Diffuse Thickening Research Articles

1539 Drug-Induced Colitis as a Side Effect of Calcitonin Gene-Related Peptide (CGRP) Antagonist for Migraines

INTRODUCTION: Drug-induced colitis is a documented though relatively uncommon side effect of several drug classes, and is under-recognized and under-reported. In May 2018, an injectable calcitonin gene-related peptide (CGRP) antagonist, erenumab, was approved by the FDA for migraine prophylaxis. In the following case, this new medication is implicated as a possible cause for severe colitis resembling an inflammatory bowel disease-like picture both clinically and endoscopically. CASE DESCRIPTION/METHODS: A 33-year-old Caucasian female presented with complaints of rectal bleeding, diarrhea, and abdominal pain 2 weeks after starting a CGRP antagonist, erenumab, for chronic refractory migraine headaches. Pertinent laboratory studies showed leukocytosis with bandemia. CT Abdomen/Pelvis showed diffuse colonic wall thickening. Stool studies did not indicate any infectious etiology and an IBD panel was negative. She underwent a colonoscopy that showed severe, deep punched out ulcerations throughout the entire colon. Biopsy showed active colitis. She had a prior colonoscopy in 2009 for work up of IBS, which was endoscopically and microscopically normal. The patient's symptoms and clinical findings resolved after discontinuing the drug. A follow up colonoscopy performed one month after discharge showed evidence of ulcer healing with no new areas of inflammation. DISCUSSION: Blocking CGRP receptors of the central nervous system may reduce headaches, however blocking those of the enteric system can promote breakdown of the colonic mucosal barrier and induce a systemic inflammatory reaction. In animal models, extensive mucosal damage was noted in the subjects receiving CGRP receptor antagonists. This case implies that certain patients may be at risk for developing mucosal barrier disruption from the blockade of the calcitonin gene-related peptide receptor with erenumab or similar drugs. Further research may be needed to elucidate risk factors that may contribute to susceptibility to these deleterious effects. Once those patients that are more at risk are identified, there may be a role for endoscopic surveillance in certain patients taking these medications. While drug-induced colitis is likely to be an uncommon manifestation of blocking CGRP, clinicians should be aware of this potential side effect of targeted therapy and counsel patients receiving this therapy.

2101 Non-Necrotizing Granulomas of the GI Tract and Abdominal Lymph Nodes. Crohn’s Disease, Sarcoidosis or Both?

INTRODUCTION: Digestive tract involvement of sarcoidosis is rare (<2%) with few case reports of gastric and colon involvement and requires the presence of non-necrotizing granulomas. Non-necrotizing granulomas of the GI tract are typically associated with either Crohn’s disease or sarcoidosis. Distinguishing between the two can be a diagnostic dilemma. CASE DESCRIPTION/METHODS: A 25 y.o African American male presented with nausea, vomiting and weight loss. CT showed diffuse nodular airspace disease, lymphadenopathy, and thickening of the antrum and proximal duodenum. Lymphadenopathy involved the porta hepatis, stomach, head of the pancreas, abdominal mesentery, retroperitoneum and groin. EGD/EUS revealed mild erosive gastritis and duodenitis. Gastric and duodenal biopsies revealed non-necrotizing granulomas. Lymph node FNA showed reactive lymphocytes and non-necrotizing granulomas. Flow cytometry was normal. Colonoscopy with biopsy found nodular lymphoid hyperplasia in the terminal ileum (TI). Serum ACE level, lysozyme, and IgG4 were normal. IgG 3 was elevated. PFTs showed mildly reduced DLCO. Infectious work up was negative. He was treated with steroids and improved clinically. Three years later he presented with recurrent epigastric pain, anorexia, early satiety and profound weight loss. CT revealed increased abdominal lymphadenopathy. EGD showed inflammation and thickening of the stomach and duodenum. CSY revealed TI lymphoid hyperplasia, erosions over the IC valve and mild right sided colitis (Figure 1). Histology showed non-necrotizing granulomas with acute/chronic inflammation in the right colon, duodenum and stomach. CT chest showed regression of findings compared to 2016. Work-up revealed normal amyloid, ACE and soluble IL2 Ab receptor. Hypergammaglobulemia and hypoalbuminemia were present. He was treated with prednisone with rapid improvement. DISCUSSION: Our case demonstrates difficulty clarifying the diagnosis of non-necrotizing granulomas and distinguishing between atypical Crohn’s and sarcoidosis. Evidence of pulmonary involvement, low DLCO, lymphadenopathy and hypergammaglobulinemia argue in favor of sarcoidosis. Progression of inflammation to the TI and colon suggest Crohn’s. Pathology noted that granulomas were more abundant than commonly seen with Crohn’s disease alone. Additionally, there was more background inflammation than typically seen in GI sarcoid alone. A multidisciplinary team determined that this was consistent with Crohn’s and sarcoidosis occurring concomitantly.

