A Rare Presentation of Small Bowel Hemolymphangioma

ABSTRACT

Hemangiomas and lymphangiomas are both benign tumors and lesions that can be present at birth. Hemolymphangiomas are a rare malformation composed of both vascular and lymphatic tissue. These malformations are more commonly seen in infancy and are much more rarely identified within the adult population. We present a unique case report of a patient who presented with melena and after extensive diagnostic evaluation, was found to have a hemolymphangioma isolated to the small bowel and its mesentery.

INTRODUCTION

Hemangiomas are one of the most common benign tumors seen in infancy and are often the result of endothelial cell proliferation.¹ Lymphangiomas are another type of benign lesion of the lymphatic system that are believed to result from blockage of the venolymphatic system during fetal development.² Hemolymphangiomas are both vascular and lymphatic congenital malformations that are not commonly seen in the clinical setting. Diagnosis is often made before the age of 2 years, and these tumors are rarely found in the adult population. These malformations can be found in various anatomical sites with cases often reported in the head and neck region.³ Other locations that have been uncommonly reported in the literature include the pancreas, liver, gallbladder, and stomach.³ To our knowledge, there have been 14 cases of small bowel hemolymphangiomas reported in the literature, which are compared and represented in Table 1. This case highlights a patient with chronic iron deficiency anemia and melena found to have a hemolymphangioma isolated to the small bowel and its mesentery.

Table 1.

Comparison of cases of small bowel hemolymphangioma reported in the literature

Reference	Age	Presentation	Location	Diagnostic modality	Management
Du et al.	54	Abdominal mass	Small bowel mesentery/jejunum	CT/MRI/Exploratory laparoscopy	Surgical resection
Du et al.	52	Abdominal mass	Small bowel mesentery/jejunum	CT/exploratory laparotomy	Surgical resection
Antonio et al.	24	Anemia of unknown etiology	Duodenum	EGD/MRI	Surgical resection
Gomez-Galan et al.	43	Chronic anemia of unknown etiology	Duodenum/jejunum	Capsule endoscopy/enteroscopy	Surgical resection
Iwaya et al.	70	Iron deficiency anemia	Proximal small bowel	Capsule endoscopy/Double-balloon enteroscopy	Surgical resection
Yang et al.	20	Anemia and melena	Proximal jejunum	CT/enteroscopy	Surgical resection
Xiao et al.	42	Melena and symptomatic anemia		Capsule endoscopy/enteroscopy	Enteroscopic injection sclerotherapy
Blanco et al.	45	Recurrent melena and anemia	Jejunum	Capsule endoscopy/enteroscopy	Surgical resection
Fang et al.	57	Melena and anemia	Proximal small bowel	Enteroscopy	Surgical resection
Teng et al.	55	Abdominal pain	Jejunum	CT/enteroscopy	Surgical resection
Yu et al.	16	Abdominal pain, vomiting, diarrhea	Mesentery of proximal ileum	Abdominal ultrasound, CT, MRI, PET/CT	Combined laparoscopic and open resection
Ajmi et al.	23	Colicky abdominal pain, constipation, vomiting	Mesentery of small bowel	CT/MRI/Diagnostic laparoscopy	Referral to specialized center
Xu et al.	32	Iron deficiency anemia	Jejunum	EGD/Colonoscopy/CT/Double-balloon enteroscopy	Surgical resection
Lim et al.	55	Abdominal pain with palpable mass	Mesentery of ileum	Abdominal ultrasound/CT	Surgical resection

CT, computed tomography; EGD, esophagastroduodenoscopy; MRI, magnetic resonance imaging; PET, positron emission tomography.

CASE REPORT

A 49-year-old woman with a medical history significant for iron deficiency anemia on iron infusion therapy presented for further evaluation with gastroenterology because of the chronic nature of her anemia. The patient was initially evaluated by hematology after requiring intermittent transfusions of packed red blood cells. She reported intermittent melena. Abdominal/pelvic computed tomography (CT) was obtained by hematology for nonspecific abdominal pain, which did not reveal an acute intra-abdominal pathology. Given negative findings, further gastrointestinal evaluation was deemed necessary with esophagastroduodenoscopy and colonoscopy. Initial esophagastroduodenoscopy was without acute pathology, and biopsies of the small bowel and stomach were negative for celiac disease and Helicobacter pylori. Colonoscopy revealed dark stool throughout the colon and terminal ileum, limiting complete assessment. Subsequent video capsule endoscopy revealed multiple sessile and polypoid/nodular lymphangiectasias in the small bowel with some of the lymphangiectasias clustered and bleeding (Figure 1). With these findings, the patient underwent single-balloon enteroscopy (SBE) for additional endoscopic evaluation as an outpatient. Before scheduled SBE, the patient was evaluated in the emergency department for chest pain. Laboratory test results at that time revealed a hemoglobin of 4.7, hematocrit of 15.6, ferritin of 3, iron of 406, and iron saturation of 81%. She was transfused and discharged home with SBE performed the following week. SBE confirmed the presence of a large lobulated cluster of yellowish lymphangiectasias located in the ileum measuring roughly 5 × 2 cm with diffuse oozing (Figure 2). The diffuse oozing was not amenable to endoscopic therapy given the extent of bleeding and limited maneuverability of the enteroscope at maximal insertion. Biopsies were collected, and tattoos were placed. The patient was referred urgently to surgical oncology for segmental small bowel resection because of the frequency of blood transfusions and active oozing seen on SBE. Robotic-assisted small bowel resection was performed in which a 6.5 cm segment was removed. Postoperatively, the patient's hemoglobin and hematocrit were 7.7 and 25.8, respectively. Pathology report from the resected small bowel noted a 5.4 × 2.8 × 1.0 cm circumferential tan-gray to tan-white polypoid granular mass that was consistent with benign vascular neoplasm with mixed features of lymphangioma and hemangioma (Figure 3). Immunohistochemical staining with D2-40 highlighted dilated lymphatic channels within the resected mass. The patient has had close outpatient follow-up with hematology for her iron deficiency anemia with most recent laboratory test results revealing a hemoglobin and hematocrit of 12.3 and 38.6, respectively (Figure 3).

