bluuebunny/crossref_metadata_2025_split huggingface dataset:
2613 A Rare Case of Undifferentiated Malignant Epithelioid Neoplasm
Authors: Prakash Sameer , Sindi Ghassan , Suliman Abdelrazig 
Published: October 2019

INTRODUCTION:
The small bowel is rarely a site for development of tumor malignancy and accounts for less than 1% of all GI malignancies. We present a case of a 53-year-old male with recurrent episodes of gastrointestinal bleeding and negative upper and lower GI endoscopies found to have high-grade undifferentiated high-grade malignant epithelioid neoplasm.

CASE DESCRIPTION/METHODS:
A 53-year old male with history of peptic ulcer disease, duodenal ulcer perforation presented to the ED with right upper and lower extremity pain for last few weeks along with black stools. He had required several units of blood transfusion with recurrent admissions to hospital and had also lost about 20 lbs in last 2 months. EGD and colonoscopy 3 months before had shown duodenal angiectasia and colonic polyps which were removed. Outpatient capsule endoscopy was recommended but patient did not follow up. Bleeding scan was negative and CT abdomen pelvis with contrast showed thickening in distal jejunum concerning for mass-like lesion. Patient underwent laparoscopic small bowel resection of 18 cm and pathology showed high-grade undifferentiated malignant epithelioid neoplasm with macro-metastatic tumor. The tumor showed diffuse staining for vimentin,pancytokeratin, CK7 with subset moderate cytoplasmic staining for calretinin and CK8/18.Port-A-Cath was placed and patient was started on standard adjuvant chemotherapy including combination of 5-FU, leucovorin, and oxaliplatin.

DISCUSSION:
Small bowel tumors are very rare and aggressive and have poorer prognosis than colon cancer. They present a diagnostic challenge as access to small bowel is limited during conventional diagnostic procedures and symptoms present late. We present a patient who was having recurrent gastrointestinal bleeding requiring multiple blood transfusions and found to have high-grade undifferentiated high-grade malignant epithelioid neoplasm. The differential for this includes monophonic synovial sarcoma, malignant epithelioid mesothelioma, and large cell lung carcinoma vs others. Video capsule endoscopy has rapidly become the conventional standard for small bowel evaluation, followed by other methods such as magnetic resonance enteroclysis. Standard chemotherapy treatment closely resembles that of large bowel cancer, including combinations of 5-FU, leucovorin, and oxaliplatin/irinotecan/capecitabine.
2615 Treating Gas With Gas: A Case of Benign Extensive Pneumatosis Intestinalis
Authors: Anugwom Chimaobi , Singh Dupinder , Levitt Michael 
Published: October 2019

INTRODUCTION:
Radiological evidence of extensive gas collections in the bowel wall and, particularly, the portal vein commonly suggests a life-threatening process. We present a patient with massive intramural and portal gas that was adjudged to be of benign origin and managed conservatively with high flow nasal oxygen.

CASE DESCRIPTION/METHODS:
A 74-year-old male with a history of coronary artery bypass surgery and a 40 pack-year history of smoking was admitted with a two-week history of abdominal discomfort with food ingestion, bloating and belching. History was negative for gastrointestinal bleeding, prior abdominal surgeries or fever. On examination, he was afebrile, hemodynamically stable, and had no abdominal tenderness, rebound or guarding. Initial laboratory measurements were normal and notable for normal white blood cell count of 9180/mm3 and a lactic acid of 1.8 mmol/L. A stool exam for pathogens was obtained. A Computed tomography (CT) scan of the abdomen showed extensive pneumatosis involving the upper small intestine and the stomach, and a relatively massive collection of portal venous gas (see Figure 1). He was admitted to the hospital with the diagnosis of possible ischemia of the small bowel and/or stomach. Despite the ominous abdominal imaging findings, he had a normal physical exam and lack of evidence of sepsis which suggested a benign etiology of the pneumatosis. The patient was essentially asymptomatic throughout his 6-day hospital course. The stool screen for 20 pathogens revealed enteropathogenic E. coli. On the second day of hospitalization, high inhalational oxygen (>50%) via nasal cannula was instituted in addition to an elemental diet. A repeat CT scan on day 5 of hospitalization showed virtually complete resolution of intramural small bowel and gastric gas, and only small residual portal venous gas remained. He was discharged home on antibiotics for 3 weeks and has continued to do well

