By Huguet, Kevin L Metzger, Philip P; Menke, David M
Lymphangiomas of the colon are historically rare benign tumors. Only 331 cases have been reported in the world medical literature between 1931 and 2004. With widespread use of colonoscopy, however, they are being found more frequently. We report the case of a 74- year-old woman in whom a colonoscopy revealed a 3 x 4-cm submucosal lesion in the cecum that was eventually diagnosed as a lymphangioma. A CT of the abdomen showed a soft-tissue mass in the cecum and a low-density hepatic lesion. An endoscopic ultrasound of the colon showed a 3 x 4-cm hypoechoic lesion with internal septa arising from the submucosal layer of the cecum. This lesion resembled a vascular malformation; therefore a biopsy specimen was not taken. Pathologic findings of a specimen taken after a subsequent right hemicolectomy identified a submucosal lymphangioma. Published reports indicate that colonoscopy cures most lesions smaller than 2.5 cm in diameter. Resection should be reserved for larger lesions or those in patients exhibiting proteinlosing enteropathy. LYMPHANGIOMAS ARE BENIGN tumors most COmmonly found in the head, neck, or axillae during childhood. Intra-abdominal lymphangioma is rare, composing less than 5 per cent of all cases. Colorectal lymphangioma is even more unusual. We report the diagnosis and treatment of a patient with lymphangioma of the cecum, and we review the Englishlanguage medical literature on this entity.
A 74-year-old woman with a history of breast cancer and osteoporosis had a colonoscopy to investigate potential causes of a recent unexplained weight loss. The colonoscopy revealed a 3 ? 4-cm submucosal lesion in the cecum. The mass was bluish black and moderately soft when probed. An 8-mm tubular adenoma was also found in the ascending colon and was excised. Because of concern that the cecal mass represented a vascular lesion, biopsy specimens were not obtained.
A CT of the abdomen and pelvis confirmed the 3 ? 4-cm mass in the cecum (Fig. 1) and also identified a small, apparently benign, cystic lesion in the dome of the right lobe of the liver. An endoscopie ultrasonogram of the cecal mass showed multiple hypoechoic regions and several areas of mixed heterogenicity.
The lesion seemed to be within the submucosa rather than arising from the muscularis propria. Findings of general laboratory screening tests were within normal limits, including those from liver function tests and serum carcinoembryonic antigen.
A right hemicolectomy was conducted, and a 2.5 ? 3-cm tumor was removed from the cecum. Histopathologic findings identified a vascular tumor with benign lymphoid nodules in the wall and benign endothelium. No continuous mural, smooth muscle, or elastic lamina was identified. Pathologic features were diagnostic of lymphangioma (Fig. T). The patient’s postoperative course, recovery, and subsequent follow-up were unremarkable.
Lymphangiomas are usually found in the head, neck, or axillae. These sites account for 50 per cent to 75 per cent of all cases. Intra-abdominal lymphangiomas occur in just 5 per cent of all cases. Intraabdominal lymphangiomas are frequently located in the mesentery. Colorectal lymphangiomas such as the one we report here are extremely rare.
In 1932, Chisholm and Hillkowitz1 reported what they identified as the first case of a rectal lymphangioma. The rarity of this lesion is highlighted by the fact that in 1943, Helwig2 identified no lymphangiomas among 154 benign colonie tumors found during 1460 consecutive autopsies. Similarly, in 1969, Stout and Turell3 reported only 4 colonie and rectal lymphangiomas among 292 tumors of the colon and rectum, excluding adenomas, carcinoid tumors, and adenocarcinomas.
Colorectal lymphangiomas are being reported with increasing frequency, perhaps because of the increased use of colonoscopy. Our search of the Englishlanguage medical literature (Medline and PubMed using the terms colon, rectum, and lymphangioma) identified a total of 83 cases reported between 1932 and 1988.4 After 1988, 248 additional cases were reported.5″12 Thus, we reviewed a total of 331 published reports of colorectal lymphangioma.
FIG. l. CT of the abdomen shows a soft-tissue mass (arrows) in the cecum.
FIG. 2. Pathologic findings of a biopsy specimen identified dilated lymphatic spaces (arrowheads) and adjacent lymphoid nodules (arrows) consistent with submucosal lymphangioma (hematoxylin-eosin stain; x20).
Worldwide, colorectal lymphangioma has been reported predominantly in Eastern countries. Only 36 cases were documented in Western countries compared with 295 cases in Eastern countries. Of the 295 cases in Eastern countries, almost all (281; 95%), or 85 per cent of the overall total, occurred in Japanese patients.