Figure 1.

Video capsule endoscopy revealing hemolymphangioma.

Figure 2.

Hemolymphangioma visible on single-balloon enteroscopy.

Figure 3.

Postsurgical hemolymphangioma specimen.

DISCUSSION

Hemolymphangiomas are a rare vascular and lymphatic malformation that are commonly found in soft tissues of the head and neck.³ More uncommonly, there have been reported cases of hemolymphangiomas of the gastrointestinal tract such as the small bowel and/or its mesentery.³ Outside of infancy and childhood, these benign tumors are uncommonly seen in the adult population. These tumors can be classified as either primary or secondary, with primary being congenital and secondary related to issues with lymphatic obstruction that is often a result of trauma, surgery, or chronic inflammation.⁴ In our case, the patient did not have a history of the above, indicating that the hemolymphangioma is likely congenital in origin.

Clinically, signs and symptoms of hemolymphangiomas vary based on the location and size. Until this benign tumor approaches a clinically significant size, it may be asymptomatic for a period. In some cases, gastrointestinal bleeding may be the first symptom that arises.⁵ Our patient had been experiencing several episodes of symptomatic anemia such as fatigue, palpitations, dizziness, and lightheadedness that necessitated evaluations in the emergency department for blood transfusions. Initial differential diagnosis at the time included abnormal uterine bleeding, poor nutrition, peptic ulcer disease, and gastrointestinal malignancy. To identify these benign tumors, imaging is often implemented in the form of CT or magnetic resonance imaging for further classification as well as potential planned interventions.⁴ Interestingly, our patient had several abdominal and pelvic CT imaging studies, which were repeatedly negative for an intra-abdominal process or pathology. Video capsule endoscopy and single-balloon enteroscopy ended up being the most effective diagnostic tools for identification of the tumor.

After successful identification of the tumor, appropriate surgical planning ensued with the patient undergoing robotic-assisted small bowel resection with side-to-side end-to-end anastomosis. Surgery is often the treatment of choice for these rare congenital malformations with robotic or laparoscopic interventions deemed viable options. In a meta-analysis performed by Choi et al, robotic-assisted abdominopelvic surgery was associated with shorter length of stay, lower open conversion rates, and lower intraoperative complications when performed by experienced surgeons.⁶ Robotic-assisted surgery has been increasingly used in gastrointestinal procedures because of improved instrument articulation, enhanced visualization, and ergonomic advantages for the surgeon. However, current evidence, primarily derived from colorectal surgery, suggests that robotic approaches are generally comparable with conventional laparoscopy in terms of safety and overall outcomes, with some studies demonstrating associations with lower conversion rates and faster return of bowel function in selected cases. In this case, a robotic approach was selected based on surgeon preference and anticipated technical advantages in precise dissection and intracorporeal anastomosis within a confined segment of the small bowel. Complete surgical excision of the tumor is often the treatment of choice for localized disease because this has the lowest rate of recurrence compared with other modalities of treatment such as cryotherapy, injection of sclerotic agents, and radiotherapy.⁷

Hemolymphangiomas can be difficult to diagnose because of their rarity and nonspecific presentation. It is important to correctly identify hemolymphangiomas after biopsy or surgical resection to rule out potential malignant etiologies. Immunohistochemistry staining can help differentiate hemolymphangiomas from other potential etiologies such as adenocarcinoma, neuroendocrine tumor, cavernous lymphangioma, or cystic lymphangioma. Commonly, hemolymphangiomas stain positive for CD31 and D2-40, which reflect vascular endothelial cells and lymphatic channels, respectively.^8,9 In our case, pathology was able to visualize the vascular architecture of the resected mass and, with positive D2-40 staining, further supporting the diagnosis of hemolymphangioma.

Despite its rare occurrence, consideration should be given to hemolymphangiomas as a potential cause of persistent anemia and gastrointestinal bleeding as in the case of our patient. A thorough workup is crucial in identifying these tumors, especially if they arise in uncommon anatomic locations. Endoscopic evaluation can be an important diagnostic modality, especially if imaging is unable to identify the lesion or if imaging is inconclusive. Endoscopic evaluation serves as an important step in both the diagnosis and surgical planning for these tumors. Robotic-assisted resection represents a feasible minimally invasive approach for small bowel lesions; however, its advantages over conventional laparoscopy in this setting remain uncertain and warrant further study.

DISCLOSURES

Author contributions: All authors listed above have made substantial contributions to the conception of the work, drafting the work or revising it critically for important intellectual content, final approval of the version to be published, and agreement to be accountable for all aspects of the work. R Molina is the article guarantor.

Financial disclosure: None to report.

Informed consent was obtained for this case report.