Multiple Hepatic Masses Research Articles

Hepatic Recurrence of Neuroendocrine Tumor Component in Gallbladder Cancer.

A 60-year-old man underwent an extended cholecystectomy with regional lymphadenectomy for T2N0M0 gallbladder cancer (GBC), which showed significant FDG uptake (SUVmax = 6.5). However, CT scan at 24 months postsurgery revealed multiple hepatic masses. Unlike the primary GBC, the hepatic masses showed limited FDG uptake (SUVmax = 3.7). A tumor biopsy revealed the diagnosis of a grade 3 neuroendocrine tumor. Furthermore, the hepatic masses showed significant uptake on somatostatin receptor scintigraphy. Upon reevaluation, the primary resected specimen was found to include approximately 10% of neuroendocrine tumor components. This case suggests that different FDG uptake between primary and metastatic cancer may necessitate differential diagnosis.

6590 Androgen Derived Hepatocellular Adenoma And Reinitiation Of Gender Affirming Hormone Therapy

Abstract Disclosure: A.L. McKenna: None. Y. Huang: None. L. Yang: None. N. Loo: None. C. Ritchie: None. A. Metcalfe: None. R. Nakhleh: None. M. Krishna: None. S. Thota-Kammili: None. J. Eleazer: None. A.Y. Chang: None. Hepatocellular adenomas (HCA) are uncommon, benign epithelial tumors of the liver. Risk factors include exogenous estrogen or androgen use, and genetic and metabolic syndromes. Management of HCA is delineated by sex assigned at birth; Observation is recommended in an HCA less than 5 cm in an individual assigned female at birth, while resection of an HCA in an individual assigned male at birth is recommended no matter the size due to risk of malignant transformation. What is not known is whether risk is different for individuals with gender incongruence on gender affirming hormone therapy (GAHT). There is limited data on the risk of HCA in this population. One case report of a transgender man who continued GAHT after diagnosis of an HCA though no follow-up information was available. We present follow-up on a transgender man assigned female at birth with multiple hemorrhagic HCA that was re-initiated on GAHT after stabilization of adenomas off GAHT. Unlike prior cases, the molecular profile showed ß-catenin negative, estrogen and androgen receptors positive. This is the first case report to our knowledge reporting detailed follow-up after restarting masculinizing GAHT after diagnosis of HCA. A 24-year-old transgender man presented to the emergency department with right upper quadrant pain. Initial labs were notable for ALT of 239 units per liter (U/L) and AST 165 U/L. Magnetic resonance imaging (MRI) of the abdomen and liver elastogram revealed multiple bilobar hepatic masses, the largest measuring 9.9 cm concerning for hemorrhagic HCA which was subsequently confirmed on liver biopsy. Immunostains were positive for estrogen and androgen receptors. The patient had no history of alcohol or illicit drug use, tattoos, or family history of liver disease. The patient had been treated with 100 mg of intramuscular testosterone cypionate weekly for the past six years for gender incongruence locally. No testosterone concentrations were available from prior to or during acute presentation. Discontinuation of hormone therapy was recommended along with conservative management with frequent MRI. Over the next 19 months, monitoring revealed improvement/resolution of the auto infarcting adenomas with the last MRI noting two stable HCA measuring 2.1 cm and 0.8 cm without new or enlarging liver lesions. Due to gender dysphoria off GAHT, reinitiation of hormone therapy with low dose AndroGel 1.62% topically was prescribed at 18 months after presentation. The patient has been monitored with labs monthly and plans to obtain MRI abdomen every three months. To date, the patient has tolerated reinitiation of therapy. This case demonstrates that patients on masculinizing GAHT who develop HCA can be conservatively managed and monitored based on pathology and that testosterone does not necessarily explain sex differences in the presentation/malignant transformation of HCA cisgender men as opposed to cisgender women. Presentation: 6/3/2024

Yolk sac tumor of the liver: Presentation, diagnosis, and treatment review.

e16371 Background: Yolk Sac Tumor of the Liver (YSTL) is an extremely rare extragonadal germ cell tumor with gonadal origin and histology which presents in the liver. While uncommon, this tumor is often misdiagnosed due to the similarities between YSTL, HCC, and Hepatoblastoma (HB). As this cancer can present with serious mortality, our review aimed to evaluate clinical findings, diagnostic workup, and treatments in patients with YSTL. Methods: We performed a literature search in PubMed from 1950 to October 2023 using filters English language and full text with keywords “Yolk sac tumor of the liver”, “Endodermal Sinus tumor of liver”, “Hepatic Yolk sac tumor”, and “Hepatic Endodermal sinus tumor”. We also conducted a combined search query with all these search terms and “primary”. Patients were included in the study if they had primary YSTL and no other malignancy. Results: Our results looked at 19 patients ranging from 15 months to 64 years with median age being 27 years, including 13 female (68%) and 6 male (32%) patients, percentages reflect the total number of patients. The key presenting symptom in adults was abdominal pain (55%), while the key presenting symptom in children was abdominal distention (63%). All patients in the study had a diagnostic workup that included imaging and pathology. Imaging included CT Abdomen (68%) and MRI (37%), in 58% of patients a solitary mass was seen on imaging, while in 42% of patients multiple hepatic masses were seen. Histology workup mainly noted Shiller Duval Bodies(SDBs)(76%). Molecular Markers included AFP(42%), Alpha-1 anti-trypsin(A1AT)(26%), SALL4(21%), Pancytokeratin(PK)(11%), and Placental-like alkaline phosphatase(PALP)(11%). Treatment included chemotherapy(58%), surgery(47%), transplant(11%), and trans-arterial chemoembolization(5%). Out of the 11 patients who were treated with chemotherapy, 91% received the bleomycin, etoposide, and cisplatin (BEP) regimen. The remaining 9 % of patients received a regimen of cisplatin, adriamycin, and pepleomycin. Conclusions: YSTL is a unique tumor presenting in patients of all ages, tending to present vaguely often with abdominal pain or distension. Diagnostic workup often includes abdominal imaging (MRI and CT Abdomen) and pathology with a focus on SDBs on histologic examination and stains like AFP, A1AT, SALL4, etc. Treatment often focuses on surgery and chemotherapy, notably BEP. Though both HCC and HB traditionally present as a multifocal liver mass on imaging along with positive staining for AFP and PK, our study helps differentiate this tumor from others by emphasizing the importance of SDBs in diagnosis and highlighting new molecular markers to help make the diagnosis easier.

