CLOVES (congenital lipomatous overgrowth, vascular malformations, epidermal nevi, and skeletal anomalies) syndrome is associated with regional bony and/or soft tissue overgrowth, capillary malformation, and an increased risk for Wilms tumor.
To evaluate the frequency of Wilms tumor in patients with 2 similar conditions: diffuse capillary malformation with overgrowth (DCMO) and macrocephaly–capillary malformation (M-CM).
Culling our Vascular Anomalies Center database, we retrospectively reviewed patients in whom DCMO and M-CM had been diagnosed and who were evaluated between 1998 and 2016 for possible development of Wilms tumor. Patients younger than 8 years of age at their last visit and not seen in more than 2 years were contacted for follow-up.
The study comprised 89 patients: 67 with DCMO, 17 with M-CM, and 5 with an indeterminate diagnosis. No case of Wilms tumor was found in these groups.
Some patients were younger than 8 years of age at last follow-up visit and the sample size was small.
Patients with DCMO do not appear to be at increased risk for Wilms tumor. Screening is probably unnecessary in DCMO unless there is associated hemihypertrophy. Although there were no cases in our cohort, there are 2 reports of M-CM associated with Wilms tumor in the literature.Le texte complet de cet article est disponible en PDF.
Key words : diffuse capillary malformation, macrocephaly–capillary malformation, overgrowth, Wilms tumor
Abbreviations used : BWS, CLOVES, CM, CVM, DCMO, KTS, M-CM, PROS, WT
| Ms Peterman and Dr Vadeboncoeur contributed equally to this work.
| Funding sources: None.
| Conflicts of interest: None declared.
| Preliminary data from this study were presented at the 42nd Annual Meeting of the Society of Pediatric Dermatology, Minneapolis, MN; July 16, 2016.
| Reprints not available from the authors.