Hypervascular Lesions Research Articles

Use of the LI-RADS classification in patients with cirrhosis due to infection with hepatitis B, C, or D, or infected with hepatitis B and D.

ObjectiveTo evaluate liver lesions, in accordance with the LI-RADS classification, using contrast-enhanced multiphase dynamic computed tomography in patients with hepatitis B, coinfected or not with hepatitis D, or with chronic hepatitis C, as well as to determine the level of agreement between radiologists.Materials and MethodsWe evaluated 38 patients with hepatitis B, coinfected or not with hepatitis D, or with chronic hepatitis C, all of whom underwent contrast-enhanced multiphase dynamic computed tomography. For each examination, two radiologists selected up to three hepatic lesions, categorizing them in accordance with the LI-RADS classification and evaluating signs of chronic liver disease and portal hypertension. To determine the level of agreement between radiologists, we calculated the kappa statistic (κ) .ResultsRadiologist 1 and radiologist 2 selected 56 and 48 liver lesions, respectively. According to radiologist 1 and radiologist 2, respectively, 27 (71%) and 23 (61%) of the 38 patients had at least one liver lesion; 13 (34%) and 12 (32%) had a LI-RADS 5 lesion (κ = 0.821); 19 (50%) and 16 (42%) had a hypervascular lesion (κ = 0.668); and 30 (79%) and 24 (63%) had splenomegaly (κ = 0.503). Both radiologists identified chronic liver disease in 31 (82%) of the patients (κ = 1.00).ConclusionLesions categorized as LI-RADS 5 were detected in approximately 32% of the patients, with almost perfect agreement between the radiologists. The level of agreement was substantial or moderate for the other LI-RADS categories.

Is doppler ultrasonography reliable in the evaluation of solid testicular lesions?

Aim: Evaluation of using color Doppler ultrasonography in the diagnosis of testicular solid lesions. The aim of our study was to determine the rate of benign lesions in patients with hypervascularized solid intratesticular lesions. Material and Methods: The data of 88 patients who underwent inguinal orchiectomy and who were diagnosed to have a scrotal mass in color Doppler ultrasound examination between March 2013 and March 2018 were retrospectively evaluated. All patients’ age, complaints during their admission to hospital, size of solid lesions, preoperative tumor markers (AFP, bHCG and LDH) and post operative pathology results were evaluated. Two-tailed tests were used to determine the value of preoperative numerical parameters and Fisher’s exact test to compare preoperative non-numerical parameters of both malignant and benign solid lesions, and p0.05 was considered significant.Results: The mean age of the patients was 38.54±19.09 years. The mean lesion size was 4.21±2.65 cm. The high levels of tumor markers (AFP, bHCG, LDH) were detected in 24 (27.2%) patients before the operation. In 72 patients, the size of the lesion was greater than 1 cm and in 16 patients it was less than 1 cm. As a result of the pathological evaluation of the testicular masses, it was found that 29 (32.9%) were malignant and 59 (67.1%) were benign. High levels of tumor markers, palpability and large solid lesion size were found as parameters predicting malignancy.Conclusion: In the evaluation of testicular masses with scrotal USG and Doppler USG, the rate of benign mass detection is relatively higher. In small masses, non-palpable masses and in patients with no tumor marker elevation, testicular biopsy or testicular preventive surgery should be considered.

A suspected insulinoma with the unexpected histopathological finding of a nesidioblastosis – A case report and literature review

Introduction: Nesidioblastosis is a hyperplasia of the beta cells that causes persistent hyperinsulinemic hypoglycemia, unfortunately, it is difficult to diagnose through imaging studies. Case presentation: A 68-year-old woman with palpitations, diaphoresis, dizziness and alterations in the level of consciousness that improve with food intake. Laboratories: glucose 35.0 mg/dl, insulin 12.5 mUI/ml, proinsulin 14.1 pmol/L and peptide C 2.55 ng/ml. Octreoscan reveals an abnormal epigastric uptake area and the tomography shows a focal hypervascular lesion in pancreatic body of 12×11 mm. A distal pancreatectomy was performed without palpating the tumor in transoperative period, and a diffuse nesiodioblastosis was reported in the histopathological study. The patient persists with hypoglycemia and an additional pancreatic resection was performed, resecting 90% of the organ. Although an insulinoma was not located in the piece, an area surrounded by connective tissue was observed where islet hyperplasia was more accentuated. Discussion: The pathophysiology of nesidioblastosis is unknown, its main differential diagnosis is insulinoma and this should be suspected when the tumor can not be identified. Most agree that a pancreatectomy that involves 60-80% of the total organ can control glucose levels with low risk of diabetes and pancreatic insufficiency. Conclusion: The nesidioblastosis should be suspected when a hyperinsulinemic hypoglycemia is difficult to control and when a tumor can not be identified. The extension of the pancreatectomy should be individualized and if an insulinoma is not localized and a nesidioblastosis is suspected, an intraoperative histopathological examination with frozen section evaluation for the margins could determine the extent of pancreatectomy.