1956 Never Assume, Always Ask: Beyond Infectious and Inflammatory Proctocolitis

INTRODUCTION: Chemical colitis can occur as a result of accidental or intentional exposure to various chemicals including alcohol. Ethanol enemas, practiced either to avoid hangovers and upper gastrointestinal (GI) symptoms or as klismaphilia appears to be dangerous practice. We report a rare case of alcohol-induced proctocolitis in association with sexual fetish. CASE DESCRIPTION/METHODS: 38-year-old female with depression, bipolar disorder, polysubstance abuse, presented with lower abdominal and rectal pain, hematochezia, and tenesmus upon waking up. On questioning, she said that night prior to presentation, she was intoxicated (vodka, methamphetamine), participated in consensual intercourse when her partner “spit” vodka into rectum. She was mildly tachycardic. Examination revealed a distressed, tearful, dehydrated female with lower abdominal tenderness. Labs revealed leukocytosis, metabolic acidosis and hyperchloremia. Urine toxicology was positive for amphetamines, benzodiazepines, opiates, cannabinoids. Blood alcohol level was zero. CT demonstrated diffuse wall thickening of rectum and distal sigmoid colon. She was admitted for hydration, analgesia, systemic antibiotics and was placed nil per oral. Colonoscopy revealed longitudinal ulcers predominantly in the rectum with erythema and erosions extending up to 20 cms above the anus. Biopsies showed mild nonspecific inflammation. Hydrocortisone enemas, stool softeners and oral diet were commenced. Follow-up sigmoidoscopy in six weeks was advised, patient did not attend. DISCUSSION: Intentional administration of alcohol enemas has been implicated in sexual practices, suicidal attempts, bowel cleansing, hemorrhoidal pain relief. Symptoms of abdominal pain, diarrhea, hematochezia, tenesmus develop within 24 hours. Endoscopic evaluation reveals edema, friable, hemorrhagic or necrotic mucosa with ulcerations. Histologic features include mild erythema, extensive mucosal sloughing, necrosis. Diagnosis can be challenging if pertinent history is not obtained, given the non-specific nature of clinical, endoscopic and histological findings. Ischemic colitis, perforation and peritonitis, respiratory depression and CO2 narcosis causing noncardiogenic pulmonary edema and death are potential complications. Severity depends on the amount and speed of administration. GI symptoms usually resolve within 7–10 days. Careful history taking, particularly sexual practices, is crucial when evaluating young healthy individuals with lower abdominal pain and colitis of unclear etiology.

2778 A Cytomegalovirus Masquerade

INTRODUCTION: Plasmablastic lymphoma (PBL) is a very rare but highly aggressive lymphoma seen in HIV patients. It is typically seen in the oral cavity and extraoral involvement is even rarer. We present a unique case of gastric plasmablastic lymphoma initially thought to be cytomegalovirus (CMV) gastritis. CASE DESCRIPTION/METHODS: A 30-year-old HIV positive female presented with epigastric pain. She had an esophagogastroduodenoscopy (EGD) that showed non-bleeding erosive gastropathy 6 weeks before presentation. Biopsy showed CMV gastritis. She was given valganciclovir. Repeat EGD 4 weeks later showed a cardia ulcer with an adherent clot and oozing that was treated with epinephrine. She was given a proton pump inhibitor and valganciclovir. Her pain worsened after discharge despite treatment leading her to present to our facility. She was tachycardic and tender to palpation of the abdomen. Labs revealed CD4 246, viral load of 2760 and platelets of 1.1 million. A CT scan showed diffuse gastric thickening up to 2 cm (Figure 1.) EGD revealed an edematous, friable and oozing mucosa with scattered Forrest III ulcerations (Figure 2). Gastric biopsies were consistent with plasmablastic lymphoma without H pylori. She had six cycles of chemotherapy with complete response at 6 months. Repeat EGD at 6 months showed erythematous gastropathy (Figure 3.) Biopsies revealed chronic active gastritis with H. pylori and no lymphoma. She completed quadruple therapy for the H. pylori infection. DISCUSSION: PBL is a rare disorder seen in the HIV population, usually involving the oral cavity. EBV is seen in 74% of AIDS-related PBL. There has been no association with CMV or Helicobacter pylori reported in the literature. PBL is associated with an aggressive course and poor survival. Despite good initial response to chemotherapy, relapse rates are as high as 54% within the first year. The endoscopic appearance of gastric lymphoma is nonspecific presenting as tumor lesions, thickened folds, ulcerative, infiltrating, bulky, or as gastritis-like changes. CMV gastritis is typically seen with CD4 count < 50. A high index of suspicion was required to make an alternative diagnosis of PBL in this patient with a CD4 count > 200.

1965 A Case of Gastric Amyloidosis Manifested as Gastrointestinal Bleeding

INTRODUCTION: Gastrointestinal amyloidosis (GIA) ensues from the deposition of insoluble extracellular protein fragments. GIA is most frequently associated with chronic inflammatory disorders (AA amyloidosis), plasma cell dyscrasias like multiple myeloma (AL amyloidosis), and end-stage renal disease (Beta-2 amyloidosis). CASE DESCRIPTION/METHODS: A 60-year-old female with a history of diastolic heart failure, light-chain multiple myeloma complicated with systemic amyloidosis and CKD stage III presented with acute epigastric abdominal pain associated with vomiting and non-bloody diarrhea that started a week after she had chemotherapy with bortezomib and elotuzumab. She was on chronic steroid and PPi therapy. On exam, she was noted to have tachycardia, dry mucous membranes, macroglossia and epigastric tenderness with no rebound. Her labwork showed a drop in hemoglobin from 8 g/dL to 5.9 g/dL in a period of 2 days and a platelet count of 48000/mL. Coagulation profile, liver tests and kidney tests were all normal. In the ED, her stool was positive for occult blood. CECT abdomen showed diffuse small and large bowel wall thickening, including the stomach and duodenum. Gastroscopy showed diffuse discontinuous congestion, friability, and ulceration of the mucosa. Clots overlying ulcers were removed with forceps revealing mild oozing only. The gastric biopsy revealed mucosal cells with extracellular amorphous deposits, staining positive for congo red stain, consistent with amyloid. She was started on palliative chemotherapy given her multi-system involvement of malignancy. DISCUSSION: GIA involves the buildup of insoluble proteins, most commonly in the second part of duodenum which impedes the normal organ structure and function. Weight loss and GI bleeding are the most common presentations of GIA. Bleeding is a sequela of mucosal lesions, wall ischemia, or vascular friability. The endoscopic appearance of amyloidosis is nonspecific. The mucosa may have a finely granular appearance, polypoid protrusions, erosions, ulcerations, and thickening of the wall. Tissue biopsy is required to make the diagnosis of GI amyloidosis. Treatment depends on a sufficient suppression of the predisposing inflammatory disorder, or malignancy, along with supportive therapy. The prognosis of patients with AL amyloidosis is worse with GI involvement.

