bluuebunny/crossref_metadata_2025_split huggingface dataset:
2387 Desmoplastic Tumor: Rare and Advanced Presentation
Authors: Khudyakov Aleksandr , Arroyo-Mercado Fray , Sumbly Vikram , Ahmed Rafsan , Wang Donghai , Shao Charles , Frank David 
Published: October 2019

INTRODUCTION:
Desmoplastic small round cell tumors (DSRCT) are a rare and aggressive variant of soft-tissue sarcomas. Data suggest that occurs as a consequence of a translocation of (11;22) (p13; q12) resulting in a fusion of EWS/WT1 genes. The incidence of DSRCT is more common in young Caucasian males between the ages of 15 and 25 [6,7].

CASE DESCRIPTION/METHODS:
A 26-year-old male with no past medical history complaining of shortness of breath, anorexia, unintentional weight loss of 20 pounds and increased abdominal girth associated with pain starting two months prior to presentation. Physical exam was remarkable for cachexia, mild diffuse tenderness to palpation, protuberant abdomen without a distinct mass. Initial workup showed the following abnormality; normocytic anemia with hemoglobin of 12.6, ALP 189, AST 58, ALT 73 and HIV negative. Abdominopelvic CT scan revealed a large mass measuring 20 × 10 × 10 cm in the left upper quadrant, nodular deposits in the anterior abdomen concerning for carcinomatosis and multiple liver metastases, largest measuring 10 × 8 cm (Figure 1). Mesenteric lymph node biopsy showed findings consistent with desmoplastic fibromyxoid stroma and immunohistochemically positive for AE1/AE3, EMA, vimentin, desmin, synaptophysin, and CD99 (Figures 2–3).

DISCUSSION:
DSRCT's are aggressive soft tissue sarcomas that are extremely rare. They predominantly involve the pelvis, retroperitoneum, and mesentery. Upon diagnosis, it is crucial to exclude desmoid tumors and Ewing sarcomas, since, these malignancies that can be confused with DSRCT. Accumulating data suggest that is more prevalent in young adults, making the diagnosis challenging at the early stages, since, it can be potentially confused with less aggressive conditions such as gastritis, dyspepsia or gastroesophageal reflux disease. Prognosis is frequently poor given a late presentation of disease; five-year survival is estimated at 15 to 30% [2]. The exact mechanism of DSRCT is not well understood, but research suggest it arises from a translocation. Some promising therapies are undergoing phase trials as surgical interventions and hyperthermic intraperitoneal chemotherapy [2]. Systemic chemotherapy with aggressive surgical resection has shown improved outcomes [6]. Nonetheless, systemic therapy tends to be very toxic, requiring frequent admissions to control potential adverse events. Further research is needed to characterize the exact pathogenesis and treatment of this uncommon malignancy.
2388 Peliosis Hepatis Progressing to Acute Liver Failure
Authors: Engelberg Rachel , Mehta Amit , Wan David 
Published: October 2019

INTRODUCTION:
Peliosis hepatis is characterized as a vascular condition in which blood-filled cystic cavities are distributed diffusely throughout the liver parenchyma. The condition is rare, with the pathogenesis poorly described in the literature. It is believed that this disease is secondary to an underlying malignancy, immune disorder, infection, or a drug/toxin mediated effect. We present a case of a patient diagnosed with peliosis hepatis complicated by the development of acute liver failure.

CASE DESCRIPTION/METHODS:
A 71-year-old man with a past medical history of coronary artery disease, hypertension, hyperlipidemia, and prostate cancer treated with radiosurgery was admitted to our hospital with symptoms of fatigue, dysphagia and anorexia. He was in his usual state of health until four weeks prior to admission, when he began experiencing early satiety with poor oral intake, nausea, diarrhea, right upper quadrant abdominal pain, and weight loss. He was evaluated by his primary care provider who prescribed him a five day course of levofloxacin with no resolution of symptoms. Upon admission, labs were notable for leukocytosis and a mild transaminitis. Imaging revealed hepatomegaly with hepatic hemangiomatosis and splenic hemangiomas. A liver MRI was performed which returned consistent with diffuse hepatic peliosis with splenic peliosis. He underwent an intensive hematologic/oncologic, rheumatologic, and infectious workup that was significant for a positive quantiferon TB gold and he was subsequently started on empiric TB therapy. Synthetic liver function continued to worsen consistent with acute liver injury, which progressed to acute liver failure. The patient continued to decline and ultimately passed from multi-organ system failure.

