Ariyo Ihimoyan

A Rare Occurrence of Primary Hepatic Leiomyosarcoma Associated with Epstein Barr Virus Infection in an AIDs Patient

Primary hepatic leiomyosarcoma is exceedingly rare accounting for less than 1% of the hepatic tumors. Close to 45 cases have been reported in the English literature. Presentation is usually nonspecific and diagnosis is often delayed until tumors reach a large size. This leads to a dismal prognosis. The tumors are not yet fully understood, hence the standard of care is not well defined. Curative resection remains the mainstay of management. Close association of Epstein Barr virus (EBV) induced soft tissue sarcomas is proven, especially in the presence of immunosuppression encountered in HIV/AIDS patients and in posttransplant patients. We herein present a case report of a 54-year-old man diagnosed to have HIV/AIDS and EBV infection admitted to our hospital with complaints of intractable hiccups for more than a week. Extensive workup revealed primary leiomyosarcoma of the liver.

Systemic Reactive Amyloidosis Associated with Castleman's Disease

We report this case of secondary amyloidosis associated with Castlemans disease. A 51-year-old man presented with systemic symptoms of generalized weakness, fatigue, unintended weight loss, anorexia and progressively worsening abdominal distension. On examination he was found to have an indurated right-sided submandibular mass and tense ascites. He was found to have multiorgan dysfunction with deranged liver function tests and renal failure. Ascitic fluid analysis revealed evidence of spontaneous bacterial peritonitis. Biopsy of the submandibular mass revealed angiofollicular lymph node hyperplasia consistent with a diagnosis of Castlemans disease. A subsequent liver biopsy showed extensive deposition of amyloid protein. Bone marrow biopsy also showed the presence of amyloid and increased kappa light chain-restricted plasma cells. The patient was not considered a candidate for chemotherapy or solid organ transplantation in view of active sepsis and poor physical condition. Secondary systemic amyloidosis complicating Castlemans disease is very rare. Untreated secondary systemic amyloidosis often has a rapidly fatal course, such as seen in our patient.

Systemic vascular resistance in cirrhosis: a predictor of severity?

Background The aim of this study was to investigate whether systemic vascular resistance (SVR) correlates with validated prospective scoring systems such as Model for End-stage Liver Disease (MELD) and its modifications. Methods Patients with cirrhosis, who were admitted to hospital with decompensation (as defined by development of ascites, hepatic encephalopathy, and variceal bleeding) and underwent echocardiography were included in this study. Laboratory data required for computing MELD score, serum bilirubin, serum creatinine, international normalized ratio, and serum sodium were collected for every patient. We tabulated hemodynamic and echocardiography parameters that enabled calculation of SVR. We analyzed the correlation between SVR and each of the individual prognostic scores. Results A total of 771 patients with a diagnosis of decompensated cirrhosis were included in the study. Two hundred and sixty-two patients were found to have a low sodium level (<135 mEq/L) and 509 were found to have a normal sodium level (>135 mEq/L). In the patients with hyponatremia, we found statistically significant inverse correlations between SVR and validated liver severity models. However, these correlations were not seen in patients with normonatremia. Conclusion We observed a statistically significant inverse correlation between SVR and all the validated liver disease severity models used in this study among patients with hyponatremia but not in those with normonatremia.

Ileoileal Intussusception Secondary to an Ileal Fibroma

Intussusception is defined as the telescoping of a segment of the gastrointestinal tract (intussusceptum) into an immediately adjacent distal bowel (intussuscipiens). Intussusception is a relatively rare cause of intestinal obstruction in adults. Unlike in children, a lead point is present in 90% of adult cases. The most common causes of small bowel intussusception are benign, usually hamartomas, lipomas, inflammatory polyps, adenomas and leiomyomas, in contrast to the large intestine where malignant tumors, usually adenocarcinomas, are more common. The clinical presentation of adult intussusception is non-specific with variable manifestations, predominantly those of intestinal obstruction, often making the diagnosis a challenge. The onset of symptoms may be acute, intermittent or chronic. We present a rare case of an ileal fibroma presenting with intussusception. A 43-year-old woman presented to our outpatient clinic with a history of recurrent abdominal pain. The clinical presentation and CT scan findings led to the diagnosis of ileoileal intussusception. Subsequently she underwent laparotomy which revealed an ileal fibroma as the lead point of the intussusception. Surgical exploration remains essential for diagnosis and treatment since in the majority of cases a pathologic lead point is identified. Ileal fibroma is an uncommon benign neoplasm of the small bowel and must be considered in the differential diagnosis for small bowel intussusception.

An inflammatory fibroid polyp in the hepatic flexure of the colon treated with argon plasma coagulation, endoscopic clipping and polypectomy.

Inflammatory fibroid polyp (IFP) is a rare benign polypoid lesion of the gastrointestinal tract. Most IFPs occur in the stomach and colonic occurrence is very rare. Histologically IFP is characterized by a mixture of numerous small vessels, fibroblasts and edematous connective tissue associated with marked inflammatory infiltration by eosinophils. We present a rare case of a pedunculated IFP in the hepatic flexure of the colon treated successfully with a combination of argon plasma coagulation, endoclipping and polypectomy. A 74-year-old asymptomatic female underwent a screening colonoscopy in our hospital. A 12-mm pedunculated polyp was found at the hepatic flexure of the colon. After saline injection, we attempted to remove the polyp with a hot snare. However the polyp stalk was extremely difficult to resect despite several attempts with the hot snare. We placed an endoclip at the base of the stalk and then applied argon plasma coagulation at 1.0 l/min and 40 W. After these measures we were able to resect the stalk and the polyp was retrieved. Histologically the polyp was located in the submucosa of the gastrointestinal tract. Proliferation of spindle cells and infiltration of inflammatory cells such as plasma cells and eosinophils were observed. The spindle cells were positive for CD34 and S100 but negative for c-kit and muscle markers. These findings are consistent with a histopathological diagnosis of IFP.

