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Lymphangioma of the ileocecal valve clinically masquerading as a submucosal small intestinal GIST: Report of a case and literature review.
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PMID:  25038213     Owner:  NLM     Status:  Publisher    
Lymphangiomas are rare tumors affecting the gastrointestinal tract, and may be seen in the bowel, gall bladder, and pancreas. They resemble hemangiomas, but consist of spaces of variable sizes containing lymph. In this report, we describe the case of a 53-year-old male who presented with abdominal pain and constipation. Computerized tomography (CT) scan showed a polypoidal lesion at the ileocecal valve which was thought to be a gastrointestinal stromal tumor. Resected specimen did, however, show a lymphangioma. We also describe the clinicopathologic features of gastrointestinal lymphangiomas with a literature review.
Omar A Al-Obeed; Maha-Hamadien Abdulla
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Journal Detail:
Title:  Saudi journal of gastroenterology : official journal of the Saudi Gastroenterology Association     Volume:  20     ISSN:  1998-4049     ISO Abbreviation:  Saudi J Gastroenterol     Publication Date:    2014 July-August
Date Detail:
Created Date:  2014-7-19     Completed Date:  -     Revised Date:  -    
Medline Journal Info:
Nlm Unique ID:  9516979     Medline TA:  Saudi J Gastroenterol     Country:  -    
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Languages:  ENG     Pagination:  262-264     Citation Subset:  -    
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Journal Information
Journal ID (nlm-ta): Saudi J Gastroenterol
Journal ID (iso-abbrev): Saudi J Gastroenterol
Journal ID (publisher-id): SJG
ISSN: 1319-3767
ISSN: 1998-4049
Publisher: Medknow Publications & Media Pvt Ltd, India
Article Information
Copyright: © Saudi Journal of Gastroenterology
Received Day: 06 Month: 2 Year: 2014
Accepted Day: 08 Month: 2 Year: 2014
Print publication date: Season: Jul-Aug Year: 2014
Volume: 20 Issue: 4
First Page: 262 Last Page: 264
PubMed Id: 25038213
ID: 4131310
Publisher Id: SJG-20-262
DOI: 10.4103/1319-3767.136986

Lymphangioma of the Ileocecal Valve Clinically Masquerading as a Submucosal Small Intestinal Gist: Report of a Case and Literature Review
Omar A. Al-Obeedaff1
Maha-Hamadien Abdullaaff1
Colorectal Research Center, Department of Surgery, King Saud University, Riyadh 11472, Saudi Arabia
Correspondence: Address for correspondence: Prof. Maha-Hamadien Abdulla, Assistant Professor, Director of Colorectal Research Center, Department of Surgery, King Khalid University Hospital, King Saud University, P.O.Box 7805 (37), Riyadh 11472, Saudi Arabia. E-mail:

Lymphangioma is a benign tumor commonly found in children. It usually occurs in the head, neck, and axillary region.[1] Many lymphangiomas are congenital malformations of the lymphatic system and are considered to be lymphatic hamartomas.[2] They are masses consisting of flat endothelial cells and supporting connective tissue. Lymphocytes that are surrounded by islands of fat cells are also usually present.[3] Colorectal lymphangioma was first reported by Chisholm and Hillkowitz in 1932.[4] A case of lymphangioma of the ileocecal valve clinically masquerading as small submucosal nodule of the ileocecal valve and mimicking gastrointestinal stromal tumors (GISTs) was encountered in our hospital.


A 56-year-old Saudi male was admitted to King Khalid University Hospital (KKUH) with the chief complaint of epigastric pain for 3 months associated with feeling of fullness, heartburn, and constipation. He had a past medical history of a surgical operation for renal stones and tympanoplasty, but with no significant family history. The vital signs recorded upon admission were as follows: Temperature 36.8°C, blood pressure 147/76 mmHg, pulse rate 72 beats/min, respiratory rate 20 breaths/min, and oxygen saturation 97%. His weight was 64 kg and height was 162 cm. Upon examination, the patient's abdomen was soft and lax with no tenderness, rigidity, or guarding noted.

Laboratory tests revealed an increase in white blood cells (WBC) and bilirubin with decreased red blood cells (RBC), hematocrit (HCT), and sodium level. Upper and lower endoscopic procedures were done, and colonoscopic findings were non-significant up to terminal ileum while gastroesophageal endoscopy showed a moderate hiatus hernia with peptic esophagitis Grade A and thickening of the mucosal folds at the pylorus. Ultrasound of the upper abdomen showed small cysts in both kidneys. Abdominal computerized tomography (CT) showed a small polypoidal lesion at the ileocecal valve [Figure 1]. The patient was later on discharged with home medication of Pantoprazole.

