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A case report of vanishing bile duct syndrome after exposure to pexidartinib (PLX3397) and paclitaxel.

ABSTRACT: Pexidartinib (PLX3397) is a small molecule tyrosine kinase and colony-stimulating factor-1 inhibitor with FDA breakthrough therapy designation for tenosynovial giant-cell tumor, and currently under study in several other tumor types, including breast cancer, non-Hodgkin's lymphoma, and glioblastoma. Here, we report a case of severe drug-induced liver injury requiring liver transplantation due to vanishing bile duct syndrome (VBDS) after exposure to pexidartinib in the I-SPY 2 Trial, a phase 2 multicenter randomized neoadjuvant chemotherapy trial in patients with Stage II-III breast cancer. We also review the current literature on this rare, idiosyncratic, and potentially life-threatening entity.

SUBMITTER: Piawah S

PROVIDER: S-EPMC6570645 | biostudies-literature | 2019

REPOSITORIES: biostudies-literature

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A case report of vanishing bile duct syndrome after exposure to pexidartinib (PLX3397) and paclitaxel.

Piawah Sorbarikor S Hyland Colby C Umetsu Sarah E SE Esserman Laura J LJ Rugo Hope S HS Chien A Jo AJ

NPJ breast cancer 20190614

Pexidartinib (PLX3397) is a small molecule tyrosine kinase and colony-stimulating factor-1 inhibitor with FDA breakthrough therapy designation for tenosynovial giant-cell tumor, and currently under study in several other tumor types, including breast cancer, non-Hodgkin's lymphoma, and glioblastoma. Here, we report a case of severe drug-induced liver injury requiring liver transplantation due to vanishing bile duct syndrome (VBDS) after exposure to pexidartinib in the I-SPY 2 Trial, a phase 2 mu ...[more]

PMID: 31240240

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Laparoscopic management of Rhabdomyosarcoma of common Bile duct, Case report

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| S-EPMC7519210 | biostudies-literature

Giant duodenal diverticulum with mucinous carcinoma of distal bile duct, mimmicking Lemmel syndrome: A rare case report.

Project description:IntroductionGiant duodenal diverticulum is a very rare case. There are only few cases reported. We reported a case of giant duodenal diverticulum with biliary obstruction caused by mucinous carcinoma of distal common bile duct (CBD), that mimicking Lemmel syndrome.Case presentationA 68-years-old man admitted to hospital with recurrent epigastric pain, jaundice and fever. Magnetic resonance cholangiopancreatography showed dilated intrahepatic and extrahepatic biliary tree, dilated gallbladder and cystic mass in pancreatic head that pushed the pancreatic duct ventrally. Emergency laparotomy was performed. Distended edematous gallbladder with necrotic spot, dilated of CBD and compressible bulging of the pancreatic head were found. Duodenotomy in 2nd-3rd part was made and found a giant duodenal diverticulum filled with food and mucus. Tight adhesion to the ampula of Vater, common bile duct, and pancreas due to fibrosis, met difficulties in dissection with a lot of bleeding, hence the diverticulum was not removed. Gastrojejunostomy, cholecystectomy and choledocho-duodenostomy were also done. Pathologic examination of CBD mucus was accordance with mucinous carcinoma.DiscussionPeriampullary duodenal diverticulum can cause obstructive jaundice, known as Lemmel syndrome. This case was different as the giant duodenal diverticulum located in the 3rd part filled with food and mucin that compressed both distal CBD and pancreatic duct. The cause of obstructive jaundice could be fibrotic tissue in distal CBD and mucinous carcinoma.ConclusionGiant duodenal diverticulum with bile obstruction is very rare and challenging in diagnosis and treatment. The other cause of obstruction should be considered such as mucinous carcinoma of distal CBD.

| S-EPMC8760494 | biostudies-literature

Phase Ib study of the combination of pexidartinib (PLX3397), a CSF-1R inhibitor, and paclitaxel in patients with advanced solid tumors.