PRIMARY HEPATIC NEOPLASIA IN THREE ADULT BAMBOO SHARKS (CHILOSCYLLIUM PLAGIOSUM AND CHILOSCYLLIUM PUNCTATUM) IN AN AQUARIUM POPULATION.

Neoplasia in elasmobranchs is uncommonly documented. This report describes primary hepatic neoplasia in three adult female bamboo sharks under managed care: biliary adenocarcinoma in a whitespotted bamboo shark (Case 1; Chiloscyllium plagiosum), biliary adenocarcinoma in a brownbanded bamboo shark (Case 2; Chiloscyllium punctatum), and hepatocellular carcinoma in a whitespotted bamboo shark (Case 3). Case 1 presented with extensive cutaneous papillomas and was electively euthanized. At necropsy, a 4-cm-diameter, pale-tan, firm hepatic mass was identified and diagnosed histologically as a biliary adenocarcinoma. Case 2 presented with decreasing body condition despite normal food intake. Coelomic ultrasound and exploratory surgery revealed several large masses in both hepatic lobes, and the patient was euthanized. At necropsy, nine, 1-6-cm-diameter, black to tan, firm hepatic masses were identified and diagnosed histologically as biliary adenocarcinoma with branchial intravascular neoplastic emboli. Case 3 presented for routine health examination and was euthanized for diagnostic purposes after coelomic ultrasound revealed multiple hepatic masses. At necropsy, two 1-3-cm-diameter, brown- and-black mottled, firm hepatic masses were identified and diagnosed histologically as hepatocellular carcinoma. Immunohistochemistry was performed in two of these cases and was noncontributory, likely because of a lack of cross reactivity between antibodies (antipancytokeratin) and elasmobranch tissues.

THU577 Acth Dependent Cushing Syndrome Identification And Management Challenges

Abstract Disclosure: B.C. Barreto: None. S. Patrick: None. H. Oktaei: None. H.O. Steinberg: None. Endogenous Cushing Syndrome(CS) is rare with an incidence of 0.2-5.0 per million people per year. 70 to 80% of CS is ACTH dependent and subclassified into Cushing Disease, Ectopic ACTH Syndrome and Ectopic CRH Syndrome. Ectopic ACTH secretion(EAS) has a variable clinical presentation. Atypical features like refractory hypokalemia in a patient with a tumoral lesion should raise suspicion. When imaging is inconclusive, differentiating between pituitary or ectopic disease includes invasive and noninvasive methods. We report a case of EAS. Case: A 57-year-old male with history of metastatic small-cell lung-cancer on palliative chemotherapy was admitted for acute coronary syndrome. Endocrinology evaluated the patient’s uncontrolled hypertension, hyperglycemia, and hypokalemia (1.5 mmol/L). He had no suppression with low dose dexamethasone (cortisol>75mcg/dL, ACTH 926pg/mL). Examination was pertinent for an elevated BMI 36.1kg/m2, hyperpigmented anterior thorax but no other stigmata of hypercortisolism. Laboratory analysis showed baseline cortisol 146.1mcg/dL(8.7-22), ACTH 590pg/ml(6-50), 24hr Urine Free Cortisol 736mcg/24h(5-50), which were not suppressed by high dose dexamethasone suppression test(HDDST). Aldosterone and PRA were undetectable. Brain MRI showed an enlarged pituitary gland, but no focal mass. CT abdomen pelvis showed bilateral adrenal hyperplasia, multiple hepatic masses, and bone lesions. Since the patient was a poor surgical candidate, medical therapy was initiated with ketoconazole 200mg TID, aldactone 50mg BID and potassium chloride 40mEq TID. After twelve days cortisol levels decrease to 65.2 mcg/dL, potassium 3.8 mmol/L and glucose 146 mg/dL. Hospitalization was complicated by a small bowel ileus. Medications were held due to poor gastrointestinal absorption and parenteral octreotide 200mcg TID was initiated. Once the ileus resolved, ketoconazole 200mg TID, eplerenone 50mg daily, and dexamethasone 0.5mg daily was started. Despite medical care, the patient eventually expired. Discussion: The HDDST can help distinguish pituitary from an ectopic ACTH overproduction. HDDST with >50% suppression, has been described with sensitivity-specificity of 60 to 100% for pituitary ACTH source. Symptomatic treatment of CS requires rapid normalization of hypercortisolism action. Surgery is the first line approach. When surgery is not an option, medical therapy should be considered. Adrenal steroidogenesis inhibitors, such as ketoconazole, are the first line due to rapid onset of action. Agents such as glucocorticoid receptor antagonists have limited availability in the inpatient setting. Somatostatin analogs and etomidate that inhibit 11-ßhydroxylase are potential options but require close monitoring. Severe hypercortisolism in the critically ill prompts the use of noninvasive evaluation tools and alternative medical approaches. Presentation Date: Thursday, June 15, 2023

