Abdominal cystic lymphangioma

The patient was a 46-year-old black man who presented with an 18-kg (40-lb) weight gain during the previous 4 months and a 1- to 2-month history of increasing abdominal girth. His abdomen became painful and significantly enlarged during the 2 weeks prior to admission. His symptoms were relieved by postural changes (kneeling forward). He reported no change in his appetite or his bowel habits. He denied nausea, vomiting, melena, hematochezia, diarrhea, fever, and chills.

The patient's past medical history revealed a left pontine cerebrovascular accident in 1981, hyperbilirubinemia since 1990, and long-standing hypertension, but he was on no medications. Physical examination revealed a well-nourished man in minimal distress. The man's abdomen was tense and markedly distended, and he felt mild tenderness to palpation. There was no fluid wave and no circumscribed mass. A diagnostic paracentesis was performed and revealed blood and blood clot, consistent with a resolving hematoma. Laboratory values showed normal electrolyte, amylase, and lipase levels. The patient's total bilirubin was increased at 2.6 mg/dL, his ceruloplasmin value was 72 mg/dL, and the prothrombin time was 13.9 seconds with the international normalized ratio 1.33.

Radiologic studies were performed, and a plain abdominal radiograph showed a mass occupying much of the abdomen. An abdominal computed tomographic scan revealed the mass was wrapped around the terminal ileum and extended to the bladder in the midline. Septa were seen along the left aspect of the mass, suggesting a cystic or multicystic mass (Figure 1).

The patient was taken to the operating room, and surgery revealed a large cystic mass occupying the majority of the abdomen. The lesion was in the mesentery, arising at the base of the ileum and coming to within 3 cm of the ileocecal valve. The mass, with the attached small intestine, was sent to the surgical pathology department for evaluation.

The specimen consisted of a large, lobulated, multicystic mass (Figure 2), which measured 44 X 30 X 21 cm and weighed 16.7 kg. The attached 88-cm segment of small intestine was uninvolved. The mass was filled with bloody, dark brown fluid and blood clot. There were no papillary excrescencies, and the cysts ranged from 1 to 20 cm in diameter. Microscopic examination revealed the cysts were of varying size and shape and were lined by thin, attenuated endothelial cells (Figure 3). Focal portions of organizing hematoma were adherent to the endothelial lining. Within the cystic walls were focal aggregates of lymphocytes, also of varying size. A few vascular spaces were seen, and small amounts of smooth muscle were present within the walls of the vessels. The endothelial cells demonstrated immunoreactivity for factor VIII (Figure 4) and were negative for keratin.

What is your diagnosis?

Pathologic Diagnosis: Multicystic Intra-abdominal Lymphangioma

Lymphangiomas are benign lesions with a proliferation of dilated lymphatic spaces lined by thin, attenuated endothelial cells.1 They can occur throughout the body, but are most common in the neck of children and are called cystic hygromas.2 Cystic lymphangiomas were originally described by Redenbacker in 1828.3 Rokitansky described a chylous cystic angioma in 1842,4 and Wernher coined the term cystic hygroma in 1843.3

Lymphangiomas are rare intra-abdominal tumors.1 The majority are present in patients younger than 5 years of age, but occasionally these tumors do not produce symptoms until adult life. Within the abdomen, the most common site for a lymphangioma is the mesentery, followed by the omentum, mesocolon, and retroperitoneum.2 The symptoms typically produced depend on the site of involvement and include an abdominal mass or an acute abdomen. Complications can include volvulus, intestinal obstruction, infarction, perforation, and hemorrhage. Perforation and hemorrhage may be spontaneous or may be caused by injury or trauma. Pain in the right lower quadrant can mimic acute appendicitis.5

The diagnosis of a lymphangioma can be deduced by multiple radiologic studies. An ultrasound can show a multiloculated cystic structure within the abdomen.6 A computed tomographic scan shows multiple, homogeneous, nonenhancing areas with variable attenuation. An arteriography can show that the lesion is poorly vascularized.7