DISCUSSION:
The majority of patients with peliosis hepatis will present asymptomatically and are found incidentally, while a subset of patients will present with abdominal pain or stigmata of liver disease. A liver biopsy is useful for diagnosis and is used in the context of the clinical picture, laboratory data, and imaging findings. Once the underlying offending etiology is recognized and appropriately treated, symptoms can improve and regression of the hepatic lesions can be seen. Our patient is unique in that his presentation was atypical and rapid, and a true causal etiology was never concretely identified, leading to progression to acute hepatic failure.
2391 When Breast Cancer Comes Back With Vengeance: Death Within Three Weeks Secondary to Acute Liver Failure
Authors: Rozner Raquel N. , Wan David 
Published: October 2019

INTRODUCTION:
Breast cancer (BC) is the most common cancer in women in the developed and developing world. Here we report a patient with treated stage II HER2+ BC presenting with abnormal liver enzymes and jaundice, found to have innumerable hepatic lesions consistent with metastatic BC, with rapid progression to acute liver failure(ALF).

CASE DESCRIPTION/METHODS:
A 63-year-old female with history of stage II HER2+ BC who underwent six cycles of neoadjuvant chemotherapy, lumpectomy, radiation, letrozole for four years, presented with one week of abdominal distension, dark urine, pale stools, and jaundice. She denied a history of hepatitis, intravenous drug use, or herbal supplement use but used marijuana and was a former smoker and heavy alcohol user. Six years prior to admission, two tubulovillous adenomas, 1.0 × 1.0 × 0.8 cm and 1.5 × 1.5 × 1.2 cm were resected. Surveillance colonoscopy, six months later, revealed a tubular adenoma, 0.2 × 0.2 × 0.2 cm and a tubulovillous adenoma, 1.2 × 1.2 × 1.2 cm. She missed her repeat surveillance colonoscopy. Physical examination was notable for jaundice, scleral icterus, hepatomegaly, and abdominal distension. Workup included negative hepatitis panel, platelet count 121,000/uL, total bilirubin 14.2mg/dL, direct bilirubin 10.7mg/dL, alkaline phosphatase 1367 IU/L, aspartate aminotransferase 621 U/L, alanine aminotransferase 252 U/L, albumin 1.5 g/dL, prothrombin time 14.7 seconds, activated partial thromboplastin time 41.5 seconds, international normal ratio 1.2, CA 15-3 3900 U/mL, CA 19-9 7200 U/mL, and CEA 844 U/mL. CT Abdomen/Pelvis revealed innumerable hepatic metastases, hepatomegaly, and small volume ascites (image 1). She underwent IR-guided biopsy of a hepatic lesion, confirmed as metastatic HER2 equivocal BC. Within one week, she became encephalopathic and died whilst deciding between hospice and systemic chemotherapy.

DISCUSSION:
BC represents about 30% of all cancer diagnoses in women each year. Of those diagnosed at early stages, 20-30% will relapse with metastatic BC. HER2+ BC has a higher risk of relapse than other subtypes, and is more likely to spread to the brain, liver, bone, and lung. This patient’s hepatic involvement was concerning for metastatic BC or colon cancer, based on past tubulovillous adenomas and missed surveillance colonoscopy. Within three weeks of presentation, she died. This case highlights the fulminant progression of HER2+ BC causing ALF due to tumor infiltration of liver parenchyma with cessation of synthetic capacity.
2394 Development of Hepatic Infarct After Successful Transjugular Intrahepatic Portosystemic Shunt (TIPS) Placement
Authors: Mavuram Meher S. , Herlevic Vincent , Samant Hrishikesh , Morris James D. , Pandit Sudha , Manas Kenneth J. , Jordan Paul 
Published: October 2019

INTRODUCTION:
Transjugular intrahepatic portosystemic shunt (TIPS) placement has now been well established as an effective and considered relatively safe treatment for the management of sequelae of portal hypertension. Hepatic infarction is an uncommon, potentially fatal complication after a TIPS procedure but rarely reported in cirrhosis patients with a low MELD (Model for End Stage Liver Disease). Here, we present a case of large hepatic infarct developing within a day of successful placement of TIPS in a patient with low MELD, which resolved with supportive treatment.

