A very rare case of duodenal hemolymphangioma presenting with iron deficiency anemia

Abstract

Introduction: Intraabdominal lymphangiomas account for less than 5% of all lymphangiomas and small intestinal hemolymphangioma is a very rare benign tumor.

Presentation of case: Here we describe the first case of primary ulcerated duodenal hemolymphangioma in a 24-year-old woman, causing occult bleeding from gastrointestinal tract. She presented with an unexplained refractory iron-deficiency anemia and gastroduodenoscopy revealed an ulcerated and polypoid lesion of the second portion of the duodenum. Partial resection of the duodenum was thus performed and the final pathological diagnosis was hemolymphangioma.

Discussion: There were only two reports, one of a hemolymphangioma of the pancreas invading to the duodenum and another of a small intestinal hemolymphangioma, presenting with gastrointestinal bleeding until May 2012.

Conclusion: The aim of this case report is to highlight the difficulty in making an accurate preoperative diagnosis and describe the surgical management of an unusual location for a very rare tumor. To arrive at a definitive diagnosis and exclude malignancy, partial resection of the duodenum was considered to be the required treatment.

Keywords: Anemia; Duodenum; Hemolymphangioma; Occult gastrointestinal bleeding; Small intestine.

Fig. 1

Gastroduodenoscopy revealing a bleeding, ulcerated polypoid mass in the second portion of the duodenum and precisely at the papilla of Vater region. (Kindly granted by Dr. Raffaele Bennato, Gastroenterology Unit, “Antonio Cardarelli” Hospital, Naples, Italy.)

Fig. 2

Cholangio-MRI in coronal plane on T2-weighted images showing a solid, polypoid mass (40 mm × 15 mm) at the lateral wall of the second/third portion of the duodenum with no evidence of ampullary obstruction or periduodenal tissue infiltration. (Kindly granted by Dr. Francesco Di Pietto, Department of Radiology, “Antonio Cardarelli” Hospital, Naples, Italy).

Fig. 3

Wide local resection of the tumor via duodenotomy (A), with preservation of the ampulla (B).

Fig. 4

Histopathology showing thin walled vascular channels lined by flattened endothelium containing acellular eosinophilic amorphous material. Hematoxylin and eosin stain, 250×. (Kindly granted by Dr. Amedeo Boscaino and Dr. Giovanna Carrillo, Department of Anatomic Pathology, “Antonio Cardarelli” Hospital, Naples, Italy.)