Most lymphangiomas were identified in patients older than 40 years of age (peak incidence, 41-50 years and 61-70 years; Table 1). Thus, there did not appear to be a correlation between age and peak incidence. The male-to-female ratio was 1.39:1, for a slight predominance in men. The most common site of colorectal lymphangioma appears to be the transverse or ascending colon (Table 2).
TABLE 1. Frequency of Colorectal Lymphangiomas by Age Reported between 1931 and 2004a
As early as 1877, Von Wegner13 reviewed the classifications of lymphangioma and identified three main types: simple, cavernous, and cystic. In the early 1950s, four types of chylous cysts of the abdomen were described by Beahrs et al.:14 developmental, traumatic, neoplastic, and infected. Chylous cysts would now be identified as lymphangiomas. Almost 100 years after Von Wegner’s report, Berardi15 recommended that, although any localized cystic tumor (simple or multicystic) could be a lymphangioma, the term “lymphangiectasia” should be restricted to 4. tumor with diffuse involvement.
The exact histogenesis of a lymphangioma is not completely known. Willis16 suggested that lymphangiomas are hamartomas rather than true neoplasms. The rationale for this differentiation is that these lesions have not shown any malignant potential and appear to be inborn errors in tissue development.
Tumor size was recorded in 245 of the 331 published reports we reviewed, as well as in the medical record of our patient. The diameter of colorectal lymphangiomas varied from 0.4 cm to 23 cm. As many as 80 per cent of the lymphangiomas were multilocular.
TABLE 2. Anatomical Location of Colorectal Lymphangioma by World Region
TABLE 3. Treatment of Colorectal Lymphangioma
Most lymphangiomas are usually diagnosed preoperatively as submucosal tumors. Colonoscopy is the main diagnostic study. Lymphangiomas are typically pinkish, translucent, and tense with a lustrous surface. Their shape may change with peristalsis, compression, or a shift in the patient’s position. As many as 50 per cent may have a waist or stalk. Obtaining a biopsy specimen from a lymphangioma may result in an efflux of lymphatic fluid. Radiologie studies often reveal a sharply demarcated oval or round colonie defect. The differential diagnosis should include other benign submucosal tumors such as lipoma, leiomyoma, or colitis cystica profunda, as well as carcinoid tumors, carcinoma, or mucocele.
There have been two case reports of colorectal lymphangioma associated with colonie carcinoma.17- 18 Lymphangioma has also been associated with adenomas, hemorrhoids, anal fistulae, or anal fissures. These associated lesions are incidental and probably unrelated to the primary lymphangioma. Of note, however, were five patients who had proteinlosing enteropathy. The lymphangiomas of these patients were larger than typical and also were associated with surface ulceration or erosion.
The most common clinical signs and symptoms of lymphangioma are intermittent, cramping abdominal pain, or rectal bleeding. Less frequent findings include diarrhea, fullness, abdominal mass (invagination), or, as previously noted, protein-losing enteropathy. Some patients with lymphangioma may be asymptomatic.
The most common types of therapy include segmentai or radical resection, wedge resection, or snare polypectomy (Table 3). Endoscopie therapy has increasingly been indicated for treatment. Since about 1980, a lymphangioma of 2.5 cm or less in diameter has usually been treated by endoscopie snare polypectomy. Of the treatment methods reported for 224 patients since 1980, 52 per cent were endoscopie compared with 13 per cent before 1980. The most important aspect of either treatment approach is removal of the entire lesion rather than its puncture and drainage. Larger lesions or lesions that cause proteinlosing enteropathy characteristically have deeper muscular wall or subserosal penetration and should be treated by wedge or segmental resection.
Lymphangiomas of the colon and rectum are uncommon benign tumors with little malignant potential. Diagnosis is usually best accomplished with colonoscopy, and therapeutic colonoscopy can be used for most lesions that are less than 2.5 cm in diameter. Resection is best reserved for larger lesions or for those resulting in protein-losing enteropathy.
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KEVIN L. HUGUET, M.D.,* PHILIP P. METZGER, M.D.,* DAVID M. MENKE, M.D.t
From the *Department of Surgery and the tDepartment of Laboratory Medicine and Pathology,
Mayo Clinic, Jacksonville, Florida
Address correspondence and reprint requests to Philip P. Metzger, M.D., Department of Surgery, Mayo Clinic, 4500 San Pablo Road, Jacksonville, FL 32224.
Copyright Southeastern Surgical Congress Apr 2007
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