Multiple Hepatic Masses in a Patient With Crohn’s Disease

Multiple Hepatic Masses in a Patient With Crohn’s Disease

Insulin therapy in a dog diagnosed with a metastatic insulin‐secreting neuroendocrine tumour

AbstractA 10‐year‐old, male, neutered Jack Russell terrier cross presented for a 24‐hour history of ataxia and collapse, progressing to generalised seizures in hospital at which a severe hypoglycaemia (2.9 mmol/L; 3.5–6.7) and hyperinsulinaemia (122 mU/L; 5–40 mU/L) were documented. Fine‐needle aspirates taken from multiple hepatic masses confirmed the diagnosis of a neuroendocrine neoplasm and probable metastatic insulinoma. Initial medical treatment consisted of intravenous dextrose 5%, glucagon continuous‐rate infusion, prednisolone, diazoxide, S‐adenosylmethionine and silybin, and toceranib phosphate. Hyperglycaemia (16.9 mmol/L; 3.5–6.7 mmol/L) was documented 157 days after commencing therapy with consistent clinical signs of diabetes mellitus. All medical treatments for insulinoma were discontinued; however, the dog's hyperglycaemia persisted. Therefore, caninsulin therapy commenced at a starting dose of 0.25 IU/kg, and was gradually increased to 0.6 IU/kg in the morning and 0.5 IU/kg in the evening. The dog was treated with insulin for 140 days, before relapsing hypoglycaemia and hyperinsulinaemia, with subsequent euthanasia 320 days after the initial diagnosis.

Anaplastic oligodendroglioma with nasal invasion and systemic metastasis in a dog.

An 11-year-old spayed female French bulldog was referred on suspicion of nasal tumor. Anaplastic oligodendroglioma in the olfactory bulb that was suspected to have invaded the nasal cavity was diagnosed from imaging and histopathology. Metastasis to cervical lymph nodes was suspected, with no other metastases identified. The brain-to-nasal lesion and lymph nodes were treated with hypo-fractionated radiation therapy. Nasal congestion soon resolved. About 3 months later, follow-up computed tomography revealed multiple hepatic and splenic masses, which were cytologically suspected as metastatic oligodendroglioma. Nimustine, followed by toceranib phosphate, seemed to have no effect, and the dog died on day 167. Postmortem examination revealed the primary tumor disappearance and systemic metastases. Canine oligodendroglioma can grow outside the cranial vault, and systemically metastasize.

Posterior Reversible Encephalopathy Syndrome: A Rare Side Effect of Nivolumab

Introduction: Posterior reversible encephalopathy syndrome (PRES) is a novel clinico-radiological syndrome which is characterized by variable symptoms including visual disturbances, headache, vomiting, seizures and altered consciousness, usually accompanied by hypertension. Several cases of immunotherapy-related PRES have been reported as a rare cause of PRES. We present a case of a 52-year-old male who was started on the immunotherapy nivolumab for his esophageal cancer. He came to the hospital with progressive vision loss and was diagnosed with PRES along with optic neuritis. Case Presentation: A 52-year-old male with no significant past medical history presented to the hospital with left sided numbness and spatial recognition issues. A computed tomography (CT) of the head revealed a 3 cm right parietal mass with vasogenic edema later confirmed with a magnetic resonance imaging (MRI) of the brain. He was started on steroids along with 500 mg Keppra twice a day. CT of the chest, abdomen, and pelvis showed multiple hepatic masses and retroperitoneal lymphadenopathy. He had a colonoscopy about a year prior that was unremarkable. He underwent right frontal parietal craniotomy for single brain metastatic lesion. The biopsy of both the brain and liver mass revealed metastatic adenocarcinoma. He was discharged and started on Fluorouracil, Folinic Acid, Oxaliplatin (FOLFOX) chemotherapy as outpatient for possible upper gastrointestinal (GI) cancer. After two cycles of chemotherapy, a positron emission tomography (PET) scan revealed suspected primary esophageal cancer. Though patient had zero PDL-1 biomarker, nivolumab was added to cycle 3 of FOLFOX with the expectation of having a better response early in the disease. He continued to have stereotactic radiosurgery (SRS) along with steroid taper for local control of brain metastasis. Nivolumab was held after cycle 2 as he developed mild headache, fever and elevated lipases. He was found to be hypertensive to 144/85 mmHg. MRI brain showed improving post-surgical changes. On improvement of symptoms, Nivolumab was reintroduced to cycle 6 of FOLFOX. Unfortunately, a week later he developed progressive vision loss more on right eye. He was sent to optometrist who referred patient to emergency room (ER) because of concern for bilateral papilledema. On admission to ER, he was hypertensive to 182/104 and tachycardic to 107 beats per minute. Physical exam was significant for left inferior quadrantanopia more prominent in right eye. CT head was unrevealing for any infarct. MRI of the orbit and brain showed increased patchy T 2 signal in bilateral parieto-occipital lobes without enhancement suggestive of posterior reversible encephalopathy syndrome (PRES) along with right optic neuritis. These findings were attributed to the immunotherapy nivolumab. He was started on a high dose of prednisone, 1mg/kg/day along with 20 mg lisinopril for strict blood pressure control with a goal of 120/80 mmHg. He was discharged with tapering steroids dose and his symptoms improved within 1 week of treatment. Discussion: Posterior reversible encephalopathy syndrome is a clinico-radiological diagnosis. The underlying pathophysiology involves hypertension and endothelial dysfunction. Hypertension leads to cerebral hyper-perfusion which may cause vascular damage and vasogenic edema leading to blood brain barrier dysfunction. The posterior circulation is the most susceptible to blood pressure changes, thus it is the most common imaging abnormality. PRES frequently develops in the context of cytotoxic medication, immunotherapy, (pre)eclampsia, sepsis, renal disease or autoimmune disorders. MRI is an important diagnostic tool and displays hyperintense lesions in T2-weighted or FLAIR sequences in the parietal-occipital lobes in about 70% of cases but other parts can also be involved. Electroencephalogram (EEG) and lumbar puncture (LP) can be done to rule out other causes. Differentiation is important from an ischemic event as management is different in both cases. Treatment of PRES is symptomatic. Recommendations are to avoid fluctuations in blood pressure with a goal of 130-150/80-100mmHg. If any triggering factor or condition is identified like immunotherapy, it should be avoided or treated accordingly. Figure 1View largeDownload PPTFigure 1View largeDownload PPT Close modal

