Painless Jaundice Research Articles

Incidental Finding of Liver Metastasis in a Case of Pancreatic Adenocarcinoma

Exocrine cancer of pancreas is the fourth leading cause of death in the United States. Surgical resection is the only curative treatment option. Contrast enhanced multidetector-row computer tomography (MDCT) is the current modality of choice for the detection of distant metastasis in pancreatic cancer. We present a first ever reported case of an incidental liver metastasis in a patient with pancreatic adenocarcinoma. The patient is a 73 year old Caucasian woman who presented with pruritus and painless jaundice. Past medical history was significant for Hypertension, and Type II Diabetes Mellitus but had no significant surgical history. Social history was negative for tobacco, alcohol, and illicit drug use. Family history was noncontributory. Review of system was negative. Liver chemistry revealed Aspartate Aminotransferase 104 U/L, Alanine Aminotransferase 98 U/L, Alkaline Phosphatase 176 U/L, Total Bilirubin 5.7 mg/dL and albumin 3.9 g/dL. Carbohydrate antigen 19-9 was elevated at 278 u/L. Endoscopic retrograde cholangiopancreatogram showed distal common bile duct stricture. Endoscopic ultrasound showed 25 mm pancreatic head mass. Fine needle biopsy of the mass showed adenocarcinoma. Contrast enhanced CT of pancreas and liver and PET /CT scan for staging was negative for distant metastasis. Case was discussed in multidisciplinary team. Patient underwent Whipple procedure. Surgical pathology was positive for poorly differentiated pancreatic ductal adenocarcinoma with perineural invasion. 6/28 regional lymph nodes were positive for malignancy. Lymph nodes involving celiac access and hepatic artery were negative. Intraoperatively liver parenchyma looked grossly abnormal with ultrasonographic findings of early fibrosis. Random wedge biopsy from lateral segment of the liver was positive for 2 mm pancreatic adenocarcinoma.This case clearly suggests that MDCT/PET-CT can miss distant small metastasis. A pilot study done in 2011 by Holzapfel and Reoser-Erken et.al. showed that diffusion weighted magnetic resonance imaging (DW-MRI) is better than MD-CT for detection of small metastatic lesion, with good histopathological correlation. Hence, we need more studies of same kind in future to consider DW-MRI as a part of staging workup in pancreatic lesions that could potentially change the management of pancreatic adenocarcinoma. In addition, it argues for implementation of neo-adjuvant chemotherapy in surgically resectable localized pancreatic adenocarcinoma.2910_A Figure 1. Contrast enhanced CT with pancreatic protocol. Pancreatic head mass (red arrow)2910_B Figure 2. H&E stain, 400 X original magnification.. Wedge Liver biopsy. Histology diagnostic of pancreatic adenocarcinoma.2910_C Figure 3. PET/CT scan. Pancreatic head mass with Fludeoxyglucose (FDG) uptake. (red arrow head)

Severe Cholestatic Jaundice: A Rare Adverse Reaction of Methimazole

Methimazole (MMI) is a widely used and generally well-tolerated antithyroid medication. Generally, adverse reactions to antithyroid drugs occur in less than 10% of patients being treated for hyperthyroidism. In this case report, we present a patient who developed hepatotoxicity from MMI treatment. A 51-year-old female with Hashimoto's thyroiditis and Graves' orbitopathy presented to the emergency room with painless jaundice. Her Graves' orbitopathy was refractory to orbital decompression surgery and steroids, thus she was started on MMI six weeks prior to presentation. On admission, her skin exam was significant for jaundice and her abdomen was nontender. Laboratory studies showed alanine aminotransferase (ALT) 1366 IU/L, aspartate aminotransferase (AST) 853 IU/L, total bilirubin 26.2 mg/dL, alkaline phosphatase 954 IU/L, and albumin 3.4 g/dL. Her coagulation profile was normal. Magnetic resonance cholangiopancreatography showed normal biliary tree anatomy without obstruction and computed tomography (CT) of her abdomen showed a normal-appearing liver. Extensive serologic work-up for other causes of liver disease including viral hepatitis, autoimmune hepatitis, primary biliary cholangitis, alpha-1 antitrypsin deficiency, and Wilson's disease were negative. Thus, the patient's liver disease was attributed to drug-induced liver injury from MMI. The drug was held throughout her hospitalization and she was started on ursodiol. ALT peaked at 1,847 IU/L, AST 1,028 IU/L, alkaline phosphatase 1,173 IU/L, and total bilirubin 34.6 mg/dL. Upon discharge one week later, ALT was 340 IU/L, AST 66 IU/L, alkaline phosphatase 466 IU/L, and total bilirubin 7.6 mg/dL. Hepatic synthetic function remained intact and the patient remained asymptomatic.There have been less than 30 case reports of MMI-induced liver injury reported in the literature. Propylthiouracil (PTU) is considered to be more hepatotoxic than methimazole, as PTU is known to cause hepatocellular necrosis. However, while there are case reports of MMI leading to severe hepatitis or liver failure, liver toxicity is not a widely acknowledged side effect of MMI. The mechanism of MMI-induced liver injury is unclear, but it appears to have potential to cause severe cholestasis and hepatitis. Fortunately, the drug-induced liver injury appears to be reversible, as demonstrated by this case in which liver enzymes decreased dramatically with cessation of MMI and administration of ursodiol.

