Vien X. Nguyen

Usefulness of PET/CT Imaging in Systemic IgG4-related Sclerosing Disease. A Report of Three Cases

Autoimmune pancreatitis is the pancreatic manifestation of a novel clinicopathological disorder called systemic IgG4-related sclerosing disease. Beside the pancreas, this entity affects other sites (salivary glands, orbit, lung, thyroid, gallbladder, biliary tree system, kidney, abdominal aorta, retroperitoneum, prostate, and lymph node) by infiltration with IgG4-positive plasma cells. Several case reports and small case series have demonstrated the utility of integrated positron emission tomography/computed tomography (PET/CT) in monitoring therapy and documenting relapse and flare-up of autoimmune pancreatitis. However, there are no reports on the usefulness of PET/CT in selecting extrapancreatic sites for tissue sampling. We herein demonstrate the clinical utility of integrated PET/CT in 3 cases of systemic IgG4-related sclerosing disease for targeting extrapancreatic biopsy sites.

Appropriate use of endoscopy in the diagnosis and treatment of gastrointestinal diseases: up-to-date indications for primary care providers

The field of endoscopy has revolutionized the diagnosis and treatment of gastrointestinal (GI) diseases in recent years. Besides the ‘traditional’ endoscopic procedures (esophagogastroduodenoscopy, colonoscopy, flexible sigmoidoscopy, and endoscopic retrograde cholangiopancreatography), advances in imaging technology (endoscopic ultrasonography, wireless capsule endoscopy, and double balloon enteroscopy) have allowed GI specialists to detect and manage disorders throughout the digestive system. This article reviews various endoscopic procedures and provides up-to-date endoscopic indications based on the recommendations of American Society for Gastrointestinal Endoscopy and American Cancer Society for primary care providers in order to achieve high-quality and cost-effective care.

18F-FDG PET/CT Imaging of the Pancreas: Spectrum of Diseases

Since the introduction of integrated positron emission tomography-computed tomography (PET/CT), it has a great impact on the field of oncology. Comparing to other conventional scanners, only PET/CT is capable of providing important information on accurate detecting, staging/restaging, and post-therapeutic monitoring of many cancers. Many studies have demonstrated that PET/CT changes the management in approximately 30% of all cancer patients. Because 2-((18)F)-fluoro-2-deoxy-D-glucose (FDG) is a nonspecific tracer, understanding the PET/CT limitations and pitfalls for various pancreatic conditions can lead to more accurate interpretation of PET/CT images, which ultimately would impact patient care. As a result, it is important for radiologists and other clinicians to familiarize themselves with a wide spectrum of pancreatic PET/CT findings simulating cancer from benign entities.

Digital Image Analysis Is a Useful Adjunct to Endoscopic Ultrasonographic Diagnosis of Subepithelial Lesions of the Gastrointestinal Tract

Objective. The purpose of this study was to explore the role of digital image analysis in differentiating endoscopic ultrasonographic (EUS) features of potentially malignant gastrointestinal subepithelial lesions (SELs) from those of benign lesions. Methods. Forty‐six patients with histopathologically confirmed gastrointestinal stromal tumors (GISTs), carcinoids, and lipomas who had undergone EUS evaluation were identified from our database. Representative regions of interest (ROIs) were selected from the EUS images, and features were extracted by texture analysis. On the basis of these features, an artificial neural network (ANN) was built, trained, and internally validated by unsupervised learning followed by supervised learning. Outcomes were the performance characteristics of the ANN. Results. A total of 106, 111, and 124 ROIs were selected from EUS images of 8, 10, and 28 patients with lipomas, carcinoids, and GISTs, respectively. For each ROI, 228 statistical parameters were extracted and later reduced to the 11 most informative features by principal component analysis. After training with 50% of the data, the remainder of the data were used to validate the ANN. The model was “good” in differentiating carcinoids and GISTs, with area under the receiver operating characteristic curve (AUC) values of 0.86 and 0.89, respectively. The model was “excellent” in identifying lipomas correctly, with an AUC of 0.92. Conclusions. Digital image analysis of EUS images is a useful noninvasive adjunct to EUS evaluation of SELs.