Robotic central pancreatectomy for a well-differentiated neuroendocrine tumor

In this video, we show a robotic central pancreatectomy for a well-differentiated neuroendocrine tumor. This is the case of a 50-year-old patient admitted to the emergency department for acute pancreatitis. CT-scan and MRI demonstrate the presence of a hypervascularized lesion of approximately 15mm in diameter in the pancreatic isthmus. Octreoscan does not evidence any intense uptake. The colon and the omentum are detached and the stomach is suspended laparoscopically. The robot is docked using a lateral approach. A retropancreatic passage is achieved on the mesenteric-portal axis. An intraoperative ultrasonography is performed to visualize the tumor and delimitate the resection margins. After the dissection, the anastomosis is performed between the distal part of the pancreatic remnant and the posterior gastric wall. The postoperative period was characterised by a grade B pancreatic fistula. Pathologist report confirmed the presence of a well-differentiated neuroendocrine tumor. The patient was discharged on postoperative day 22.

Differential diagnosis of non-hypervascular pancreatic neuroendocrine tumor and pancreatic ductal adenocarcinoma by MRI

Objective To investigate the value of MR unenhancement and dynamic enhancement scans for distinguishing non-hypervascular pancreatic neuroendocrine tumor (PNET) from pancreatic ductal adenocarcinoma (PDAC). Methods Thirty five patients (45 lesions) with pathologically confirmed PNETs and 52 patients (53 lesions) with PDACs were retrospectively analyzed before surgery. All patients underwent MR unenhanced and dynamic enhanced scans (including arterial, venous and delayed phase). Based on arterial enhancement, PNETs were divided into hypervascular and non-hypervascular lesions. The morphologic characteristics (including location, size, quantity, margin and signal intensity) and enhancement patterns of non-hypervascular PNETs and PDACs were evaluated. Involvement of the pancreatic duct and bile duct, vascular invasion, peripancreatic infiltration and other organs metastasis were observed. Independent sample ttest was used to compare signal intensity ratio of nonhypervascular PNET and PDAC. Chi-square test was used to compare MRI characteristic and secondary signs. Results PNET included 20 hypervascular and 25 nonhypervascular lesions. Enhancement degree of non-hypervascular PNET was higher than PDAC in the arterial, venous and delayed phase (P<0.01). Non-hypervascular PNET showed significantly higher frequencies (P<0.01) of venous hyper-or isoenhancement (20/25), delayed hyper-or isoenhancement (23/25) and a well-defined margin (17/25), but lower frequencies of ductal stricture and dilatation (P<0.01), pancreas atrophy (P<0.05), bile duct stricture (2/25), peripancreatic infiltration (8/25, P<0.01) and vascular invasion (8/25, P<0.05), when compared with PDAC. Conclusion A well-defined margin, hyper-or isoenhancement in the venous and delayed phase,and without ductal dilatation and pancreas atrophy are more common in non-hypervascular PNET, which may be distinguished from PDAC. Key words: Pancreatic neoplasms; Magnetic resonance imaging; Neuroendocrine tumor

1668 A Rare Cause of Cyclical Rectal Bleeding: Sigmoid Endometriosis

INTRODUCTION: Endometriosis can frequently involve the bowels with rectosigmoid involvement being the most common. Although serosal infiltration is not uncommon, mucosal involvement is rare, hence presentation with rectal bleeding is rare. Diagnosis without pathology is difficult given symptoms frequently mimic IBS, IBD or malignancy. Here, we present a case of a woman with a history of breast cancer who presented with rectal bleeding ultimately found to have infiltrating sigmoid endometriosis. CASE DESCRIPTION/METHODS: A 40-year-old woman with a history of ER+/PR-/HER2+ breast cancer in remission on maintenance tamoxifen presented with worsening rectal bleeding for one year. Interestingly, hematochezia occurred cyclically every month seemingly coinciding with menses. It was associated with dyschezia, abdominal pain, loose stools, and dysmenorrhea. Hemoglobin and rectal exam were normal. Due to her history of breast cancer, a CT scan was done which demonstrated a focal area of sigmoid wall thickening and luminal narrowing with a 3 cm × 4 cm filling defect. Primary colonic malignancy and metastatic lesions were considered in the differentials. Colonoscopy revealed an area of extreme tortuosity in the sigmoid colon that could not be traversed. Several hypervascular polypoid lesions were noted in the proximal sigmoid colon at the site of narrowing. Cold forceps biopsy of the lesions showed endometriosis on pathology. Immunohistochemical stains positive for CD10 and estrogen receptor further supported the diagnosis. The patient was treated with Goserelin acetate with complete resolution of her rectal bleeding. After a year, she discontinued Goserelin due to side effects of fatigue and joint pains and has been on Danazol suppositories. Surgery was deferred as she did not have colonic obstructive symptoms. DISCUSSION: Here, we report a rare case of sigmoid endometriosis presenting as cyclical rectal bleeding with a lesion that masqueraded as malignancy on CT scan. Gastroenterologists should be aware of this differential in women in their reproductive years and be familiar with its unusual endoscopic appearance. This case also emphasizes good history taking, where the cyclical nature of rectal bleeding coinciding with menses is a clue for diagnosis. While symptoms of colonic endometriosis are typically chronic, late diagnoses can lead to unfortunate sequelae including acute bowel obstructions. Early diagnosis is clinically important to attempt medical therapies before complications necessitate surgical intervention.