Necrotizing hepatitis caused by Clostridium novyi type B in a dog with no predisposing liver lesions: a case report

BackgroundInfectious necrotic hepatitis (INH) is typically a disease of ruminants caused by Clostridium novyi type B. Growth of the causative agent is supported by development of an anaerobic environment within the liver. In dogs, C. novyi is rare and has only been previously reported as a post-mortem diagnosis. In one case, infection was secondary to metastatic pancreatic adenocarcinoma and the other was presumptively diagnosed on histopathology of a hepatic lesion in a dog initially presented for acute collapse.Case presentationAn 8-year-old spayed, female mixed breed dog was presented for acute onset of hyporexia and vomiting. Serum biochemistry revealed elevated hepatocellular injury and cholestatic liver enzymes. Ultrasound revealed peritoneal fluid accumulation and multiple hepatic masses. Cytologic examination of liver aspirates and peritoneal fluid revealed frequent 4 × 1 μm bacilli with a terminal endospore. Anaerobic bacterial growth isolated from the fluid sample could not be identified using typical laboratory identification techniques. Long-read, whole genome sequencing was performed, and the organism was identified as Clostridium novyi type B. Antimicrobial and hepatic support treatment were initiated. The patient re-presented 27 days later, and the follow up liver aspirate with cytology revealed no appreciable bacteria and anaerobic culture was negative. The patient was presented four months later and a large hepatic mass and peritoneal fluid were again identified on abdominal ultrasound. Cytologic examination of the peritoneal fluid revealed bacilli similar to those identified on initial presentation. The patient was euthanized. The most significant finding on necropsy was necrotizing hepatitis with intralesional endospore-forming bacilli compatible with recurrence of Clostridium novyi type B. There was no identifiable cause of an anaerobic insult to the liver.ConclusionsThis case demonstrates the diagnostic utility of using cytology as part of the initial diagnostic work up for infectious hepatitis. The cytologic findings coupled with whole genome sequencing and anaerobic culture were crucial for the identification and classification of the organism identified on fine needle aspirate. Clostridium novyi type B should be considered when bacilli organisms containing a terminal endospore are identified on liver aspirates collected from canine patients.

Diagnostic and public health investigation of Mycobacterium tuberculosis infection in a dog in Ontario, Canada

A 4-y-old, female mixed-breed dog was presented to the Ontario Veterinary College for further evaluation of multiple pulmonary and hepatic masses, intrathoracic lymphadenitis, and recent development of a pyogranulomatous pleural effusion. Along with other comprehensive tests, a thoracic lymph node biopsy was performed, and Mycobacterium tuberculosis complex infection was confirmed by real-time PCR. The dog’s condition declined post-operatively, and euthanasia was elected. Postmortem examination confirmed severe granulomatous pneumonia, hepatitis, intrathoracic and intraabdominal lymphadenitis, omentitis, and nephritis. Line-probe assays performed on samples collected postmortem confirmed the species as M. tuberculosis. 24-loci MIRU-VNTR genotyping, spoligotyping, and whole-genome sequencing revealed relations to known human isolates, but no epidemiologic link to these cases was investigated. Given the concern for potential human exposure during this animal’s disease course, a public health investigation was initiated; 45 individuals were tested for M. tuberculosis exposure, and no subsequent human infections related to this animal were identified. Our case highlights the need for more readily available, minimally invasive testing for the diagnosis of canine mycobacteriosis, and highlights the ability of canid species to act as potential contributors to the epidemiology of M. tuberculosis infections.