Finding a Signet Ring in the Biliary System

65 year old female comes in with painless jaundice and weight loss of greater than 40 lbs in the past year, Patient found to have signet ring carcinoma of the stomach with metastasis to the ampulla of vater. SRCC in the ampulla of vater is a rare entity.1 While the incidence of gastric cancer is decreasing, the incidence of signet ring cell carcinoma (SRCC) is only increasing with about a 10-fold increase from 1970 to 2000.2 65 yo F with PMH of HTN, DM, non-ischemic cardiomyopathy s/p AICD 2013 (EF 45% 9/2015), CVA with expressive aphasia, cervical cancer s/p hysterectomy admitted for painless jaundice (total bili of 21) and unintentional weight loss (about 40lbs within the past year). CT abd/pelvis w/out contrast and RUQ ultrasound showed intrahepatic and extrahepatic biliary ductal dilatation (Image 1,2 and 3). EUS was significant for wall thickening in the entire body and antrum of the stomach with wall thickness measuring 14mm in the antrum and 19mm in the body; impression consistent with Linitis plastica or infiltrating gastric cancer. EUS guided FNA showed signet ring carcinoma of stomach with local metastasis to ampulla of vater. ERCP unable to be performed due to edematous mucosa; had percutaneous biliary drain placed through IR. Bili trended downward after placement of drain. She had surveillance CT chest and abdomen/pelvis w/ contrast for further staging (negative for any metastasis or lymphadenopathy). Patient was not a candidate for chemotherapy/RT/surgery. Only 26 cases previously have been described with SRCC in the ampulla of vater.1 Prognosis in early gastric cancer is better in SRCC compared to non SRCC gastric carcinomas while prognosis is less clear between SRCC and non-SRCC gastric cancers in advanced gastric cancer.2Prognosis with SRCC in the ampulla is even less clear, with >5 year survival reported in only 4/26 cases.1 Our patient was advanced gastric cancer of SRCC and given metastasis and poor functional status was not a candidate for further therapeutic measures.21365_A.tif Figure 1: No Caption available.1365_B.tif Figure 2: No Caption available.1365_C.tif Figure 3: No Caption available.

Pancreatic Retroperitoneal Fibrosis Masquerading as a Pancreatic Mass: A Diagnostic Quandary

Retroperitoneal fibrosis (RPF) is a cryptic condition affecting 0.1 per 100,000 patients per year. The pathophysiologic etiology is believed to be autoimmune in nature, resulting in fibrotic tissue deposition within multiple organs. This case highlights an unusual primary pancreatic manifestation of an already exceedingly rare disease. The patient is a 45 year-old male with no significant past medical history who presented with painless jaundice. Computerized tomography revealed a 3 cm pancreatic-head mass encasing the vasculature and ductal system. An endoscopic ultrasound with fine-needle-biopsies (EUS-FNB) of the mass and endoscopic retrograde cholangiopancreatography (ERCP) were performed. The EUS characterized the lesion as a 34 x 28 mm ill-defined heterogeneous mass with multiple collateral blood vessel in the pancreas, suggestive of underlying portal hypertension, correlating with an endoscopic finding of esophageal varices. On ERCP, the common bile duct was dilated to 14mm with a shelf-like distal stricture, treated with a 10 French, 5cm plastic biliary stent. Initial fine needle biopsy results, while inconclusive, could not rule out malignancy or autoimmune pancreatitis. Repeat EUS-FNB with 7 needle passes again returned with non-malignant findings. He subsequently underwent laparoscopic biopsy of the pancreatic mass which revealed sclerosing collagenous fibrosis, displaying lymphoplasmacytic inflammation, suggestive of RPF. Infectious and malignant etiologies for RPF were excluded. He was started on steroids with symptomatic improvement and mass stabilization on subsequent imaging. Through extensive review of the literature, RPF encasing the pancreas without ureteral or periaortic involvement has scarcely been documented. In this case, the mass effect of the fibrosis resulted in biliary obstruction and pre-sinusoidal portal hypertension, and highlights the difficulty in diagnosing an exceedingly rare condition masquerading as pancreatic malignancy. This patient had a protracted course involving multiple sub-specialties, numerous endoscopic procedures, and ultimately a diagnostic surgery. The seemingly elusive diagnosis of RPF conclusively requires histologic certainty prior to treatment. When finally diagnosed, steroids are the mainstay of therapy for RPF.1424_A.tif Figure 1: Dense fibrosis with keloid-type collagen fibers1424_B.tif Figure 2: PET scan showing avid FDG uptake at pancreatic lesion1424_C.tif Figure 3: No Caption available.

