Volume 203, Issue 4 , Pages e9-e11, October 2010
Intestinal lymphangiectasia mimicking primary peritoneal carcinoma
Article Outline
Intestinal lymphangiectasia is an obstruction of the lymphatic system. We report on a patient with mesenteric adenopathy and an elevated CA125 level, which were suspicious for peritoneal carcinoma. Further evaluation and bowel resection identified intestinal lymphangiectasia. This disease should be considered in patients with mesenteric adenopathy and a small bowel mass.
Key words: carcinomatosis, intestinal lymphangiectasia, mesenteric adenopathy
We present the case of a 46-year-old woman with abdominal pain after undergoing a liver biopsy for elevated liver function tests. Primary biliary cirrhosis was diagnosed on liver biopsy. In addition to a liver subcapsular hematoma, computed tomography (CT) identified mesenteric adenopathy that was thought to be consistent with carcinomatosis. Further assessment revealed a CA-125 level of 120 U/mL (range, 0–34 U/mL), a carcinoembryonic antigen level of 1.4 ng/mL (range, 0–5.4 ng/mL), a normal transvaginal ultrasound scan, and a right upper quadrant ultrasound scan that showed a fatty liver. Upper and lower gastrointestinal endoscopy demonstrated mild antral gastritis and sigmoid diverticulosis.
Case Report
Her medical history was significant for stage II primary biliary cirrhosis (newly diagnosed on liver biopsy), hypothyroidism, and chronic back pain. Surgical history was significant for back surgery and cervical fusion. She has no significant family medical history. She quit smoking approximately 1 year ago.
Abdominal CT scans reported multiple small mesenteric nodules and hypodense, lobulated, nonenhancing masses that abutted and encased the jejunum and ileum without intravenous contrast enhancement and multiple enlarged mesenteric lymph nodes (Figure 1). These findings were suspicious for small-bowel lymphoma. However, these CT findings were unchanged in comparison to a scan that had been performed 9 months earlier. The uterus, fallopian tubes, and ovaries were normal.
FIGURE 1.
Axial computed tomography image through the upper pelvis shows mesenteric nodularity and jejunal mass
Red arrowheads show mesenteric nodules. Yellow arrowheads show a nonenhancing, low-density mass abutting the jejunum.
Steines. Intestinal lymphangiectasia mimicking primary peritoneal carcinoma. Am J Obstet Gynecol 2010.
The patient was taken to the operating room for a diagnostic laparoscopy and mesenteric lymph node biopsy. At the time of laparoscopy, there was significant mesenteric fullness and a nodular mass that involved a 10-cm segment of mid jejunum. No mesenteric adenopathy was identified, as noted on preoperative CT scan. Frozen sections of multiple mesenteric biopsy specimens were reported as fibroadipose tissue. A small-bowel resection was performed because of the concerns of impending obstruction and for definitive diagnosis.
Surgical pathologic findings showed yellow and focal white patches in the mesentery, with dilated semitranslucent cysts that ranged from 0.3–4.0 cm in size throughout the bowel wall and mesentery (Figure 2). Milky-white fluid drained from these cysts on incision. Histologically, there are dilated lymphatic spaces throughout the full-thickness of the bowel wall (Figure 3).
FIGURE 2.
Grossly nodular area in jejunal mucosa
The jejunal mucosa is studded with raised yellow-to-white soft nodules; in addition, there are numerous cysts within the mesenteric fat (inset).
Steines. Intestinal lymphangiectasia mimicking primary peritoneal carcinoma. Am J Obstet Gynecol 2010.
FIGURE 3.
Photomicrographs of intestinal tissue that shows intestinal lymphangiectasia
Dilated, thin-walled lymphatic spaces (arrows in A) involve all layers of the bowel wall from mucosa to serosa. Original magnification: A, ×20; B, ×100; C, ×40; D, ×400.
Steines. Intestinal lymphangiectasia mimicking primary peritoneal carcinoma. Am J Obstet Gynecol 2010.
Comment
Intestinal lymphangiectasia (IL) is a rare protein-losing enteropathy that usually is diagnosed by 3 years of age and is reported later in life.1 Although the cause of IL is currently unknown, it is probably caused by congenital or acquired obstruction of the lymphatic drainage system.1
Common symptoms include chronic diarrhea, lower extremity edema, fatigue, vomiting, and abdominal pain. Some patients may be asymptomatic. In children and adolescents, a common finding is growth restriction.2 Malabsorption may be significant. It is common to have laboratory abnormalities, which include low albumin and total protein levels. In addition, CD4+ and CD8+ levels usually are decreased.
