Duodenal intussusception due to a giant neuroendocrine carcinoma in a patient with Peutz–Jeghers syndrome

Abstract

Duodenal intussusception is a rare entity. To date, only a few cases have been reported in the literature. In this report, a case of duodenal intussusception due to an unusual tumor was presented and the clinical features of this entity were discussed. A 42-year-old man with Peutz-Jeghers syndrome presented with epigastric pain, vomiting, and severe anemia. Computed tomography scan revealed synchronous duodenojejunal and jejunojejunal intussusceptions. An emergency laparotomy revealed a polypoid mass originating from the lateral wall of the descending duodenum with intussusception of the distal duodenum. Histological examination demonstrated a poorly differentiated neuroendocrine carcinoma with muscularis infiltration, vascular invasion, and a Ki-67 index of 20%. A comprehensive literature search revealed 44 English reports that provided adequate descriptions of an additional 47 such cases. Clinical presentation was usually chronic and nonspecific. Diagnostic modalities included ultrasonography, upper gastrointestinal series, computed tomography, and endoscopy. Five patients were due to a non-neoplastic lesion; however, the other 43 patients were secondary to a tumor, benign in 35 cases and malignant in eight cases. Only one patient was treated by endoscopic polypectomy, whereas the remaining underwent open surgeries. Duodenal intussusception is a challenging condition due to its rarity and nonspecific presentation. It should be considered in the differential diagnosis of gastric outlet obstruction, upper gastrointestinal bleeding, pancreatitis, and obstructive jaundice.

Jejunoduodenal intussusception caused by a solitary polyp in a woman with Peutz-Jeghers syndrome: a case report

“…Abdominal distention and local tenderness are the most frequent findings. We located a palpable mass in our patient but an abdominal mass has been noted in only 12.5% of cases [5]. Abdominal CT has been reported to be the most useful imaging modality.…”

“…This clinical presentation has been observed in 47% to 69% of adult patients with PJS and most of them were due to polyps located in the small intestine [3]. The majority of PJS intussusceptions reported in the literature are ileal or jejunal [2,5-7]. Colo-colonic intussusception is reported in only a few cases [8].…”

Taking the Lead: A Case Report of a Leiomyoma Causing Duodeno-Duodenal Intussusception and Review of Literature

“…2 More commonly, patients present with nonspecific symptoms including intermittent abdominal pain, nausea, vomiting, and anemia. 3 Given the wide range of diseases with this constellation of symptoms, physicians must have a strong clinical suspicion to make the diagnosis. Work-up may include hemoglobin levels, fecal occult blood tests, cross-sectional imaging (CT or MRI), and endoscopy (EGD, colonoscopy, capsule endoscopy, or push endoscopy).…”

A 16-Year-Old Female with Peutz-Jeghers Syndrome

“…17 The majority of intussusceptions reported in the literature are in ileal or jejunal regions. 18,19 Colo-colonic intussusception is reported in only a few cases. 20 In this case, the patient developed jejuno-jejunal and ileo-colic intussusceptions.…”