Liver Fibrosis Assessment Using Transient Elastography by FibroScan and Shear Wave Elastography by Sonography: A Comparative Cross-sectional Study in an Outpatient Liver Clinic

Background: Non-alcoholic fatty liver disease (NAFLD) is the second most common cause of liver transplantation in the United States, with a continuously growing prevalence. There are several non-invasive methods to detect liver fibrosis, which is defined as the accumulation of extracellular matrix proteins, particularly collagens. It is most commonly associated with chronic liver diseases, such as NAFLD. Objectives: This study aimed to investigate the concordance between transient elastography (TE) and shear wave elastography (SWE) for liver fibrosis staging and also to examine the congruence between the controlled attenuation parameter (CAP) and the B-mode hepatorenal ratio for hepatic steatosis grading in patients with NAFLD. Patients and Methods: In this cross-sectional study conducted during March 2018 - 2019, NAFLD patients, referred to the liver clinic of our center for the non-invasive assessment of hepatic fibrosis, were enrolled. However, patients with sonographic features of cirrhosis, multiple hepatic masses, or moderate to large ascites were excluded; also, patients who were uncooperative during the tests were excluded. Measurements obtained by different tools were recorded. Kolmogorov-Smirnov test, Chi-square test, independent t-test, or Mann-Whitney tests, as well as Pearson’s correlation coefficient test, were used to analyze the data. Results: Sixty-five patients (male-to-female ratio, 1:13), with a median age of 47 years, were included in the study. The tools for assessing fibrosis (r = 0.9538, 95% CI: 0.9252 - 0.9717, P < 0.0001) and steatosis (r = 0.429, 95% CI: 0.2048 - 0.6104, P < 0.0001) were perfectly and moderately correlated, respectively. Sex, age, and body mass index (BMI) did not affect the results. Conclusion: The two elastography modalities showed a strong correlation for fibrosis staging in our study population. Also, the CAP and B-mode hepatorenal ratio were moderately correlated for grading hepatosteatosis. Overall, selection of the best assessment method among the studied modalities depends on factors other than internal validity.

S1555 Stage IV Tail of the Pancreas Tumor Presenting With Obstructive Jaundice Secondary to Liver Metastasis

Introduction: Pancreatic cancer is an aggressive disease with a poor prognosis. It is most commonly found in the head of the pancreas and 20-25% in the body and tail. The clinical manifestations vary based on the location and spread of the tumor. Case Description/Methods: Our patient is a 55-year-old male with a past medical history of hypertension and nicotine use disorder who presented with dull epigastric abdominal pain of 3-month duration. The review of systems was significant for anorexia, unintentional weight loss of 40 pounds, tea-colored urine, and acholic stool. He was vitally stable. Physical examination showed scleral icterus and a diffusely tender abdomen without peritoneal signs. Laboratory work showed elevated AST, ALT, alkaline phosphatase, total bilirubin, direct bilirubin, and lipase. A computerized tomography (CT) scan of the abdomen revealed a mass in the pancreatic tail, multiple heterogeneous hypodense hepatic masses, and basilar lung nodular opacities. An abdominal ultrasound (US) showed ascites, multiple hepatic masses, intrahepatic biliary dilatation, and a common bile duct of 7 mm. US-guided liver and pancreas biopsies were performed. Magnetic resonance cholangiopancreatography (MRCP) revealed a 3.5 cm mass in the tail of the pancreas, multiple hepatic masses that were compressing the common hepatic duct, and retroperitoneal lymphadenopathy (Figure 1). Histopathology studies confirmed stage IV pancreatic adenocarcinoma with metastasis to the liver, lung, and peritoneum. Given the patient's extensive metastatic disease, he was not a candidate for chemotherapy or surgical intervention. Hospice and palliative care were pursued. Discussion: Tumors of the body and tail of the pancreas have been associated with poor outcomes with a 0-25% 5-year survival rate even after surgical resection. Its late presentation prompts an increased risk of distant lymphatic and hematogenous dissemination when compared with pancreatic head neoplasms. Consequently, patients with tumors originating in the head of the pancreas often present early with symptomatic obstructive jaundice. Interestingly, our patient presented with obstructive jaundice, but it was caused by intrahepatic cholestasis secondary to liver metastasis compressing the common hepatic duct. This case highlights that even as obstructive jaundice is most frequently related to pancreatic head malignancies, pancreatic body/tail neoplasms may have this clinical presentation in the presence of liver metastasis.Figure 1.: MRCP of abdomen showing a mass in the tail of the pancreas (Black arrow) and multiple hepatic masses (White arrow).

68. Hemorrhagic shock due to ruptured hepatic adenoma secondary to oral norethindrone acetate in a patient with type 3 von Willebrand disease: a case report