DISCUSSION:
Pneumatosis intestinalis should be recognized as a clinical sign and not a diagnosis and should warrant careful review of the clinical context, signs/symptoms and laboratory data. The counter-perfusion supersaturation theory has been proposed as the pathophysiology in most benign cases. It involves increased hydrogen production by intestinal bacteria which supersedes the nitrogen tension in the blood stream. High flow or hyperbaric oxygen may help in the establishment of a nitrogen gradient between intramural gas and blood with improvement of intramural gas
2616 Seronegative Adult Autoimmune Enteropathy
Authors: Lundholm Michelle D. , Wanta Kaitlin , Ding Xianzhong , Palmer Lena , Abegunde Ayokunle T. 
Published: October 2019

INTRODUCTION:
Autoimmune enteropathy (AIE) is a rare disorder of the immune system characterized by 6 or more weeks of severe diarrhea with malabsorption and histologic changes on small intestinal biopsy. This condition is primarily seen in neonates and children, but rarely has been reported in adults.

CASE DESCRIPTION/METHODS:
A 75-year-old woman presented with 4 months of intractable diarrhea, vomiting, and 30-pound weight loss. Prior workup was unrevealing including celiac testing, stool enteric pathogens, Cytomegalovirus, Herpes simplex virus, Helicobacter Pyloritesting, and colonoscopy without biopsies. Non-specific acute and chronic inflammation without villous blunting was seen in duodenal biopsies. She was treated supportively but her symptoms progressed requiring admission. She appeared emaciated with stable vital signs and a benign abdominal exam. She had severe hypoalbuminemia and hypomagnesemia. C-reactive protein, liver and thyroid tests, anti-nuclear antibody, HIV, and immunoglobulin levels were normal or negative. Repeat esophagogastroduodenoscopy (EGD) showed duodenal scalloping and friability with contact (Figure 1). Colonoscopy appeared endoscopically normal to the terminal ileum. Biopsies of the duodenum and terminal ileum showed glandular destruction, epithelial apoptosis, and goblet cell depletion without significant intraepithelial lymphocytosis (Figures 2 and 3). Periodic acid-Schiff stain and random colonic biopsies were negative for Whipple's disease and microscopic colitis. Anti-enterocyte antibodies were negative. She was diagnosed with AIE and started on high-dose steroids with a prolonged taper. At her 2-month follow-up she had gained back 10 pounds with complete resolution of her nausea, vomiting, and diarrhea.

DISCUSSION:
AIE should be included in the differential diagnosis for adult patients with intractable diarrhea. Diagnostic criteria include partial or complete blunting of small intestinal villi, deep crypt lymphocytosis, increased crypt apoptosis and minimal intraepithelial lymphocytosis. Anti-enterocyte antibodies are positive in approximately 80% of cases. In this case, the small intestinal histopathology was not diagnostic for AIE 2 months into her symptomatology but was diagnostic on EGD 9 weeks later, highlighting the utility of repeat EGD and biopsy in patients with persistent symptoms. This emphasizes that villous blunting may be patchy and chronic inflammatory changes of AIE may evolve over time. More research is needed to better understand this emerging condition.
2621 Gastrointestinal and Hepatic Amyloidosis: An Unusual Cause for Abdominal Pain and Elevated Liver Enzymes
Authors: Rai Puja , Dhingra Rohit , Sterling Mark 
Published: October 2019

INTRODUCTION:
Amyloidosis is a complex, multi-system disorder involving the extracellular deposition of insoluble protein fragments. Symptomatic gastrointestinal and hepatic involvement are relatively rare, often leading to a challenging and delayed diagnosis.

CASE DESCRIPTION/METHODS:
We describe a case of gastroduodenal and hepatic amyloidosis in a 61-year-old male with a history of plasma cell myeloma with systemic AL (amyloid light chain) amyloidosis with cardiac involvement who presented with 3 months of epigastric pain associated with nausea, vomiting, and 20-pound weight loss. Laboratory testing revealed transaminitis including a significantly elevated alkaline phosphatase (ALP). Right upper quadrant ultrasound was normal. Computed tomography (CT) showed diffuse mesenteric haziness and heterogenous edematous appearance of the liver. Since his diagnosis of amyloidosis, the patient had been treated with cyclophosphamide, bortezomib, and dexamethasone (CyBorD) therapy with appropriate light chain suppression. Despite treatment, he continued to have persistent gastrointestinal symptoms. He therefore underwent upper endoscopy with biopsies of the esophagus, stomach and small intestine notable for positive Congo red staining, with classic green birefringence under polarized light, indicating extensive amyloid deposition within the lamina propria. Subsequent transjugular liver biopsy demonstrated marked centrilobular sinusoidal dilation and increased fibrosis with amyloid deposition within the vessels of the portal tract. His symptoms were medically managed, and he was continued on chemotherapy while undergoing evaluation for cardiac transplant prior to consideration for autologous stem cell transplant.