IL is divided into primary and secondary forms. Primary IL is diagnosed in children and adolescents and usually is due to abnormal development of the lymphatic system.3 Secondary IL is more common in adults and associated with systemic lupus erythematosus, hepatic cirrhosis, previous abdominal surgeries, lymphoma, postradiation injury,2 right-sided heart failure, and retroperitoneal fibrosis.4
On radiographic barium studies, IL appears as thickened irregular mucosal folds with tiny nodules that represent lymphatic dilation.5 CT imaging of IL has shown bowel wall thickening; a CT “halo sign” may be present. The halo consists of a thickened, low-attenuation inner ring, which represents lymphatic dilation in the mucosa and submucosa, and a higher attenuation outer ring, which consists of muscularis propria and serosa.6, 7 Nonenhanced, fluid-sensitive magnetic resonance imaging sequences may show bright signal intensity, which corresponds to lymphangiectasia in the mucosa/submucosa and mesentery.8
Macroscopic examination shows variably sized cysts in the mesentery and bowel wall and a creamy-yellow appearance that exudes milky, chylous fluid on sectioning (Figure 2). Histologic evidence shows dilated lymphatic spaces throughout the layers of the intestinal wall from mucosa to serosa (Figure 3).
Diagnosis by endoscopy is difficult, because the disease is located most commonly within the jejunoileum region. Therefore, capsule endoscopy and double-balloon enteroscopy are more useful in obtaining a diagnosis.2, 9
Treatment for IL includes dietary changes and symptomatic treatment. Specific recommendations include a diet that is supplemented with medium-chain triglycerides, as these bypass the lymphatic system and help to avoid increased lymphatic pressure, thereby decreasing the amount of protein lost.2, 3 In addition, a low-fat, high-protein diet is recommended. Other treatment options include antiplasmin, octreotide, corticosteroid, or albumin infusions; however, these rarely have been found to be helpful. Small-bowel resection may be useful in the treatment of recurrent or bulky disease.
Although there are certain aspects of this case that mimic peritoneal carcinoma, there are many signs and symptoms that the patient did not have (such as ascites, omental caking, or any gastrointestinal or genitourinary symptoms, which often are seen in patients with peritoneal carcinoma). As Ozan et al10 and others11, 12 have demonstrated, peritoneal tuberculosis is another condition that should be included in the differential diagnosis, in addition to IL, in patients with signs and symptoms that are suggestive of peritoneal carcinoma.
Given the patient's elevated CA-125, peritoneal carcinoma was noted to be considerably high on the differential diagnosis list. Interestingly, this patient was found to have no ascites on CT scan, which is not consistent with primary peritoneal carcinoma nor with peritoneal tuberculosis, in which 92% of cases have notable ascites.11 In addition, the most common findings on CT scan for both peritoneal carcinoma and peritoneal tuberculosis are omental thickening and ascites.12
An Ovid Medline literature search from 1950 to the present with the use of the search terms pelvic mass, cancer, carcinomatosis, adenopathy, gynecology, and CA-125 identified no articles in which IL mimicked peritoneal carcinoma. In conclusion, IL should be included in the differential diagnosis of any patient with known risk factors (such as cirrhosis, chronic diarrhea, lower extremity edema, fatigue, vomiting, or abdominal pain) who has an elevated CA-125 level and radiographic evidence that is suggestive of mesenteric adenopathy.
References
- . Primary intestinal lymphangiectasia (Waldmann's disease). Orphanet J Rare Dis. 2008;3:5
- . A primary intestinal lymphangiectasia patient diagnosed by capsule endoscopy and confirmed at surgery: a case report. World J Gastroenterol. 2007;13:2263–2265
- . Primary intestinal lymphangiectasia: case report with radiological and ultrastructural study. Digestion. 1981;21:107–114
- . Lymphangiectasia in Waldmann's disease. Clin Gastroenterol Hepatol. 2005;3:xxxiii
- . Pattern approach for diseases of mesenteric small bowel on barium studies. Radiology. 2008;249:445–460
- . The CT halo sign: a new finding in intestinal lymphangiectasia. J Comput Assist Tomogr. 1997;21:1005–1007
- . Localized intestinal lymphangiectasia: CT findings. AJR Am J Roentgenol. 2003;180:213–214
- . Cross-sectional imaging findings in congenital intestinal lymphangiectasia. J Comput Assist Tomogr. 2002;26:526–528
- Secondary lymphangiectasia of the small bowel: utility of double balloon enteroscopy for diagnosis and management. Dig Dis. 2008;26:383–386
- . Peritoneal tuberculosis mimicking peritoneal carcinomatosis. Eur J Gynaecol Oncol. 2009;30:426–430
- Tuberculous peritonitis: reports of 26 cases, detailing diagnostic and therapeutic problems. Eur J Gastroenterol Hepatol. 2001;13:581–585
- . US and CT findings in the diagnosis of tuberculous peritonitis. Acta Radiol. 1996;37:517–520
Authorship and contribution to the article is limited to the 5 authors indicated. There was no outside funding or technical assistance with the production of this article.
PII: S0002-9378(10)00875-6
doi:10.1016/j.ajog.2010.07.009
© 2010 Mosby, Inc. All rights reserved.
Volume 203, Issue 4 , Pages e9-e11, October 2010