2553 A Rare Discovery of Duodenal Noncaseating Granulomas

INTRODUCTION: Duodenal involvement of sarcoidosis is infrequently reported. It can manifest with a spectrum of symptoms and mimic other gastrointestinal diagnoses. CASE DESCRIPTION/METHODS: This is the case of a 59-year-old man with a history of pulmonary sarcoidosis on hydrochloroquine and deep venous thrombosis on apixaban who presented with weight loss with associated abdominal pain, decreased appetite, early satiety, and intermittent dysphagia to both solids and liquids. He reported a colonoscopy 2 years prior that was normal. Labs were notable for a hemoglobin of 11.5 with an MCV of 77. CMP, TSH and HIV were normal. Physical exam revealed a thin male, unremarkable vital signs and normal cardiac, pulmonary and abdominal evaluations. He underwent upper endoscopy and was found to have white esophageal plaques suggestive of candidiasis, gastritis with gastric nodularity, and a normal duodenum. Gastric biopsies were consistent with gastritis with positive staining for H.pylori for which he was subsequently treated. He underwent abdominal CT revealing diffuse small bowel wall thickening and enlarged retroperitoneal and mesenteric lymph nodes. Decision was made to proceed with repeat upper endoscopy as well as endoscopic ultrasound. Upper endoscopy noted gastritis and new duodenitis. Gastric biopsies showed chronic gastritis, negative staining for H.pylori, and positive CD20 and CD3 staining consistent with a reactive process. Duodenal biopsies showed noncaseating granulomas and villous blunting (Figure 1). EUS was unrevealing except for peripancreatic and porta hepatis lymphadenopathy for which he underwent fine needle aspiration. Flow cytometry was negative. The patient was started on high dose steroids given the duodenal involvement of his sarcoidosis followed by a taper over a few months. During the taper, he returned with worsening anemia and diarrhea. Repeat upper endoscopy revealed more extensive involvement of the duodenum with severe inflammation and plan for outpatient colonoscopy and follow up with his sarcoidosis specialist to discuss treatment regimen. DISCUSSION: Although duodenal sarcoidosis is rare, it is important to include this in the differential diagnosis in the appropriate clinical setting. It's symptoms and pathology can have overlap with some common diseases such as celiac, Crohn's disease, and even lymphoma. Tissue acquisition is imperative to confirm the diagnosis and can be performed at the time of endoscopy with treatment aimed at management of the underlying disease process.

2595 Metastatic Urothelial Carcinoma to the Duodenum: An Unusual Cause of Duodenal Stenosis

INTRODUCTION: Bladder cancer typically presents within the confines of the bladder wall with less than 4% of cases spreading to distant lymph nodes and organs. We present a unique case of gastric outlet obstruction with early satiety and weight loss secondary to usual mass effect from metastatic bladder cancer in the duodenum. CASE DESCRIPTION/METHODS: An 81-year-old male presented with two weeks of bilious vomiting, early satiety, and 20 lb weight loss. Medical history significant for prostate cancer status post prostatectomy with biochemical recurrence and bladder cancer status post radical cystectomy. On physical exam, he had mild epigastric tenderness. Labs were significant for metabolic alkalosis, acute kidney injury, and normal liver function tests. MRI of the abdomen showed diffuse thickening of the duodenal wall. He subsequently underwent an esophagogastroduodenoscopy (EGD), which revealed gastric outlet obstruction at the second portion of the duodenum. An endoscopic ultrasound (EUS) was performed which revealed no pancreatic head mass. However, a two centimeter hypoechoic thickening of the second portion of the duodenum with resultant peri-duodenal biopsies via fine needle aspiration revealed metastatic urothelial carcinoma. Patient was managed conservatively with nasogastric tube placement. With gradual improvement to a soft diet, he was discharged home with plans for outpatient palliative chemotherapy. However, he was readmitted two weeks later with similar complaints and underwent an exploratory laparotomy and palliative gastrojejunostomy. The patient was discharged on a soft diet and plans for platinum chemotherapy. DISCUSSION: Bladder cancer ranks as the ninth most frequently-diagnosed cancer worldwide. It typically spreads via the lymphatic and hematogenous routes with the most common sites involving: regional lymph nodes, liver, lung, and bone. Metastasis to the duodenum is extremely rare and to the best of our knowledge is only reported in 4 cases in the literature to date. Three of these cases presented as duodenal obstruction and one case presented as a massive upper GI bleeding. We describe an uncommon presentation of gastric outlet obstruction from bladder metastasis to the duodenum in a patient whose cancer was in remission. Tumor metastasis should be considered in a patient with a history of cancer and presenting with gastric outlet obstruction despite a normal EGD. We also highlight the utility of EUS as a safe and minimally invasive procedure to establish the diagnosis.

Hypertrophic pachymeningitis an unusual manifestation of systemic lupus erythematosus: A case report

Hypertrophic pachymeningitis is a rare inflammatory disease of the dura matter characterised by localized or diffuse thickening of the dura mater with fibrosis. Hypertrophic pachymeningitis is often secondary to infections, carcinomatosis or inflammatory diseases. It may also be idiopathic. We report a case of pachymeningitis which was A 45-year-old female presented with left-extremity focal seizure. MRI revealed diffuse pachymeningeal thickening showing intense post contrast enhancement involving falx cerebri and along bilateral fronto-parietal convexities. These findings suggest either Neurosarcoidosis, Behcet’s disease, SLE,Idiopathic hypertrophic pachymeningitis or IgG4 related hypertrophic pachymeningitis.