Cholangioscopy-Assisted Guidewire Placement in a Malignant Biliary Stricture

Cholangioscopy is an adjunct to fluoroscopic evaluation during endoscopic retrograde cholangiopancreatography (ERCP), with uses ranging from diagnostic evaluation of strictures to therapeutic lithotripsy of stones. However, cholangioscopy has not been described previously to assist guidewire placement in malignant biliary strictures. Here we describe a case of guidewire placement through a difficult malignant biliary stricture using single operator cholangioscopy. A 74-year-old female presented with painless jaundice and weight loss. Endoscopic ultrasound (EUS) and ERCP by other endoscopists demonstrated pancreatic adenocarcinoma with a markedly dilated cystic duct (CD) and common bile duct (CBD). The associated distal CBD stricture was initially dilated and stented. However, the patient persisted to have jaundice and subsequently developed cholangitis two days later, prompting a referral for a repeat ERCP. The previously-placed biliary stent migrated distally to the confluence of the markedly dilated cystic duct and high-grade CBD stricture (Figure 1). Despite using multiple hydrophilic guidewires, the stricture could not be traversed due to preferential wire passage into the dilated cystic duct. Cholangioscopy was then used to visualize the orifices of the CD and CBD stenosis (Figure 2), enabling the guidewire to be placed directly through the stricture into the proximal CBD (Figure 3). A covered metal stent was successfully placed in the CBD, resulting in the resolution of the patient's cholangitis and jaundice. Most biliary strictures are successfully traversed with standard techniques using hydrophilic guidewires during ERCP, but challenges can occur when the biliary tree consists of multiple strictures and sharp angulations. Cholangioscopy provides direct visualization of the ducts which can allow wire access to a selective duct, significantly increasing success rate and preventing repeat procedures. This method has been described previously in difficult anastomotic strictures in liver transplant patients, with technical success. To our knowledge, this is the first case to describe guidewire placement using cholangioscopy for a malignant biliary stricture. Cholangioscopy is an effective tool for accurate guidewire placement in difficult benign and malignant biliary strictures and can be considered in anatomically challenging patients.2118_A Figure 1. Dilated cystic duct with looped guidewire.2118_B Figure 2. Direct visualization of CBD stenosis with guidewire (arrow) and dilated cystic duct (asterisk).2118_C Figure 3. Successful placement of guidewire in CBD (arrow).

Pancreatic Head Mass: A Rare Manifestation of Granulomatosis With Polyangiitis

A solid pancreatic mass is found to be malignant 95% of the time, while inflammation accounts for the remaining cases. We report a case of granulomatosis with polyangiitis (GPA) presenting as an inflammatory mass initially suspected to be metastatic pancreatic cancer. This is a 73-year-old male who presented to the ED with weakness, fevers, night sweats, and weight loss. His medical history was significant for severe chronic sinusitis and nasal polyps. On physical examination, he was noted to have bilateral lower extremity nodular purple plaques with necrotic centers. Laboratory studies were significant for leukocytosis, mildly elevated alkaline phosphatase, normal kidney function, transaminases and bilirubin. CT imaging demonstrated a low-attenuation mass in the pancreatic head along with multiple cavitary pulmonary nodules without hepatic lesions. Our differential diagnoses included metastatic pancreatic cancer versus an autoimmune process. CA 19-9 and CEA were within normal limits. An autoimmune work-up showed negative ANA, positive RF, elevated WSR, elevated IgE, and an elevated C-ANCA. Skin biopsy yielded leukocytoclastic vasculitis with granular fluorescence of C3 and IgG. Biopsy of lung nodules revealed extensive necrosis with acute neutrophilic infiltrates, fibrin thrombi, multinucleated giant cells, consistent with GPA. He was started on high dose steroids. He was discharged with plans for repeat imaging and biopsy of the pancreatic mass. A pancreatic mass in an elderly patient is highly concerning for cancer, which was our primary differential diagnosis. The absence of hepatic involvement and normal tumor markers decreased the likelihood of a primary pancreatic cancer with lung metastases. Due to his history of recurrent sinusitis, nasal polyps, and skin lesions, an autoimmune etiology was explored. A positive C-ANCA coupled with the lung biopsy findings established the diagnosis of GPA, a very rare cause of pancreatic masses. Most cases detailing GI manifestations of GPA present with pancreatitis or painless jaundice, but few have reported pancreatic masses. Necrotizing vasculidities can mimic pancreatic carcinoma and should be considered in the differential diagnosis of atypical cases. It is important to distinguish a pancreatic mass caused by GPA from pancreatic cancer due to the significant differences in treatment and prognosis. More studies are needed to determine whether these pancreatic masses should be biopsied or followed with imaging.1406_A.tif Figure 1: ßLungßonstrating extensive necrosis with acute neutrophilic infiltrates, fibrin thrombi, and multinucleated giant cells.1406_B.tif Figure 2: Low attenuation mass anteriorly in the pancreatic head measuring approximately 2.0 cm. The margins of the mass are inseparable from posterior wall of the duodenal bulb.1406_C.tif Figure 3: Bilateral lower extremity lesions described as nodular purple plaques with necrotic centers.