Project description:PurposeTo evaluate the safety, recommended phase II dose (RP2D) and efficacy of pexidartinib, a colony stimulating factor receptor 1 (CSF-1R) inhibitor, in combination with weekly paclitaxel in patients with advanced solid tumors.Patients and methodsIn part 1 of this phase Ib study, 24 patients with advanced solid tumors received escalating doses of pexidartinib with weekly paclitaxel (80 mg/m2). Pexidartinib was administered at 600 mg/day in cohort 1. For subsequent cohorts, the dose was increased by ⩽50% using a standard 3+3 design. In part 2, 30 patients with metastatic solid tumors were enrolled to examine safety, tolerability and efficacy of the RP2D. Pharmacokinetics and biomarkers were also assessed.ResultsA total of 51 patients reported ≥1 adverse event(s) (AEs) that were at least possibly related to either study drug. Grade 3-4 AEs, including anemia (26%), neutropenia (22%), lymphopenia (19%), fatigue (15%), and hypertension (11%), were recorded in 38 patients (70%). In part 1, no maximum tolerated dose was achieved and 1600 mg/day was determined to be the RP2D. Of 38 patients evaluable for efficacy, 1 (3%) had complete response, 5 (13%) partial response, 13 (34%) stable disease, and 17 (45%) progressive disease. No drug-drug interactions were found. Plasma CSF-1 levels increased 1.6- to 53-fold, and CD14dim/CD16+ monocyte levels decreased by 57-100%.ConclusionsThe combination of pexidartinib and paclitaxel was generally well tolerated. RP2D for pexidartinib was 1600 mg/day. Pexidartinib blocked CSF-1R signaling, indicating potential for mitigating macrophage tumor infiltration.

| S-EPMC6589951 | biostudies-literature

Intrahepatic intraductal papillary cystic neoplasm of the bile duct: A case report.

Project description:Introduction and importanceIntraductal papillary neoplasm of the bile duct (IPNB) is a tumour with a very low incidence in the Western world, characterised by a high risk of malignant transformation and unknown prognosis. It is a new entity which was adopted by the WHO in 2010 as a precursor lesion of cholangiocarcinoma. Intrahepatic bile duct is the most common site of origin for IPNB.Case presentationHereby, we present a case of an asymptomatic 63- year-old man, referred to our department after routine ultrasonography showing a multifocal cystic lesion on the left hepatic lobe. Further screening modalities (CT, MRI abdo) confirmed a complex cystic liver lesion with atypical features. The patient underwent left hepatectomy. Histopathology showed a cystic type intrahepatic IPNB, which was completely resected (R0). The follow up in 2 yrs post-operation showed no signs of recurrence.Clinical discussionThe diagnosis and management of IPNB remain challenging. A multimodality imaging approach is essential in order to diagnose IPNB, assess tumour location and extent and plan the optimal treatment strategy.ConclusionComplete surgical resection (R0) with close postoperative follow-up offers long-term survival.

| S-EPMC7900680 | biostudies-literature

Repeat hepatectomy for liver metastases from bile duct neuroendocrine tumor: a case report.

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| S-EPMC7415062 | biostudies-literature

Small-cell neuroendocrine carcinoma arising from an extra-hepatic bile duct: a case report.

Project description: Not available

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Persistent Mullerian duct syndrome with transverse testicular ectopia: A case report.

Project description:Persistent Mullerian duct syndrome (PMDS) is a rare entity of internal male pseudohermaphroditism. Transverse testicular ectopia (TTE) is the condition in which one testis moves to the other side and both testes pass the same inguinal canal. The combination of PMDS with TTE is rarer. Here, we present a case a phenotype male with left inguinal hernia and right undescended testis. On exploration of left inguinal region, uterus like tissue with its tubal structures were found. Both testes were in same side, one in left inguinal region and the other in the left scrotum.

| S-EPMC6503297 | biostudies-literature

Long-term observation and treatment of a widespread intraductal papillary neoplasm of the bile duct extending from the intrapancreatic bile duct to the bilateral intrahepatic bile duct: A case report.

