Large, Vascular Cecal Mass. (Case Histories).

ABSTRACT: A 74-year-old woman had a recent weight loss and history of breast and cutaneous malignancy. Medical evaluation included colonoscopy, which showed a 4 x 3 cm, vascular-appearing mass in the cecum. It was blue-tinged, soft, and easily deformed by pressure. Endoscopic ultrasonography characterized the lesion as a fluid-filled submucosal mass. Because of the suspicion of neoplasm, the patient had exploratory laparotomy and right hemicolectomy. Histopathologic examination revealed a lymphangioma. We review the literature regarding diagnosis and management of colonic lymphangiomas and describe a distinctive case.

Lymphangiomas are benign tumors arising from lymphatic channels, usually in the head and neck or axillary region. While gastrointestinal involvement is rare, this report describes a patient diagnosed with a lymphangioma of the colon.

CASE REPORT

A 74-year-old woman came for a medical evaluation, with her only complaint being an unexplained loss of 10 lb body weight. She had a remote history of unilateral mastectomy for breast cancer and had recently had excisional biopsy of an unknown type of cutaneous malignancy, which was done elsewhere. She had no gastrointestinal symptoms. An evaluation, including a physical examination, standard laboratory tests (including carcinoembryonic antigen level), and chest radiograph, was unremarkable. The patient then had a colonoscopy (Olympus CF-QI40L, Olympus America Inc, Melville, NY), which showed a large (4 x 3 cm) submucosal, vascular-appearing mass with central umbilication located in the cecum. The mass was blue-tinged, soft, and easily deformed by pressure. The endoscopist believed its appearance to be that of a vascular lesion or possibly a metastatic melanoma (Fig 1). There was also an 8 mm adenoma in the middle of the ascending colon, which was removed by hot biopsy forceps.

Computed tomography (CT) of the abdomen and pelvis found the soft tissue mass in the region of the cecum, as well as multiple low-density hepatic lesions, the largest measuring 1.9 cm in diameter (Fig 2). Endoscopic ultrasonography (EUS), with the 12 MHz Olympus UM-2R probe passed through the working channel of the standard colonoscope, documented a hypoechoic lesion, 4 x 3 cm in diameter, with internal septations located in the submucosal layer (Fig 3). Areas of mixed heterogeneity were also seen in the lesion. Since this was considered to be a vascular lesion, EUS-guided fine-needle aspiration biopsy was not attempted. Based on the large size of this lesion and the multiple low-density liver lesions seen on CT, surgery was recommended.

Subsequently, the patient bad exploratory laparotomy with the preoperative diagnosis of a vascular cecal tumor with possible hepatic metastases. Operative findings included a soft and mobile lesion in the cecum, without lymphadenopathy or other masses. Only a few small cysts in the liver were found. A right hemicolectomy with ileotransverse colon anastomosis was done, and the patient recovered uneventfully. Histopathologic analysis showed a 3 x 3 x 2.5 cm, blue-tinged, soft, vascular-appearing tumor adjacent to the ileocecal valve. On sectioning, clear fluid was found in the tumor. Microscopic examination revealed dilated lymphatic channels, confirming the diagnosis of submucosal lymphangioma (Fig 4).

DISCUSSION

Lymphangiomas are benign tumors arising from dilated lymphatic channels and are commonly found in the head and neck or axillary region. Previously, gastrointestinal involvement was thought to be rare. However, with increasing numbers of patients having endoscopic evaluations, this condition is now more frequently recognized. Lymphangioma of the colon was first described by Chisolm and Hillkowitz (1) in 1932. In recent years, more than 100 cases have been described in the Japanese literature and a fewer number in the western literature. (2,3) The patients are usually asymptomatic, and the lesion is often discovered during a procedure for investigation of an unrelated condition. Some patients may complain of abdominal pain, alteration in bowel habits, or rectal bleeding. Rarely, complications such as obstruction, intussusception, and protein-losing enteropathy have been reported. (4,5)

Findings on colonoscopy include a smooth-surfaced mass with a translucent appearance. (6) The shape can alter with a change in position, respiration, or direct pressure with a probe (pillow sign). It can occur anywhere in the colon, but right-sided lesions are more common. The size can vary from 0.5 to 10 cm. Lymphangiomas are usually solitary. When multiple lesions are present, the term lymphangiectasia has been used. Endoscopic ultrasonography is useful in the diagnosis and shows an anechoic lesion, usually with septations located in the submucosal layer. (3,7,8)

The differential diagnosis for submucosal tumors of the colon includes lipoma, lymphangioma, and leiomyoma. Carcinoid tumors and metastatic lesions can also manifest as submucosal masses, as can non-neoplastic lesions such as endometriosis and pneumatosis intestinalis. The BUS may be useful in distinguishing among these lesions by determining their layer of origin. Myogenic tumors such as leiomyoma and leiomyosarcoma arise in the fourth layer, as opposed to the third (submucosal) layer. In our case, the tumor arose from the submucosal layer, but the overlying mucosa appeared so vascular that we suspected a hemangioma or a metastatic melanoma.