Pancreatic Head Mass: A Textbook Case

Pancreatic Cancer is one of the deadliest malignancies, accounting for over 330,000 deaths per year. Unfortunately, due to its vague symptoms, the diagnosis is often initially missed. Thus it is important to be familiar with some of the classic findings associated with this malignancy. Here we report an interesting case of a patient with a textbook presentation of pancreatic cancer. A 51-year-old male with no significant past medical history presented with dehydration, jaundice, pruritus and loose, grey bowel movements (Image 1) that began 3 weeks prior to presentation. The patient reported severe pruritus leading to scratching and extensive excoriations involving his entire body. He reported 2-3 episodes of “explosive” clay-white diarrhea daily. He denied hematochezia, melena, nausea, vomiting, unexpected weight loss, fevers, and chills. He reported drinking 4 beers a night and has a 45-pack year smoking history. On physical exam, the patient was ill appearing with moderate scleral icterus and jaundice. Initial lab work revealed elevated alkaline phosphatase, total and direct bilirubin, and lipase levels. CT abdomen and pelvis showed significant intra- and extrahepatic biliary ductal dilatation with a distended gallbladder and dilated pancreatic duct known as the “double duct sign” (Image 1). ERCP was performed and a biliary stent was placed for symptomatic relief. Biopsy from this procedure was highly suspicious for pancreatic adenocarcinoma. Given lack of metastasis, the patient underwent a pancreaticoduodenectomy to remove the mass and plans were made chemoradiation. Pancreatic adenocarcinoma is a highly morbid condition that can present with painless jaundice, pruritus, and Thomas' Sign (Image 2). Thomas' sign is the production of silver stool from combination of acholic stool and melena. This is diagnostic for pancreatic cancer that also affects the ampulla of vater. 5 year survival for patients who undergo surgery is 34.5%. Treatment options include surgical resection of the tumor, chemotherapy, radiation therapy or a combination of all of these. Given the poor prognosis in these patients, early identification of signs and symptoms such as the triad seen in this patient is important in allowing for prompt diagnosis and initiation of treatment.1306_A.tif Figure 1: No Caption available.1306_B.tif Figure 2: No Caption available.

Painless Jaundice: A Rare Presentation of Uterine Leiomyosarcoma With Pancreatic Metastasis

Painless Jaundice: A Rare Presentation of Uterine Leiomyosarcoma With Pancreatic Metastasis

Signet Ring Cell Carcinoma of the Stomach Presenting With Painless Obstructive Jaundice: Do We Need to Think out of the Box?

Signet Ring Cell Carcinoma of the Stomach Presenting With Painless Obstructive Jaundice: Do We Need to Think out of the Box?

Idiopathic Intrahepatic Cholestasis Associated With Monoclonal Gammopathy of Undetermined Significance