ADRENAL TB AS A CAUSE OF PRIMARY ADRENAL INSUFFICIENCY

SESSION TITLE: Monday Medical Student/Resident Case Report Posters SESSION TYPE: Med Student/Res Case Rep Postr PRESENTED ON: 10/21/2019 02:30 PM - 03:15 PM INTRODUCTION: Addison’s disease refer to a chronic endocrine disorder characterized by primary adrenal insufficiency. Adrenal involvement in tuberculosis is most often the result of hematogenous spread of Pulmonary tuberculosis. Isolated Adrenal Tuberculosis was noted in only 1- 2% of Addison’s Disease and one such rare case is presented here CASE PRESENTATION: A 36 yr old male, admitted with complaints of weight loss, generalised fatigue and discoloration of the body for past 6months. On admission, his BP 80/60mmHg, HR 64/min, temperature 101F & RR 22/min. His skin over the face, arms, palms and soles were darkly pigmented. Clinical examination of respiratory, cardiac, abdominal and nervous system were normal. Blood results revealed RBS 76mg/dl, urea 37, creatinine 2.2, serum Na 126mEq/L, serum K 6.5 and other parameters were normal. Serum Cortisol is 2.57 μg/dl [ 6.2 to 19.4] and ACTH is >1250 pg/ml [7.2to 63.3]. A chest radiograph and chest CT revearled no abnormalities. Bronchial wash CBNAAT MTB not detected. Mantoux 11mm. CECT of abdomen showed bilateral diffuse thickening of adrenal glands without calcifications. CT guided biopsy of Adrenals showed granulamatous adrenalitis thus confirming the diagnosis of primary adrenal tuberculosis DISCUSSION: PAI is caused by destruction of Adrenocortical tissue due to Autoimmume ds.,TB, Cryptococcosis, Fungal infections, Primary or Metastatic tumours,etc. The symptoms of PAI manifests when >90% of the Adrenals have been destroyed. Primary Tubercular Adrenalitis is a rare clinical entity & based on the clinical, serological, radiological & biopsy findings diagnosis was made. A prompt clinical & radiological response was seen in our patient. CONCLUSIONS: Isolated adrenal tuberculosis is a rare cause of Addison’s disease especially with enlargement of adrenal glands causing diagnostic problems and require differentiation from primary or secondary neoplastic disease. It is a rare but important disease entity that must be identified early and treated promptly and aggressively Reference #1: Textbook of Tuberculosis by Surendra K Sharma, 2nd Edition Reference #2: Textbook of Tuberculosis & Non Tuberculous Mycobacterial Infections by David Schlossberg, 5th Edition Reference #3: Nakaoka K, Branch J, Ozeki T, et al (2012) Acase of Primary Adrenal TB – Endocrinal Metabolic Syndrome DISCLOSURES: No relevant relationships by ALLWYN VIJAY GOWRI SHANKAR, source=Web Response No relevant relationships by Sathya Natarajan, source=Web Response

3022 Metastatic Colon Adenocarcinoma Presenting as a Hilar Obstructing Lesion Diagnosed With EUS-FNB

INTRODUCTION: Bile duct obstruction from periportal masses has a broad differential diagnosis.1 Cross-sectional abdominal imaging can help to detect periportal masses, but have low sensitivity in the diagnosis of proximal biliary and hilar lesions.2 Endoscopic ultrasound (EUS) allows for evaluation of periportal and biliary lesions and enables tissue diagnosis via fine needle biopsy (FNB). We present a case of a young patient who was presumed to have primary metastatic ovarian cancer; with EUS and ERCP guided sampling we arrived at a specific diagnosis of metastatic adenocarcinoma of colonic origin. CASE DESCRIPTION/METHODS: A 41-year-old female with a history of ovarian cancer s/p TAH and BSO and jejunal resection presented with painless jaundice, hematochezia, and high fever. Abdominopelvic CT scan showed a 2 cm mass in the region of the porta hepatis invading the portal vein and diffuse thickening of the rectosigmoid colon (Figure 1). Colonoscopy from a year prior was unremarkable, and subsequent colonoscopy was significant for hemorrhoids and hyperplastic polyps. Due to clinical suspicion for cholangitis, an ERCP was performed primarily for biliary drainage; a common hepatic duct (CHD) stricture and right hepatic duct dilation were found (Figure 2). Two 7 French 12 cm plastic stents were deployed across the common bile duct (CBD) stricture with their proximal end within the dilated right main hepatic duct. EUS showed a 2 cm lesion adjacent to the hilum that was invading the CHD and biliary bifurcation extending 1-2 cm distal and proximal to the bifurcation, portal, and hepatic artery. EUS-FNB demonstrated adenocarcinoma of jejunal origin based on cytokeratin markers (Figure 3). DISCUSSION: Specific diagnosis of periportal lesions involving the hilum of the liver is a limitation of cross-sectional imaging. EUS offers better visualization of the periportal region and enables tissue sampling for cytological or histological diagnosis. EUS-FNB has high sensitivity for diagnosis of lesions involving the common hepatic duct. It also enables assessment of vascular invasion and loco-regional staging, which is essential prior to therapy. Our case highlights, how EUS-FNB of a periportal lesion offered a specific diagnosis of metastatic colonic adenocarcinoma to the common hepatic duct. ERCP at the same session enabled biliary drainage that was essential prior to appropriate chemotherapy. The EUS FNB and ERCP with stent placement made a significant impact on the management of our patient.