<h3>Background</h3> Patients with von Willebrand's disease (vWD) often require menstrual suppression in order to avoid episodes of heavy bleeding. While multiple hormone-based options are appropriate for these patients, specific side effects and clinical considerations based on the type and severity of vWD need further study. We discuss refinement of treatment options for menorrhagia for an adolescent with type 3 vWD who presented with a complication likely related to chronic progestin exposure. <h3>Case</h3> Patient C.D. was diagnosed with type 3 vWD in infancy and presented at 11 years-old for management of menorrhagia with a goal of amenorrhea. She was initially started on oral norethindrone acetate alone 3 times daily, which was continued for years to control breakthrough bleeding while receiving injectable progestins followed by the etonorgestrel-releasing implant. At 15 years-old, C.D. presented to the emergency room after 1 day of severe abdominal pain. She was found to be hypotensive with peritoneal signs on exam. CT demonstrated multiple hepatic masses, the largest being 9 cm with active contrast extravasation suggesting a bleeding adenoma with associated hemoperitoneum. She underwent an emergent right hepatic lobe embolization by interventional radiology. She recovered in the pediatric ICU with stabilization of her bleeding. The etiology of her hepatic adenomas was suspected to be chronic progestin use. After department discussions with hematology and gynecology, the oral norethindrone acetate was discontinued during her hospital course and she experienced withdrawal bleeding. On hospital day 13, the patient's contraceptive implant was removed and a levonorgestrel-releasing IUD was placed, which expelled 2 days later in the setting of heavy vaginal bleeding. C.D. received depot-medroxyprogesterone and was discharged home on daily recombinant VWF. At 1-month follow-up, the patient was amenorrheic and had stable hepatic masses on repeat imaging. The patient was maintained on injectable progestins with plans to trial a progestin IUD in the future. <h3>Comments</h3> Hepatic adenoma is a known complication of estrogen-containing medications and a rare side effect from peripheral conversion of oral progestins. A few case reports have shown regression of hepatic adenomas after cessation of progestin therapy. Furthermore, menses suppression for patients with severe vWD is critical to avoid menorrhagia. Patients with type 3 vWD particularly have limited pharmacologic options for preventing heavy menses. Through this case report, we discuss the management options for menstrual suppression and control of menorrhagia in patients with severe vWD with consideration of potential morbid side effects, and explore areas of further research of treatment regimens.

IMHOTEP: cross-professional evaluation of a three-dimensional virtual reality system for interactive surgical operation planning, tumor board discussion and immersive training for complex liver surgery in a head-mounted display

BackgroundVirtual reality (VR) with head-mounted displays (HMD) may improve medical training and patient care by improving display and integration of different types of information. The aim of this study was to evaluate among different healthcare professions the potential of an interactive and immersive VR environment for liver surgery that integrates all relevant patient data from different sources needed for planning and training of procedures.Methods3D-models of the liver, other abdominal organs, vessels, and tumors of a sample patient with multiple hepatic masses were created. 3D-models, clinical patient data, and other imaging data were visualized in a dedicated VR environment with an HMD (IMHOTEP). Users could interact with the data using head movements and a computer mouse. Structures of interest could be selected and viewed individually or grouped. IMHOTEP was evaluated in the context of preoperative planning and training of liver surgery and for the potential of broader surgical application. A standardized questionnaire was voluntarily answered by four groups (students, nurses, resident and attending surgeons).ResultsIn the evaluation by 158 participants (57 medical students, 35 resident surgeons, 13 attending surgeons and 53 nurses), 89.9% found the VR system agreeable to work with. Participants generally agreed that complex cases in particular could be assessed better (94.3%) and faster (84.8%) with VR than with traditional 2D display methods. The highest potential was seen in student training (87.3%), resident training (84.6%), and clinical routine use (80.3%). Least potential was seen in nursing training (54.8%).ConclusionsThe present study demonstrates that using VR with HMD to integrate all available patient data for the preoperative planning of hepatic resections is a viable concept. VR with HMD promises great potential to improve medical training and operation planning and thereby to achieve improvement in patient care.

S1734 Where's the Primary Site?

INTRODUCTION: Cancer of unknown primary site consist of around 5% of all malignancies. Initial workup frequently fails to detect the primary location. After light microscopic evaluation of a tissue biopsy, patients can be placed in one of four categories: neuroendocrine carcinoma, squamous cell carcinoma, adenocarcinomas, and poorly differentiated tumors. The diagnostic approach and treatment vary between these groups. We present a challenging case of liver metastasis with an unknown primary site in a patient with a past medical history of 2 primary malignant neoplasms. CASE DESCRIPTION/METHODS: An 86 y/o male with PMHX of alcohol abuse, tobacco use, thyroid cancer, and colon cancer presented to the ER with anorexia, dysphagia to solids, and 30lbs of unintentional weight loss for 6 months without any other symptoms. Chest CT revealed a 3 cm spiculated mass in the left upper lobe and multiple hepatic masses. Abdominopelvic CT revealed a nonspecific thickening of the duodenal bulb. Clinical findings suggested metastatic disease with an unknown primary site for which the gastroenterology service was consulted for a colonoscopy due to the patient’s history of colorectal cancer. Due to the patient’s advanced age and frail state, they recommended an interventional radiology consult for assessment of the patient’s liver lesions prior to scheduling a colonoscopy. Initial liver biopsy was positive for malignant cells and immunohistochemistry was positive for CK-7, CDX-2, CK19, Napsin A and TTF-1 and negative for CK 20 and Arginase 1, suggestive of primary lung adenocarcinoma with aberrant expression of CDX-2. DISCUSSION: This case presented a diagnostic challenge as the patient had a history of 2 primary neoplasms and workup showed 2 possible primary sites, lung versus gastrointestinal tract. Initial workup for metastatic cancer’s primary site must include a comprehensive medical history and a thorough physical exam, along with laboratory studies like basic chemistries, complete blood count, and imaging studies of the abdomen, pelvis, and chest. Pelvic examination and mammography might be warranted in female patients, whereas PSA and prostate exam should be considered in male patients. Appropriate tissue biopsy results help determine subsequent testing in order to establish the primary site and specific treatment. In this case, liver biopsy played a key role in the proper identification of the primary site and in avoiding unnecessary invasive workup such as endoscopic procedures in this frail elderly patient.Figure 1.: Chest CT with contrast showed 3 cm spiculated mass in the left upper lobe.Figure 2.: Non-specific thickening of the duodenal bulb showed in abdominal and pelvic CT.Figure 3.: Liver mass shown in CT scan suggesting metastatic disease.

