Chylous Ascites as a Serious Complication of the Neuroendocrine Tumor of Ileum – Case Report

Czech version

Authors: V. M. Matějka ¹; O. Fiala ¹; R. Tupý ²; L. Holubec ¹; J. Fínek ¹
Authors‘ workplace: Onkologické a radioterapeutické oddělení, FN Plzeň ¹; Klinika zobrazovacích metod LF UK a FN Plzeň ²
Published in: Klin Onkol 2013; 26(5): 358-361
Category: Case Report

Overview

Background:
Chylous ascites is a rare complication of the gastrointestinal neuroendocrine tumor. There are two mechanisms of its origin: mechanical obstruction by the tumor mass and fibrosis of the surrounding tissue due to overproduction of serotonin. Its presence restricts treatment options.

Case:
We report a case of 66‑year old man suffering from recurrent diarrhoea and ascites. We found elevated tumor marker Chromogranin A and elevation of hydroxyindoleacetic acid (5- HIAA) in the urine. A subsequent whole‑ body scintigraphy scan by octreoscan confirmed multinodal process with increased somatostatin receptors activity in the wall of the ileum, rectosigmoideum, lymph nodes of the retroperitoneum and mesenterium and left supraclavicular area. We performed biopsy from the lymph node of supraclavicular area, and there was metastasis of the neuroendocrine tumor. Start of cytostatic therapy was repeatedly complicated by recurrent massive chylous ascites. The patient underwent only one series of palliative chemotherapy. Another procedure was again complicated by chylous ascites that caused hospitalization at the internal department, and the patient died four months after diagnosis.

Conclusion:
Chylous ascites is a very rare complication of gastrointestinal neuroendocrine tumor. It is not only a marker of poor prognosis, but also a complication that makes systemic treatment very difficult.

Key words:
case report – neuroendocrine tumors – chylous ascites – malignant carcinoid syndrome

The authors declare they have no potential conflicts of interest concerning drugs, products, or services used in the study.

The Editorial Board declares that the manuscript met the ICMJE “uniform requirements” for biomedical papers.

Submitted:
25. 5. 2013

Accepted:
27. 6. 2013

Sources

1. Modlin IM, Sandor A. An analysis of 8 305 cases of carcinoid tumors. Cancer 1997; 79(4): 813– 829.

2. Oberndorfer S. Karzinoide Tumoren des Dunndarms. Frankfurter Z Pathol 1907; 1: 426– 430.

3. Bosman FT, Carneiro F, Hruban RH et al (eds). WHO Classification of Tumours of the Digestive System. 4th ed. World Health Organization classification of tumours. IARC: Lyon 2010.

4. Mandys V. Neuroendokrinní nádory trávícího ústrojí – histologická klasifikace. Farmakoterapie 2011; 7 (Spec. příl.): 8– 11.

5.Thorson AH. Heamodynamic changes during flush in carcinoidosis; the carcinoid syndrome. Am Heart J 1956; 52(3): 444– 461.

6. Feldman JM. Carcinoide tumors and the carcinoid syndrome. Curr Probl Surg 1989; 26(12): 835– 885.

7. Makridis C, Oberg K, Juhlin C et al. Surgical treatment of mid‑ gut carcinoid tumors. World J Surg 1990; 14(3): 377– 385.

8. Varma JS. Acute chylous ascites with carcinoid of the pancreas. Scott Med J 1985; 30(2): 111.

9. Mortensen RM, Medoff J, Feldman JM. Case report: chylous ascites and carcinoid tumors: possible association of 2 rare disorders. Am J Med Sci 1988; 296(4): 272– 274.

10. Dumont AE, Libby N. Thoracic duct lymph in a patient with chylous ascites and carcinoid tumor. Lymphology 1989; 22(4): 199– 201.

11. Scully RE, Mark EJ, McNeely BU. Case records of the Massachusetts General Hospital; case 20– 1986. A 68 year old man with malabsorption, chylous ascites and amesenteric mass. N Engl J Med 1986; 314(21): 1369– 1378.

12. Sherry S. Clinical pathologic conference: diarrhea, abdominal pain, gastrointestinal bleeding, chylous ascites and an intra‑ abdominal tumor. Am J Med 1959; 26(6): 919– 928.

13. Hodgson HJF, Sharp EJ, Waxman J et al. Grand Rounds‑ Hammersmith Hospital. A case of chylous ascites. BMJ 1993; 307: 495– 497.

14. Kelly ML Jr, Butt HR. Chylous ascites: an analysis of its etiology. Gastroenterology 1960; 39: 161– 170.

15. Bigler D, Lublin HK. Chylous ascites. Ugresk Laeger 1981; 143(9): 551.

16. Feldman JM. Carcinoid tumor and the carcinoid syndrome. Curr Probl Surg 1989; 26(12): 831– 898.

17. Aliaga L, Herrera F, Sarmiento C et al. Chylousascites secondary to a carcinoid tumor. An Med Interna 1990; 7(8): 419– 421.

18. Kypson AP, Onaitis MW, Feldman JM et al. Carcinoid and chylous ascites: an unusual association. J Gastrointest Surg 2002; 6(5): 781– 783.

19. Ayers R. Chylous ascites and jejunal carcinoid: a diagnostic challenge. ANZ J Surg 2005; 75(7): 618– 619.

20. Warner RR, Croen EC, Zaveri K et al. A carcinoid tumor associated with chylous ascites and elevated tumor markers. Int J Colorectal Dis 2002; 17(3): 156– 160.

21. Portale TR, Mosca F, Minona E et al. Gastrointestinal carcinoid tumor and chylous ascites, a rare association with a poor prognosis. A case report. Tumori 2008; 94(3): 419– 421.

22. Modlin IM, Moss SF, Oberg K et al. Gastrointestinal neuroendocrine (carcinoid) tumours: current diagnosis and managment. Med J Aust 2010; 193(1): 46– 52.

23. Deftos LJ. Chromogranin‑A: its role in endocrine function as an endocrine and non‑endocrine tumor marker. Endocr Rev 1991; 12(2): 181– 187.

24. Modlin IM, Gustafsson BI, Moss SF et al. Chromagranin A‑ biological fiction and clinical utility in neuro endocrine tumor dinase. Ann Surg Oncol 2010; 17(9): 2427– 2443.

25. Bergmann JF, Beaugrand M, Labadie H et al. CA 125 (ovarian tumour‑associated antigen) in ascitic liver diseases. Clin Chim Acta 1986; 155(2): 163– 165.

26. Sevinc A, Buyukberber S, Sari R et al. Elevated serum CA‑ 125 levels in patients with nephrotic syndrome‑induced ascites. Anticancer Res 2000; 20(2B): 1201– 1203.

27. Sevinc A, Buyukberber S, Sari R et al. Elevated serum CA‑ 125 levels in hemodialysis patients with peritoneal, pleural, or pericardial fluids. Gynecol Oncol 2000; 77(2): 254– 257.

28. Buamah P. Benign conditions associated with raised serum CA‑ 125 concentration. J Surg Oncol 2000; 75(4): 264– 265.