3183 Explanted Liver Confirming Hereditary Hemochromatosis in an African American Male

INTRODUCTION: Hereditary hemochromatosis (HH) is a disorder in which intestinal iron absorption leads to iron overload. It is associated with mutations in the HFE gene. Most commonly seen in Caucasian individuals; however, it is infrequent to see it present in those of African descent. CASE DESCRIPTION/METHODS: A 52-year-old African American male with a history of decompensated alcoholic cirrhosis with ascites, portosystemic encephalopathy (PSE), and non-bleeding esophageal varices presented to hepatology clinic with a MELD score of 16. He quit drinking ten years before the presentation. He was evaluated for a liver transplant, was subsequently deemed a candidate, and placed on the list. As a part of transplant evaluation, an MRI of the abdomen was obtained approximately which showed cirrhotic liver morphology with no hypervascular liver lesions, but the presence of diffuse T2 hypointensity suggestive of iron overload and fibrosis, located in the liver, pancreas and myocardial tissue (Figure 1). A dedicated cardiac MRI with iron protocol was done to confirm findings and give quantification, which showed iron overload with an iron concentration of 1.8 mg/g of dry weight. Serology showed serum iron 125 ug/dL (normal), ferritin 1281 ng/mL (very high), 95% transferrin saturation (very high), and total iron binding capacity of 132 ug/dL (very low). Hereditary hemochromatosis (HH) was suspected, and genetic testing was conducted. The patient was found to be negative for HFE mutations C282Y, H63D, and S65C. Two months following the serological workup, he underwent a liver transplant with an ABO identical allograft liver. The explanted liver (Figure 2) showed severe iron overload in the hepatocytes confirmed by iron stain with pericanalicular accentuation (Figure 3). The diagnosis was confirmed to be HH. DISCUSSION: HH was confirmed when the presence of multi-organ iron overload was seen in imaging, serology showed significant ferritinemia with 95% transferrin saturation, and biopsy showed the presence of severe iron overload in the hepatocytes. While genetic testing for C282Y, H63D, and S65C was negative, this does not mean HH can be ruled out. Identifying these mutations is not necessary for the diagnosis of HH, but the mutations are predominantly positive in Caucasians. For other ethnicities, including Africans, there are rare HH mutations that can return positive but were not tested. This case describes a rare phenomenon of biopsy proven HH found in an African American male.

2666 Disseminated Renal Cell Carcinoma Presented as Obscure Gastrointestinal Bleeding

INTRODUCTION: Metastatic disease to the gastrointestinal tract is frequently observed in patient with known disseminated malignancies. Among those, melanoma, breast and lung cancer are commonly reported. Renal cell carcinoma (RCC) metastasis to small bowel is rare and the presentation varies from obstructive symptoms, intussusception, as well as, gastrointestinal bleeding. CASE DESCRIPTION/METHODS: A 85 years old male with medical history of pulmonary embolism on apixaban and progressive stage IV RCC despite multiple lines of therapy on lenvatinib, presented with progressive fatigue and melenic stools. Denied abdominal pain, change in bowel habits, prior episodes of bleeding, or NSAIDs use. Vital signs were unremarkable. Physical exam was pertinent for non-tender abdomen, no distention, active bowel sounds, no rebound or guarding, and a rectal exam that revealed melenic stools. Labs: Hemoglobin of 6.8 g/dL (Baseline 10.3 g/dl), electrolytes, kidney and liver function tests were unremarkable. Computerized tomography (CT) of the abdomen and pelvis revealed multiple hepatic, retroperitoneal and mesenteric metastases. He received two units of packed red blood cells with appropriate response. Apixaban was held and he was admitted for workup of gastrointestinal bleeding. Esophagogastroduodenoscopy was unrevealing, while colonoscopy showed a few non-bleeding colonic and rectal angioectasias. A subsequent capsule endoscopy demonstrated an actively bleeding jejunal lesion (Figure 1). He continued to have large melenic bowel movements, and his hemoglobin decreased to 5.5 g/dL. A computed tomography angiography showed a blush of contrast in the jejunum, near the site of a tumor implant (Figures 2 and 3). An interventional radiology embolization was performed, with successful hemostasis. DISCUSSION: One autopsy series reported that only 2 out of 44 metastatic tumors to the small intestine were duodenal implants of RCC. In recent years, however, the number of published reports of RCC small bowel metastasis has been increasing. Retrograde venous spread to the paravertebral vessels has been propose as the route of dissemination of RCC to the intestine and solitary, bulky hypervascular lesions might be diagnostic if seen on CT scan. Luminal evaluation may localize the bleeding and offer potential treatment, however, angiography with embolization is the preferred therapeutic approach for small intestine lesions. Small bowel metastases must be considered as an etiology of bleeding when patients with RCC present with melena.