1728 A Spastic Spiral: Paraneoplastic Pseudoachalasia in an Elderly Male With Multiple Malignancies

INTRODUCTION: Pseudoachalasia is an uncommon condition that is difficult to distinguish from primary achalasia. Pseudoachalasia is usually caused by malignancy involving the gastroesophageal junction (GEJ); however, a paraneoplastic etiology has been reported in rare cases. Our aim is to describe the diagnosis and management of a case of paraneoplastic pseudoachalasia resulting from acetylcholine receptor (AchR) ganglionic (a3) antibodies. CASE DESCRIPTION/METHODS: A 72-year-old male with a history of lung adenocarcinoma, bladder transitional cell carcinoma and prostate adenocarcinoma presented with acute chest pain, regurgitation, and dysphagia. Positron emission tomography scan showed increased distal esophageal uptake prompting a referral for endoscopy. Endoscopy showed a tortuous, spiraling esophageal body, distal esophageal ulcer, and narrowed GEJ (Figure 1). Ulcer biopsies returned benign, and endoscopic ultrasound (EUS) was performed. EUS of the GEJ showed diffuse thickening of the muscularis mucosa with no discrete mass. Motility evaluation was performed. Barium esophagram demonstrated a corkscrew esophagus and persistently narrowed GEJ (Figure 2). Esophageal manometry was attempted, but the catheter was unable to traverse the lower esophageal sphincter. Tracings of the esophageal body revealed spastic contractions with occasional pan-esophageal pressurization suggestive of possible mixed Type II/III achalasia (Figure 3). A paraneoplastic serum autoantibody panel was obtained and returned positive with AChR a3 antibody level of 220 pmol/L (normal < 53). Evaluation for recurrent malignancy was negative. Trial of a cholinesterase inhibitor and calcium channel blocker were attempted without improvement. Intravenous immunoglobulin therapy was declined by the patient. Endoscopic botulinum toxin injection to the esophageal body and LES was performed and resulted in sustained symptom relief. DISCUSSION: Paraneoplastic pseudoachalasia is rare, and requires a high index of suspicion for diagnosis. It may be the initial clinical sign of malignancy, or as in our case, a finding concerning for recurrent malignancy prompting re-evaluation. Prior reports suggest AChR antibodies are associated with adenocarcinomas. In patients with known malignancy who present with achalasia-type symptoms, a paraneoplastic autoantibody panel can assist with diagnosis. Treatment of the malignancy is the primary therapy, while medications and endoscopic interventions remain anecdotal.

A CARCINOMATOUS CALVARIAL COMPLAINT

SESSION TITLE: Wednesday Medical Student/Resident Case Report Posters SESSION TYPE: Med Student/Res Case Rep Postr PRESENTED ON: 10/23/2019 09:45 AM - 10:45 AM INTRODUCTION: Scalp lesions have a wide spectrum of causes and in one study 7.8% were malignant, most commonly dermatologic neoplasms[1]. Of patients with any skull lesions who underwent surgery for diagnosis, cure, or palliation of symptoms, 13% of those masses were first presenting symptom of metastatic cancer[2]. We present a patient with a scalp lesion from a less recognized primary to reinforce that patients may not have presenting symptoms from their primarily affected organ. CASE PRESENTATION: 61 year old male who initially presented to his primary care physician with a scalp nodule that had been present for 7 months. He had initially attributed the lesion to a mild head trauma. Past medical history was significant for gastroesophageal reflux, coronary artery disease, ischemic cardiomyopathy, and tobacco use. The patient also complained of left elbow pain and fatigue. The initial work-up included a CT head, which demonstrated a 4.2cmx4cmx2.7cm dural-based mass eroding through the calvarium left of midline with dystrophic calcifications and associated edema and numerous dural and cerebral lesions consistent with metastatic cancer. X-ray of his left elbow showed a proximal ulnar osteolytic lesion. CT chest/abdomen/pelvis was significant for small hiatal hernia with diffuse thickening of the lower esophagus and prominent mediastinal and gastric lymph nodes. Neurosurgery performed a biopsy on the prominent brain mass, which showed metastatic poorly differentiated carcinoma with adenosquamous features of enteric origin. PET-CT showed likely origin of cancer in distal esophagus with suspicious gastroesophageal lymphadenopathy. No lesions in liver, lungs, or adrenal glands. There were multiple metastases in the brain and vertex calvarial areas in addition to another hypermetabolic lesion in the left ulna. The patient was started on palliative chemotherapy with FOLFOX and whole brain radiation. DISCUSSION: Esophageal cancer is a disease associated with high mortality and an increasing incidence in the United States. Risk factors include Barrett esophagus, GERD, smoking, and obesity. Common presenting symptoms include dysphagia, weight loss, heartburn, regurgitation, retrosternal pain, fatigue, and anemia. Esophageal cancer is metastatic in 18% of cases at diagnosis. The most common sites of metastasis are abdominal lymph nodes, liver, and lung. Brain metastases were present in only 2%[3]. Screening for esophageal cancer is not recommended for the general population in the United States because it is rare and there is a lack of evidence that screening improves survival. CONCLUSIONS: Clinicians should have a high suspicion for slowly growing head masses and bone pain in patients with significant risk factors for cancer. Esophageal cancer is a highly aggressive malignancy that does not always present with dysphagia or heartburn. Reference #1: Turk CC, Bacanli AB, Kara NN. Incidence and clinical significance of lesions presenting as a scalp mass in adult patients. Acta Neurochirurgica. 2015 Feb, 157(2): 217-223. Reference #2: Stark AM, Eichmann T, Mehdorn HM. Skull metastases: clinical features, differential diagnosis, and review of the literature. Surgical Neurology. 2003 Sep; 60(3): 219-25. Reference #3: Quint LE, Hepburn LM, Francis IR, Whyte RI, Orringer MB. Incidence and distribution of distant metastases from newly diagnosed esophageal carcinoma. Cancer. 1995 Oct 1; 76(7): 1120-5. DISCLOSURES: No relevant relationships by jayanth keshavamurthy, source=Web Response No relevant relationships by Eugene Quarshie, source=Web Response No relevant relationships by Varsha Taskar, source=Web Response No relevant relationships by Thomas Whitton, source=Web Response

