year-old male who developed benign biliary stricture after photodynamic therapy using the photosensitizer photofrin.

Papilomatose das vias biliares / Biliary papillomatosis

A papilomatose das vias biliares (PVB) é uma afecção rara, descrita inicialmente por Caroli, em 1959. Até o ano de 2001, apenas cerca de 50 casos foram descritos na literatura mundial. O diagnóstico da PVB dificilmente é realizado no pré-operatório, pois os achados de exames de imagem e endoscópicos quase sempre são inespecíficos, e muitas vezes apenas sugerem o diagnóstico de neoplasia das vias biliares. O objetivo da presente publicação é relatar um caso de PVB tratado por hepatectomia esquerda com o diagnóstico provável de colangiocarcinoma da confluência dos hepáticos. O diagnóstico definitivo de PVB só foi estabelecido após estudo anatomopatológico da peça cirúrgica. São comentados aspectos relacionados ao diagnóstico diferencial, às opões de tratamento propostas para a PVB, bem como o prognóstico dos pacientes submetidos à ressecção.

Biliary papillomatosis (BP) is a rare disease, initially described for Caroli, in 1959. Until the year of 2001, only about 50 cases had been described in world-wide literature. The diagnosis of the BP hardly is carried through in pre-operatory, therefore the findings of image examinations and endoscopies almost always are not specifics, and many times only suggest diagnosis of neoplasia of biliary ducts. The objective of present publication is to relate a case of BP treated for left hepatectomy with probable diagnosis of cholangiocarcionoma of hepatics ducts confluence. Definitive diagnosis of BP alone was established after anatomopathological study of surgical specimen. Aspects related to differential diagnosis, treatment options proposals for the BP, as well as prognostic of the patients submitted to surgery are commented.

Diagnosis and treatment for 23 cases of biliary papillomatosis / 中华内分泌外科杂志

Objective To investigate the diagnosis and treatment for biliary papillomatosis. Methods 23 patients who were pathologically diagnosed as biliary papillomatosis and treated in our hospital from Jan. 2003 to Mar. 2008 were enrolled in this study. Their ages, genders, clinical manifestations, diagnostic approaches,histopathological findings, intraoperative findings, treatments and prognosis were analyzed retrospectively. Recal manifestations were abdominal pain (83.3%) and obstructive jaundice (72.2%). All the patients were diagnosed pathologically through intraoperative frozen section. No patient was diagnosed preoperatively. Anatomically, solitary lesion was observed in 10 patients (43.5%) and multiple lesions in 13 patients (56.5%), with a median tumor size of 2.6 cm. Microscopically, carcinomatous change was found in one patient, mild atypical hyperplasia in three, and medium atypical hyperplasia in one. Therapies mainly included radical excision and palliative external drainage. All but one patient recovered well postoperatively. This patient had anastomotic leak. All 23 patients were followed up with a mean period of 34 months. During the follow-up, three patients died as a consequence of biliary malignancy, one patient had the recurrence and others without recurrence. Conclusions Biliary papillomatosis is very difficult to diagnose preoperatively and the definitive diagnosis mainly depends on intraoperative and postoperative pathologic examination. To obtain a better prognosis, management should be aimed at radical excision. Intraoperative cholangioscopic evaluation and frozen section are strongly recommended to guide the choice of appropriate therapeutic approach.

Double Intrahepatic and Extrahepatic Cholangiocarcinomas Arising from Biliary Papillomatosis: A Case Report

Biliary papillomatosis is a rare disease entity characterized by multiple papillary adenomas along the bile duct mucosa. It is widely accepted that the adenoma-carcinoma sequence represents the process by which most, if not all, cholangiocarcinomas of an intraductal-growing type arise. Interestingly, friable papillary projections easily detached from the primary site can be implanted into the other sites in the bile duct in suitable animal models, resulting in multiple tumors. A 76-year-old male was referred to our hospital due to intermittent abdominal discomfort. Imaging workups revealed two lesions: wall thickening in the proximal portion of the left interhepatic duct and abrupt narrowing of the distal common bile duct. A hepatopancreaticoduodenectomy was carried out and pathological analysis demonstrated a well-differentiated adenocarcinoma of the left hepatic duct and carcinoma in situ of the distal common bile duct on a background of biliary papillomatosis. Six days after the operation, the patient received a re-exploration due to ruptured mesenteric vessels. Unfortunately, 3 months after the initial operation, the patient died of aspiration pneumonia. Biliary papillomatosis can present with a broad spectrum of disease characteristics ranging from adenoma to, usually, low-grade adenocarcinoma. If there is a complete excision, and a favorable postoperative course is secured, excellent survival can be expected after surgery.

A case of photosensitivity reaction after photodynamic therapy for biliary papillomatosis / 대한내과학회지

Photodynamic therapy has been widely used in the treatment of various cancerous diseases. However, photodynamic therapy with a photosensitizer can cause a photosensitivity reaction on the skin. We report a case of a 64-year-old woman diagnosed with photosensitivity reaction after photodynamic therapy using the photosensitizer, porfimer. She presented to the emergency room with a skin rash on her face, neck, and both upper and lower extremities nine days after the photodynamic therapy. She denied any outdoor activity during that period. After administration of systemic corticosteroid and anti-histamine, her skin lesions resolved completely within seven days.

Endoscopic retrograde cholangiopancreatography in biliary papiliomatosis / 中华消化内镜杂志

Objective To evaluate the diagnostic and therapeutic effects of endoscopic retrograde cholangiopancreatography (ERCP) in biliary papillomatosis. Methods Data of 6 patients, who underwent ERCP and diagnosed as biliary papillomatosis from 2000 to 2008, were retrospectively analyzed. Results There were 3 males and 3 females, with the mean age of onset at 72.8 years (range 52-83 years). Recurrent cholangitis and jaundice were common presentations in all patients, with 5 patients having right upper abdominal pain and 3 others exhiting fever and algor. History of partial hepatectomy was observed in 2 patients. Endoscopic findings included dilated papillary orifice with mucin discharge in 5 patients and papillary-occupying lesion in 1 patient. Multiple filling defects in the lumen of the biliary system in dilated common bile duct were detected in all patients, accompanied with extra-hepatic ducts dilatation in 3, right intra-hepatic duct dilatation in 1, and major pancreatic duct dilation in 1. Of 6 patients, 5 underwent multiple ERCP, inclucling stents and endoscopic nasobiliary drainage (ENBD), and have survived for 10-30 months. Another 83-year-old patient underwent palliative endoscopic treatment with balloons, baskets and ENBD, but died of cholangitis 10 days after the procedure. Conclusion This case series reports the typical endoscopic findings of biliary papiliomatosis. For inoperable or postoperative recurrent patients, endoscopic palliative treatment is a safe, convenient and effective procedure.