CASE DESCRIPTION/METHODS:
We present a 55 y/o Caucasian lady with Laennec cirrhosis with MELD 15 and normal bilirubin, who underwent elective TIPS placement for refractory ascites at our tertiary care center. Patient tolerated the procedure well with normal monitored vitals post procedure. However, she started complaining of right upper quadrant abdominal pain on next day with elevated transaminases and INR (40- fold and 2- fold increase from her baseline, respectively) with a stable bilirubin, CBC and creatinine. CT triple phase of the liver revealed a large hypodensity involving segments 6, 7 of the liver, suggestive of massive hepatic infarction and a patent TIPS. Patient not being a suitable candidate for liver transplant (with a history of recently diagnosed bladder carcinoma), was managed with supportive measures- Fluid, albumin, pain control and antibiotics. Liver enzymes continued to improve with return to baseline in 2 weeks. Subsequent clinic follow up showed complete control of ascites.

DISCUSSION:
Potential sequelae of TIPS are reported more frequently in cirrhotic with a MELD score greater than 18. Hepatic infarction is an uncommon development after a TIPS procedure. However, physicians must be aware of this virtually serious, post TIPS complication in cirrhotics even with a low MELD, which may progress to acute liver failure, with a need for liver transplant. Though our patient responded to supportive care, we think, an ideal patient planned for elective TIPS should be aware of this rare phenomenon. Prompt diagnosis is imperative to decrease the mortality from hepatic infarction.
2402 A Rare Case of IPEX Syndrome With Hepatic Complications
Authors: Ahmad Bareeqah , Masood Umair , Sharma Anuj , John Savio 
Published: October 2019

INTRODUCTION:
Immunodysregulation, polyendocrinopathy and enteropathy X-linked (IPEX) Syndrome is a rare autoimmune illness affecting only males starting in the early few months of life. It can be associated with autoimmune hepatitis among other systemic ailments and ultimate treatment is immunosuppression or bone marrow transplant. We present a case of undiagnosed IPEX Syndrome leading to liver failure requiring eventual liver transplantation.

CASE DESCRIPTION/METHODS:
A 19 year-old male patient had undergone a liver transplant at age 2 for suspected autoimmune hepatitis resulting in acute liver failure. No specific cause was identified at time of diagnosis. He remained on chronic immunosuppression thereafter, eventually progressing to chronic active autoimmune hepatitis in the transplanted liver. He was diagnosed with IPEX Syndrome at age 16, for which he underwent a bone marrow transplant. He presented to the hospital with neutropenia, and hepatology was consulted for liver transplant rejection in light of mild transaminitis. On admission, vital signs were normal and pertinent labs include: AST/ALT 50/74 units/L, Alkaline Phosphatase 811 IU/L, Albumin 3.4 g/dL, WBC 0.6 × 109 cells/L. He reportedly had multiple episodes of acute rejection in the past which were treated with steroids. Chronically, he was on mycophenolate mofetil, tacrolimus, and prednisone. His liver biopsy did not show evidence of acute rejection, but suggested chronic rejection and liver fibrosis. A prior liver biopsy had suggested autoimmune hepatitis, but no evidence of a flare on current biopsy. He was continued on his current immunosuppression regimen, and he was subsequently discharged home.

DISCUSSION:
Due to its rarity, incidence of IPEX syndrome is not entirely known, but is estimated to be 1 in 1,609,490. Generally, it is diagnosed in infancy and is often fatal. Our case is unique as the diagnosis was missed for more than a decade during which period the patient had even received a liver transplant for autoimmune hepatitis. Autoimmune hepatitis reportedly occurs in 20% of patients with IPEX Syndrome. There are currently no reports of liver failure requiring liver transplant in such scenarios. The fact that our patient developed recurrent autoimmune hepatitis in the transplanted liver highlights the underlying defect in his genetic disorder. This case should serve as a reminder to clinicians to consider IPEX syndrome in the differential for early onset autoimmune hepatitis, as early diagnosis could reduce significant morbidity.
2407 Clinical Challenges of Diagnosis and Management: A Case of Malignant Insulinoma With Hepatic Metastasis
Authors: Khan Adil , Lu Zheyu , Panghal Ritvika , Naeem Raza 
Published: October 2019

INTRODUCTION:
Insulinoma is a rare type of neuroendocrine tumor, about 4 cases per 1 million person-years. It can present at varied sites of body and metastasize extensively and progress rapidly. We present a case of rare and complicated duodenal insulinoma with significant hepatic metastases requiring integrated diagnostic workup and multidisciplinary management with specialties- gastroenterology, pulmonary, oncology, endocrinology and interventional radiology.

