August Georg University Augusta, GA, United States
Shreyans Doshi, MD1, Pooja Mude, MD2, Pearl Princess Uy, MD1, Loc Ton, MD3, John Erikson Yap, MD4 1August Georg University, Augusta, GA; 2Medical College of Georgia, Augusta, GA; 3Kaiser Permanente Sacramento, Sacramento, CA; 4Medical College of Georgia at Augusta University, Augusta, GA
Introduction: Cystic fibrosis (CF) is the most common life limiting recessive genetic disorder of Caucasian. The pancreas is one of the organs earliest and most seriously affected by CF. It is also known that CF presents with an increased risk for the development of gastrointestinal cancers. There are only two reported cases of pancreatic mucinous cystic neoplasm (MCN) in patients with CF. We present a rare case of a CF patient found to have a giant MCN causing abdominal distention.
Case Description/Methods: A 22-year-old female with CF (F508del/G551D) and exocrine pancreatic insufficiency sought consultation when her husband noticed abdominal distention in her left upper quadrant when she laid flat. She denied any trauma or any recent surgery. Physical examination was consistent with a large firm non-tender left upper quadrant mass on palpation. Her blood tests including complete blood count, electrolytes as well as liver function tests were normal. Pregnancy test was negative. A computed tomography and magnetic resonance imaging of her abdomen revealed a 16.5 x 12.5 x 17.9 cm cystic lesion with septations anterior to the left kidney between the stomach and the spleen. Patient underwent surgical resection for removal of the 15-pound cystic structure with pathology consistent with mucinous cystic neoplasm with multifocal low grade and high grade dysplasia. Patient did well post- surgery with no complications and had resolution of her symptoms.
Discussion: Patients with CF present with a variety of pancreatic disorders which includes both functional (endocrine and exocrine) insufficiency as well as structural abnormalities (fibrofatty replacement of parenchyma and pancreatic cyst). The architectural changes especially pancreatic cyst becomes a challenge in ruling out malignancy given the altered anatomy. A high index of suspicion is needed in evaluating CF patients due to the known increased risk of GI malignancy. Some literature even advocates for early surgical evaluation of suspicious pancreatic cystic lesions. Our case is the biggest pancreatic MCN reported in literature of a CF patient that was successfully treated by complete resection of the MCN via distal pancreatectomy.
Shreyans Doshi indicated no relevant financial relationships.
Pooja Mude indicated no relevant financial relationships.
Pearl Princess Uy indicated no relevant financial relationships.
Loc Ton indicated no relevant financial relationships.
John Erikson Yap indicated no relevant financial relationships.
Shreyans Doshi, MD1, Pooja Mude, MD2, Pearl Princess Uy, MD1, Loc Ton, MD3, John Erikson Yap, MD4. P0048 - Honey, I am Not Pregnant! Giant Pancreatic Mucinous Cystic Neoplasm in a Cystic Fibrosis Patient Causing Abdominal Distention, ACG 2021 Annual Scientific Meeting Abstracts. Las Vegas, Nevada: American College of Gastroenterology.