The Association of Ménétrier Disease with Ulcerative Colitis: A Case Report with Implications on the Pathogenesis of Ménétrier Disease

Ménétrier disease (MD) is a rare hypertrophic condition of the gastric mucosa. The unusual association of MD with ulcerative colitis (UC) has been reported in the literature in eight cases. Transforming growth factor-alpha (TGF-alpha) is overexpressed in UC and appears to play a role in colonic healing and repair. Overproduction of TGF-alpha in murine stomach has been shown to induce gastric hypertrophy similar to MD. It can be hypothesized that increased expression of TGF-alpha may occur in the gastric mucosa in patients with UC and may lead to MD. We report the ninth case of MD associated with UC. The role of TGF-alpha and treatment with cetuximab are discussed.

Gastrointestinal: Gastrointestinal involvement of mantle cell lymphoma

A 58 year-old Caucasian man, with a history of hyperlipidemia and benign prostate hypertrophy, complained of 3-week rectal bleeding. He also had a 6-month history of left lower quadrant pain, diarrhea, unintentional 14-pound weight loss, and urticaria. Examination revealed no lymphadenopathy, palpable mass, or organomegaly. Abnormal laboratory results were as follows: gamma globulin 1.7 (normal: 0.6–1.6 g/dL), immunoglobulin A 439 (50–400 mg/dL). Other studies included white blood cell, hemoglobin, albumin, lactate dehydrogenase, uric acid, beta-2 microglobulin, C-reactive protein, C1 esterase inhibitor, and other immunoglobulins were within normal limits. Computed topographic (CT) colonography (Figure 1, left) and colonoscopy (Figure 1, right) demonstrated innumerable sessile polyps (2–4 mm) throughout the entire colon. Esophagogastroduodenoscopy and video capsule endoscopy also revealed multiple small nodules in the duodenal bulb and ileum, respectively. Immunohistochemical staining of excised colonic polyps was positive for Cyclin D1 (Figure 2, 200x). The chest and abdomen CT scans showed no hepatosplenomegaly or lymphadenopathy, and the bone marrow examination was normal. The patient decided to return to his native state for further treatment. Mantle cell lymphoma (MCL) is a subtype of the B-cell nonHodgkin lymphomas (NHL) and comprises about 7% of adult NHL. MCL is characterized by the chromosomal translocation t(11:14), resulting in overexpression of Cyclin D1. Cyclin D1 is a nuclear protein that promotes cell proliferation. Positive immunohistochemical staining for this protein is diagnostic for MCL. Approximately 25% of patients with MCL present with extranodal disease. Extranodal sites include bone marrow (>60%), liver and spleen (45–60%), Waldeyer’s ring, and gastrointestinal tract (20%). Multiple lymphomatous polyposis (MLP) is a rare primary gastrointestinal (GI) manifestation of MCL. It occurs predominately in male (65%) with mean age of 63 years. Endoscopy shows small polyps (0.5–2 cm) along one or more segments of the GI tract. Common symptoms are weight loss, abdominal pain, diarrhea, and hematochezia. Clinical presentation of chronic urticaria in our patient is atypical. MLP is the least common type of primary GI lymphomas. Differentiating MLP from follicular and mucosaassociated lymphoid tissue (MALT) lymphomas is crucial because MLP has one of the poorest prognoses (median survival of 8–20 months) of all NHL subtypes and there is no accepted therapeutic approach.

Positron emission tomography/computed tomography imaging of adrenocorticotropic hormone-producing small-cell neuroendocrine carcinoma of the cervix

Ectopic ACTH-dependent Cushing syndrome-inducing small-cell neuroendocrine carcinoma of the cervix, undiagnosed by computed tomography and whole-body octreotide scintigraphy, was identified using F-18 FDG PET/CT imaging. This article is protected by copyright. All rights reserved.

