P1822 - POSTER WITHDRAWN

Zhongqian Lin, DO, Aruni Rahman, MD, Samuel Quintero, MD
New York-Presbyterian / Brooklyn Methodist Hospital, Brooklyn, NY

Introduction: Heterotaxy, a rare condition caused by disruption of left-right axis orientation during embryonic development, results in the atypical arrangement of intrathoracic and abdominal organs. It is often linked to congenital cardiac malformations but can involve organs such as the spleen, stomach, and liver. Data on non-cardiac heterotaxy and its association with gastrointestinal malignancy are limited. We present a case of an elderly adult with pancreatic ductal adenocarcinoma and heterotaxy of the stomach and spleen.

Case Description/Methods: A 69-year-old woman with a recent finding of a pancreatic head mass presented with four months of abdominal pain. Exam revealed moderate epigastric tenderness but unremarkable vital and routine blood work. A contrasted CT scan showed a 5.3 x 6.3 x 6.7 cm hypodense mass in the pancreatic head, abutting the superior mesenteric artery, right kidney, adrenal gland, duodenum, jejunum, and the lesser curvature of the stomach. Interestingly, stomach was located in the right upper quadrant (RUQ) with associated mural thickening as well as polysplenia in the RUQ. Old records indicated stomach and spleen in the location and severe pancreatic atrophy. An inpatient EUS with biopsy confirmed pancreatic ductal adenocarcinoma. After discharge, she completed two cycles of FOLFIRINOX but experienced worsening functional status and recurrent ED visits for pain, eventually enrolling in hospice. A follow-up CT revealed an increased mass size (9.0 x 8.1 x 7.6 cm) with central necrosis, complete circumferential encasement of the proximal duodenum with fistulization, and new spleen invasion in the RUQ.

Discussion: Heterotaxy is associated with congenital heart abnormalities in neonates with high mortality, but rarer for abdominal organs. Our patient has incidental findings of stomach and spleen malposition. Mentions of an association between pancreas and heterotaxy were only sporadic in the literature and none with pancreatic malignancy. Pancreatic atrophy is typically linked with pancreatic cancer, usually from the same atrophy location, but opposite in this case. We hypothesize that abdominal heterotaxy patients may have increased pancreatic malignancy risk. Extra organs complicate tumor resection, leading to poorer prognosis, as seen with the tumor invading the stomach and spleen in the RUQ. Despite known heterotaxy and atrophy, no screening occurred due to lack of guideline. Clinicians should recognize potential malignancy risks with heterotaxy and promote further research.



Disclosures:


Zhongqian Lin, DO, Aruni Rahman, MD, Samuel Quintero, MD. P1822 - POSTER WITHDRAWN, ACG 2024 Annual Scientific Meeting Abstracts. Philadelphia, PA: American College of Gastroenterology.