Received: 10 December 2001Accepted: 20 April 2002Published online: 22 June 2002� Springer-Verlag 2002

A. Paterson (&)Department of Radiology,Royal Belfast Hospital for Sick Children,180 Falls Road, Belfast BT12 6BE, UKE-mail: anne.paterson@royalhospitals.n-i.nhs.ukTel.: +44-28-90894963Fax: +44-28-90313798

R. GrundyDepartment of Oncology,Birmingham Children’s Hospital,Birmingham, UK

J. de Ville de GoyetDepartment of Surgery,Birmingham Children’s Hospital,Birmingham, UK

F. RaafatDepartment of Histopathology,Birmingham Children’s Hospital,Birmingham, UK

S. BeathDepartment of Hepatology,Birmingham Children’s Hospital,Birmingham, UK

A. McCarthyChildren’s Haematology Unit,Royal Belfast Hospital for Sick Children,Belfast, UK

An 8-month-old boy presented witha 2-week history of dyspnoea,anorexia and increasing abdominaldistension. Physical examinationrevealed a markedly distendedabdomen, which was dull to per-cussion. US demonstrated a hetero-geneous increase in hepaticechogenicity, with multiple intrahe-patic cystic lesions (Fig. 1). Therewas extensive ascites. CT confirmedreplacement of most of the right lobeof the liver by a multiloculated cysticlesion. The coeliac axis vessels andsuperior mesenteric artery were en-cased by the mass, as were the

splenic, main and right portal veinsand the IVC (Fig. 2). Aspiration ofthe liver cysts and peritoneal fluidgave chylous fluid, which containedclusters of ‘atypical’ cells and somereactive mesothelial cells. A liverbiopsy was non-diagnostic, showingexcess fibrosis and compressed he-patic parenchyma. Laparotomydemonstrated a retroperitoneal tu-mour that enveloped the IVC andinvaded the liver via the portal vein.Subsequent histology revealed a di-agnosis of congenital malignantperitoneal mesothelioma (MPM).The patient received palliative

PICTORIAL INTERLUDE

Fig. 1 Longitudinal US through the right lobe of the liver showing abnormalechotexture and multiple cystic lesions. There is a small right-sided pleural effusion andsubdiaphragmatic ascitic fluid

Pediatr Radiol (2003) 33: 73–74DOI 10.1007/s00247-002-0751-2

Anne Paterson

Richard Grundy

Jean deVille de Goyet

Faro Raafat

Susan Beath

Anthony McCarthy

Congenital malignant peritoneal mesothelioma

care and died 1 month after presen-tation.

Mesotheliomas are rare tumoursarising from the surface mesotheliallayer that covers the pleural andperitoneal cavities. Less frequentlythe site of origin may be the serosalsurface of the pericardium or tunicavaginalis. Only 2–5% of all cases ofmalignant mesothelioma occur inthe first two decades of life [1, 2], andof these, 15–20% originate in theperitoneum [2, 3]. There is no clearassociation between childhood ma-lignant mesothelioma and asbestos,radiation or isoniazid exposure [1, 3].Childhood malignant mesothelio-

mas are aggressive tumours andtheir prognosis is poor [1, 3, 4]. Areview of the literature has revealedonly two previously documentedcases of congenital MPM [4, 5].

Clinically, peritoneal mesothelio-mas present with symptoms such asvague abdominal pain and disten-sion, weight loss and ascites. Cross-sectional imaging with either CT orMRI is required to define the extentof the disease. Previously notedimaging findings in both adult [6]and childhood [7] MPM includeperitoneal and omental thickeningand nodules, occasional discrete,solid intra-peritoneal mass lesions

and variable quantities of ascites.Direct organ invasion has not beendescribed radiologically or at post-mortem in childhood MPM.

This is the first reported case of acongenital MPM in the radiologyliterature. Direct hepatic invasion inchildhood MPM has not previouslybeen documented. Although rare,these tumours should be consideredin the differential diagnosis of in-traperitoneal neoplasms in child-hood, including those cases in whichthere is evidence of solid organinvasion.

References

1. Brenner J, Sordillo PP, Magill GB(1981) Malignant mesothelioma in chil-dren: report of seven cases and review ofthe literature. Med Pediatr Oncol 9:367–373

2. Kelsey A (1994) Mesothelioma ofchildhood. Pediatr Hematol Oncol11:461–462

3. Fraire AE, Cooper S, Greenberg SD,et al (1988) Mesothelioma of childhood.Cancer 62:838–847

4. Nishioka H, Furusho K, Yasunaga T,et al (1988) Congenital mesothelioma. Acase report and electron-microscopicstudy. Eur J Pediatr 147: 428–430

5. Silberstein MJ, Lewis JE, Blair JD, et al(1983) Congenital peritoneal mesotheli-oma. J Pediatr Surg 18:243–245

6. Guest PJ, Reznek RH, Selleslag D, et al(1992) Peritoneal mesothelioma: the roleof computed tomography in diagnosisand follow up. Clin Radiol 45:79–84

7. Haliloglu M, Hoffer FA, Fletcher BD(2000) Malignant peritoneal mesotheli-oma in two pediatric patients: MRimaging findings. Pediatr Radiol 30:251–255

Fig. 2 Contrast-enhanced CT at the level of the pancreas. The rightlobe of the liver is expanded by the low-density cystic mass. Theabdominal vessels are encased by the tumour. Extensive ascitic fluidis present

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