2774 Gastritis After Combination Ipilimumab and Nivolumab: A Rare Adverse Event After Immunotherapy

INTRODUCTION: Immunotherapies are commonplace in the treatment of many neoplasms, and have an array of commonly reported toxicities. Among the reported gastrointestinal side effects, diarrhea and colitis are most frequently cited. However, there have only been a rare handful of reported cases of Immune-checkpoint modulator toxicity affecting only the upper GI tract. In this paper we report a case of Ipilimumab-Nivolumab associated Gastritis in a patient treated with these medications at our facility. CASE DESCRIPTION/METHODS: The patient is a 78-year-old male with Stage IV Melanoma who presented to the hospital with nausea, vomiting, weight loss, and reduced oral intake for two weeks. The patient had most recently received 2 rounds of Ipilimumab and Nivolumab for his Melanoma 2 weeks prior to his presentation to our hospital, concurrent with the start of his symptoms. There had been no history of NSAID usage during this time, and physical exam was notable for mild epigastric tenderness. A Computed Tomography scan of the Chest/Abdomen/Pelvis showed diffuse thickening of the stomach, compatible with gastritis. Upper Endoscopy revealed diffuse severely erythematous mucosa with bleeding on contact in the entire examined stomach, and patchy, mildly erythematous mucosa without bleeding was found in the duodenal bulb, with the second portion of the duodenum being normal. The pathology report revealed subacute gastritis with acute inflammatory exudate consistent with an area of mucosal ulceration, with other etiologies of gastritis ruled out. The patient was begun on glucocorticoid therapy, with rapid resolution of his symptoms. Repeat endoscopy 2 weeks later revealed resolution of the previously noted gastritis and inflammation. Pathologic examination of biopsies confirmed resolution of the inflammatory process. DISCUSSION: On review of the literature, gastritis resulting from immune-checkpoint modulators has rarely been reported on. Some common side effects associated with these immune modulating medications, colloquially called immune-related adverse events (irAEs), include diarrhea, colitis, and hepatitis. However, there are a few case reports concerning the development gastritis without enterocolitis after Nivolumab treatment alone. Approximately 3 case reports and 1 case series of 20 patients describe such a side effect, To date, there are no reported cases of a patient treated with both Nivolumab and Ipilimumab who developed gastritis as the only adverse event.

2740 IgG₄-Related Disease: A Gastric Mass With an IgG4 Surprise

INTRODUCTION: IgG₄-related disease is a rare autoimmune syndrome that causes fibrosis of glands, most commonly of the pancreas and biliary tract1,3. The overall prevalence is unknown, with an estimated less than 1:100,000 of the general population typically found in middle-aged males2,3. Diagnosis is made with IgG₄ levels at least 2x the upper limit of normal and dense lymphoplasmacytic infiltrate, however, it may be seen with normal IgG₄ levels6. Although rare, it can present similar to GI malignancies and should be considered in the differential for patients with autoimmune disease1,3,4,5. Here we present one of those unusual cases. CASE DESCRIPTION/METHODS: A 65-year-old lady with PMH of DM2 and Sjogren's Syndrome (SS) who presented to the hospital with intractable nausea, vomiting, and failure to thrive for over 1 month. The physical exam, labs that included CMP, LFTs, and serology was unremarkable; previous EGD revealed diffuse thickening of the gastric submucosa. Biopsy via open gastrectomy revealed chronic inflammation with multiple IgG₄ plasma cells and no signs of lymphoma, suspicious for IgG₄-RD. After the partial gastrectomy, serum Na levels were 162, which improved with DDAVP and was later confirmed to be diabetes insipidus (DI). Further workup and imaging were negative and baseline IgG and IgG₄ levels were 1178 and 61, respectively. Treatment included steroid therapy and DDAVP which improved her symptoms and preceded an uneventful hospital course. DISCUSSION: IgG₄-RD is a disease with numerous presentations based on the organs involved. It presents as a GI-specific illness due to its affinity for the pancreas and biliary tract1,2,3. As seen in our patient, although rare, it may also affect the stomach, form mass lesions and cause symptomatology similar to a GI malignancy3,5. In patients with autoimmune diseases, where GI malignancy is considered, it is reasonable to include IgG₄-RD in the differential. IgG₄-RD may mimic symptoms of SS, and therefore case-patients with SS should also be considered for IgG₄-RD. This patient also experienced DI which may be a manifestation of IgG₄-RD, as the syndrome may affect the pituitary4. This case is one of the few instances reported to have pituitary involvement after the onset of multiorgan involvement4. Like other autoimmune disorders, IgG₄-RD is responsive to steroid therapy or rituximab. Therefore, a timely diagnosis can help slow down or prevent symptomatic manifestations, as seen in the case. Clinicians should be aware of this clinical presentation.