2609 Use of Dotatate Scan to Establish Diagnosis of a Neuroendocrine Tumor

INTRODUCTION: Neuroendocrine tumors (NETs) are rare malignancies, defined at a cellular level by the presence of somatostatin receptors, which has recently been exploited in the development of positron-emitting somatostatin analogs for use in the 68-Gallium DOTATATE PET scan (hereafter “Dotatate scan”). Compared to previously-used technology, the Dotatate scan has shown dramatic improvements in sensitivity for lesion detection. The Dotatate scan exposes patients to lower doses of radiation, and is completed much more quickly. We present a case of a new diagnosis of a neuroendocrine tumor that illustrates multiple benefits of Dotatate scan including improved lesion detection and characterization which quickly led to changes in the treatment plan for a patient who previously was given a presumptive diagnosis of advanced pancreatic adenocarcinoma. CASE DESCRIPTION/METHODS: A 52-year-old man with history of two episodes of pancreatitis presented with one day of acute-onset epigastric pain, diarrhea, and nausea. Initial ultrasound and computed tomography (CT) scan demonstrated numerous hypervascular lesions in the liver, a mesenteric mass with associated lymphadenopathy, and a possible pancreatic tail lesion, leading to clinical concern for pancreatic adenocarcinoma. Upon further questioning, the patient reported 12–18 months of intermittent flushing and rash. A Dotatate scan revealed increased avidity in the hepatic and mesenteric lesions suggestive of carcinoid malignancy. The improved lesion characterizations provided by the Dotatate scan were useful for identifying the larger small bowel lesion as the likely primary tumor, as well as for surgical planning for debulking. Subsequent serum and urine tests were consistent with carcinoid malignancy, and a biopsy of a liver lesion confirmed the diagnosis of metastatic carcinoid tumor. The pancreatic lesion also demonstrated uptake on the scan, most consistent with a metastasis to the pancreas, with a confirmatory biopsy via endoscopic ultrasound pending. DISCUSSION: We demonstrate the utility of the Dotatate scan to support the rapid diagnosis of a neuroendocrine tumor, to assist in identification of the likely primary lesion, and to better characterize the extent of disease. Dotatate scans have been infrequently used due to novelty and cost, but cases such as this demonstrate that the cost can be well-justified in the right clinical setting.

Hypo-vascular hepatocellular carcinoma and liver transplantation: Morphological characteristics and implications on outcomes.

The clinical importance of hypovascular liver lesions in cirrhotic patients awaiting liver transplantation (LT) has not been fully investigated. The objective of this study was to characterize the clinicopathologic features and management of these tumors and to assess their impact on post-LT outcomes. We performed a retrospective review of cirrhotic patients with lesions suspicious for hypovascular hepatocellular carcinoma (HCC) who underwent LT at a single institution from 2011- 2017. We identified 22 pre-LT patients with radiologic diagnosis of a lesion(s) suspicious for hypovascular HCC. There were 28 hypovascular lesions within the 22 patient cohort; 9 lesions (32%) converted to hypervascular HCC before LT and 19 lesions remained hypovascular at LT. 88% of hypovascular lesions were HCC on explant pathology. Compared to patients with hyper-vascular HCC lesions, hypovascular HCC lesions underwent less preoperative tumor ablation (58% vs 89%; P < .01). Hypovascular HCC were more likely to be well-differentiated (67% vs 11%; P < .01), but there were no differences in the microvascular invasion, tumor recurrence, or survival post-LT. Hypovascular HCC has similar clinical outcomes and needs for transplantation as hypervascular HCC. The high prevalence of HCC within suspicious hypovascular lesions supports a similar monitoring and locoregional therapy strategy as for hypervascular HCC.