DISCUSSION:
Gastrointestinal and hepatic amyloidosis are unusual diagnoses that present with variable and nonspecific symptoms, and often late in the disease course. Clinicians should maintain a high index of suspicion of amyloidosis in patients with previously diagnosed plasma cell or inflammatory disease. Our case demonstrates that despite high-dose chemotherapy, patients may develop refractory disease with multi-organ involvement. Prognosis depends on the amyloid subtype, and whether there is gastrointestinal infiltration as these patients typically have worse outcomes, emphasizing the importance of prompt diagnosis. Management aims to treat the underlying amyloid type of pathology, with supportive therapy to alleviate the specific gastrointestinal symptoms.
2625 Intussusception After Classic Pancreaticoduodenectomy With a Roux-en-Y Pancreatico-hepaticojejunostomy
Authors: Alexandre Cassidy , El Jabbour Tony , Parsa Nour , Mansoor Muhammad Sohail , Gumustop Bora 
Published: October 2019

INTRODUCTION:
Intussusception in adults is uncommon, accounting for 1%–5% of all intussusception cases. It usually occurs because of a lead point, frequently malignancy or adhesions from previous surgery. Here we report an exceedingly rare case of jejunal intussusception into the gastric lumen in a patient after classic pancreaticoduodenectomy with a Roux-en-Y pancreatico-hepaticojejunostomy.

CASE DESCRIPTION/METHODS:
A 49 year-old male with Down syndrome, celiac disease and surgical history significant for classic pancreaticoduodenectomy (Whipple procedure) with a Roux-en-Y pancreatico-hepaticojejunostomy for pancreatic mass with distal bile duct stricture and duodenal stricture presented with one-day history of abdominal pain. He was minimally verbal due to Down's syndrome however pointed to his upper and lower abdomen. Associated symptoms included mild nausea but no emesis, melena or hematochezia. Abdominal examination was negative for any mass or peritoneal signs. Initial labs were remarkable for only mild anemia; while blood cell count and lactic acid were normal. A CT scan demonstrated an approximately 7-cm length of jejunum protruding into the lumen of the stomach at the gastrojejunostomy site concerning for intussusception, but no high-grade mechanical obstruction observed. EGD showed intussusception of the blind jejunal limb with healthy appearing mucosa and jejunum biopsies were obtained to rule out ischemia; examination of gastrojejunal anastomotic site and the jejunum (efferent limb) was otherwise normal. Endoscopic reduction of intussusception was attempted but was unsuccessful. Remaining hospital course was uneventful. He was tolerating diet, having proper bowel movements and his abdominal pain resolved. No surgical intervention for repair was planned as he continued to improve clinically and he was safely discharged home. He was seen in surgery clinic a few months later and remained asymptomatic.

DISCUSSION:
Intussusception is a rare entity in adults and only a few cases have been reported after Whipple procedure. Diagnosis of intussusception in adults, regardless of previous surgeries, is primarily through imaging because presenting symptoms are often vague and nonspecific. Surgical management is often required. However, in our patient, due to the absence of bowel obstruction or ischemia, surgical intervention was not needed. This case highlights that intussusception should be considered as a potential diagnosis in patients with history of Whipple presenting with vague abdominal symptoms.
2627 Small Bowel Adenocarcinoma With Right Atrial Metastasis: A Case Combining Two Rare Entities
Authors: Wang Patty , Weinberg Richard B. 
Published: October 2019

INTRODUCTION:
Small bowel adenocarcinoma comprises less than 2% of all gastrointestinal malignancies. Given its rarity, little is known about its clinical features, treatment modalities, or prognosis. Early diagnosis remains a challenge due to the absence or nonspecific nature of its symptoms. The heart is a rare site for metastasis of any malignancy, and cardiac metastases are likewise often clinically silent. Here we present a case of jejunal adenocarcinoma with metastasis to the heart that was ultimately diagnosed by pancreatic protocol CT imaging.

