Linear nevi, hemihypertrophy, connective tissue hamartomas, and unusual neoplasms in children

Neoplasms in proteus syndrome

“…Types reported include papillary adenocarcinoma of the testis [Hornstein et al, 1987, case 21, mesothelioma of the tunica vaginalis of the testis [Malamitsi-Puchner et al, 19901, and probable mesothelioma involving the peritoneal surfaces (our patient 1). Although no frank malignancies have occurred in patients with multiple tumors (Table 11) to date, our patient 2 had bilateral serous cystadenomas with nuclear atypia that may represent premalignant change.…”

Compromise of the spinal canal in Proteus syndrome

“…Literature review showed that to date, compromise of the spinal canal is rare. Hornstein et al [1987, patient 11 reported a patient who, on myelography and CT scan, demonstrated complete blocks in the lower thoracic and lumbar spinal canal resulting from marked vertebral overgrowth. Recovery of neurologic dysfunction did not occur after surgical decompression.…”

Risk of tumorigenesis in overgrowth syndromes: A comprehensive review

“…Nonetheless, an annual physical examination and Cases reported by Kontras, 1974;Cohen and Hayden, 1979;Kousseff, 1986;Hornstein et al, 1987;Viljoen et al, 1987;Cohen, 1988;Alysworth et al, 1988;Nishimura and Kozlowski, 1990;Malamitsi-Puchner et al, 1990;Maassen and Voigtlander, 1991;Bouzas et al, 1993;Bale et al, 1993;Pearson and Hoyme, 1993;Skovby et al, 1993;Gordon et al, 1995;Horie et al, 1995;Whitley and Flannery, 1996;Zachariou et al, 1996;Boccone et al, 1997 (Tables XIII and XIV). Females were affected more commonly than males (1.72:1), and the right-to-left sidedness ratio was 1.29:1.…”