99MTC-MAA HEPATIC PERFUSION IMAGING PRIOR TO 90Y RADIOEMBOLIZATION: HOW THORACIC UPTAKE CHANGES MANAGEMENT

SESSION TITLE: Medical Student/Resident Imaging Posters SESSION TYPE: Med Student/Res Case Rep Postr PRESENTED ON: October 18-21, 2020 INTRODUCTION: Targeted radioembolization with 90Y (yttrium 90) has shown a survival advantage in advanced stage hepatocellular carcinoma1. Interventional radiologists perform this procedure, however nuclear, thoracic, and abdominal imagers play a key role in determining candidacy for this treatment via interpretation of 99mTc-MAA (macroaggregated albumin) hepatic perfusion imaging and accompanying CT scans2. It is critical for pulmonologists and thoracic radiologists to recognize normal and abnormal studies to avoid potential deadly complications in patients who are poor candidates for this therapy. CASE PRESENTATION: The patient is a 70-year-old male who presented with abdominal pain and was found to have multiple hepatic masses and bile duct dilation, which on biopsy was consistent with aggressive multifocal hepatocellular carcinoma. Additionally, he was found to have a partial portal vein thrombosis. Because of the extent of the disease he was deemed not to be a surgical candidate and therefore was referred for evaluation for potential 90Y chemoembolization. To evaluate candidacy, the patient underwent 99mTc-MAA hepatic perfusion imaging with chest and abdominopelvic CT scans. Results are shown in Figure 1. Scintigraphy demonstrated a pulmonary shunt fraction of 80%. DISCUSSION: It is essential to recognize that these results are highly abnormal and an absolute contraindication to 90Y chemoembolization. 90Y undergoes B-decay which can result in radiation pneumonitis when spheres containing the radiopharmaceutical lodge in the pulmonary capillaries. For 90Y sphere embolization, <10% lung shunting is considered acceptable for therapy. For patients with shunt fractions <10-20%, a reduction of spheres delivered is necessary, and shunts >20% are a contraindication to the procedure3. Notably, portal vein thrombosis is not a contraindication however should be considered in planning. CT and angiography are key tools for identifying the shunt. CONCLUSIONS: 90Y embolization may be a helpful tool to attenuate the progression of hepatocellular carcinoma, but carries the risk of dangerous radiation pneumonitis in patients with significant pulmonary shunts. It is essential for clinicians to be aware of the potential danger and able to recognize abnormalities on preprocedural imaging studies to prevent harming the patient. Reference #1: Jordi Bruix, Margarita Sala, Josep M. Llovet. Chemoembolization for hepatocellular carcinoma. Gastroenterology; Volume 127, Issue 5, Supplement 1, 2004, S179-S188, https://doi.org/10.1053/j.gastro.2004.09.032. Reference #2: Kao, Y., Steinberg, J.D., Tay, Y. et al. Post-radioembolization yttrium-90 PET/CT - part 1: diagnostic reporting. EJNMMI Res 3, 56 (2013). https://doi.org/10.1186/2191-219X-3-56. Reference #3: Livnat Uliel, Henry D. Royal, Michael D. Darcy, Darryl A. Zuckerman, Akash Sharma and Nael E. Saad. From the Angio Suite to the γ-Camera: Vascular Mapping and 99mTc-MAA Hepatic Perfusion Imaging Before Liver Radioembolization—A Comprehensive Pictorial Review. J Nucl Med November 1, 2012 vol. 53 no. 11 1736-1747. doi: 10.2967/jnumed.112.105361. DISCLOSURES: No relevant relationships by Kathleen Capaccione, source=Web Response Speaker/Speaker's Bureau relationship with Genentech Please note: $20001 - $100000 Added 06/11/2020 by Mary Salvatore, source=Web Response, value=Honoraria research grant relationship with Boehringer Ingelheim Please note: >$100000 Added 06/11/2020 by Mary Salvatore, source=Web Response, value=Grant/Research Support

S3320 A Rare Case of Metastatic Pancreatic Neuroendocrine Tumor (PNET) in a Young Man

