East Carolina University Medical Center Greenville, NC
Jinye Liu, DO1, Abdulazeez Swaiti, MD2, Christin Wilkinson, MD2, Stephanie Ibarra Lepe, MD1, Ahmed Hebishy, MD1, Christina Santana, MD1, Amy Chang, MD1, Saeed Graham, MD2, Michelle Cooley, BS3, Matthew Tugman, BA3, Catherine Gray, BS3, Rahul Pamarthy, MD2, Zarak Khan, MD4, Hassam Ali, MD2, Shiva Poola, MD1, Kara Regan, MD4 1East Carolina University Medical Center, Greenville, NC; 2East Carolina University, Greenville, NC; 3East Carolina University Brody School of Medicine, Greenville, NC; 4East Carolina Gastroenterology, Greenville, NC
Introduction: Follicular lymphoma is a B-cell lymphoma characterized by the t(14;18) chromosomal translocation, originating from germinal centers in lymph nodes. It is the second most common lymphoma in the United States after diffuse large B-cell lymphoma. Neuroendocrine tumors of the rectum are rare. The combination of gastrointestinal follicular lymphoma and neuroendocrine tumor is exceedingly rare, with only one case reported in the literature.
Case Description/Methods: A 68-year-old female with a history of pulmonary embolism and deep vein thrombosis was evaluated for nausea, vomiting, dysphagia, and changes in bowel habits. Recent imaging revealed enlarged mesenteric and retroperitoneal lymph nodes compared to prior. Colonoscopy and endoscopy were performed for further evaluation. Endoscopy showed multiple enlarged lymph nodes in the peri-gastric and porta-hepatis regions, all biopsied. Colonoscopy showed nodular patchy mucosa throughout the colon and a yellow submucosal nodule in the rectum, all biopsied. Pathological examination revealed low-grade follicular lymphoma in the stomach and right and left colon, and a well-differentiated, low-grade neuroendocrine tumor in the rectum involving the submucosa and lamina propria.
Discussion: The gastrointestinal tract (GIT) is the most common site for extranodal lymphoma, yet primary follicular lymphoma of the GIT is uncommon and poorly defined, typically presenting in the small intestine and accounting for 1-3% of GI non-Hodgkin’s lymphoma (NHL). Neuroendocrine tumors (NETs) are ubiquitous, with up to 55% occurring in the GIT, primarily in the small intestine (45%) and rectum (20%). Secondary primary malignancies (SPMs) are associated with NETs, with one study noting that among 78 individuals with NETs and a secondary primary malignancy, five had lymphoma, including one with follicular lymphoma. What marks our case unique is that there has been no documented incidence of follicular lymphoma and NET in different segments of the GI tract in the same patient. Some theorize that neuron-specific enolase (NSE) is a shared immunohistochemical marker between NETs and NHLs. This case highlights the unclear pathogenesis of NETs associated with SPMs and underscores the need for further research and the development of formal guidelines for diagnostic workup and treatment plans.
Figure: Figure 1: Yellowish submucosal small nodule in rectum, found to be neuroendocrine tumor (top left). Nodular patchy mucosa suggestive of lymphoid follicles at the sigmoid colon (top right), transverse colon (middle right), and ascending colon (bottom left). Several small nodules can be seen in the gastric body as well (bottom right).
Disclosures:
Jinye Liu indicated no relevant financial relationships.
Abdulazeez Swaiti indicated no relevant financial relationships.
Christin Wilkinson indicated no relevant financial relationships.
Stephanie Ibarra Lepe indicated no relevant financial relationships.
Ahmed Hebishy indicated no relevant financial relationships.
Christina Santana indicated no relevant financial relationships.
Amy Chang indicated no relevant financial relationships.
Saeed Graham indicated no relevant financial relationships.
Michelle Cooley indicated no relevant financial relationships.
Matthew Tugman indicated no relevant financial relationships.
Catherine Gray indicated no relevant financial relationships.
Rahul Pamarthy indicated no relevant financial relationships.
Zarak Khan indicated no relevant financial relationships.
Hassam Ali indicated no relevant financial relationships.
Shiva Poola indicated no relevant financial relationships.
Kara Regan indicated no relevant financial relationships.
Jinye Liu, DO1, Abdulazeez Swaiti, MD2, Christin Wilkinson, MD2, Stephanie Ibarra Lepe, MD1, Ahmed Hebishy, MD1, Christina Santana, MD1, Amy Chang, MD1, Saeed Graham, MD2, Michelle Cooley, BS3, Matthew Tugman, BA3, Catherine Gray, BS3, Rahul Pamarthy, MD2, Zarak Khan, MD4, Hassam Ali, MD2, Shiva Poola, MD1, Kara Regan, MD4. P0344 - Double Trouble in the GI Jungle: Navigating Follicular Lymphoma and Neuroendocrine Tumor, ACG 2024 Annual Scientific Meeting Abstracts. Philadelphia, PA: American College of Gastroenterology.