In cases such as ours, surgical resection may be required to provide definitive diagnosis and treatment. Other, minimally invasive treatment options for lymphangiomas have also been described. Lesions in the head and neck area are typically managed by sclerotherapy. However, the use of sciero therapy has only been reported once for a colonic lymphangioma and was unsuccessful. (9) Several researchers have advocated endoscopic polypectomy for pedunculated or semipedunculated lymphangioma lesions less than 2 cm in diameter. (2,7,10,11) However, in one case, attempted polypectomy of a 1 cm nonpedunculated lymphangioma resulted in colonic perforation. (12) Other authors have developed a technique of deroofing the lymphangioma lesion with cyst drainage. (9) Variable results have been reported with aspiration of the lesion, with some authors noting a high recurrence rate. In addition, it is not clear that asymptomatic small lesions require any intervention. In our case, the endoscopic appearance and the large tumor size guided our choice of surgical resection.

In summary, we have reported an unusual case of large cecal lymphangioma, which was well-studied with colonoscopy, EUS, and CT and confirmed with surgical resection. Our review of the literature supports endoscopic management of small, symptomatic lymphangiomas (<2 cm in diameter), though asymptomatic lesions may not require intervention.

From the Division of Gastroenterology and Hepatology, Mayo Clinic, Jacksonville, Fla.

Reprint requests to Herbert C. Wolfsen, MD, Mayo clinic, 4500 San Pablo Rd, Jacksonville, FL 32224.

References

(1.) Chisholm AJ, Hillkowits P: Lymphangioma of the rectum. Am J Surg 1932; 17:281-282

(2.) Sato K, Maekawa T, Yabuki K, et al: Cystic lymphangiomas of the colon, Gastroenterology 1999; 34:520-524

(3.) Kochman ML, Wiersema MJ, Hawes RH, et al: Preoperative diagnosis of cystic lymphangioma of the colon by endoscopic ultrasound. Gastrointest Endosc 1997; 45:204-206

(4.) Matsui A, Okajima K, Ishii M, et al: Lymphangioma of the cecum with intussusception: a case report and review of literature [in Japanese/English abstract]. Nippon Rinsho Geka Gakkai Zasshi 1985; 47:234-239

(5.) Zilko PJ, Laurence BH, Sheiner H, et al: Cystic lymphangiomyoma of the colon causing protein-Losing enteropathy. Am J Dig Dis 1975; 20:1076-1080

(6.) Camilleri M, Satti MB, Wood CB: Cystic lymphangioma of the colon. endoscopic and histologic features. Dis Colon Rectum 1982; 25:813-816

(7.) Fujimura Y, Nishishita C, Iida M, et al: Lymphangioma of the colon diagnosed with an endoscopic ultrasound probe and dynamic CT. Gastrointest Endosc 1995; 41:252-254

(8.) Kameyama H, Niwa Y, Arisawa T, et al: Endoscopic ultrasonography in the diagnosis of submucosal lesions of the large intestine. Gastrointest Endosc 1997; 46:406-411

(9.) Mimura T, Kuramoto S, Hashimoto M, et al: Unroofing for lymphangioma of the large intestine: a new approach to encloscopic treatment. Gastrointest Endosc 1997; 46:259-263

(10.) Kuramoto S, Sakai S, Tsuda K, et al: Lymphangioma of the large intestine, report of a case. Dis Colon Rectum 1988; 31:900-905

(11.) Nakagawara G, Kojima Y, Mai M, et al: Lymphangioma of the transverse colon treated by transendoscopic polypectomy: report of a case and review of literature. Dis Colon Rectum 1981; 24:291-295

(12.) Wakabayashi T, Suzuki K, Tanabe K, et al: A case of lymphangioma of the ascending colon diagnosed by polypectomy [in Japanese]. Nippon Naishikyo Gakkai Ski 1986; 28:440

RELATED ARTICLE: KEY POINTS

* Colonoscopy done in an elderly patient with weight loss found a 4 x 3 cm cecal mass.

* Computed tomography and endosonography characterized the mass as a vascular, submucosal lesion.

* Surgical resection and histopathology analysis documented a submucosal lymphangioma.

* Review of the medical literature supports endoscopic management of small, symptomatic lymphangiomas (<2 cm in diameter), while asymptomatic lesions may not require intervention.