INTRODUCTION: Pancreatic cancers are very aggressive cancers with high metastasizing potential. Pancreatic neuroendocrine tumors (PNETs) are rare cancers that account for 1-2% of pancreatic tumors. We present an unusual case of metastatic PNET occurring in a 32-year-old man. CASE DESCRIPTION/METHODS: A 32-year-old male without past medical history presented with worsening diffuse abdominal pain and nausea for 3 weeks. He also reported clay-colored stool, intermittent diarrhea, and stabbing abdominal pain. Labs showed total bilirubin 3.1 (0.3-1.2), direct bilirubin 2.2 (0-0.3), alk phos 173 (50-136), AST 124 (15-40), ALT 200 (4-47), and lipase 707 (59-393). Hepatomegaly was noted on the physical exam. Abdominal US demonstrated hepatomegaly with multiple isoechoic hepatic masses. CT A and P showed pancreatic body neoplasm with diffuse hepatic metastasis. Ca-19 and CEA tumor markers were within normal limits. Endoscopic US revealed an irregular pancreatic body mass measuring 30 mm × 25 mm. FNA showed high-grade neuroendocrine carcinoma with small cell features. FNA malignant cells were positive for CD56, Claudin4, pankeratin, with high proliferative activity (Ki-67 proliferation index is 60%). The patient was diagnosed with metastatic Stage 4 pancreatic cancer. The patient was discharged and later admitted to a different hospital with intractable abdominal pain and worsening jaundice. MRI of the pancreas showed 3.6 cm × 2.9 cm primary pancreatic neoplasm and innumerable hepatic metastasis replacing x >50% of the liver parenchyma. US-guided FNA of hepatic metastasis revealed small cell carcinoma with the Ki67 proliferation index greater than 90%. The tumor was positive for pancytokeratin (AE1/AE3), epithelial membrane antigen, synaptophysin, and CD56. Few tumor cells were positive for GI markers CDX2 and SATB2. Unfortunately, the patient was not a candidate for any intervention, and he was discharged to continue palliative chemotherapy at home. DISCUSSION: PNETs are malignant tumors with an insidious presentation. PNETs have a varied clinical manifestation, including abdominal pain, nausea, jaundice, and anorexia. Unfortunately, as demonstrated in this patient, pancreatic cancers often present with non-specific early symptoms and are identified at advanced stages with multiple metastases. Treating metastatic pancreatic cancer, especially in a young man, is challenging as the mainstay of treatment remains palliative.Figure 1.: PNET with diffuse hepatic metastasis on CT abdomen and pelvis.Figure 2.: PNET with diffuse hepatic metastasis on CT abdomen and pelvis.

SAT-115 A Rare Case of Metastatic Insulinoma in a Patient with Huntington’s Disease

A 49-year-old female with past medical history of Huntington’s disease presents to the ED after being found unresponsive at her assisted-living facility. She was noted to be hypoglycemic with a glucose of 21 and responded to dextrose. For the past several months, patient had symptoms of dizziness, tremors. She has no history of diabetes, and only home medication is Zyprexa. Patient required D10 drip to maintain glucose levels. Given patient’s psychiatric history, there was concern for surreptitious use of diabetic medications. Labwork showed an insulin level of 163 and an elevated C-peptide of 7.0 consistent with endogenous insulin secretion. Patient underwent a 72-hour fasting protocol which revealed a glucose of 23, C-peptide of 4.1 and insulin of 101, undetectable beta-hydroxybutyrate levels and pro-insulin level of 782 which is consistent with a diagnosis of insulinoma. She was started on diazoxide, which diminishes insulin secretion, to treat the hypoglycemia. Patient underwent a pancreas CT protocol which showed multiple hypervascular hepatic masses up to 9 cm and one 4 cm mass in pancreatic tail concerning for metastasis. Pathology revealed metastatic well-differentiated neuroendocrine tumor grade 3. She was not a candidate for surgical resection of tumors and she was started on Octreotide 200 mcg TID. Patient underwent two Y90 radioembolization treatments, 2 months apart with improvement of hypoglycemia and discontinuation of diazoxide. Patient remains on Octreotide LAR injection every 4 weeks. Insulinoma, although rare, occurs in 1-4% of the population and is the most common type of functional neuroendocrine tumor of the pancreas. It secretes insulin which results in hyperinsulinemic hypoglycemia. Diagnosis involves persistent hypoglycemia with glucose <50 mg/dL, neuroglycopenic symptoms and prompt relief after glucose administration. Gold standard diagnosis is a 72-hour fast and measurement of plasma insulin, C-peptide and pro-insulin, which detects 99% of insulinomas. After lab confirmation, imaging is necessary with either CT, MRI or EUS. Surgical resection is curative in most patients. Other treatment modalities include injection of octreotide, EUS-aided alcohol ablation, radio-frequency ablation or embolization of insulinoma, as well as targeted therapy with everolimus or sunitinib. Malignant insulinomas are extremely rare and often invade into surrounding soft tissue or have lymph node or liver metastasis. They are usually unresectable and require targeted therapy. We present the case of a 49 YO patient with Huntington’s disease who presented with severe persistent hypoglycemia secondary to metastatic insulinoma. Diagnosis of insulinoma is often delayed or missed as symptoms may be attributed to psychiatric, cardiac or neurological disorders or medication misuse. Clinicians should be aware of insulinoma as a cause of life-threatening hypoglycemia.

Prevalence of portal vein thrombosis in patients with chronic liver disease using triphasic computed tomography scan as diagnostic modality: A study in Jos, North-Central, Nigeria

Background: Portal vein thrombosis (PVT) is one of the complications of chronic liver disease (CLD), and the prevalence varies from country to country as well as the method utilized in determining the presence of the thrombosis. Objective: The present study was aimed at determining the prevalence of PVT in patients with CLD in our locality using triphasic computed tomography as a diagnostic modality. Materials and Methods: This was a retrospective study. The triphasic computed tomography scan of the abdomen of 121 patients from January 2019 to April 2020 was retrieved and reviewed for the presence of PVT. Results: The mean age for the study population was 54.4 ± 13.4 years with age range of 22–100 years. Out of the 121 patients, 46 patients had PVT constituting the prevalence of 38.0%. The PVT is more in males (80.4%) than females (19.6). The thrombosis is more in the age group of 41–50 years. Conclusion: There is a high prevalence of PVT in our locality possibly because patients present in the later stage of CLD. PVT is associated with ascites and multiple hepatic masses.