Laparoscopic right hemicolectomy was later performed. Histopathology of the resected specimen showed dilated lymphatic vessels consistent with a lymphangioma. Excess fat and congested blood vessels were also seen near the described lymphangioma. There was no evidence of malignancy and the excision appeared complete [Figures 1 and 2]. This finding confirms the lymphatic nature of the vessels shown in Figure 2.

Patient was discharged without complications and he has been keeping well until this date.


Lymphangiomas probably represent vascular malformations rather than tumors. They may occur in the skin, subcutaneous tissue, and deep soft tissues or in visceral organs. These lesions exist in three forms: Capillary, cavernous, and cystic (cystic hygroma) variants. Intra-abdominal lymphangiomas are rare, and 60% of the tumors are present in patients under the age of 5 years, but a significant percentage of them does not manifest until adult life.[1]

The most common location is in the mesentery, followed by the omentum, mesocolon, and retroperitoneum. In addition to a palpable mass, patients often develop symptoms of an acute condition in the abdomen caused by the common complications of intestinal obstruction, volvulus, and infarction. In fact, a provisional diagnosis of acute appendicitis is frequently entertained because of the common occurrence of right lower quadrant pain. In contrast, retroperitoneal tumors produce few acute symptoms, but ultimately are diagnosed by virtue of a large palpable mass causing displacement of one or more organs.

In the past, an abdominal lymphangioma was seldom diagnosed preoperatively. Currently, the diagnosis can usually be suspected with a combination of radiologic studies.[5, 6, 7, 8]

Ultrasonography is useful for localizing and determining the cystic nature of the tumors. On CT scans, the tumors appear as homogeneous, non-enhancing lesions with variable attenuation values depending on whether the fluid is chylous or serous.

GISTs may arise in the small intestine, colon, or anorectum. These tumors may develop within any portion of the gut wall, but most are centered within the submucosa or the muscularis propia. Most GISTs appear to be well circumscribed, although some may be multinodular. Most GISTs are asymptomatic, but some may present with abdominal pain and bleeding.

Our case represents a rare and perhaps unique example of a submucosal lymphangioma of the ileocecal region which mimicks GISTs both clinically and radiologically.

Furthermore, it distinctly differs from other adult intestinal lymphangiomas of the intestine which usually present with intussusceptions.[9]


Source of Support: Nil

Conflict of Interest: None declared.


The authors would like to express their gratitude to Dr. Ammar Rikabi for helping with histology and his valuable discussion and input that greatly improved this case report. Also we would like to thank Dr. Sharif Sharqawei for his help with the radiological images.

1. Chung JH,Suh YL,Park IA,Jang JJ,Chi JG,Kim YI,et al. A pathologic study of abdominal lymphangiomasJ Korean Med SciYear: 1999142576210402167
2. Singh S,Baboo ML,Pathak IC. Cystic lymphangioma in children: Report of 32 cases including lesions atrrare sitesSurgeryYear: 197169947515578458
3. Kittredge RD,Finby N. The many facets of lymphangiomaAm J Roentgenol Radium Ther Nucl MedYear: 1965955666
4. Chisholm AJ,Hillkowitz P. Lymphangioma of the rectumAm J SurgYear: 1932172812
5. Kochman ML,Wiersema MJ,Hawes RH,Canal D,Wiersema L. Preoperative diagnosis of cystic lymphangioma of the colon by endoscopic ultrasoundGastrointest EndoscYear: 19974520469041015
6. Begos DG,Sandor A,Modlin IM. The diagnosis and management of adult intussusceptionAm J SurgYear: 199717388949074370
7. Gayer G,Apter S,Hofmann C,Nass S,Amitai M,Zissin R,et al. Intussusception in adults: CT diagnosisClin RadiolYear: 1998535379464437
8. Wan YL,Lee TY,Hung CF,Ng KK. Ultrasound and CT findings of a cecal lymphangioma presenting with intussusceptionEur J RadiolYear: 1998277799587772
9. Matsuba Y,Mizuiri H,Murata T,Niimi K. Adult intussusception due to lymphangioma of the colonJ GastroenterolYear: 200338181512640534


[Figure ID: F1]
Figure 1 

Axial and coronal CT with IV and oral contrast. CT abdomen shows small well-defined intracecal polypoidal mass lesion, related postero-superior to the ileocecal valve

[Figure ID: F2]
Figure 2 

Microscopic findings of lymphangioma of the ileocecal valve. (a) Colonic mucosa with underlying proliferating and irregular lymphatic channels consistent with lymphangioma [hematoxylin and eosin (H and E) stain, ×100]. (b) Lymphangioma high-power view showing lymphatic channels with few scattered lymphocytes in their wall (H and E stain, ×200). (c) Lymphangioma - D2-40 immunohistochemical (IHC) stain for podoplantin (D2-40) showing positive staining of the lining cells (IHC, D240, ×100)

Article Categories:
  • Case Report

Keywords: Gastrointestinal stromal tumors, laparoscopic surgery, lymphangioma.

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