Epithelioid hemangioendothelioma of the liver—case report

Epithelioid hemangioendothelioma of the liver (EHL) is a rare vascular tumor of unknown etiology and unpredictable biological outcome. We describe the case of a 59-year-old Caucasian male patient admitted to University Hospital Merkur for diagnostic work-up and liver transplantation (LT). The patient was presented with sudden loss of consciousness, fatigue and right upper abdominal pain. The only laboratory abnormality was mild increase of transaminases. The MSCT of the abdomen and pelvis showed multiple hypervascular lesions throughout the liver, and the ultrasound-guided biopsy confirmed the pathohistological diagnosis. An orthotopic LT was performed, and the patient was clinically well, without any signs of hepatic transplant dysfunction or tumor recurrence a year after. Multifocality is a common feature of EHL, therefore LT was the treatment of choice. As the multislice computed tomography (MSCT) or magnetic resonance (MR) presentation is frequently inconclusive, the final diagnosis of the tumor has to be confirmed histologically to avoid misdiagnosis of a metastasis or other liver tumors.

A Case Report of Duodenal Gangliocytic Paraganglioma – Is Duodenopancreatectomy Necessary?

Summary Gangliocytic paraganglioma (GP) is an uncommon finding in the periampullary region of the duodenum. In the Department of General and Digestive Surgery of the Albacete University Hospital Complex, a 45-years-old patient was diagnosed with a 17x13mm hypervascular nodular lesion in the descending part of the duodenum during an examination for hepatic hemangiomas. Ampullectomy was performed, followed by a favourable postoperative period. Histological analysis proved GP. There were no involvement of lymph nodes and a malignancy potential, so the clinical and radiological follow-up opted. In conclusion, because such tumours have unpredictable behavior, we recommend local resection of the tumour, except in cases in which local or ganglionic progression is suspected or confirmed, and thus cephalic pancreaticoduodenectomy (CPD) and lymphadenectomy are suggested.

Hypervascular hepatic focal lesions on dynamic contrast-enhanced CT: preliminary data from arterial phase scans texture analysis for classification

To investigate the ability of computed tomography texture analysis (CTTA) to distinguish different hypervascular hepatic focal lesions. CTTA software was used to analyse retrospectively 18 cases of focal nodular hyperplasia, 10 cases of hepatic adenoma, 20 cases of haemangioma, 20 cases of hepatocellular carcinoma, and 20 cases of hepatic metastases using arterial phase scans. A list of texture features was generated for lesion classification. Fisher's discriminant analysis (FDA) was used to construct a predictive model from these parameters and to estimate the discriminant accuracy. Receiver operating characteristic (ROC) curve analysis was used to assess the diagnostic performance of texture analysis of benign and malignant tumours. Fifteen texture features were significant differences between the five different histopathological types of all lesions. The total discriminant accuracy was 69.3%, with 55.7% cross-validation accuracy. Seven texture features showed significant differences between the benign and malignant tumours. The total discriminant accuracy in the sample was 83%, with 77.3% cross-validation accuracy. The area under the ROC curve (AUROC) of united texture features was 0.927 (95% confidence interval [CI]=0.875-0.979). CTTA can be used as an aid in the differential diagnosis of hypervascular solidfocal hepatic lesions, especially the differential diagnosis between benign and malignant lesions.

The feasibility of contrast-enhanced spectral mammography immediately after contrast-enhanced CT.

Contrast-enhanced spectral mammography (CESM) is a digital mammography method that requires an intravenous injection of iodinated contrast material to detect hypervascular lesions. In patients undergoing evaluation for metastases before breast tumor surgery, a contrast material must be injected for computed tomography (CT) and CESM studies. The purpose of our study was to investigate the feasibility of performing CESM immediately after contrast-enhanced CT, without injecting additional contrast material. We enrolled 77 women with 88 breast carcinomas. Immediately after contrast-enhanced CT, we performed CESM without injecting additional contrast material. The patients were divided into two groups based on the length of the interval between contrast material injection and the start of mammography. In group A (n = 51), it was less, and in group B (n = 26) it was more than 7min. We measured the tumor gland contrast of each tumor on the CESM images and recorded the tumor opacification on a 4-point visual scale. The mean interval between the start of contrast material injection for CT and the acquisition of mammograms in groups A and B was 5.41 and 10.40min, respectively. All lesions were detectable on the CESM images. There was no significant difference in the visual evaluation between the two groups (p = 0.21). CESM immediately after contrast-enhanced CT without the injection of additional contrast material is feasible and cost-effective.