Right-sided intra-thoracic kidney associated to Bochdalek hernia in an adult patient

Diaphragmatic hernia of Bochdalek (BH) and intra-thoracic kidney (IK) are both rare congenital, developmental anomalies. In some extremly rare occasion, these two congenital anomalies can be associated in the same patient. The kidney typically exits the retroperitoneal space through the foramen of Bochdalek. The majority of IK and BH are discovered incidentally. A conservative approach can be proposed in asymptomatic cases. In this report, the case of a 35-year-old man presenting with 1-week of acute abdominal pain is presented. Chest radiography revealed a posterior mass in the right hemithorax, which was later confirmed by chest computed tomography and magnetic resonance imaging to be a congenital IK associated with a BH.

Gastrointestinal: Gastrointestinal involvement of mantle cell lymphoma: Education and Imaging

A 58 year-old Caucasian man, with a history of hyperlipidemia and benign prostate hypertrophy, complained of 3-week rectal bleeding. He also had a 6-month history of left lower quadrant pain, diarrhea, unintentional 14-pound weight loss, and urticaria. Examination revealed no lymphadenopathy, palpable mass, or organomegaly. Abnormal laboratory results were as follows: gamma globulin 1.7 (normal: 0.6–1.6 g/dL), immunoglobulin A 439 (50–400 mg/dL). Other studies included white blood cell, hemoglobin, albumin, lactate dehydrogenase, uric acid, beta-2 microglobulin, C-reactive protein, C1 esterase inhibitor, and other immunoglobulins were within normal limits. Computed topographic (CT) colonography (Figure 1, left) and colonoscopy (Figure 1, right) demonstrated innumerable sessile polyps (2–4 mm) throughout the entire colon. Esophagogastroduodenoscopy and video capsule endoscopy also revealed multiple small nodules in the duodenal bulb and ileum, respectively. Immunohistochemical staining of excised colonic polyps was positive for Cyclin D1 (Figure 2, 200x). The chest and abdomen CT scans showed no hepatosplenomegaly or lymphadenopathy, and the bone marrow examination was normal. The patient decided to return to his native state for further treatment. Mantle cell lymphoma (MCL) is a subtype of the B-cell nonHodgkin lymphomas (NHL) and comprises about 7% of adult NHL. MCL is characterized by the chromosomal translocation t(11:14), resulting in overexpression of Cyclin D1. Cyclin D1 is a nuclear protein that promotes cell proliferation. Positive immunohistochemical staining for this protein is diagnostic for MCL. Approximately 25% of patients with MCL present with extranodal disease. Extranodal sites include bone marrow (>60%), liver and spleen (45–60%), Waldeyer’s ring, and gastrointestinal tract (20%). Multiple lymphomatous polyposis (MLP) is a rare primary gastrointestinal (GI) manifestation of MCL. It occurs predominately in male (65%) with mean age of 63 years. Endoscopy shows small polyps (0.5–2 cm) along one or more segments of the GI tract. Common symptoms are weight loss, abdominal pain, diarrhea, and hematochezia. Clinical presentation of chronic urticaria in our patient is atypical. MLP is the least common type of primary GI lymphomas. Differentiating MLP from follicular and mucosaassociated lymphoid tissue (MALT) lymphomas is crucial because MLP has one of the poorest prognoses (median survival of 8–20 months) of all NHL subtypes and there is no accepted therapeutic approach.

A Fishy Nodule

Question: A 65-yearold patient presented with a painful epigastric mass. It had been diagnosed as a hematoma after a blunt abdominal trauma 3 months prior. Since then, the mass remained and continued to cause the patient pain. Past medical history included chronic alcohol consumption and benign prostatic hyperplasia. Upon physical examination, e was determined to have a hard, irregular mass in the epigastric area associated with an ulcerated, 1.5 cm large nodule of the umbilicus Figure A). Laboratory tests revealed a mild anemia (10 g/dL) with a low prothrombin time (63%) and a total bilirubinemia of 26 mg/L. is serum alpha-fetoprotein concentration was 16.8 ng/mL. Thoracoabdominopelvic computed tomographic (CT) was performed. It evealed a 12 7-cm solid mass in the epigastric region associated with a hypertrophy of the left hepatic lobe (Figure B). What is your diagnosis? the GASTROENTEROLOGY web site (www.gastrojournal.org) for more information on submitting your favorite image to Clinical Challenges and Images in GI.