CASE DESCRIPTION/METHODS:
An 82-year-old female with a history of atrial fibrillation presented with a left middle cerebral artery stroke after her warfarin was held due to iron deficiency anemia. She underwent successful thrombectomy, but her initial evaluation with a transthoracic echocardiogram incidentally revealed a 4 cm × 3.9 cm right atrial mass. Further evaluation with a cardiac MRI and a standard CT of the chest/abdomen/pelvis showed a large hypoattenuating mass in the right atrium that extended into the inferior IVC and left hepatic vein; no tumor primary was identified. Cardiac biopsy found a moderately differentiated adenocarcinoma positive for CK-20 and CDX-2, suggesting a GI origin. She then underwent an EGD and colonoscopy, neither of which found evidence of a malignancy, although the EGD noted a prominent ampulla with normal biopsies. Given this finding and the presence of an adenocarcinoma of unknown origin, she was scheduled for an EUS and capsule endoscopy. However, before these procedures were performed, a CT with pancreas protocol revealed a 5 cm circumferential mass in the proximal jejunum causing low grade obstruction; the morphology was consistent with adenocarcinoma. The patient was treated with radiation therapy.

DISCUSSION:
To the best of our knowledge, this is the first case report of a small bowel adenocarcinoma metastatic to the heart. After the discovery of an atrial adenocarcinoma, a small bowel origin was not initially considered given its rarity and nonspecific presentation. The first CT scan may have failed to detect a jejunal primary for the same reason, although differences in contrast infusion technique may have played a role. Cardiac metastases are similarly very rare and have most often been reported in lung cancer, melanoma, lymphoma, breast cancer, sarcoma, and renal cell carcinoma. This case suggests that although rare, small bowel adenocarcinoma should be considered in patients found to have cardiac metastatic disease.
2628 Incidental Isolated Small Intestinal Melanoma in a Patient With Newly Diagnosed Celiac Disease
Authors: Amornsawadwattana Surachai , Huebner Elizabeth 
Published: October 2019

INTRODUCTION:
It is believed that patients with Celiac disease (CD) are at increased risk of certain malignancies. However, CD patients are not associated with cutaneous malignant melanoma and thus additional screening is not indicated. We report an unusual case of a patient with first diagnoses of simultaneous CD and isolated small intestinal melanoma.

CASE DESCRIPTION/METHODS:
A 55-year-old female without history of malignant melanoma (MM) presented with chronic diarrhea. Physical exam and laboratory results were unremarkable except a tissue transglutaminase IgA antibody was elevated at 126.4 units (normal 0.00–19.99.). An esophagogastroduodenoscopy was performed and demonstrated scalloping of the duodenal folds. A hyperpigmented mass was incidentally found in the proximal jejunum (Figure). Duodenal biopsies showed villous blunting, chronic inflammation and increased intraepithelial lymphocytes, which was consistent with CD. Biopsies of the jejunal mass revealed a densely pigmented nodule of atypical cells with large hyperchromatic nuclei, abundant cytoplasm, and mitoses. An immunohistochemical stain was positive for Melan-A and S100, which confirmed a diagnosis of MM of the small bowel.Additional imaging revealed no signs of metastatic disease. Subsequently, the patient underwent laparoscopic resection of the small bowel mass and the pathology showed malignant MM with negative margins. Then, she was started on Trametinib and Dabrafenib for about 5 months, but was discontinued due to side effects. Currently, the patient remains disease-free 36 months after surgery. Diarrhea is also much improved after starting a gluten free diet.

DISCUSSION:
Small bowel malignancies are rare, accounting for 3% of GI neoplasms and the majority of small bowel neoplasms represent metastatic disease. The small intestine is also considered the most common site of melanoma metastases to the GI tract. Primary small intestinal MM is characterized by MM of the small bowel without evidence of a primary extra-intestinal lesion. Whether this represents a true primary malignancy or a metastatic disease from an unrecognized or a regressed lesion elsewhere remains debatable. The main treatment of primary small intestinal melanoma is surgical resection. Although patients with CD in general have increased risk of malignancy, there is no known association between CD and MM. This case highlights a rare incidental primary small intestinal melanoma in the setting of a patient who is symptomatic from CD.
2635 Gastrointestinal Hereditary Angioedema Causing Recurrent, Self-Resolving Abdominal Pain
Authors: Gupta Kamesh , Mayigegowda Kavya K. , Hassan Tuyyab 
Published: October 2019

INTRODUCTION:
Hereditary angioedema (HAE) is a type of bradykinin-mediated isolated angioedema, characterized by temporary swelling that can affect skin or walls of hollow viscera such as the oropharynx, or gastrointestinal tract (in 43 to 93% of HAE cases respectively) and occurs without pruritus. This case is reported due to its rare presentation that often results in unnecessary invasive procedures.

