ABSTRACT
Turner syndrome patients are reported to have no increase in the relative risk of
cancer, and hepatocellular carcinoma in particular has never been reported in this
syndrome. However, Turner syndrome patients are known to have an increased prevalence
of liver lesions, some of which, like focal nodular hyperplasia, are related to vascular
anomalies. We report the case of a young patient with Turner syndrome, treated with
recombinant human growth hormone, who was found to have absence of the portal vein,
focal nodular hyperplasia, and hepatic adenoma and who developed a large hepatocellular
carcinoma requiring liver transplant.
KEYWORDS
Turner syndrome - absence of portal vein - focal nodular hyperplasia - hepatocellular
carcinoma
REFERENCES
- 1
Sybert V P, McCauley E.
Turner's syndrome.
N Engl J Med.
2004;
351
1227-1238
- 2
Roulot D, Degott C, Chazouilleres O et al..
Vascular involvement of the liver in Turner's syndrome.
Hepatology.
2004;
39
239-247
- 3
Kim T, Murakami T, Sugihara E, Hori M, Wakasa K, Nakamura H.
Hepatic nodular lesions associated with abnormal development of the portal vein.
AJR Am J Roentgenol.
2004;
183
1333-1338
- 4
Motoori S, Shinozaki M, Goto N, Kondo F.
Case report: congenital absence of the portal vein associated with nodular hyperplasia
in the liver.
J Gastroenterol Hepatol.
1997;
12
639-643
- 5
Guariso G, Fiorio S, Altavilla G et al..
Congenital absence of the portal vein associated with focal nodular hyperplasia of
the liver and cystic dysplasia of the kidney.
Eur J Pediatr.
1998;
157
287-290
- 6
Wanless I R, Albrecht S, Bilbao J et al..
Multiple focal nodular hyperplasia of the liver associated with vascular malformations
of various organs and neoplasia of the brain: a new syndrome.
Mod Pathol.
1989;
2
456-462
- 7
Laurent C, Trillaud H, Lepreux S, Balabaud C, Bioulac-Sage P.
Association of adenoma and focal nodular hyperplasia: experience of a single French
academic center.
Comp Hepatol.
2003;
2
6
- 8
Edmondson H A, Henderson B, Benton B.
Liver-cell adenomas associated with use of oral contraceptives.
N Engl J Med.
1976;
294
470-472
- 9
Resnick M B, Kozakewich H P, Perez-Atayde A R.
Hepatic adenoma in the pediatric age group: clinicopathological observations and assessment
of cell proliferative activity.
Am J Surg Pathol.
1995;
19
1181-1190
- 10
Espat J, Chamberlain R S, Sklar C, Blumgart L H.
Hepatic adenoma associated with recombinant human growth hormone therapy in a patient
with Turner's syndrome.
Dig Surg.
2000;
17
640-643
- 11
Ishak K G, Zimmerman H J.
Hepatotoxic effects of the anabolic/androgenic steroids.
Semin Liver Dis.
1987;
7
230-236
- 12
Serke S, Dienemann D, Speck B, Zimmermann R, Baer U, Huhn D.
Hepatocellular carcinoma and focal nodular hyperplasia associated with norethandrolone-therapy:
a case report.
Blut.
1986;
52
111-116
- 13
Moore S W, Millar A J, Hadley G P et al..
Hepatocellular carcinoma and liver tumors in South African children: a case for increased
prevalence.
Cancer.
2004;
101
642-649
- 14
Siqueira M A, Hayashi T, Yoshinaga K, Saisho S, Utsunomiya J, Sugihara K.
Colon cancer in a 14-year-old female with Turner syndrome: report of a case.
Dis Colon Rectum.
2003;
46
1560-1562
- 15
Ferrell L D.
Hepatocellular carcinoma arising in a focus of multilobular adenoma: a case report.
Am J Surg Pathol.
1993;
17
525-529
Raffaella A MorottiM.D.
Department of Pathology, Box 1194, Mount Sinai School of Medicine
One Gustave L. Levy Place, New York, NY 10029
eMail: raffaella.morotti@mountsinai.org
