Title : Acute neurological deficit as initial presentation of extranodal marginal zone lymphoma
Abstract:
Extranodal marginal zone lymphomas (EMZL) originate in the post germinal centers of the B-cell marginal zone and have a tendency to develop in various epithelial tissues. Gastric marginal zone lymphoma (GMZL) is the most common subtype, accounting for 75% of Gastrointestinal lymphomas. It typically involves a single site, but has potential for systemic spread and progression to B cell lymphoma. Risk factors that tend to play a key role in EMZL include genetic and environmental factors; being H. Pylori infection the most common. Although usually misdiagnosed, regression can be achieved with eradication therapy during the early stages of disease when secondary to Helicobacter Pylori infection. This is the case of a 62-year-old male, former smoker, with unremarkable past medical history, who came to the Emergency Department with a complaint of right labial commissure deviation and right-sided lower extremity weakness. Patient was admitted due to Acute Neurological Deficit (AND) and treated accordingly. During inpatient admission patient presented complaint of severe left lower quadrant abdominal pain associated with non-bloody diarrhea. Abdominopelvic CT scan revealed nonspecific soft tissue through the mesentery suggestive of carcinomatosis, and an hepatic mass lesion involving posterior segment of right lobe. Subsequent colonoscopy revealed sigmoid diverticulosis and internal hemorrhoids. Upper endoscopy was remarkable for clean base gastric ulcers and a fundic gland polyp (Figure 1) with histological evidence of gastric mucosa with EMZL and antral biopsies with chronic antral gastritis negative for H. Pylori (Figure 2). Immunohistochemical testing also revealed CD45, and B-cell markers (CD20, CD79a, CD10). These immunohistochemical data, confirms that the gastric fundus lesion is EMZL. Unfortunately, our patient presented a very aggressive course of disease and expired within 2 weeks of diagnosis. EMZL abides a rare pathology and can easily be misdiagnosed due to its unspecific symptoms, such as in our patient, who presented with AND due to underlying malignancy and hypercoagulable state. Although primary lymphomas of GI tract may be rare, it is of utmost importance to screen for risk factors. In our patient, H. Pylori staining, the most common cause for developing such a tumor, was confirmed negative. Because of its rapid progression, other etiologies such as viral diseases (HIV, Hepatitis B virus, Epstein-Barr virus) and genetic disorders were unable to be ruled out. Our aim is that this case may serve for educational purpose, in order to help recognize gastric cancer with unusual presentation.
Audience Take Away:
- Will help recognize gastric cancer with unusual presentation. Our aim is to educate the gastroenterology community about rare presentations of GI cancers, in order to improve screening and diagnostic procedures and improve quality of life of our patients.