CASE DESCRIPTION/METHODS:
70 year old male with PMHx. of tobacco who presented with fall. When EMS arrived, patient was found to be hypoglycemic. Noted to have increased liver enzymes, CT of Abdomen showing multiple liver lesions and heterogenous lesion near or arising from the distal stomach/pylorus. Due to refractory hypoglycemia, he was started on D20W on a high rate. GI performed EGD which showed large penetrating duodenal ulcer at posterior wall of duodenal bulb and biopsy showed reactive changes but negative for intestinal metaplasia. IR guided Liver Biopsy was performed which showed Metastatic Neuroendocrine tumor, grade 3. Immunostains reveal neoplastic cells positive for synaptophysin. Ki67 show high proliferation (up to 50%). B-OH gastrin level, VIP, proinsulin were normal, insulin and c-peptide were interestingly low. Also, ACTH sim test was negative. Chromogranin was elevated. On repeat EGD with EUS and biopsy obtained directly from duodenal bulb was consistent with neuroendocrine tumor diagnosis. Also, Octreoscan scan did not display of Octreoscan uptake. Given this finding and the high KI-67 index, the hepatic metastases were thought likely somatostatin receptor negative. IR decided to perform embolization. IR angiography, showed very large hyper vascular tumor occupying the entire left and anterior right lobe of the liver and 2/3 left hepatic artery. It did persist despite left, right gastric artery and 2/3 left hepatic artery embolization. Later, patient was weaned off dextrose drip and blood sugars stabilized. Patient discharged home with follow up with IR, gastroenterology and Oncology team.

DISCUSSION:
This is rare case of hepatic metastatic insulinoma with paradoxical negative diagnostic parameters and atypical location, successfully diagnosed by EUS endoscope and liver biopsy and managed by multidisciplinary specialties.
2409 A Rare Complication of Gastric Bypass in the Setting of Chronic Liver Disease
Authors: Kaur Randeep , Naffouj Sandra , Jafri Syed-Mohammed 
Published: October 2019

INTRODUCTION:
Porto-mesenteric venous thrombosis (PMVT) is a known and potentially fatal complication of general surgery. However, it has been rarely reported as a complication of worsening liver disease after undergoing bariatric surgery. This is a case of a patient who presented with mesenteric thrombosis following Roux-en-y gastric bypass (RYGB).

CASE DESCRIPTION/METHODS:
Patient is a 42-year-old African American female with a history of RYGB who presented with episodes of post-operative abdominal pain for 5 years. She was found to have recurrent high-grade small bowel obstruction's (SBO) necessitating a small bowel resection. Her course was further complicated by intra-abdominal abscesses requiring computed tomography (CT)-guided drainage, and wound dehiscence requiring open surgical intervention and debridement. Four months after discharge, she presented with right-sided abdominal pain and acute encephalopathy. Liver enzymes were elevated. Abdominal ultrasound revealed portal vein thrombosis (PVT). Dual-phase CT of the abdomen revealed sub-acute to chronic complete diffuse mesenteric thrombosis with collateralizations involving the portal, splenic, and distal superior mesenteric veins. Given that previous imaging suggested steatosis and hepatomegaly, sub-acute liver injury secondary to thrombosis was established in the setting of chronic liver disease. The patient's underlying non-alcoholic fatty liver disease was likely worsened by severe malnutrition and short gut syndrome following the RYGB. Her hepatic encephalopathy resolved after initiating lactulose and rifaximin. Outpatient hematology evaluation for all causes of thrombophilia remained inconclusive and the patient had no recurrences of thrombosis.

DISCUSSION:
RYGB remains the gold standard for bariatric surgery as it results in the most durable weight loss. However, complications may occur such as gastrointestinal leak, fistulas, marginal ulcers, SBO, and dumping syndrome. Mechanisms of thrombosis formation in this setting could be related to intra-operative vascular manipulation, increased intra-abdominal pressure, venous stasis, and possibly deteriorating liver function secondary to rapid weight loss and malnutrition. PMVT hasn't been reported consistently as a complication in various bariatric surgery studies, which leads us to conclude that it's either under or mis-diagnosed. It is extremely important to consider PMVT as a complication of RYGB in patients who have evidence of underlying liver disease prior to undergoing RYGB.
2416 A Case of Fever, Rash, and Abdominal Pain in an Immunocompromised Host
Authors: Colbenson Gretchen , Kamboj Amrit K. , Dines Virginia 
Published: October 2019

INTRODUCTION:
Varicella Zoster Virus (VZV) is a DNA virus that leads to two clinical syndromes: varicella (primary) and herpes zoster or shingles (secondary). Disseminated infection is most common in immunocompromised hosts and can lead to multi-organ involvement including hepatitis, pneumonitis, encephalitis and others. Here, we describe a case of a young woman with human immunodeficiency virus (HIV) and primary varicella infection leading to VZV hepatitis.