SUN-315 Ketogenic Diet Unmasking a Case of Insulinoma

Background: Insulinoma is a rare, sporadic neuroendocrine tumor, with incidence of 4 cases per million people (1). It is often difficult to diagnose given presentation of vague symptoms and often lack of localization of tumor on initial imaging. Timely diagnosis is usually necessary as there is commonly total resolution with surgery. We present a unique case of ketogenic diet unmasking insulinoma in a young healthy male. Clinical Case: An obese otherwise healthy 47-year male presented to our institution with rapid onset altered mental status followed by syncope and witnessed seizure-like activity. He denied previous episodes, although recalled symptoms of diaphoresis and confusion at night since beginning the ketogenic diet one week prior. Initial evaluation demonstrated normal metabolic panel, thyroid function, cortisol level, and HbA1C value of 4.8%, however plasma glucose level was 30 mg/dL with a corresponding insulin level to 87.9 (2.6-24.9) mlU/mL. Initial trauma protocol CT did not reveal injuries and the pancreas appeared normal. Hypoglycemia resolved initially following dextrose administration, however, he had several repeat episodes of symptomatic hypoglycemia despite continuous IV dextrose infusion and eating an oral diet. Two hours into 72 hour fasting test, he developed symptoms of neuroglycopenia, glucose level of 38 mg/dl with insulin level of 76.6 mlU/mL and C-peptide level of 6.7 ng/mL (1.1-1.4 ng/mL). MRI abdomen revealed 2.2x1.5 cm T2-hyperintense, hypervascular lesion in the pancreatic body with restricted diffusion, compatible with neuroendocrine tumor. Endoscopic ultrasound was completed for surgical planning. Diazoxide was initiated while awaiting surgery. He underwent successful enucleation of solitary pancreatic head lesion with resolution of episodic hypoglycemia. Histopathology confirmed neuroendocrine tumor, immunostaining positive for insulin. Conclusion: While clinicians have used ketogenic diet in aiding patients with epilepsy, the recent trend of this very low carbohydrate, high-fat diet for weight loss has been controversial. Insulinoma can be a diagnostic challenge, given the typical clinical presentation is a prolonged course of vague symptoms. Our case is atypical given the rapid onset of symptoms, diagnosis and appropriate treatment within two weeks of starting ketogenic diet. Reference: (1) Mathur, A, et al. “Insulinoma”. Surg Clin North Am. 89.5. (2009)

SUN-322 Worsening of Carcinoid Syndrome during Lutetium Therapy in a Patient with Bronchial Carcinoid and Acromegaly

Introduction: Neuroendocrine tumors (NET) are uncommon, particularly when affecting the lungs having an incidence of 0.2 to 2 per 100,000. However, they represent 1-2% of all lung malignancies and up to 30% of all NET. In the context of Multiple Endocrine Neoplasia type 1 (MEN1), bronchial carcinoid account for 2% of tumors and have also been reported in MEN4. Case: A 22 year-old male presented with an incapacitating headache after a dental extraction. Brain MRI showed a 3.3x2.4x1.4 cm lesion, suggestive of pituitary apoplexy. Of note, he had a normal brain MRI performed 20 months before due to mental confusion. He complained of abdominal cramps and diarrhea that started six months before, and had an unexplained weight loss of 10 kg during the last two months. He exhibited mild acromegalic features. On pre-operative evaluation, a routine chest X-ray was performed that showed a central lesion in inferior left pulmonary lobe (ILL). A CT scan revealed a 4.5x4.0x3.5 cm lesion in ILL. On biochemical assessment, he had panhypopituitarism with basal GH levels of 2.05 ng/mL and IGF-I of 823 ng/mL (normal range 99-289). He was submitted to transsphenoidal surgery and the histopathological analysis revealed a somatotropinoma and not somatotroph hyperplasia. A bronchoscopy showed a vegetative and hypervascularized lesion, obstructing the left bronchial segment, compatible with bronchial carcinoid. 18F-FDG PET CT and 68Ga-DOTATOC PET CT scans revealed discrete increase in glycolytic metabolism and significant increase of SSTR expression in the ILL lesion, hepatic lesions and multiple bone lesions consistent with a well differentiated metastatic bronchial carcinoid. At baseline, chromogranin A level was 17,906 ng/mL (normal range 25-140), serotonin 806.8mcg/L (normal range 30-200) and urinary 5-hydroxyindolacetic acid 19.3 mg/24h (normal range 2.0-9.0). MEN1 sequencing was negative for mutations. In the post-operative evaluation, IGF-I level fell to 474 and GH was 2.48. Pegvisomant and octreotide LAR were started with normalization of IGF-I levels. Peptide receptor radionuclide therapy (PRRT) was initiated with lutetium worsening dramatically his abdominal pain, which resolved after rescue subcutaneous octreotide and reintroduction of octreotide LAR. Since the second cycle, octreotide LAR has been given after 177Lu dose. BNP levels are within normal range and the patient has a normal echocardiography. Conclusion: The patient has two NET that can be part of a yet undiagnosed syndrome. Well-differentiated NET may synthesize humoral factors that can cause carcinoid syndrome. Excessive circulating serotonin levels may lead to carcinoid heart disease, diarrhea and cramping. During PRRT, hormone-related crisis can occur as a severe side effect. Therefore, octreotide LAR should not be stopped during Lutetium therapy.