1544 Klippel-Trenaunay-Weber: Rare Congenital Syndrome Causing Persistent Rectal Bleeding

INTRODUCTION: Klippel-Trenaunay Weber syndrome (KTS) is a rare congenital disorder characterized by a triad of cutaneous capillary malformation (port-wine stain), venous malformations and limb hypertrophy. Diffuse vascular malformations of the gut in KTS can lead to gastrointestinal (GI) bleeding ranging from minor to life threatening hemorrhage necessitating surgical intervention. We describe a case of persistent rectal bleeding and iron dependent anemia in a patient with KTS. CASE DESCRIPTION/METHODS: A 32-year-old female with previous diagnosis of KTS was seen for recurrent hematochezia and iron deficiency anemia. A recent colonoscopy demonstrated non-bleeding arteriovenous malformations (Figure 1), presumed to be the source for the recurrent rectal bleeding episodes. On exam, she appeared pale, had a right hip deep purple telangiectasia, right posterior thigh palpable mass without visible varicosities and no limb hypertrophy. Her laboratory tests revealed: Hb: 9 g/dl, mean corpuscular volume: 75.0 fl, ferritin: 3 ng/ml and serum iron: 22 mcg/dl. CT abdomen/pelvis showed diffuse thickening of the rectum and sigmoid colon with scattered mural calcifications. A repeat colonoscopy demonstrated non-bleeding large circumferential vascular malformation extending from the dentate line to about 20 cm in the rectum (Figure 2) and multiple soft, pale lymphatic blebs in the colon and rectum (Figure 3). Her iron deficiency anemia from chronic blood loss was treated with oral and IV iron therapy. Despite conservative therapy, patient continues to bleed intermittently but has decided against surgical intervention at this time. DISCUSSION: GI hemorrhage occurring in association with KTS is an uncommon cause of rectal bleeding but is the most common symptom and potentially life-threatening emergency of KTS involving the gut. Investigation should begin with visualization of the entire GI tract for localization and extension of the disease. Angiography remains the gold standard and carries the advantage of allowing therapeutic interventions to control bleeding. The management of GI vascular malformations in KTS depends on the frequency and severity of blood loss requiring transfusions. Conservative management and iron supplements may be sufficient in the patient who presents with occasional non-debilitating bleeding. Transfusion dependency and life-threatening bleeding episodes necessitate definitive surgical therapy which involves resection of the diseased bowel.

A Rare Case of Desmoplastic Mesothelioma With Good Survival Despite Lymph Node Metastasis

Abstract Introduction Desmoplastic mesothelioma (DMM), a rare histological subtype of malignant pleural mesothelioma (MPM), is lethal and has very poor prognosis. Metastasis is commonly reported in DMM compared to other histological subtypes. Here we report a case of DMM with good survival (>1 year) despite lymph node metastasis. Case Report A 62-year-old female with a history of smoking and possible asbestos exposure presented with cough and wheeze in 2012. Computed tomography (CT) revealed left-sided pleural plaques and multiple ground-glass pulmonary nodules. In 2017, repeat CT showed increased diffuse left nodular pleural thickening. Positron emission tomography revealed hypermetabolic, nodular pleural thickening in the hemithorax, compatible with neoplasia. Needle biopsy in January 2018 showed pleural tissue with haphazard, nodular growth of spindled mesothelial cells, suspicious for desmoplastic mesothelioma. Definitive diagnosis was not possible due to lack of fat invasion and absence of supportive evidence by immunohistochemical stains. The patient underwent pleurectomy in February 2018. On histopathology, the majority of the tumor showed a desmoplastic pattern, composed of malignant spindled cells arranged haphazardly in a dense hyalinized stroma with chronic inflammatory infiltrate. AE1/AE3, calretinin, and D2-40 immunohistochemical stains highlighted the infiltrating neoplastic cells, which were negative for WT-1. BAP-1 was retained. The pattern of immunoreactivity supported the diagnosis of DMM. The tumor also involved visceral pleura, pulmonary parenchyma, and pericardium. It invaded into fat and displayed lymphovascular invasion. A metastatic focus of DMM was present in a lymph node. The tumor was staged as pT3N1. On recent examination, the patient only had complaints of mild breathlessness and stable hydro-pneumothorax, without any other comorbidities. Conclusion Our finding is unusual, since among the mesothelioma subtypes, DMM has the shortest survival, usually not more than 6 months. We report an extremely rare case of DMM with survival of >1 year despite invasion of lung parenchyma and lymph node metastasis.

Optical coherence tomography use for estimation of the efficacy of anti VEGF therapy in diabetic retinopathy and diabetic macular edema.

Objectives: To observe the role of optical coherence tomography in patients receiving anti-vascular endothelial growth factors for the treatment of diabetic retinopathy. Study Design: Observational descriptive study. Setting: The study was conducted at Department of Ophthalmology, Shahida Islam Teaching Hospital, Lodhran. Period: January 2018 to December 2018. Material & Methods: 177 eyes of 156 patients with diabetic retinopathy were analyzed with optical coherence tomography to quantify and explore changes in macula and inner retinal structures and to see different patterns of DME pre and post intra-vitreal anti-VEGF injections. All eyes had baseline OCT, received anti-VEGF intra-vitreal injection Avastin (Bevacizumab) 1.25mg/0.05ml. Follow up OCT imagining was done 6 weeks after last injection. Results: The patients had mean age of 58.36 ± 3.67 years with the mean diabetes duration of 9.30 ± 2.76 years. Before intra-vitreal injection, two different patterns of DME were recognized and analyzed i.e. diffuse thickening of macula (n=117, 66.10%) and cystoids macular edema (n=60, 33.90%). Base line OCT showed mean ± SD central foveal thickness 416 ± 54µm (n=177). On post intra-vitreal injection OCT, mean ± SD macular thickness was 212 ± 35 µm (n=177). Conclusion: OCT is latest non-invasive imaging modality that currently helps in quantifying and understanding the anatomy of DME and inner retinal damage due to diabetes mellitus. Although vascular leakage in DME is assessed qualitatively with Fluorescein angiography, actual macular thickness calculated with OCT is very helpful in yielding efficacy of treatment. This tool should always be used in monitoring the effect of therapies in future studies.