Cholestasis is a common presenting symptom in various hematologic diseases. We present a patient who had non-obstructive jaundice without demonstrable malignant cell infiltration and intact intrahepatic bile ducts, which was related to monoclonal gammopathy of undetermined significance (MGUS). A 58- year- old male had history of painless jaundice, anorexia and weight lost for 2 months. He had dark urine and pale stool. The patient denied alcohol and medicine intake. Physical examination found unrecognized fever, pale conjunctiva, icteric sclera and splenic dullness on percussion. Otherwise were unremarkable. Laboratory workups were significant for hemoglobin of 8.9, a TB of 65.7, a DB of 34.1, a SGOT of 170, a SGPT of 109, a ALP of 144. Peripheral blood smear was compatible with cold-type AIHA. CT of the whole abdomen showed normal parenchyma of liver without intrahepatic duct and CBD dilatation. The spleen size was 11 cm. No intra-abdominal lymph node enlargement. Workups for liver disease including viral hepatitis (HAV, HBV, HCV, HEV), ANA, ASMA, and AMA were negative. No atypical cell was detected from bone marrow study. Serum free light chain was significantly high level for Kappa monoclonal gammopathy. Liver biopsy found mixed inflammatory cells infiltration in hepatic lobules and portal tract. There was no significant bile duct loss. No malignancy was detected. The diagnosis of idiopathic intrahepatic cholestasis associated with MGUS was made. Intravenous dexamethasone 20 mg/day was prescribed. The LFTs improved dramatically after treatment and was allowed to be discharged with prednisolone 60 mg/day. Most cases of paraneoplastic-related cholestasis are caused by cytokines-induced vanishing bile duct syndrome. However, liver biopsy results from our patient did not show significant decrease in number of bile duct. Cholestasis in our patient was likely caused by dysregulation of bile duct transporters insulted from inflammatory mediators. This phenomenon is called idiopathic intrahepatic cholestasis, which is extremely rare paraneoplastic event. To the best of our knowledge, our patient has been the first case reported in MGUS. Treatment with systemic steroids is highly effective. In conclusion, paraneoplastic syndrome presenting as idiopathic intrahepatic cholestasis is associated with MGUS. Systemic steroid is effective for the treatment of this condition.2399_A Figure 1. The hepatic lobuls showed severe cholestasis with feathery degeneration. There was mild mixed inflammatory cell infiltrate in hepatic lobules and portal tracts. Bile duct was observed without bile duct injury.2399_B Figure 2. Immunohistochemical study for CK7 demonstrated insignificant bile duct loss.2399_C Figure 3. The liver function test improved dramatically after steroids administration.

Treatment Dilemma: A Case of Hepatitis C Virus Autoimmune Hepatitis (HCV-AIH) Overlap Syndrome

Hepatitis C virus-autoimmune hepatitis (HCV-AIH) overlap syndrome is a challenging diagnosis that mandates clinical, immunological and histological manifestations of both diseases. Management is controversial and lacks consensus due to the discordant approach to treatment of each separate condition. A 57-year-old woman presented to the Emergency Room with a 2-day history of painless jaundice after her friends noticed her skin and eyes appeared yellow. At presentation her total bilirubin was 12.9 mg/dL and her direct bilirubin was 9.3 mg/dL. Her AST and ALT were 1631 and 1003 U/L, respectively, and her ALP was elevated to 183 U/L. Her Hep C Ab screen came back Grayzone with a reflex RNA PCR of 860,000 IU/mL. CT abdomen showed a normal-appearing liver without focal lesions, and a subsequent MRCP was negative for biliary obstruction. Ultrasound with Doppler was negative for any vascular occlusion. Further workup showed an elevated IgG to 2485 mg/dL, a positive ANA titer of 1:2560 and a positive anti-smooth muscle Ab to 1:320. The patient underwent a liver biopsy that showed lymphoplasmacytic infiltration along the portal ducts and within lobular parenchyma, consistent with autoimmune hepatitis and concomitant acute viral hepatitis vs. drug reaction. The patient denied recent ingestion of any hepatotoxic medications or alcohol. Her aggregate score based on the revised scoring system of the International Autoimmune Hepatitis Group was 17, placing her in the category of definite diagnosis. Prednisone was initiated prior to discharge and follow-up was arranged in clinic. At her follow-up appointment, her transaminitis had largely improved and her hyperbilirubinemia completely resolved. HCV-AIH overlap syndrome requires confirmation of diagnosis of each entity independently with a thorough workup. Management of this syndrome is controversial as treatment of one condition could exacerbate the other. One concept is to determine the predominant insult between the two at presentation and target this insult with initial therapy. This patient's histology lacked bile duct damage and steatohepatitis which is seen in acute HCV infection. Her immunologic markers combined with the biopsy results favored an autoimmune-predominant chronic HCV-AIH overlap syndrome, prompting initiation of immunosuppressive treatment with systemic steroids. The patient's subsequent rapid clinical improvement supports this strategy for initial treatment of HCV-AIH overlap syndrome.