Primary Systemic Amyloidosis with Extensive Gastrointestinal Involvement

We report this case of a 42-year-old woman who presented with a debilitating illness manifested by intractable nausea, vomiting, diarrhea and unchecked weight loss. The patient had multisystem involvement that presented as anemia, abnormal liver function tests and progressively deteriorating renal function necessitating dialysis. She was found to be profoundly hypoalbuminemic secondary to malabsorptive and protein-losing enteropathy in tandem with nephrotic range proteinuria. Intolerance to enteral feeding led the patient to be dependent on parenteral nutrition. Serum immunofixation revealed IgG lambda monoclonal protein. The patient underwent endoscopic evaluation with biopsies taken from the gastrointestinal tract that confirmed the diagnosis of primary systemic light-chain amyloidosis. A subsequent bone marrow biopsy revealed normocellular bone marrow with deposition of amyloid. The patient was not considered for autologous stem cell transplantation as the outcomes in patients with multisystem involvement are often poor, with a high mortality risk. Diffuse primary systemic light-chain amyloidosis involving the gastrointestinal tract is a rare entity and is to be considered among differentials in patients presenting with unexplained malabsorptive symptoms.

Hypertriglyceridemia Induced Pancreatitis in a Non-Diabetic Pregnant Patient Requiring the Use of Total Parenteral Nutrition.

Hypertriglyceridemia induced pancreatitis in pregnancy is established and has been widely reported. However there are very scanty reports of cases involving the use of total parenteral nutrition. We report the case of a 37-year-old gravida 3, para 2 woman at 34 weeks of gestation who presented with one day of severe epigastric pain radiating to the back, nausea and bilious vomiting caused by pancreatitis induced by hypertriglyceridemia. Her initial serum triglyceride, amylase and lipase concentration were 6,552 mg/dl, 314 U/L and 537 U/L respectively. She initially received intravenous fluids and insulin with reduction of serum triglyceride levels to 583 mg/dl on the fifth day of admission. However attempts to refeed the patient with solid food resulted in induction of hypertriglyceridemia and relapse of pancreatitis. Lipid free total parenteral nutrition was commenced in the third week of admission and lead to a better control of triglyciderides and resolution of pancreatitis until delivery of a full term healthy neonate.

A Rare Cause of Abdominal Pain and Mass in an 18-Year-Old Patient: A Diagnostic Dilemma

We present a case of an 18-year-old male who presented with complains of abdominal pain, nausea and vomiting for 2 years. An esophagogastroduodenoscopy (EGD) revealed a 3 mm nodule on the lesser curvature of the stomach and prominent gastric folds. Biopsy of the nodule revealed a well-differentiated neuroendocrine tumor (NET) in lamina prop with focal extension into muscularis mucosa consistent with a gastric carcinoid. Tumor cells stained with neuron-specific enolase (NSE), chromogranin and synaptophysin only. The prominent gastric fold biopsy revealed gastric fundic mucosa with mucosal edema and focal mild chronic inflammation. Serum gastrin level was found to be 2,083 pg/mL. Abdomen CT and endoscopic ultrasound (EUS) revealed a mass near the pancreatic neck. These findings were consistent with a functional gastrin producing well-differentiated grade 1 neuroendocrine neoplasm (gastrinoma). The patient underwent exploratory laparotomy with resection of the mass and resulting in normalization of gastrin levels.

Anemia, intractable vomiting, chronic diarrhea, and syndrome of inappropriate antidiuretic secretion: a diagnostic dilemma

Rationale: Strongyloidiasis hyperinfection and disseminated disease have high mortality rates due to several complications and early detection of Strongyloides infection is therefore prudent. Patient concerns: A 37-year-old male patient came with chronic diarrhea, intractable vomiting and was found to have hyponatremia, and anemia on the initial laboratory tests. Diagnoses: Further work up revealed syndrome of inappropriate antidiuretic secretion to be the cause of the hyponatremia in addition to gastrointestinal loses. His hospital course was complicated by persistent hyponatremia and later development of partial small bowel obstruction. Interventions: Considering his symptoms we had a suspicion of small bowel pathology for which he underwent an esophagogastroduodenoscopywith biopsies that revealed strongyloidosis as the cause of his symptoms. He was also found to have human T-cell lymphotropic virus infection, likely contributing to the disseminated disease. Outcomes: He was started on ivermectin with complete resolution of symptoms and improvement of hyponatremia. Lessons: It is very important to suspect Strongyloides infection in a patient presenting with syndrome ofinappropriate antidiuretic secretion as hyperinfection and disseminated disease can be life threatening without antihelmintic therapy.

Viekira Pak Induced Fatal Lactic Acidosis: A Case Report of an Unusual Side Effect

Viekira Pak is a new direct-acting antiviral agent that has an excellent efficacy in treating patients with chronic HCV. FDA released a safety warning that Viekira Pak can cause serious liver injury mostly in patients with underlying advanced liver disease. We report the first case of fatal lactic acidosis presenting 3 days after initiating therapy with Viekira Pak. Although it is very hard to precisely determine the cause of lactic acidosis, our case highlights an unusual side effect that ensued after starting the medication. Given the complexity of drug-drug interactions that can happen with the new direct-acting antiviral agents and the paucity of data regarding coadministration and methods of monitoring, a thorough review should be pursued prior to initiating these medications.