CASE DESCRIPTION/METHODS:
A 25-year-old female with a past medical history of hypothyroidism and asthma presented with acute on chronic intermittent abdominal pain and vomiting for a day bearing no relation with meals or defecation. She had recurrent hospitalizations for similar complaints but workup till date showed enteritis on imaging and exploratory laparoscopy showed adhesions with colonic biopsy negative. Extensive history taking revealed prodromal symptom of anxiety and a previous episode of self-resolving finger swelling, retrospectively suspected to be due to angioedema. Labs showed leukocytosis and elevated CRP. Repeat CECT abdomen displayed thickening of jejunal wall. Given her history of prodromal symptoms, finger swelling and a family history of similar self-resolving abdominal pain of unclear etiology in her mother prompted further testing for complements due to concern for hereditary angioedema, which was positive with low C4 and C1 inhibitor. She was referred to an allergy specialist for further management.

DISCUSSION:
HAE is an autosomal dominant disease that is due to decreased or impaired C1 Inhibitor. Typical GI symptoms are recurrent abdominal pain, vomiting and diarrhea caused by transient small bowel edema. Inciting events include stressful situations like fasting and infections and medicines like ACE-inhibitors. Diagnosis is clinical from history of skin swelling or respiratory distress during attacks, documenting possible triggers and family history of HAE. Physical exam and imaging is useful only during acute attacks. Recommended lab tests include C4 levels, C1 inhibitor antigenic levels, and C1INH functional levels. Endoscopy is not recommended as it may precipitate laryngoedema. Treatment includes C1 INH replacement or icatibant (bradykinin receptor antagonist) for acute attacks and attenuated androgen or lanadelumab for prophylaxis. HAE should be considered when tackling cases of undiagnosed abdominal pain in the right clinical context to prevent years of suffering, unnecessary invasive procedures and life-threatening complications from laryngeal edema or hypovolemic shock.
2636 Brunner's Gland Hyperplasia: A Massive Duodenal Lesion
Authors: Bhatti Sundus , Omer Endashaw 
Published: October 2019

INTRODUCTION:
Brunner’s glands are alkaline-fluid secreting duodenal glands in the proximal duodenum submucosa. Brunner’s gland hyperplasia is a rare entity that accounts for 10% of all benign duodenal tumors, with an estimated incidence of 0.008%. Underlying etiology is postulated to be secondary to excessive acid secretion, H. pylori infection or inflammation.

CASE DESCRIPTION/METHODS:
A 57-year-old male with PMH of Myasthenia Gravis, GERD and esophageal strictures with prior dilations, presented with melena and intermittent abdominal pain for a few weeks and labs notable for iron deficiency anemia. EGD showed a large 5 cm polypoid mass in the proximal duodenal bulb with shallow surface ulcerations and a thick stalk causing ball-valve like obstruction of the pyloric channel. CT abdomen showed an elongated tubular mass arising from the proximal duodenum projecting antegrade within the duodenal lumen, measuring 5.3 × 1.7 × 1.5 cm, with no evidence of metastasis. Endoscopic ultrasound (EUS) showed the lesion appeared to be arising from the submucosal layer, with a large blood vessel feeding the lesion. A fine needle biopsy revealed benign histology. Due to its large size, the lesion was not amenable to endoscopic removal. He underwent laparotomy with pyloroplasty, duodenotomy and partial duodenal resection due to concern for premalignant potential. Surgical pathology showed Brunner’s gland hyperplasia and hamartoma that was negative for malignancy. The patient is doing well with resolution of abdominal pain.

DISCUSSION:
Brunner’s gland hyperplasia is usually an incidental finding during endoscopy. Symptomatic presentations include gastrointestinal bleeding and intestinal obstruction. Literature search shows reports of Brunner’s hyperplasia usually 1–2 cm in size, there are few reported that are larger than 5 cm. Diagnosis requires histological examination of the mass, usually by endoscopy with excision or biopsy. CT scan and EUS can demonstrate the submucosal origin of the mass. Mode of excision depends on the size, location and presentation of the lesion. Upper gastrointestinal endoscopy with polypectomy is preferred. Laparoscopic polypectomy is also an option. Laparotomy is reserved for large, sessile tumors, failure of endoscopic approach or unstable bleeding patients. Few cases reported in literature have been managed with laparotomy. The potential cause of our patient’s large Brunner’s gland mass could be gastric acid hypersecretion as he had severe GERD with peptic esophageal strictures.