Histologically, a lymphangioma can be a well-circumscribed lesion composed of 1 or multiple large cysts, which can interconnect.8 These are typically called a cavernaus lymphangioma. A lymphangioma can also be composed of microscopic cysts producing an ill-defined, compressible, spongelike lesion, known as a cystic lymphangioma.2 The walls of the small or large lymphatic spaces are thin and contain fibrous tissue, smooth muscle, and aggregates of lymphoid tissue. The lymphatic channels and spaces are lined by one row of flat endothelial cells.9 There is no discrete capsule. In the small bowel mesentery, the spaces may be filled with chyle and are called chylous lymphangioma.5

The main differential diagnosis for cystic structures in the mesentery includes lymphangioma, cavernous hemangioma, and cystic mesothelioma (mesenteric cysts).2 The cystic mesothelioma often affects a large area of the peritoneum as a multicystic mass. Repeated surgery is necessary for local recurrence.10 The mesothelioma has gland-- like spaces that have a greater variation in size than the vascular spaces of the lymphangioma.1 Attenuated mesothelial cells line the cystic mesothelioma, and these mesothelial cells are immunoreactive for cytokeratin and are negative for factor VIII. A cavernous hemangioma has cystic spaces lined by endothelial cells, which are immunoreactive for factor VIII and are negative for keratin. The presence of lymphoid aggregates in the stroma favors lymphangioma over hemangioma.2

In conclusion, lymphangiomas are rare benign tumors that typically occur in the head and neck of children. These tumors are referred to as cystic hygromas. Lymphangiomas also occur as rare intra-abdominal tumors in the small or large bowel mesentery. Depending on the site, symptoms can include a movable mass or an acute abdomen. Radiologic studies, including computed tomographic scan and ultrasound, can suggest a diagnosis. Histologically, multiple cystic spaces are seen lined by flat, attenuated endothelial cells. Lymphoid aggregates, smooth muscle, and fibrous tissue are seen in the wall. The endothelial cells are positive for factor VIII and are negative for keratin. The main differential diagnoses include a cavernous hemangioma and a mesenteric cyst.

References

1. Fenoglio-Preiser CM, Pascal RR, Perzin KH. Tumors of the Intestines. Washington, DC: Armed Forces Institute of Pathology; 1990:494-509. Atlas of Tumor Pathology; 3rd series, fascicle 27.

2. Enzinger FM, Weiss SW. Soft Tissue Tumors. 3rd ed. St Louis, Mo: MosbyYearbook; 1995:679-689.

3. Ravitch MM, Rush BF. Pediatric Surgery. Vol 1. Chicago, III: Yearbook Medical Publishers; 1979:368-374.

4. Rokitansky LI. Pathologie and Anatomie. Vienna, Austria: 1842. Cited by: Oh C, Danese CA, Dreiling DA. Chylous cysts of mesentery. Arch Surg. 1967;94: 790.

5. Daniel S, Lazarevic B, Attia A. Lymphangioma of the mesentery of the jejunum: report of a case and a brief review of the literature. Am J Gastroenterol. 1983;78:726-729.

6. Roisman I, Manny J, Fields S, et al. Intra-abdominal lymphangioma. Brj Surg. 1989;76:485-489.

7. Perrot M, Rostan 0, Morel P, et al. Abdominal lymphangioma in adults and children. Brj Surg. 1998;85:395-397.

8. Ros PR, Olmstead WW, Moser RP, et al. Mesenteric and omental cysts: histologic classification with imaging correlation. Radiology. 1987;162:327-332.

9. Mahle C, Schwartz M, Popek E, et al. Intra-abdominal lymphangiomas in children and adults. Arch Pathol Lab Med. 1997;121:1055-1062.

10. Takiff H, Calabria R, Yin L, et al. Mesenteric cysts and intra-abdominal cystic lymphangiomas. Arch Surg. 1985;120:1266-1269.

Eric M. Chand, MD; Timothy W. McNeely, MD; Lawrence 1. Freant, MD

Accepted for publication December 21, 1999.

From the Medical College of Georgia, Augusta (Drs Chand and McNeely); Flowers Hospital, Dothan, Ala (Dr McNeely); and Veterans Administration Medical Center, Augusta, Ga (Dr Freant).

Reprints not available from the author.

Copyright College of American Pathologists Nov 2000
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