Turmeric: An Unlikely Culprit of Drug Induced Liver Injury

Drug Induced Liver Injury (DILI) accounts for at least 10% of adults with liver enzyme elevation [1]. Numerous herbal dietary supplements have been identified as culprits of DILI with almost no reports on Turmeric herbal supplements, a commonly used supplement [2]. We report a case of DILI in a 78 year old Caucasian female presenting with painless jaundice for 1 week. She had no history of analgesic abuse, alcohol use, travel or ill contacts. Her home medications included: aspirin, citalopram, losartan, metformin, oxybutynin, and simvastatin. A month ago, she replaced the simvastatin with a turmeric herbal supplement. She had no other changes in her medications over the last year and has been on simvastatin for 2 years with no adverse effects. Her diagnostic evaluation revealed: (a)an elevation of her AST and ALT >10xULN with a bilirubin of 12.8 mg/dl and an ALP of 171 U/L (b)Normal globulin, IgG and IgG subclass levels (c)Normal MRCP (d)ANA titer of 1:320, ASMA of 1:20, negative anti—LKM and AMA, and (e)an exclusion of viral hepatitis as an etiology of her symptoms. Discontinuation of the supplement resulted in a 40% fall of ALT noted in 7 days from her presentation and a complete normalization of her liver enzyme levels in 42—days. No re—challenge was performed and a liver biopsy wasn't obtained given the rapid improvement in the patient's clinical condition. No liver enzyme elevation was noted on a 4—month follow—up. A RUCAM score was calculated for the supplement which was 8 out of 13 indicating a probable association (with scores >8 indicating a highly probable category; that is the highest association level on the score). This case illustrates the importance of recognizing turmeric herbal supplements as a potential agent causing DILI. It also stresses the importance of a detailed evaluation of all over the counter agents and supplements received by a patient by primary care providers to prevent inadvertent DILI that can lead to significant patient morbidity.

Autoimmune Pancreatitis.

Over the course of the last 2 decades our knowledge of autoimmune pancreatitis has increased exponentially. In this review, we summarize the clinical presentation, diagnosis and treatment of AIP, to better allow general gastroenterologists and primary care providers to consider AIP as a as a rare but important cause of painless obstructive jaundice and recurrent acute pancreatitis. While steroids remain the mainstay of first line therapy, a number of patients with type 1 AIP require immunomodulators or rituximab to maintain remission; recommendations on the management of relapses continue to evolve.

Metastatic Breast Cancer Presenting as Painless Jaundice

Jaundice carries with it a wide-ranging differential diagnosis, including etiologies relating to infection, autoimmune, toxins, malignancy, and metabolic derangements. We present a case involving a female with history of breast cancer and relapse-free interval of 15 years who presented with an initial complaint of painless jaundice and was unfortunately diagnosed with metastatic disease involving the liver, bone, and lymph nodes. A 56-year-old female presented to the emergency department for evaluation of painless jaundice, first noticed by family members one day prior to arrival. The patient has a history of breast cancer at age 36, treated with chemotherapy, radiation, and right mastectomy. Physical exam revealed jaundice and scleral icterus and a firm, non-tender liver, palpable beyond the costal border with a negative Murphy's sign. Laboratory findings were consistent with a cholestatic process. A right upper quadrant abdominal ultrasound revealed a 4.1 x 3.6 cm poorly defined mass in the right lobe of the liver, common bile duct measuring 8.6 mm, and intrahepatic ductal dilatation, suggestive of distal common bile duct obstruction. A follow up MRCP showed extensive hepatic metastases with innumerable liver lesions of varying shape and size. The patient underwent an ERCP, where a pancreatic duct stent was deployed but the common bile duct could not be cannulated. The patient subsequently underwent a hepatic lesion biopsy and placement of an internal/external biliary drainage catheter by Interventional Radiology secondary to complete, abrupt obstruction of the distal common bile duct. Biopsy results were positive for primary mammary malignancy. A review of the literature revealed only a select number of cases where patients presented with painless jaundice deemed secondary to metastatic breast cancer. This is the first such case in which the patient already had a known diagnosis of breast cancer, irrespective of prior treatment. In patients with malignant obstruction, biliary stenting has been shown to improve jaundice and deliver improved quality of life. In conclusion, in patients with known malignancy, the development of jaundice may indicate hepatic or biliary metastasis with bile duct obstruction. While a multidisciplinary approach to treatment is necessary, biliary stenting to relieve obstruction is a viable option for palliation.1364_A.tif Figure 1: Papanicolaou stain, (A, 40x) showing numerous large atypical atypical cells with high nuclear to cytoplasmic ratios. H&E stain (B, 10x) of the core biopsy showing this infiltrate within the liver parenchyma. Additional immunohistochemical stains show these cells are positive for GATA3 (C, 10x), GDCDFP-15 (D, 10x), mammaglobin (E, 10x), and ER (F, 10x).1364_B.tif Figure 2: MRI of abdomen: axial image showing extensive hepatic metastases with innumerable liver lesions of varying shape and size.

