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  Table of Contents  
Year : 2011  |  Volume : 48  |  Issue : 1  |  Page : 139-140

Lymphangiomatosis of the colon mimicking acute appendicitis

1 Department of Surgery, Trakya University, School of Medicine, Edirne, Turkey
2 Department of Pathology, Trakya University, School of Medicine, Edirne, Turkey
3 Department of Gastroenterology, Trakya University, School of Medicine, Edirne, Turkey

Date of Web Publication10-Feb-2011

Correspondence Address:
Atakan Sezer
Department of Surgery, Trakya University, School of Medicine, Edirne
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/0019-509X.76651

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How to cite this article:
Yagci MA, Sezer A, Yeldan E, Coskun I, Hatipoglu AR, Altaner S, Umit H. Lymphangiomatosis of the colon mimicking acute appendicitis. Indian J Cancer 2011;48:139-40

How to cite this URL:
Yagci MA, Sezer A, Yeldan E, Coskun I, Hatipoglu AR, Altaner S, Umit H. Lymphangiomatosis of the colon mimicking acute appendicitis. Indian J Cancer [serial online] 2011 [cited 2021 Jun 12];48:139-40. Available from:


Lymphangiomatosis is a progressive condition caused by the abnormal and extensive benign proliferation of lymphatic tissue. The lymphatic proliferation can be throughout the body where lymphatics are normally found except the brain, which does not contain lymphatic tissue. The most common effected sites are bone, lung, mediastinum, liver, spleen, and skin. [1] Lymphangiomatosis are extremely rare benign tumors of the colon. Acute abdomen mimicking appendicitis is an unusual presentation of the disease. A 25-year-old female was admitted with right lower quadrant abdominal pain diagnosed as acute appendicitis. Physical examination revealed tenderness on the right lower quadrant. Abdominal ultrasonography demonstrated multiple hypoechoic and hypervascular lesions on the right lower quadrant. Computed tomography (CT) and magnetic resonance investigation (MRI) revealed a mass of ascending colon in 6×9 cm size [Figure 1]. There was no luminal pathology on colonoscopy examination. On exploration, multiple thin-walled cystic masses containing serous fluid were found adjacent to the caecum and ascend colon [Figure 2]. Right hemicolectomy was performed with adherent mass. The pathological investigation revealed colonic lymphangiomatosis [Figure 3]. The patient's postoperative course was uneventful and discharged at the seventh postoperative day. Lymphangiomas are focal proliferations of well-differentiated lymphatic tissue that present as multicystic or sponge-like accumulations. The colon is the rarest localization. [1] Clinical presentation of the tumor depends on its location and growth pattern. Abdominal pain, anemia, abnormal bowel habits, intussusception, and protein-losing enteropathy are the main symptoms of colonic lymphangiomatosis. Occasionally, colonic lymphangiomatosis were presented with abdominal symptoms mimicking acute abdomen. [2] To our knowledge, the current report is the first case of lymphangiomatosis of the colon mimicking acute appendicitis. Lipomas, leiomyomatosis, neurofibromatosis, and metastatic cancers should be in differentiated diagnosis. [3] Ultrasonography shows a sharply defined cystic or multiple cystic mass, often with internal septation.[4] CT and MRI are required for evaluation of the wall structure of the colon and coexisting with extracolonic lesions. Endoscopic ultrasound is a well-established, increasingly popular, and minimally invasive procedure for the assessment of submucosal tumors and also useful in differentiation of extramural lesions in the colon.[5] Essential diagnostic application is colonoscopy and required for histopathological examination. Colonic lymphangiomatosis with normal colonoscopy is extremely rare. Needle aspiration biopsy from a lymphangioma is not recommended in the diagnostic period. Malignant transformation of a colonic lymphangioma has not been reported. In reported cases after curative resection, there is no evidence of recurrence. The treatment modalities for gastrointestinal lymphangiomatosis are the endoscopic or surgical resection and symptomatic medications. Resection of the involved segment is the most preferred surgical procedure. Endoscopic treatment has become increasingly indicated recently with tumors measuring 2 cm or smaller in the maximal diameter. [1] Colonic lymphangiomatosis mimicking acute appendicitis is a rare abdominal emergency. The onset of the disease needs a high level of concern and steady supervision to reduce mortality and morbidity. This unusual presentation of colonic lymphangiomatosis should be kept in mind in the differential diagnosis of acute appendicitis and other submucosal colon tumors.
Figure 1: (a) Computed tomography demonstrates a hypoechoic mass adjacent to the ceacum. (b) Magnetic resonance investigation revealed a mass of ascendant colon in 6 cm × 9 cm in size

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Figure 2: Operative view of the tumor. Attention to the tumor arising from ceacum

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Figure 3: (a) Lymphangiomatosis in large bowel (arrows) (H+E, ×50). (b) At low power, each small cyst is lined by a flattened layer of lymphatic epithelium (H+E, ×100).

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AS drafted the manuscript, MAY helped in preparation of the manuscript, EY assisted surgery and did literature review, IC interpreted and correlated imaging data with text narrative, ARH helped in drafting the manuscript, SA reported prepared pathology slides and photos, HU conceived idea, organized case reporting. All authors read and approved the final manuscript.

  References Top

1.Faul JL, Berry GJ, Colby TV, Ruoss SJ, Walter MB, Rosen GD, et al. Thoracic lymphangiomas, lymphangiectasis, lymphangiomatosis, and lymphatic dysplasia syndrome. Am J Respir Crit Care Med 2000;161:1037-46.  Back to cited text no. 1
2.Kunimura T, Inagaki T, Hayashi R, Katou H, Iwaku K, Morohoshi T. A case of multiple colonic lymphangiomas causing intussusception. J Gastroenterol 2005;40:316-8.  Back to cited text no. 2
3.Kim J, Han D, Hong CH, Lee HL, Kim JP, Sohn JH, et al. Colonic lymphangiomatosis associated with protein-losing enteropathy. Dig Dis Sci 2000;50:1747-53.  Back to cited text no. 3
4.Kim HA, Min SK, Lee JH. Colonic lymphangiomatosis with normal colonoscopic finding in an Adult. J Korean Surg Soc 2005;73:514-6.  Back to cited text no. 4
5.Pabuccuoglu U, Sokmen S, Gürel D, Obuz F, Füzün M. Polyposis-like presentation of multiple lymphangiomas of the sigmoid colon. Tumori 2004;90:625-7.  Back to cited text no. 5


  [Figure 1], [Figure 2], [Figure 3]

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