SUN-LB058 Metastatic Insulinoma after Bariatric Surgery: A Diagnostic Challenge

Background: Post bariatric hypoglycemia (PBH) is an increasingly recognized complication of bariatric surgery. Onset typically occurs more than 1-year post-op with episodes typically occurring postprandially. In contrast, insulinoma causes fasting and postprandial hypoglycemia and has been described after bariatric surgery, with 9 reported cases. Case: A 69-year-old woman presented to the emergency department after syncope in the setting of hypoglycemia to 39 mg/dL. Medical history was notable for Roux-en-Y gastric bypass 9 years ago. She had been experiencing fatigue in the 8 months prior with episodes of diaphoresis, hunger and mental fogginess that occurred randomly throughout the day and resolved with hard candy. An inpatient supervised fast resulted in Whipple triad with blood glucose 32 mg/dL and insulin 28 uU/mL (N 2.6-24.4) within 3 hrs of starting the test, thus making it difficult to exclude PBH. Therefore, a repeat fast was performed after minimal carbohydrate intake. This test yielded similar results with hypoglycemia occurring within 2.5 hrs. Based on this, medical treatment with octreotide and diazoxide was initiated. CT abdomen showed atrophic pancreas with no mass and an enhancing liver mass suggestive of a hemangioma. However, MRI abdomen revealed a 2.9 cm pancreatic tail mass and 3 hypervascular liver lesions concerning for metastasis. Subsequently, biochemical results from the supervised fast confirmed hyperinsulinemic hypoglycemia likely due to metastatic insulinoma based on imaging studies: C-peptide 4.67 ng/mL (N 0.8-3.85), proinsulin >800 pmol/L (N <=18.8), ß hydroxy butyrate 0.04 mmol/L (N <= 0.28). Pancreatic mass FNA via endoscopic ultrasound was suspicious for neuroendocrine tumor (NET). Liver mass biopsy showed metastatic NET. Hypoglycemia worsened despite continuous IV dextrose infusion and medical therapy necessitating distal pancreatectomy, splenectomy and partial hepatectomy for tumor debulking. Pathology revealed a 1.4 cm grade 2 well- differentiated NET in the pancreatic tail (mitotic rate 1 per 10 hpfs, Ki67 5%) and 2 foci of metastasis in the liver (8 and 0.9 cm). Unfortunately, the patient developed unexplained refractory shock with multiorgan failure post-operatively and passed away after 7 days. Conclusion:To our knowledge, this is the first case reported of malignant insulinoma after bariatric surgery. Malignant metastatic insulinoma is very rare. It causes severe hypoglycemia that can occur in the very early PP period similar to PBH, which could delay diagnosis. Inconclusive initial imaging should not preclude comprehensive workup in severe persistent hypoglycemia. Reference: Mulla, CM et al. Insulinoma After Bariatric Surgery: Diagnostic Dilemma and Therapeutic Approaches. Obes Surg. 2016. 26:874-881 Unless otherwise noted, all abstracts presented at ENDO are embargoed until the date and time of presentation. For oral presentations, the abstracts are embargoed until the session begins. s presented at a news conference are embargoed until the date and time of the news conference. The Endocrine Society reserves the right to lift the embargo on specific abstracts that are selected for promotion prior to or during ENDO.

SAT-312 A Rare Case of Metastatic Paraganglioma with Positive SDHB Mutation: An Incidentally Found Silent Monster

Introduction: Although paragangliomas (PG) are derived from extra-adrenal chromaffin cells of sympathetic paravertebral ganglia in the thorax, abdomen, and pelvis, they can also arise from parasympathetic ganglia located along the glossopharyngeal and vagal nerves in the neck and at the base of the skull. Non-secretory PG have a low prevalence, are often silent, and can present as a malignant tumor with mass effects. Symptoms often include: unilateral hearing loss, pulsatile tinnitus, cough, hoarseness, pharyngeal fullness, swallowing difficulty, pain, and/or problems with tongue motion. Case: A 25 year old African American woman presented with 2 yrs of exertional presyncope, night sweats, subjective fevers, 20 lb. weight loss and a nontender enlarging left neck mass. Family history: Mother had thyroid cancer. Physical exam- BMI 21 kg/m2, BP 118/72 mmHg, HR 74, with indurated, fixed, nontender mass in left upper neck, inferior to mandibular angle. Laboratory-Hgb 6.9 g/dl, MCV 71.7, Iron 137 mcg/dl, TIBC 151 mcg/dl, Ferritin 155 ng/ml, CRP 65.20 mg/dl, ESR 122 mm/hr, AST 107 U/l, ALT 7 U/L, Alk Phos 81 U/l. EGD and Colonoscopy-normal. CT scan of neck, abdomen and pelvis: 6.0x3.5x2.7 cm heterogeneously enhancing mass in left neck involving skull base at carotid canal level, within the internal carotid space, causing external compression of left internal jugular vein, with multiple hypervascular lesions in bilateral lungs, liver and spleen, as well as cervical, thoracic, lumbar and sacral spine. Packed red blood cells were transfused. Liver Biopsy-metastatic paraganglioma with SDHB mutation. Laboratory tests- normal plasma catecholamines, thyroid function tests, prolactin, cortisol, ACTH, LH, FSH. A few weeks later, she reported new onset left sided tinnitus, orbital pain and vision changes. MRI Brain/orbit- 10 x 11 mm enhancing lesion centered in left cavernous sinus near orbital apex without intra or extraconal lesion. Chemotherapy was started with vincristine, cyclophosphamide and dacarbazine. After 12 weeks of chemotherapy, the left neck mass size decreased, tinnitus, and vision changes resolved. She was referred for genetic counseling and multidisciplinary care. Discussion: Our patient had malignant head and neck PG with SDHB mutation. Mutations in the gene encoding SDH subunit B (SDHB) can lead to metastatic disease in ≥ 40% of patients, are frequently familial, and are associated with other malignancies (renal cell carcinoma, pituitary adenoma, breast and thyroid cancer). Although there are current guidelines regarding diagnosis and management of non-malignant secretory paraganglioma/pheochromocytoma, screening and management of metastatic disease is not clear. Therefore, it is important to have a low threshold of suspicion for early diagnosis. Provider awareness and education regarding diagnosis and management is needed.