Response to R-CHOP in HPV-related squamous cell carcinoma of base of tongue: a case report

BackgroundSynchronous squamous cell carcinoma of the head and neck (HNSCC) and non-Hodgkin’s lymphoma is a rare clinical scenario. It is unknown whether the R-CHOP chemotherapy for lymphoma would also be active against HNSCC. Herein, we present such a case and a review of the literature.Case presentationA 64 year-old female presented with painless jaundice. CT demonstrated a retroperitoneal mass and pathology showed follicular lymphoma. A base-of-tongue HPV+ squamous cell carcinoma was found incidentally on staging CT. R-CHOP chemotherapy was initiated. After 3 cycles of R-CHOP the lymphoma had a complete metabolic response and, unexpectedly, the HNSCC also demonstrated excellent response. The patient received another 3 cycles followed by radiation to the HNSCC and to date is in remission for both cancers.ConclusionsThis case highlights the exquisite sensitivity of HPV-related HNSCC, which should be taken into consideration in treatment prioritization of a concurrent diagnosis of a second cancer.

An undifferentiated carcinoma at Klatskin-position with long-term complete remission after chemotherapy.

BackgroundNeoplasms anatomically adjacent to the bile duct usually derive from malignantly transformed cholangiocytes forming cholangiocarcinoma (CCA). CCAs are divided in extrahepatic (eCCA) and intrahepatic (iCCA) tumors. Patients with irresectable CCAs are treated with systemic chemotherapy and have an unfavorable prognosis with a median survival of about one year. Here, we report a case of an undifferentiated carcinoma in Klatskin-position with long-term remission after systemic chemotherapy.Case PresentationA 65-year-old Caucasian male presented with painless jaundice caused by an undifferentiated carcinoma in Klatskin-position (Type IIIb). Alpha fetoprotein (AFP; 3675 IU/mL) and carbohydrate antigen 19-9 (CA 19-9; 183 U/ml) were elevated. An exploratory laparotomy was carried out, but the patient was found to be irresectable due to severe fibrosis caused by biliary obstruction. Histology showed an undifferentiated carcinoma with high proliferation rate, and the patient was therefore subjected to poly-chemotherapy treatment according to the FOLFOX6-protocol. During therapy, AFP decreased to normal. Subsequent CT scans and ERC revealed a complete remission. Four years past initial diagnosis, a new suspicious lesion in the liver is visible on MRT; however, AFP and CA 19-9 are still in the normal range.ConclusionsOur case demonstrates that histopathological defined diagnosis may significantly inform therapeutic decision-making in irresectable cholangiocarcinoma even in regard to conventional systemic therapy. In case of an undifferentiated carcinoma poly-chemotherapy may provide significant success.

Mushroom Poisoning Mimicking Painless Progressive Jaundice: A Case Report with Review of the Literature.

Mushroom poisoning is common in the United States. The severity of mushroom poisoning may vary, depending on the geographic location, the amount of toxin delivered, and the genetic characteristics of the mushroom. Though they could have varied presentation, early identification with careful history could be helpful in triage. We present a case of a 69-year-old female of false morel mushroom poisoning leading to hepatotoxicity with painless jaundice and biochemical pancreatitis.

A rare variation of celiac trunk and hepatic artery complicating pancreaticoduodenectomy

Rationale:Anatomical variations of the celiac trunk and the hepatic artery are of considerable importance in hepatopancreatobiliary surgery, liver transplants, and radiological abdominal interventions.Patient concerns:Here, we report a 57-year-old man with 2 weeks of painless progressive jaundice. Preoperative imaging and cytology brush results suggested an ampullary tumor and common hepatic artery anomaly (CTA) was reported. The patient underwent pancreaticoduodenectomy (PD). Intraoperatively, the CHA and gastroduodenal artery (GDA) were abnormal. The CHA emerged from the superior mesenteric artery (SMA). Computer tomography angiography (CTA) was performed postoperatively; surprisingly, the left gastric artery (LGA) and splenic artery (SA) arising from the anterior wall of the abdominal aorta replaced the normal structure of the celiac trunk, and an accessory left hepatic artery (LHA) emerged from the LGA.Diagnoses:The patient was diagnosed with cholangiocarcinoma and accompanying extremely rare variation of celiac trunk and hepatic artery.Interventions and outcomes:The patient underwent PD and had an uneventful postoperative evolution. There was no recurrence of the tumor and with normal liver function during the 10-month follow-up.Interventions:The patient underwent PD and had an uneventful postoperative evolution.Outcomes:There was no recurrence of the tumor and with normal liver function during the 10-month follow-up.Lessons:Surgeons must keep in mind that arterial variation may be present in the vascular structures intraoperatively, even if it was not revealed in preoperative imaging. The preoperative identification of arterial variation and its relationship with the tumor is necessary to avoid intraoperative vascular injury and complications after surgery.

