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Introduction
Lymphangioma is a rare benign tumor of lymphatic vessel origin. The tumor often appears in the head and neck region at a young age and can occasionally be found in the abdomen of adults with protean degrees of symptoms depending on the tumor size and location. However, lymphangioma of the small intestine is extremely rare with only a few cases reported in the literature. As the tumor is not well-recognized, many patients with small intestine lymphangioma have been given an incorrect preoperative diagnosis. The ideal treatment for the disease is surgical excision, and the prognosis is comparatively good. In this paper, we report a rare case of ileal lymphangioma with gastrointestinal hemorrhage preoperatively diagnosed using enteroscopy and treated with surgery.
Case Report
A 50-year-old woman with an one-month history of hematochezia and fatigue was admitted to the Department of Oncology, the Fourth Affiliated Hospital of Harbin Medical University, Harbin. She had no other symptoms on presentation, except for the nonspecific finding of anemia. The patient’s past medical history and her family history were unremarkable. Physical examination did not reveal any significant positive findings. Routine blood tests showed that the patient’s red blood cell (RBC) count and hemoglobin (HGB) had decreased to 2.18 × 1012/L and 52 g/L, respectively within a week.
Gastroscopic and colonoscopic examinations were negative. However, enteroscopy showed segmental swelling in the ileum and an accumulation of clotted blood in the intestinal lumen (Fig. 1). On biopsy, histologic examination showed an archetypical lymphangioma.
Endoscopy showed areas of edema in a segment of ileum.
Laparoscopic examination revealed segmental thickening and swelling of the ileum, and focal white nodules present in the ileal mucosa (Fig. 2). A segmental resection was performed (Fig. 2), and the patient was discharged 2 weeks after surgery following an uneventful postoperative course. The results of pathologic examination were consistent with the diagnosis of ileal lymphangioma (Fig. 3). There were no reports of other abnormal findings during the subsequent 1-year follow-up.
A segment of ileum with thickening, swelling and white-tan mucosal nodules.
Microscopically, there was a single layer of flat endothelium with various amounts of fibrous and lymphoid tissue.
Discussion
Lymphangiomas are deemed to have derived from a congenital abnormality of the lymphatic system and can be classified into 3 types: capillary, cavernous and cystic. In the capillary and cavernous types, cutaneous lesions are predominant, while the cystic form is generally found in the abdomen and retroperitoneum[1]. On account of different tumor locations and tumor sizes, symptoms of intra-abdominal lymphangiomas vary from abdominal pain to that of mass effect including bowel obstruction and even intussusception[2]. However, when the lesion is localized within the small intestine it may cause severe gastrointestinal hemorrhage[3].
Auxiliary imaging diagnostic modalities such as US, CT and MRI may be helpful in the diagnosis of intra-abdominal lymphangiomas, especially when located in proximity to the mesentery and retroperitoneum. However, the application of new techniques, such as double balloon enteroscopy and capsule endoscopy, may prove essential for the diagnosis of small intestine lymphangioma[4,5].
In this case, enteroscopy combined with histopathologic examination of the biopsy specimen was very helpful in making the correct preoperative diagnosis. Further, we learned that when gastroscopic and colonoscopic examination show negative results in patients with gastrointestinal hemorrhage, enteroscopy can be vital to the correct diagnosis of rare tumors of the small intestine, such as lymphangiomas.
The ideal treatment for intra-abdominal lymphangiomas is surgical excision which consists of segmental resection and local excision. The laparoscopic procedure may be an option for some patients[6,7]. A new report suggests that select cases of small intestine lymphangiomas can be treated with double balloon enteroscopy which can avoid the potential morbidity and mortality secondary to traditional surgery[8]. A segmental resection was performed in this case, and the findings from the follow up confirmed the outcome of surgery for this type of disease.
In summary, lymphangiomas are unusual tumors and are rarely detected within the small intestine. Better understanding of this type of tumor may help in the accurate diagnosis of the disease and in choosing the best treatment option for patients.
Conflict of interest statement
No potential conflicts of interest were disclosed.
- Received December 9, 2009.
- Accepted January 11, 2010.
- Copyright © 2010 by Tianjin Medical University Cancer Institute & Hospital and Springer
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