Non-squamous cell carcinoma diseases of the larynx: clinical and imaging findings

IntroductionSquamous cell carcinoma is the most common laryngeal neoplasm and accounts for approximately 95% of all malignant neoplams of the larynx. However, various benign and malignant tumors and inflammatory diseases may affect the larynx. ObjectiveThe purpose of this study is to analyze the clinical and imaging findings of non-squamous cell neoplasms and inflammatory diseases of the larynx. MethodsThis retrospective study was conducted in 18 patients who were diagnosed with non-squamous cell carcinoma lesions of larynx at our institution between 2007-2017. Clinical symptoms, examination findings, imaging characteristics, histopathologic diagnosis and treatment modalities were analyzed. ResultsThere were 9 malignant lesions (2 chondrosarcoma, 1 neuroendocrine tumor-atipical carcinoid, 1 Natural Killer/T-cell lymphoma, 1 diffuse large B-cell lymphoma, 3 plasmocytoma-multiple myeloma involvement, 1 adenocarcinoma metastasis), 3 benign neoplasms (chondroma, paraganglioma, lipoma), 2 tumor-like lesions (Brown tumor and inflammatory myofibroblastic tumor), 3 inflammatory lesions (Wegener granulomatosis, Behçet's disease and tuberculosis involvements), and 1 vascular malformation. The most common presenting symptom was hoarseness (66.6%). Paraganglioma was seen as hypervascular lesion on computed tomography and magnetic resonance imaging and showed intense tracer uptake on 68Gallium-DOTA-peptide PET/CT. Chondroid matrix calcifications were detected in chondroma and chondrosarcoma-grade 1. In patients with vascular malformation and lipoma, the typical imaging findings made it possible to diagnose. ConclusionImaging studies may provide clues for diagnosis of non-squamous cell laryngeal lesions. Clinical and imaging findings and previous clinical history should be evaluated together in clinical management of laryngeal lesions.

Abstract P6-02-18: Hepatic complications of breast cancer

Abstract Background Breast cancer is the most common malignancy and the leading cause of cancer death in women in the United States. Nearly 30% of women with metastatic breast cancer will have liver metastases, with a median survival from time of 4.23 months from time of metastatic diagnosis. Hepatic involvement of breast cancer is common and can result from metastatic tumor burden or treatment with chemotherapy or radiation. Being able to recognize and diagnose hepatic involvement of breast cancer is of utmost importance, as it has a dramatic effect on patient prognosis and management. Learning Objectives To familiarize multidisciplinary clinical team members with the radiographic presentations of hepatic complications of breast cancer in a variety of imaging modalities including computed tomography, dual-energy computed tomography, ultrasound, and MR. This will facilitate accurate and prompt diagnosis and treatment of hepatic involvement of breast cancer. Abstract Our educational pictorial essay demonstrates the typical appearance of hepatic metastases on multiple imaging modalities, as well as atypical presentations such as hyperdense, hypervascular, or hypoechoic lesions and pseudoprogression. We will show examples of hepatic metastases presenting as pseudocirrhosis, left lobar atrophy, capsular retraction, as well as sequelae of pseudocirrhosis such as malignant ascites, varices, portal vein thrombosis, and biliary obstruction. We will discuss the radiographic appearance of the liver after being treated with locoregional liver-directed techniques such as surgical resection, ablation, and chemoembolization. Importantly, we will illustrate examples of hepatic complications from systemic therapy including steatohepatitis from tamoxifen, as well as hepatic fibrosis from gemcitabine. Conclusion Clinicians must have a thorough understanding of the hepatic complications of cancer to ensure excellent diagnostic accuracy and management.