Painless Jaundice: A Happy Ending

Significantly elevated serum bilirubin can predict malignancy. The following case discusses a patient with a very high bilirubin and our search for its cause. A 58-year-old female presented with painless jaundice. There were no new medications, supplements or weight loss. Physical exam revealed jaundice and scleral icterus. Laboratory analysis showed a total bilirubin 40.1 mg/dL, AST 110 U/L, ALT 134 U/L and alkaline phosphatase 1665 U/L. Computed tomography revealed intra and extra-hepatic biliary ductal dilatation, tapering of the common bile duct (CBD) within the pancreatic head, and no pancreatic head mass. ERCP showed two stones in the CBD, the largest being 13.4 mm in diameter (Fig. 1, Fig. 2). Three biliary stents were placed to bypass the obstructing stones and liver tests subsequently normalized. Follow-up ERCP with cholangioscopy and electrohydraulic lithotripsy successfully removed the obstructing stones (Fig. 3) and the patient underwent cholecystectomy. Choledocholithiasis commonly presents with abdominal pain and elevated liver tests. These are often modestly elevated in a cholestatic pattern, but dramatic elevations of total bilirubin (>10 mg/dL) have been described1. Elevations of this magnitude are concerning for primary liver disease or malignancy, often leading to delays in diagnosis and extensive testing. Levels of bilirubin elevation in obstructive jaundice correlate with underlying pathology, and significant elevation (>11 mg/dL) may predict malignancy2. Painless choledocholithiasis occurs in about 24% of patients. It is more likely in patients >65 years old with diabetes mellitus, hypertension, atherosclerosis, coronary artery disease and/or cerebrovascular disease. It is associated with higher morbidity and mortality3. Multiple small (< 8mm) stones are more often found with painless presentation, whereas few (< 4) large stones (>8mm) may produce typical pain. Our patient's presentation was concerning for underlying neoplasm. Thankfully, her painless jaundice had a happy ending.Figure: ERCP showing an obstruction in the common bile duct.Figure: ERCP showing a large common bile duct stone (approximately 13.4 mm in diameter).Figure: ERCP showing free flowing contrast in the common bile duct after placement of three plastic biliary stents.

A Case of IgG 4 Seronegative Type I Autoimmune Pancreatitis

Introduction IgG related diseases (IgG4-RD) are rather distinct autoimmune gastrointestinal conditions including autoimmune pancreatitis (AIP) and sclerosing cholangitis. Patients present with organomegaly or mass, lymphadenopathy, obstructive jaundice and weight loss. The most common extra-pancreatic finding of Type 1 AIP is IgG4 Sclerosing Cholangitis which resembles Primary Sclerosing Cholangitis or cholangiocarcinoma. Case 67 y.o male with PMH of GERD, presented with 2 wk H/O generalized abdominal discomfort, weight loss, fatigue and painless jaundice. Physical Exam: Vitals WNL and positive scleral icterus. Elevated LFTs, bilirubin and Ca 19-9 were detected with normal CEA. Total IgG: 12.9 and IgG4: 0.67. Autoantibody screen negative. CT Abd/pelvis showed mediastinal lymphadenopathy, bulky pancreatic head along with tiny foci of calcifications throughout and evidence of intra and extra-hepatic biliary dilatation. MRCP showed widespread intra-hepatic duct dilatation suggestive of PSC. ERCP showed 3cm stricture in CBD and biliary stricture involving both right and left hepatic ducts. Biopsy ruled out malignancy. Patient showed clinical and lab normalization after steroid therapy which was confirmed by normal internal MRCP. Based on this clinical response and radiological evidence, a seronegative AIP with extra-pancreatic IgG4 related SC was diagnosed. Discussion Type 1 AIP, the prototype of IgG4RD is prevalent worldwide, typically affecting middle aged and older men. Sensitivity and specificity of seropositive AIP have been found to be as low as 62% and 59% respectively and studies show IgG4 seronegativity in as many as 24% cases. Despite lack of serological and histological evidence, AIP with IgG4 related SC can still be diagnosed based on history, imaging and therapeutic response as also suggested by International Consensus Diagnostic Criteria (ICDC). Conclusion: This case stresses the advantage of timely identification and treatment of seronegative IgG4 AIP and SC. Normal IgG4 levels and lack of definite histology shouldn't exclude the diagnosis when there are characteristic clinical findings and extra-pancreatic manifestations, along with distinctive imaging results. It is imperative to treat patients with steroid trial and close clinical monitoring to avoid unnecessary pancreatobiliary procedures.