Project description:Few studies have reported the long-term outcomes of surgical resected intraductal papillary neoplasm of the bile duct (IPNB). Here, we describe the long-term observation and treatment of a case of widespread IPNB.A 57-year-old male was referred to our hospital due to jaundice and dilation of the intrahepatic bile duct. Computed tomography showed dilation and irregularities of the right intrahepatic and extrahepatic bile ducts together with a 3cm nodule in the common hepatic duct. Peroral cholangioscopy revealed mucinous discharge from the ampulla of Vater, which resulted in a diagnosis of IPNB. A biopsy of the nodule and the bile duct revealed papillary adenoma in all of them. Right hepatectomy, caudate lobectomy, extrahepatic bile duct resection, and left hepaticojejunostomy were performed. The nodule was histologically diagnosed as papillary carcinoma in situ, and R0 resection was performed. However, mucus production from the papillary adenoma in the B3 and B4 was observed. We carefully managed the patient's biliary tract by inserting a biliary drainage tube into the segment 2, and he has survived for more than 7 years since the initial treatment.Mucus might be produced after the surgical resection of IPNB even if s surgical margin was benign. Five-year survival rate of benign IPNB was reported from 85% to 100%. That might be caused by difference of the postoperative management of the biliary tract.Careful management of the biliary tract should be performed after surgical resection of IPNB.

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Laparoscopic completion total gastrectomy for remnant gastric cancer following pancreaticoduodenectomy for bile duct cancer: a case report.

Project description:Laparoscopic completion total gastrectomy following pancreaticoduodenectomy (PD) has not been reported. A 73-year-old male who underwent PD 25 years ago for distal common bile duct cancer visited a surgical department for remnant gastric cancer. A previous reconstruction was performed with pancreaticojejunostomy (PJ), gastrojejunostomy and Braun anastomosis, i.e., jejunojejunostomy (JJ), between the afferent and efferent jejunal limb to prevent bile reflux into the remnant stomach. Adhesiolysis was initially performed to secure the surgical view. Lymph node dissections around the splenic artery, splenic hilum, celiac axis, left gastric artery, and common hepatic artery were performed. The PJ site was well visualized and safely preserved. Esophagojejunostomy was performed with an OrVil system. Specimen retrieval, Roux-limb preparation and JJ were performed through an extended umbilicus trocar site. A final pathologic examination revealed a 5.5-cm serosa-exposed tumor (T4a) without lymph node metastasis. The patient was discharged on postoperative day 7 without any complications.

| S-EPMC4751144 | biostudies-literature

Pancreatogenic choledocholithiasis in common bile duct stump after Roux-en-Y hepaticojejunostomy: A case report.

Project description:Choledocholithiasis in common bile duct (CBD) stump after Roux-en-Y hepaticojejunostomy (RYHJ) is incredibly rare and its pathophysiology is poorly understood.A 79-year-old woman was admitted to our hospital with upper abdominal pain radiating through to the back in November 2016.Abdominal computed tomography (CT) scan and magnetic resonance cholangiopancreatography (MRCP) revealed filling defects in CBD stump, chronic pancreatitis, and dilatation of CBD stump and main pancreatic duct (MPD).During the endoscopic retrograde cholangiopancreatography (ERCP), cannulation proceeded easily from MPD to CBD through a variant pancreatic duct, and then white crushed stones extracted from the CBD stump. Elemental analysis and infrared spectrophotometry demonstrated that the main constituent of the calculi was calcium carbonate.After a therapeutic ERCP, the patient's symptoms disappeared, and a 9-month follow-up indicated no remaining stones or lithiasis relapse.This type of choledocholithiasis in CBD stump after RYHJ has never been reported before. We nominated it as "pancreatogenic choledocholithiasis," and pancreatobiliary reflux caused by a variant pancreatic duct may be the main cause.

| S-EPMC5704883 | biostudies-other

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