CASE DESCRIPTION/METHODS:
A 35-year-old woman presented with a 1-week history of fevers, right upper quadrant and epigastric pain, and a non-pruritic, non-painful rash. Her past medical history was significant for a diagnosis of HIV 5 years prior, not currently on treatment. Upon presentation, vital signs were within normal limits except for a fever of 38.8°C. Physical examination was notable for multiple crusted erosions and papules present on her face, trunk and extremities (Figures 1 and 2). Laboratory evaluation was notable for alanine aminotransferase level 291 U/L, aspartate aminotransferase level 260 U/L, alkaline phosphatase level 135 U/L, total bilirubin 0.4 mg/dL, leukocytes 8 × 109/L, and platelet count 132 × 109/L. CD4 count was 118 cells/mcL and HIV-1 RNA 4,120,000 copies/mL. Computed tomography abdomen/pelvis demonstrated diffuse heterogeneity of the hepatic parenchyma (Figure 3). She underwent liver biopsy and pathology demonstrated hepatic parenchyma with portal and lobular inflammation but no necrosis. VZV polymerase chain reaction (PCR) from the liver and facial lesion swab returned positive. She was treated with 4 days of IV acyclovir with improvement in her fevers and transaminitis and then transitioned to oral valacyclovir. HAART was also initiated two days after the start of IV acyclovir.

DISCUSSION:
The differential for fever and widespread rash in an immunocompromised individual is broad, and includes disseminated herpes simplex virus, VZV, secondary syphilis, disseminated gonococcal infection, parvovirus, cryptococcosis and others. While uncomplicated VZV can be treated with supportive care or oral antivirals, such as acyclovir, valacyclovir, or famciclovir, an immunocompromised host with disseminated VZV should typically be admitted to the hospital for intravenous acyclovir. Biopsy proven VZV hepatitis is rare and is associated with a high mortality. Notably, our patient's VZV IgM and IgG were negative, indicating the need for a high degree of suspicion if the clinical scenario is consistent with VZV.
2418 Abdominal Wall Hematoma Due to Dissection of the Deep Circumflex Iliac Artery: A Rare Complication of Ultrasound Guided Paracentesis
Authors: Amarnath Shivantha , Daoud Magda , Mulrooney Stephen 
Published: October 2019

INTRODUCTION:
Aspiration of ascitic fluid via paracentesis is performed for both diagnostic and therapeutic purposes, using a landmark based approach (LBA) or the safer ultrasound guided method. Complication rate for paracentesis is less than 2% and LBA carries a nearly four-fold increase as opposed to the sonographic approach. Complications include bowel perforation, infection/peritonitis, and bleeding. It is well documented that hemorrhagic complications due to accidental transection of the inferior epigastric artery (IEA) or its branches can lead to abdominal wall hematomas, pseudoaneurysms and hemoperitoneum. We report a rare complicated case of accidental iatrogenic laceration of the deep circumflex iliac artery (DCIA) due to altered anatomy in a patient with ascites.

CASE DESCRIPTION/METHODS:
50-year-old female with decompensated alcoholic liver cirrhosis, coagulopathy and thrombocytopenia (Child-Pugh class C and MELD 29) presented with abdominal distention due to worsening abdominal ascites. Vitals were normal. Physical exam demonstrated fluid shift and mental status was intact. Laboratory findings reported thrombocytopenia of 118 K/uL, INR of 1.8 and Albumin of 2.2 g/dL. Ultrasound revealed extensive abdomino-pelvic ascites with the largest pocket of 19 cm in diameter in the right lower quadrant. She underwent sonographic diagnostic paracentesis of the right lower quadrant with drainage of 60 cc of serous fluid. Spontaneous bacterial peritonitis was ruled out. The next day, she endorsed sharp pain near the incision site and her hemoglobin dropped by 2 units. Patient was hypotensive and underwent CT abdominal angiography. This revealed a large right anterior abdominal wall hematoma measuring 24 cm × 9 cm with active arterial extravasation from the ascending branch of the right DCIA. She underwent angiographic transcatheter coil embolization of the DCIA. She remained stable and a repeat CT angiogram demonstrated reduced size of the hematoma and was discharged.

DISCUSSION:
The literature reveals that distribution of the abdominal wall vasculature, primarily the IEA is often displaced laterally in patients with ascites. Our case proves that this holds true for the DCIA, the second branch of the external iliac artery that travels along the iliac crest. Future studies should focus on incidence of the aberrant anatomy of abdominal wall vasculature, which might help clarify if pre-procedure precautions like the color doppler mode could possibly reduce risk of all paracentesis related morbidity and mortality.