54. Binocular sight loss: uncommon, yet a devastating complication of GCA

IntroductionGCA (giant cell arteritis) affects cranial branches of the arteries from aortic arch, especially the superficial temporal artery and vessels supplying the eye. Ophthalmic manifestations of GCA are characterised by the vasculitis of the ophthalmic artery and its branches. We present a case of a patient who developed the binocular sequential permanent vision loss secondary to GCA due to the delayed diagnosis.Case descriptionA seventy-six year-old lady with a background of type two diabetes mellitus, hypertension and dyslipidemia and hemicolectomy for appendix tumour two years ago (no recurrence) was admitted with blurring of vision in the eye. She did not complain about other symptoms, however, on direct questioning she admitted six weeks history of weight loss (2.5 stones), severe tiredness and occasional night sweats, mild left sided temporal headache. She was initiated on 60mg of prednisolone. One week prior to admission she had right eye CRAO diagnosed by the ophthalmology team with preceding three weeks history of right eye blurring of vision. CRP at that time was 12 g/L and GCA was not considered likely. Following her permanent right sided vision loss, she was referred to stroke team for further management and was given aspirin. Her CT and MRI brain scans were unremarkable, USS carotid arteries revealing diffuse intimal thickening in all the arteries of the neck. On assessment in the rheumatology fast track clinic she denied any associated jaw/tongue/arms claudication and no proceeding or historical PMR symptoms. She had mild left sided temporal artery tenderness and decreased temporal artery pulses bilaterally, no vision in the right eye along with the blurring in the left eye. There were no audible bruits in carotid, axillary, femoral arteries. Her acute phase maker (CRP) was mild but persistently elevated with a maximum level of 12 g/L for one week prior the admission. USS showed features consistent with GCA with extensive non-compressible halo signs in temporal arteries and its branches bilaterally. An urgent ophthalmology review revealed AION changes in the left eye, her vision deteriorated over the next forty-eight hours despite the initiation of high dose intravenous methylprednisolone. The patient was also started on tocilizumab infusion in an attempt to reverse the sight loss.DiscussionThe ophthalmic manifestations of GCA range from AION (anterior ischaemic optic neuropathy), CRAO (anterior central retinal artery occlusion), cilioretinal artery occlusion to occipital lobe infarcts, transient monocular vision loss, photopsias or diplopia. Ophthalmic GCA is an emergency and requires urgent ophthalmological evaluations and initiation of treatment with high dose of steroids to avoid permanent loss of vision. The reported incidence of visual symptoms in GCA ranges widely from 12% to 70% of cases. GCA is often associated with constitutional symptoms such as weight loss, night sweats, fevers, systemic inflammatory response with elevated inflammatory markers. However, it had been reported that this can be very mild in ischaemic GCA.The diagnosis of GCA in this particular case was delayed for approximately six weeks due to the atypical initial presentation with mainly constitutional symptoms and unilateral vision blurring as well as mildly elevated inflammatory markers. A collaboration of three teams (namely ophthalmology, stroke and rheumatology) proved necessary to initiate high dose steroid treatment and tocilizumab. However, patient still suffered from permanent binocular vision loss. She was registered blind and will in future require extensive physical and psychological support.Key learning pointsDespite the recent advances and increased awareness with good availability of fast track GCA services, cases of bilateral blindness are still observed. This is due to a lack of awareness of atypical non-cranial symptoms that frequently accompany a GCA ischaemic presentation. Inflammatory markers are often only mildly elevated in such cases. We suggest a heightened public and professional awareness program to mitigate this irreversible disastrous complication.Conflicts of interestThe authors have declared no conflicts of interest.

AFP-producing hepatoid adenocarcinoma (HAC) of peritoneum and omentum: a case report and literature review.

Hepatoid adenocarcinoma (HAC) is a group of neoplasms with features resembling hepatocellular carcinoma. The stomach is the most commonly affected organ among the reported primary sites. We report the case of a 28-year-old man with chronic hepatitis B and a complaint of abdominal distension. The patient was examined by PET-CT and magnetic resonance imaging (MRI), which showed diffuse thickening of the peritoneum and omentum but no mass was found in the liver. Pathological examination of a biopsy of the omental nodules was consistent with moderately differentiated hepatocellular carcinoma (HCC); thus, a diagnosis of HAC of the peritoneum and omentum was established. The patient received a chemotherapy regimen consisting of oxaliplatin and capecitabine and gained remarkable effects as the AFP level dropped significantly, and the tumour nearly disappeared. When the patient shifted to the standard multikinase inhibitors, Sorafenib or Lenvatinib, both treatments were ineffective. HAC is a heterogeneous group of prognostically unfavourable tumours mimicking the histological appearance of HCC, and the treatment outcomes are still unclear.

Sonographic features of benign and malignant axillary nodes post-neoadjuvant chemotherapy.

The aim was to determine whether sonographic features of metastatic axillary lymph nodes predict pathologic nodal status post-neoadjuvant chemotherapy (NCT) and help to tailor less invasive surgical management of the axilla. Patients with biopsy-proven cN1 primary breast malignancy who received NCT between January 2011 and December 2014 and had performed ultrasound were included in this study. Sonographic features of biopsy-proven clipped metastatic axillary nodes pre- and post-NCT were retrospectively reviewed by two independent readers. Changes in lymph node shape, fatty hilum status, cortical thickness, and cortical echogenicity were compared in patients with and without nodal pathologic complete response (pCR) using univariate and multivariate logistic regression models. Inter-reader variation was analyzed to determine the reproducibility of data. Of the 195 patients included in the study, 75 (45%) showed nodal pCR and 90 (55%) persistent metastatic disease post-NCT. pCR was significantly more likely in lymph nodes with isoechoic or hypoechoic cortical echogenicity post-NCT, (P=.02), conversion to normal cortical thickness (P=.0001), and oval shape (odds ratio=0.17, P=.004), compared to lymph nodes with anechoic cortical echogenicity, persistent or diffuse cortical thickening, and irregular shape, respectively. The overall accuracy of sonographic nodal features in the prediction of pCR was 65% (95% CI: 58%-72%). The overall accuracy of sonographic features of biopsy-proven metastatic axillary lymph nodes post-NCT is not sufficiently high to predict pCR of axillary nodal status and thereby should not be solely used in guiding less invasive surgical approaches to the axilla.